Clinical Vignette Poster Session I Thursday, October 20, 2011 5pm - 7pm INTESTINE/COLON/IBD 42 PYLORIC METAPLASIA AS A HISTOLOGICAL CLUE FOR DIAGNOSIS OF CROHN'S DISEASE IN THE ABSENCE OF GRANULOMA. Paul, Adam 1; Malkani, Anjali 1; Twaddell, William 1, 2, 1. Pediatrics, University of Maryland Children's Hospital, Baltimore, MD, United States. 2. Pathology, University of Maryland Medical Center, Baltimore, MD, United States. Pyloric metaplasia of colonic mucosa is a rare pathologic finding that occurs due to replacement of inflamed and ulcerated mucosa with regenerative tissue. It is a well documented feature of Crohn’s disease in the pathology literature, but is underrepresented in pediatric gastroenterology literature. We present a 16 year old female presenting with a one month history of generalized abdominal pain, nausea, vomiting, and significant weight loss worsening over the last 3 days. Abdominal CT showed significant terminal ileum thickening, and luminal narrowing, as well as features of early intramural abscess formation. Infectious work up was negative, but acute phase reactants and stool calprotectin were elevated. She was started on piperacillin/tazobactam and metronidazole for abscess treatment. Once the abscess had improved radiographically, she underwent endoscopy and colonoscopy which showed localized granular mucosa in the cecum, and moderate inflammation at the IC valve with marked swelling, with a visually normal recto‐sigmoid and transverse colon. Intubation of the IC valve was not possible due to the degree of swelling. However, biopsies from cecum showed focal acute cryptitis as well as crypt distortion and pyloric metaplasia. The remainder of the colonic, duodenal, gastric and esophageal biopsies were normal. She was started on high dose steroids and azathioprine with resolution of symptoms. Despite being a rare and nonspecific finding, pyloric metaplasia should be looked for to support biopsy diagnosis of Crohn’s disease in the absence of granuloma formation, as it is not a typical feature of Ulcerative Colitis. 43 A SEVERE EXACERBATION OF ULCERATIVE COLITIS AFTER RITUXIMAB THERAPY IN A PATIENT WITH IDIOPATHIC THROMBOCYTOPENIC PURPURA . Raizner, Aileen 1; Phatak, Uma Padhye1; Pashankar, Dinesh S1, 1. Pediatrics, Yale University, New Haven, CT, United States. Background: Rituximab (an anti‐CD20 antibody) is widely used for autoimmune disorders including Idiopathic Thrombocytopenic Purpura (ITP). Severe ulcerative colitis (UC) has been reported to occur following rituximab therapy in a child with nephrotic syndrome (Pediatrics 2010) and an adult with Grave’s disease (Gut 2008). Effects of rituximab in patients with both UC and an autoimmune disorder have not been reported. Case Report : A 17‐year‐old male presented with a history of bloody stools and abdominal pain. His father had a colectomy for severe UC. Blood work showed Hb 10 g/dL and platelets 14,000/uL. UC was suspected but endoscopy was deferred due to thrombocytopenia. ITP was subsequently diagnosed. He failed to respond to iv immunoglobulin and Rho (D) Immune Globulin. He received 3 weekly doses of rituximab 750 mg. Two weeks after the third infusion, he presented with severe abdominal pain and progressive bloody diarrhea occurring 25 times per day (stool output 2 liters/day). Blood work showed hemoglobin 8.4g/dL, platelets 150,000/uL,C‐reactive protein 171mg/dL, a positive p‐ANCA , and negative ASCA antibody. Stool tests were negative for infection. An upper endoscopy with histology was unremarkable. A sigmoidoscopy revealed severe ulcerations. Histology revealed severe, chronic active colitis without granulomata. Flow cytometry from 6 weeks through 16 weeks after rituximab therapy revealed virtually no circulating B cells, consistent with anti CD20 therapy, and normal circulating T cells. He was hospitalized and treated with bowel rest, parenteral nutrition and high dose intravenous steroids for a week. He slowly improved and presently is doing well on tapering prednisone and 6‐MP therapy. Conclusions‐ Although rituximab therapy led to improvement in ITP, it also led to a severe exacerbation of UC in our patient. This clinical pattern of severe colitis with B cell depletion is similar to the previous reports. We recommend caution and vigilant monitoring when using rituximab in patients with both UC and autoimmune disorders. 44 MESALAMINE‐INDUCED EOSINOPHILIC PNEUMONIA IN AD 15 YEAR OL BOY WITH CROHN’S DISEASE. Lucia, Chantal 1; Garcia, Jennifer 2; Reeves‐Garcia, Jesse 1; Muinos, William 1; Gomara, Roberto 1; Ambati, Shashikanth 1; Hernandez, Erick 1, 1. Pediatric Gastroenterology, Miami Children's hospital, Miami, FL, United States. 2. Pediatric Gastroenterology, Miller School of Medicine. University of Miami, Miami, FL, United States. A 15 year‐old boy with Crohn’s Disease presented with a one day history of sharp chest pain worsened by movement and inspiration localized to left upper side of the chest. He denied cough, fever, night sweats, weight loss, diarrhea and rash. 6‐Mercaptopurine (6MP) immunosuppression and Asacol (Mesalamine) had been started five months prior. Additionally, the patient had been treated for Mycobacterium fortuitum in the stool two weeks prior and had returned back from the Dominican Republic four days prior.Physical exam was remarkable for tenderness on the left second rib. A left upper lobe nodule was discovered on chest X‐ Ray that had not been present one month previously. Computed tomography (CT) of the chest showed nonspecific bilateral non‐calcified sub‐centimeter pulmonary nodules with pleural nodules centered in the upper lung zones. Right thoracoscopy and wedge resection was performed. Biopsy results showed eosinophilic pneumonia with no discrete granulomas. Special stains were negative for acid fast and fungal stains. Electron microscopy was negative for Birbeck granules or viral inclusions. The patient remained on 6MP and Asacol was discontinued. There was complete resolution of respiratory symptoms, and repeat CT chest three months later showed no pulmonary nodule. Conclusion: Although the differential for a pulmonary nodule in an immunosuppressed patient with inflammatory bowel disease is broad. The diagnostic findings of eosinophilia, upper lobe involvement, and pulmonary eosinophilia on biopsy are highly suggestive of Mesalamine toxicity. This case illustrates how eosinophilic pneumonia should be considered in the pediatric population who develop pulmonary nodules while on Mesalamine therapy. 45 METASTATIC CROHN’S DISEASE IN A TEENAGE BOY ON INFLIXIMAB. Kutsch, Erika 1; Figueroa, Ernesto 2; Adeyemi, Adebowale 1, 1. Gastroenterology, AI duPont Hospital for Children , Wilmington, DE, United States. 2. Urology, AI duPont Hospital for Children, Wilmington, DE, United States. Case: A 16 year old male presented with weight loss, diarrhea, erythema nodosum and scrotal swelling. Colonoscopy findings included diffuse edema, pseudopolyps, exudate and aphthae. Pathology was consistent with granulomatous colitis. He underwent Infliximab induction, followed by 5mg/kg every 8 weeks. Four months later, scrotal and penile edema reoccured in the absence of bowel symptoms. Infliximab trough was therapeutic (7.6mg/ml). MRI showed marked edema of scrotal sac without evidence of fistula. He was treated with prednisone, metronidazole and topical tacrolimus with improvement over a few weeks. Three months later, he again developed scrotal and penile edema. Repeat colonoscopy revealed colonic mucosal healing. Scrotal biopsy did not show granulomas but lymphohistiocytic infiltration, consistent with cutaneous manifestation of Crohn's disease (CD). A short course of prednisone and infliximab dose increase to 10mg/kg resulted in sustained resolution of his scrotal and penile edema. Discussion: Metastatic CD of the genitourinary system is a rare extraintestinal manifestation that is histologically similar to inflammation seen in the intestinal tract. In patients with metastatic CD, cutaneous manifestation and intestinal involvement do not always follow parallel course as adequate treatment of bowel disease does not predict improvement of cutaneous lesions. Treatment of metastatic CD with different agents, including steroids, cyclosporin A, 6‐ mercaptopurine, sulfasalazine and antibiotics have shown variable success. Treatment with infliximab has shown promising results. Several case reports have suggested its efficacy in treatment when other therapies have been unsuccessful. We hope that maximizing the infliximab dose in our patient will lead to sustained remission of cutaneous findings. 46 MEGALOGASTRIA IN CROHN’S DISEASE. Ahmad, Fareed 1; Steiner, Steven 1, 1. Riley Hospital for Children, Indianapolis, IN, United States. A 16‐year‐old Caucasian female was referred with a one month history of abdominal pain, vomiting, and weight loss. Physical examination was remarkable for mild abdominal tenderness, distension and a perianal skin tag with underlying fissure. Laboratory examination revealed anemia (Hemoglobin 9 gm/dl), elevated inflammatory markers (ESR 38 mm/hr, CRP 2.8 mg/dl), and hypoalbuminemia (2.5 g/dl). Abdominal CT scan (Panel A) demonstrated megalogastria, extending into the right pelvis. Upper gastrointestinal endoscopy revealed a large, fluid‐filled stomach with a thickened, erythematous pylorus and a large duodenal bulb ulcer, with duodenal narrowing (Panel B). There was no evidence of Helicobacter pylori on gastric biopsy. Active chronic duodenitis was observed. Anti‐Saccharomyces cerevisiae antibodies were positive, suggestive of Crohn’s disease. The patient received intravenous methylprednisolone and esomeprazole. A conventional Barium study of her upper gastrointestinal tract prior to discharge revealed significant resolution ofe th megalogastria. Repeat endoscopic examination after eight weeks demonstrated resolution of the duodenal ulcer. She is currently asymptomatic and remains on azathioprine and mesalamine for maintenance therapy of Crohn’s disease. 47 SUPERIOR MESENTERIC ARTERY SYNDROME AND BOWEL ISCHEMIA ASSOCIATED WITH TAKAYASU ARTERITIS. Duh, Glenn 1, 1. Pediatrics, Southern California Permanente Medical Group, Downey, CA, United States. Takayasu arteritis is a serious vasculitis that rarely can be associated with gastrointestinal complications. This appears to be the first reported case of superior mesenteric artery syndrome in a patient with Takayasu Arteritis. Case: A 17 year old female of Japanese descent was referred for a pediatric GI consultation with suspected Crohn’s disease. She presented 8 months previously with fever of unknown origin. Laboratory findings included iron‐deficiency anemia, a ferritin level of 468.1 ng/mL [13‐150]), an ESR of 120 mm/hr, and a CRP of 213.7. Infectious disease and pediatric hematology evaluations were negative, and fever was controlled with low‐dose prednisone. The patient presented to the pediatric GI visit with a history of low‐grade fevers, malaise, weight loss, hip weakness, abdominal pain and early satiety, and examination demonstrated mild left‐lower‐quadrant abdominal tenderness. She denied a history of vomiting or abnormal bowel movements. An upper GI series with small bowel follow‐through demonstrated superior mesenteric artery syndrome with severe duodenal distension. She presented shortly afterwards to the emergency department with left sided facial and upper extremity weakness, and was hospitalized. CT of the head was negative; however, her chest radiograph demonstrated mediastinal enlargement, which on thoracic CT imaging was determined to be caused by aortic dilatation. A mural thrombus was also noted in the ascending aorta. CT angiogram demonstrated severe stenosis of the celiac and SMA arteries. The patient's inpatient EGD and colonoscopy were normal, except for a markedly dilated proximal duodenum. The patient was diagnosed with Takayasu arteritis, was started on steroids and oral methotrexate, and was referred to see a vascular surgeon. Unfortunately, she returned to the emergency department approximately one month later with acute, severe abdominal pain, was found during exploratory laparotomy to have extensive bowel ischemia and necrosis, and subsequently expired from septic shock. 48 SPECIFIC CARBOHYDRATE DIET IN FIVE PEDIATRIC PATIENTS WITH CROHN’S DISEASE. Burgis, Jennifer Clare1; Nguyen, Kaylie 1; Cox, Kathleen 1; Shah, Shamita 1; Cox, Kenneth 1, 1. Stanford University, Palo Alto, CA, United States. Traditional treatment for Crohn’s disease (CD) involves medications which cause many side effects. Enteral nutrition can be an effective alternative for both induction and maintenance of remission, but adherence to a formula‐based diet can be challenging. We report our experience in 5 patients with CD (3 with only small bowel disease, 2 with small bowel and colonic disease) who chose to follow a specific carbohydrate diet (SCD) for a minimum of 4 months. The SCD excludes all grains, gluten and lactose. It is restricted to simple carbohydrates found in fruit, honey, and vegetables as well as proteins from unprocessed meats, fish and lactose free dairy. Two patients used the SCD for both induction of remission and maintenance therapy; three started the SCD in conjunction with a steroid taper that spanned 4‐6 weeks after which the SCD was continued as monotherapy for maintenance. Tables 1 and 2 show BMI and laboratory parameters for SCD‐only patients and steroid + SCD patients, respectively. Pre values indicate time of diagnosis and post values are at between 4‐6 months after initiation of therapy. All five patients were in remission by 1 month and were symptom free with improved growth and normalization of laboratory values while they remained on the SCD. Responses demonstrate that effective dietary intervention may allow for medication‐ free remission and/or maintenance. We are planning a prospective study to evaluate the SCD in comparison with 6‐mercaptopurine as maintenance therapy for patients with CD. 49 INFLIXIMAB THERAPY IN PEDIATRIC PATIENTS 7 YEARS OF AGE AND UNDER. Kelsen, Judith 1; Gupta, Kernika 1; Grossman, Andrew 1; Baldassano, Robert 1; Mamula, Petar 1, 1. The Children's Hospital of Philadelphia, Philadelphia, PA, United States. Background: Infliximab has altered management in pediatric and adult IBD. However, it has not been studied in patients 7 years of age and younger. Our aim was to characterize efficacy and safety of infliximab in this cohort. Methods: This was a retrospective study of patients 7 years and younger with IBD treated with infliximab between 1999‐2011 at The Children’s Hospital of Philadelphia. Medical records were examined for demographics, disease location, phenotype, therapy, surgical history, infliximab dose and infusion intervals. Outcome measures included physician global assessment, AES, corticosteroid requirement, and escalation of therapy. Results: 33 children were included in the study. 19 patients were 2‐5 years old, 6 were 6 years old and 8 were 7 years old. 20 patients had CD, 4 had UC, and 9 had indeterminate colitis. All but 3 patients completed the induction regimen and 75% began maintenance therapy. Half of patients were on corticosteroids at the time of induction. At 1, 2 and 3 years 34%, 19% and 12.5%, respectively continued therapy. Of the patients who completed 1 year of therapy, 25% remained steroid‐free and demonstrated response through PGA, with either non‐active or decrease from active to mild disease at one year. Reasons for therapy discontinuation included loss or poor response, development of antibody, and parental concern for adverse events (1 patient). There were 6 infusion reactions noted. There were no malignancies, serious infections, or deaths. Conclusion: Infliximab therapy in patients 7 years oldr and younge demonstrates a low response rate and modest steroid sparing effect at one year. It further shows lower rates of continued maintenance therapy in contrast to previously published literature describing continued maintenance therapy in children 8 years old and greater of 93%, 78%, and 67% at 1, 2 and 3 years, respectively. 50 CAPSULE ENDOSCOPY IDENTIFIES MESENTERIC CASTLEMAN’S DISEASE MASQUERADING AS CROHN'S DISEASE. Bass, Lee M1; Barsness, Katherine 1; Benya, Ellen 1; Proytcheva, Maria 1; Kagalwalla, Amir 1, 1. Children's Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, IL, United States. Castleman's disease (CD) is a rare lymphoproliferative disorder of unclear etiology that often presents with anemia in children. In this case we report a novel approach using Capsule Endoscopy (CE) to identify tumor mass that was subsequently diagnosed as CD. CASE PRESENTATION: 16 year old male was seen for resistant chronic iron deficiency anemia. His clinical history was remarkable for recurrent periumbilical abdominal pain and oral ulcers. There was no history of vomiting, diarrhea, hematochezia, melanotic stools, fevers, weight loss, joint pain or skin rash. Abdominal examination was unremarkable without tenderness, masses or organomegaly. Laboratory studies demonstrated WBC 6.3, Hgb 11.4, Hct 34.9, MCV 76.5, platelets 337, ESR 98, CRP 17.8. Crohn's disease was suspected. An upper GI with SBFT, an upper endoscopy and colonoscopy with biopsies with evaluation of terminal ileum were performed, but they were unremarkable. However, Capsule endoscopy identified a large sub‐mucosal mass in the mid jejunum. This finding was confirmed by MR enterography that demonstrated a 4.8 x 4.2 x 6.3 cm mass arising from the mesentery in the mid abdomen. There was focal mass‐effect on the adjacent loops of bowel with posterior displacement of the proximal jejunum. A large peritoneum covered mass that appeared consistent with a very large lymph node was excised at laporotomy. Histopathological examination of the mass demonstrated a lymph node with angiofollicular dysplasia, numerous plasma cells and fibrosis, consistent with a diagnosis of plasma cell variant of Castleman's disease. The patient had an uneventful post surgical course without recurrence of abdominal pain and resolution of the iron deficiency anemia. Conclusion: We present a novel approach utilizing CE that was instrumental in identifying a mass that was subsequently diagnosed as CD disease. Although rare, CD should be in the differential diagnosis of an adolescent presenting with features characteristic of crohn's disease. 51 UNUSUAL PRESENTATION OF CROHN’S DISEASE WITH EYE SYMPTOMS. Maksimak, Martin 1; Maksimak, Brian 1, 1. Geisinger Clinic ‐ Janet Weis Children's Hospital, Danville, PA, United States. KR was a 13 yo girl when she presented to her primary physician with a complaint of puffiness at the right outer corner of her eye, subsequent severe eye pain for 5 days, lid droop and diplopia on upward gaze. She was seen by an ophthalmologist, who noted restriction of supraduction. MRI of the orbits revealed a soft tissue mass within the superior right orbit that appeared to arise from the superior rectus muscle. This location was confirmed by ultrasound. Biopsy of the lesion revealed that it was composed of noncaseating granulomas and other chronic inflammatory cells. No other symptoms were present such as fever, weight loss, joint symptoms, diarrhea, etc. A tentative diagnosis of sarcoidosis was made and steroids were started by the pediatric ophthalmologists at Johns Hopkins. Referral was made to a pediatric rheumatologist. Further testing revealed mild hypoalbuminemia, anemia (Hgb 10.9 g/dL), iron deficiency, and double positive ASCA antibody screening. Evaluation by pediatric gastroenterology showed a normal growth curve with no significant change in growth parameters over the past several years, heme negative stools, but prominent inflammatory anal skin tags. Colonoscopy revealed severe Crohn’s involvement of the IC valve and distal ileum which was confirmed on biopsies and small bowel radiographic studies. Aggressive treatment initially with steroids and 6 MP and eventually with infliximab led to complete resolution of eye symptoms and all laboratory abnormalities along with normalization of the eye ultrasound. Endoscopic, radiologic and pathology findings will be presented. 52 CAVITARY LUNG LESIONS: A RARE EXTRAINTESTINAL MANIFESTATION OF CROHN’S DISEASE. Vadlamudi, Narendra 1; Mestre, Jose 1; Nogueira, Janaina 1; Maclin, Jeanine 1, 1. Pediatric Gastroenterology, Children's hospital of Alabama, Birmingham, AL, United States. 17 year old female known patient with Crohn’s disease (CD) presented with a three week history of progressive skin lesions on her right wrist and left shin, diarrhea and weight loss. She was diagnosed with CD in 2006 and was treated medically with Imuran and Mesalamine. Her disease was poorly controlled due to non‐adherence with medications. On examination, she had skin lesions over her right wrist, left shin and also on the left labia majora which were ulcerated, red, swollen and tender. She was admitted for suspected cellulitis and received intravenous antibiotics with no clinical response. While on antibiotics, she developed cough and fever. Her chest X‐ray showed bilateral opacities, concerning for pneumonia. CT scan of chest revealed several cavitary lesions in the lung and two round dense lesions in liver. CBC, CMP, ESR, CRP, LDH, Uric acid, immunodeficiency workup including neutrophil burst test, quantiferon, PPD, fungal cultures, ANA, ANCA and angiotensin converting enzyme level were all normal. Hemoccult was positive and stool calprotectin was significantly high indicating an active bowel inflammation. Imuran metabolite levels drawn at admission were undetectable. Bronchoalveolar lavage showed numerous macrophages and neutrophils but negative for bacteria, fungus, pneumocystis, mycobacteria and malignant cells. Liver biopsy showed non‐specific chronic inflammation with focal necrosis. All cultures including blood, urine, wound, sputum, bronchial wash and liver tissue were all negative for bacteria and fungi. Skin biopsy showed granulomatous inflammation concerning for possible metastatic Crohn's disease of the skin. Antibiotics were stopped and she was treated with steroids and Remicade which resulted in rapid clinical improvement and resolution of radiological signs in lung and liver. CD can present with varied extraintestinal manifestations but to our knowledge, this is the first reported pediatric case of cavitary lung lesions associated with Crohn’s disease. 53 A RARE CASE OF CROHN’S DISEASE PRESENTING AS INTESTINAL PERFORATION. Vadlamudi, Narendra 1; Thame, Kirk 1; Dimmitt, Reed 1, 1. Pediatric Gastroenterology, Children's hospital of Alabama, Birmingham, AL, United States. Poorly controlled Crohn’s disease (CD) can be associated with significant complications like intestinal obstruction, abscesses, fistulae, perforation and bleeding. Intestinal perforation as a presenting feature of CD is rare, especially in children. We report a case of a 14d year ol female who presented with six day history of fever, dyspnea, abdominal pain and intermittent diarrhea. Initial labs revealed elevated inflammatory markers, liver enzymes, gamma glutamyl transferase and direct bilirubin. CT scan of her abdomen showed a markedly thickened and enlarged gall bladder, concerning for possible cholecystitis as well oas tw dense lesions in liver concerning for liver abscesses. The initial diagnosis was sepsis from cholecystitis and intravenous antibiotic therapy initiated with no significant clinical response. Three days later she developed signs of peritonitis and shock requiring fluid resuscitation and mechanical ventilation. A repeat CT scan of her abdomen confirmed signs of peritonitis and also findings suggestive of inflamed appendix. She was suspected to have perforated appendix and underwent emergent laparotomy, which revealed slightly inflamed but intact appendix, extremely diseased terminal ileal bowel wall, significant creeping fat and perforation of ileum near the ileocecal valve. Histopathology of the resected terminal ileum revealed a diffuse inflammatory infiltrate with non‐caseating granulomas and stricture, suggestive of CD. She responded well to steroids and infliximab with resolution of both gastrointestinal and hepatobiliary symptoms. Subsequent colonoscopy revealed perianal skin tags, patchy mucosal ulceration in colon but normal findings on EGD and ileoscopy. She remained asymptomatic after a 3 month follow‐up. Presentations of CD can be varied leading to challenges in its diagnosis especially in patients with extraintestinal manifestations as presenting symptoms of CD. Pediatric gastroenterologists should be vigilant and include CD in the differential diagnosis in patients with acute intestinal perforation. 54 CROHN’S DISEASE PRESENTING AS LARGE PLEURAL EFFUSION IN A PEDIATRIC PATIENT. Vadlamudi, Narendra 1; Thame, Kirk 1, 1. Children's hospital of Alabama, Birmingham, AL, United States. An 11 year old African American female was admitted to the children’s hospital with a flare of her psoriasis and also a three week history of worsening non‐productive cough. Review of systems was negative for diarrhea, vomiting, abdominal pain, hematochezia, joint pain, weight loss and dyspnea. Past medical history was significant for poorly controlled psoriasis with frequent flares. At admission, she was febrile but other vital signs were normal. Examination was remarkable for weeping and crusting lesions in her groin and arm pits and also decreased air entry on left side of chest. The rest of the systemic exam including abdomen was non‐revealing. Chest radiograph showed evidence of large left‐sided pleural effusion with no obvious pulmonary parenchymal disease. Initial CBC, CMP, CRP, ESR, ANA, DsDNA, HLA B27, C3, C4 and immune work up were normal. PPD and quantiferon testse wer negative. She continued to have daily fevers and repeat CXR showed developing lung disease concerning for pneumonia. Intravenous antibiotic therapy was initiated with no significant clinical response. Blood cultures for bacteria, virus and fungi were negative. Repeat labs were negative except for dropping albumin level. CT scan revealed a large left pleural effusion with mass effect and left lower lobe consolidation. Bronchoalveolar lavage and pleural fluid analysis were nonspecific and negative for bacteria, virus, fungi and Mycobacteria. EGD and colonoscopy were performed due to persistent hypoalbuminemia and a new onset of diarrhea. These showed gastritis, duodenitis and diffuse active colitis,t consisten with CD. While waiting for biopsy results, she developed significant respiratory distress requiring chest tube placement and transfer to intensive care. She responded well to steroids and Remicade with resolution of both gastrointestinal and respiratory symptoms. She was discharged one week later and remained symptom free on Remicade at five month follow up. Pulmonary involvement in CD has been described but to our knowledge this is the first reported pediatric case of Crohn’s disease presenting as massive pleural effusion. 55 CROHN’S DISEASE OF THE LUNG IN CHILDREN.Vadlamudi, Narendra 1; Dimmitt, Reed 1; Harris, Tom 1, 1. Children's hospital of Alabama, Birmingham, AL, United States. Background: Pulmonary involvement in children with Crohn’s disease (CD) has been described but is very rare (<0.5%). The most frequent lung problems in patients with CD include eosinophilic pneumonia, fibrosing alveolitis and bronchiolitis. Majority of these lung manifestations appear to be secondary to sulfasalazine or mesalamine therapy. Latent pulmonary disease with bronchial hyperreactivity has been shown in children. Symptomatic pulmonary disease with granulomatous lung changes is extremely rare. The aim of this study was to describe the clinical characteristics of CD of lung in children. Results: A total of fifteen pediatric cases previously reported in English literature. Age ranged from 3‐17 years and with no significant gender discordance (7 male and 8 female). The lung disease was predominantly bilateral (10 patients). One patient had normal lung parenchyma with predominant large airway disease. The most frequent respiratory symptoms were cough and dyspnea. Pulmonary symptoms occurred after the diagnosis of CD was established in majority of patients (8 patients). In the remaining patients, respiratory symptoms appeared with (3 patients) or preceded (4 patients) the onset of bowel symptoms. Among the patients with prior diagnosis, CD was stable in five and was poorly controlled in two patients. One patient developed respiratory symptoms 4 years after having colectomy for poorly controlled disease. All patients had colon involved at initial diagnosis and in five patients the disease was limited to colon. Four patients were on either sulfasalazine (1 patient) or mesalamine (3 patients) at the time of onset of pulmonary symptoms. The majority (7 of 9 reported) of patients had restrictive lung pattern on pulmonary function tests. All the patients had chronic granulomatous inflammation and all but two cases showed characteristic noncaseating granulomas on lung biopsies. Majority of patients (ten) responded to conventional therapy but three patients required infliximab. Conclusion: Awareness of pulmonary manifestations of CD in children and timely lung biopsy may lead to more prompt diagnosis, appropriate therapy and decreased morbidity. 56 EXTRACORPOREAL PHOTOPHERESIS FOR TREATMENT OF REFRACTORY STEROID DEPENDENT PEDIATRIC CROHN’S DISEASE.Cheerva, Alexandra C2; Dillard, Robert P1; Bertolone, Salvatore 2, 1. Pediatric Gastroenterology, University of Louisville, Louisville, KY, United States. 2. Pediatric Hematology & Oncology, University of Louisville, Louisville, KY, United States. We present, to our knowledge, the first use of extracorporeal photopheresis (ECP) in a pediatric patient, for treatment of Crohn’s disease uncontrolled by aggressive standard therapy. Studies of ECP in steroid dependent adults with moderate to severely active Crohn’s disease have shown response rates up to 50%, with up to 25% complete. (1‐3) Our patient, a 12 year old white male, had severe unremitting disease, despite treatment with anti‐inflammatory, immunosuppressive and biologic agents. Elemental diet enteral nutrition failed, requiring TPN. A diverting colostomy for perforation and intractable ano‐ rectal disease was required as was frequent hospitalization and homebound schooling. Repeat endoscopy prior to ECP revealed ongoing severe inflammation. ECP was begun twice weekly for 4 weeks, then twice weekly every other week for a total of 26 weeks. ECP was well tolerated and prednisone was discontinued. Azathioprine and Infliximab at 6 week intervals were continued. TPN was weaned as enteral intake improved. Disease abatement allowed a return to school and activities. Endoscopy showed normal upper tract, normal ano‐rectum and decreased, though significant, colonic disease. This response continues since finishing ECP. We conclude that ECP is useful, deserving consideration and prospective evaluation in refractory steroid dependent pediatric Crohn’s disease. References: 1 Abreu MT, von Tirpitz C, Hardi R, et al. Extracorporeal Photopheresis for the Treatment of Refractory Crohn’s Disease: Results of an Open‐Label Pilot Study. Inflamm Bowel Dis 2009;15:829‐836. 2 Bisaccia E, Palangio M, Gonzalez J. Extracorporeal Photochemotherapy for the treatment of refractory Crohn’s disease. Transfus Apher Sci 2007;37:171‐174. 3 Reinisch W, Nahavandi H, Santella R et al. Extracorporeal Photochemotherapy in patients with steroid‐dependent Crohn’s disease: a prospective pilot study. Aliment Pharmacol Ther 2001; 15:1313‐1322. 57 IN‐PATIENT BOWEL REHABILITATION (IBR) FOR CHILDREN WITH SIGNIFICANT FECAL SOILING: A 5 YEAR EXPERIENCE. Sankararaman, Senthilkumar 2; Lu, Wexin 1; Hussain, Sunny Z1, 1. WK Pediatric Gastroenterology & Research, Shreveport, LA, United States. 2. Pediatrics, LSUHSC, Shreveport, LA, United States. Objective: To assess effectiveness of an IBR program for the management of fecal soiling in children. Method: Children (5‐16 years) with significant fecal soiling were enrolled over the period of 5 years. A planned approach included an examination of anorectal under general anesthesia (EUA) and if necessary, manual fecal disimpaction. A nasogastric tube (NGT) was placed while the patient was still under the anesthesia. Colonic cleanout was performed overnight with bolus dose of magnesium citrate (6‐10oz), followed by Golytely infusion (100‐ 150 cc/hr). The caregivers were given adequate advice and counseling regarding bowel training. The patients were discharged home on twice daily polyethylene glycol (17g) which was gradually tapered. Patients were instructed to sit on the commode for 10‐15 minutes twice a day. Follow‐up visits were arranged in 2 weeks and a later one in 3 months. No control group was used as placebo was not suitable given the clinical complexity of these patients in our center. Results: Out of 131 children undergoing the IBR, 87 completed the both the visits. There were 63 boys and 24 girls, age ranging from 5 to 16.5 years. The soiling duration of soiling ranged from 1 month to 10 years. Majority (70/87) of the patients had fecal impaction. Among the 87 patients, 13 patients lived with step parents or had other family issues, 18 had bed‐wetting and 40% patients had Medicaid (n= 36). Psychological issues were noticed in 30% (n=27). The soiling‐free status was observed in 94.3% (82/87) after 2 weeks and 72.4% (63/87) after 3 months. Discussion: The high success rate for IBR materialized for three reasons. The patient and the family paid close attention as they were stepped aside from their busy schedules. Secondly, the child became more compliant to avoid another encounter with NGT Golytely. Thirdly, the prior fecal disimpaction made the colonic cleanout process much more efficient. Conclusion: IBR program is an extremely effective modality in the management of fecal soiling although psychological issues remain a barrier to long‐term successful outcome. 58 PEDIATRIC CROHN’S DISEASE PRESENTING AS UPPER GASTROINTESTINAL OBSTRUCTION. Falaiye, Tolulope 1; Warolin, Joshua P1; Martinez, J. Andres1; Gillis, Lynette 1; Arthur, Catherine 1; Acra, Sari A1; Moulton, Dedrick E1; Rosen, Michael J1, 1. Pediatric Gastroenterology, Vanderbilt University, Nashville, TN, United States. Case 1: A 15‐year‐old male with Asperger’s syndrome presented with 3 weeks of emesis and slow weight gain over last 2 years. BMI was 14.4. The patient’s father had a colectomy for ulcerative colitis. Laboratory evaluation was notable for a CRP of 55.7 mg/L, Alb of 3.1 g/dL and hemoglobin of 10.5 g/dL. Abdominal CT showed bowel thickening of the duodenum and the proximal jejunum with mucosal enhancement of the jejunum and ileum. EGD revealed severe erythema and edema in the first portion of the duodenum causing a stenosis that precluded passage of a 5.9mm endoscope. Colonoscopy revealed scattered apthous ulcers and a pseudopolyp in transverse colon and a thickened ileocecal valve. Pathology was supportive of Crohn’s disease with granulomata, duodenitis and proctitis. After two days of IV solumedrol, the patient was able to tolerate oral intake. Case 2: A 13‐year‐old male with trisomy 21 presented with 3 weeks of emesis unresponsive to PPI. Laboratory evaluation was notable for platelets of 490. EGD revealed 800ml of retained gastric fluid. The gastric mucosa appeared erythematous and edematous with ulceration of the pylorus. There was severe pyloric stenosis precluding intubation of the duodenum with a 5.9 mm endoscope. Colonoscopy revealed a severe stricture in the right colon. Pathology showed active gastritis and colitis with eosinophilia. Abdominal CT showed noncontiguous segments of bowel edema affecting the gastric antrum, duodenal bulb, terminal ileum and the cecum. Gastric CMV PCR, AFB and H pylori stains were negative. Neutrophil oxidative burst testing for chronic granulomatous disease (CGD) was negative. The patient was started on steroids and mercaptopurine. Patient improved clinically and on repeat imaging. While uncommon, Crohn’s disease with severe gastroduodenal involvement may present with persistent emesis from high grade upper GI obstruction. Other etiologies to be considered include tuberculosis, CGD, CMV, angioedema and lymphoma. NUTRITION/NUTRITION SUPPORT 91 HIGH PREVALENCE OF VITAMIN D INSUFFICIENCY IN NON‐WHITE OBESE CHILDREN AND ADOLESCENTS. Ashai‐Khan, Farhat N1, 2; Mullins, Denise 1; Stolfi, Adrienne 1, 2; Burke, Ann 1, 2; Amisola, Rogelia 1, 2; Ebert, James 1, 2, 1. Gastroenterology, Children's Medical Center, Dayton, OH, United States. 2. Department of Pediatrics, Wright State University Boonshoft School of Medicine, Dayton, OH, United States. Background: Vitamin D insuffiency/deficiency (VDI) has been well described in children with high BMI, insulin resistance, hypertension and associated comorbidities. Aim: To determine the prevalence of VDI in a pediatric lipid clinic and factors that may be associated. Method: A retrospective chart review of 229 new patients referred to our lipid clinic for obesity from May to October 2010 was conducted. Patients with vitamin D drawn at the time of initial visit were included. Age, race, gender, BMI, blood pressure, heart rate, REE, lipid panel, fasting insulin and glucose, hs‐CRP, ALT, and AST were collected. Total hours per week spent on the computer or watching television(CTV) were obtained from parent report. Patients were divided into those with Vitamin D levels ≤30 ng/ml (VDI) vs >30 ng/ml (vitamin D sufficient, VDS). Comparisons were made with chi‐square tests, two‐sample t tests and multiple logistic regression. Results: Sixty‐seven of the 229 patients had vitamin D levels drawn. Of the 67, 39% were male, 61% white, and mean (SD) age was 12.4 (3.2) years. All but one patient had BMI >95th percentile. Forty‐six (69%) were VDI. There was no association with age, gender, CTV, or laboratory values other than fasting insulin. Mean fasting insulin was 24 (13) in the VDI group vs 16 (11) in the VDS group (p=0.013). Non‐white race was significantly associated with VDI (56% vs 5% in VDS group, p<0.001). In logistic regression, only race remained significant (adjusted odds ratio 19, 95% CI 3‐166). None of the patients had prior screening for vitamin D, and none were taking viatmin D supplements. Conclusion: Prevalence of vitamin D insufficiency is high in non‐white obese children and adolescents. Vitamin D screening and supplementation should be encouraged in this high risk group. 92 BEZOAR AND FAILURE TO THRIVE. Squires, Jennifer C.1; Lukacik, Marek 2; Nguyen, Kim‐Doan K.2, 1. Pediatrics, Georgia Health Sciences University, Augusta, GA, United States. 2. Pediatrics, Division of Pediatric Gastroenterology, Georgia Health Sciences University, Augusta, GA, United States. INTRODUCTION: Bezoars are an uncommon association with failure to thrive (FTT), and their presence and significance is unknown. CASE REPORT: A 2‐year‐old Caucasian female was admitted for FTT and suspicion of neglect. Her previous extensive work‐up and evaluation provided no single etiology of FTT. EGD showed a gastric to duodenal bezoar consisting of food particles, including undigested noodles and pickles. Further questioning revealed the patient had poor mastication habits and a diet inappropriate for her age. The patient gained weight without diarrhea or vomiting on NG feeds consisting of semi‐ elemental formula, once caloric goals were met. DISCUSSION: Bezoars are very uncommonly found in FTT and can cloud the clinical picture. Review of the literature revealed one case of trichobezoar proximal to the ileocecal valve found in a patient with FTT. This is the first case report associating a food bezoar with FTT, found in the stomach and extending to the duodenum. Further studies into the association and significance of bezoars and FTT would determine whether this is a coincidental finding or related to the etiology of FTT. 93 SELF ADVANCING POST‐PYLORIC FEEDING TUBE IN CRITICALLY ILL CHILDREN. Khlevner, Julie 1; Antino, Janice 1; Panesar, Rahul 1; Chawla, Anupama 1, 1. Pediatric Gastroenterology, Stony Brook Children's , Stony Brook, NY, United States. Nutrition requirements are increased in trauma patients in the Pediatric Intensive Care Unit(PICU).Early nutrition support is an integral part of the care of critically ill children.Early enteral feeding(EF) is associated with significant advantages over late EF or total parenteral nutrition(TPN).It improves nitrogen balance,and prevents bacterial translocation and gut mucosal atrophy. Adequate EF is often not achieved as gastric feeds are not tolerated and placing postpyloric feeding tubes can be difficult.Spontaneous transpyloric passage of standard feeding tubes after 24 hours is only in the order of 30% and does not seem to be affected by tip profile or weight added to the tip of the feeding tube.The Tiger2 self‐advancing nasal jejunal feeding tube is a 14Fr polyurethane feeding tube with flaps that gently drag the catheter into the jejunum.Tiger2 tubes have been used in the adult population with great success but to our knowledge there have been no reports of its use in pediatric age group. We present 5 critically ill patients 12‐19 years old, admitted to PICU, in whom prolonged recovery,inability to tolerate gastric feeds or dependence on ventilator was predicted at the outset.Self advancing nasojejunal feeding tube was successfully placed on first try at the bedside in all of the five patients within the first 24hrs without the use of promotility agents.Radiograph confirmed the tip of the tube in the 3rd portion of duodenum in all 5 patients. Percent of nutrition goal achieved within 48hrs of admissionU to the PIC was compared between the critically ill Tiger2 tube population and those who received nutrition via nasogastric route ±TPN. Comparison of nasogastric feeds ±TPN vs postpyloric feeds (Tiger2) showed that children with postpyloric feedings reached their nutritional goal much earlier(Table1)and did not require TPN. Conclusion:Tiger2 tube can be used effectively to establish early enteral nutrition in critically ill children. 94 GASTRIC BALLOON IN AN OBESE ADOLESCENT. Jean, M. Raphaelle1; Oliveira, Stephanie B1; Monteiro, Iona M1, 1. Pediatrics, UMDNJ‐ New Jersey Medical School, Newark, NJ, United States. Childhood obesity in the United States has reached pandemic proportions. More intensive treatment regimens have been proposed for obese children and adolescents unable to control their weight, including pharmacotherapy and bariatric surgery. CASE REPORT: 17‐year‐old male was evaluated for intermittent epigastric abdominal pain, nausea and vomiting for a month. He had no diarrhea or constipation. One month prior he was started on Orlistat by his medical doctor, which was discontinued a week prior after an emergency room visit for his symptoms. He underwent intragastric balloon placement in the Dominican Republic a year earlier. His exam was unremarkable except for morbid obesity (BMI 41.39). On Esophagogastroduodenoscopy, a 3.8 inch deflated gastric balloon was noted in the fundus, which was then removed. He tolerated the procedure well with resolution of his symptoms. DISCUSSION: Complications related to intragastric balloon placement and removal have been reported. There is a significant increase in risk for complications if the balloon is left in place for more than 6 months, as in our case. Complications reported include: intolerance presenting with nausea, vomiting and abdominal pain; small bowel obstruction; pancreatitis and gastric perforation. Complications have also been reported in the first days after the insertion of balloon, including: acute abdomen, cardiac arrest, massive gastric necrosis, and death. Subacutely gastric outlet obstruction, steatohepatitis, pancreatitis and tachyarrhythmias have been reported. Most of these cases are in adults. As rates of obesity in childhood and adolescence are increasing, more intensive therapies can become more common in the this population in the near future, as well as their complications. CONCLUSION: Physicians should recommend removal of the intragastric balloon no later than 6 months post placement, because of a significant increase in risk of complications. Given the prevalence of international migration, Pediatric Gastroenterologists should be cognizant of intragastric balloons and their complications despite the fact that they are not FDA approved in the United States. 95 THE BROAD CLINICAL SPECTRUM OF HEREDITARY FRUCTOSE INTOLERANCE. Himes, Ryan W.1; Kitagawa, Seiji 1, 1. Pediatrics, Baylor College of Medicine, Houston, TX, United States. Two unrelated cases of hereditary fructose intolerance (HFI; OMIM# 229600) were diagnosed within a six week period. Widely disparate presentations and acuity are highlighted as are diagnostic clues to this uncommon entity. Patient #1 ‐A 2‐month‐old male presented to the emergency center for vomiting that began after being treated for presumed milk protein allergy by changing the formula from a lactose‐based product to a sucrose‐containing formula. ‐Because of persistent vomiting, he was supplemented with a mixture of boiled rice and sucrose, a folk remedy, which exacerbated his vomiting. ‐Initial labs: Na 143, CO2 12, BUN 5, creatinine 1.16, glucose 47, AST 444, ALT 206, INR 8.3, conjugated bilirubin 2.1, ammonia 179, albumin 3.2, lactate 10.2. UA: 3+ glucose, 1+ ketone, 4+ protein, 3+ reducing substances. ‐By history HFI was suspected and the patient was changed back to a lactose‐based formula pending ALDOB gene sequencing; full recovery was made following diet modification. ‐He is a compound heterozygote for two mutations described in patients with HFI, c.448G>C (p.A150P) and c.178C>T (p.R60X). Patient #2 ‐A 12‐year‐old female was seen in the clinic for abdominal pain and malaise after ingesting “sugar” ‐She began with vomiting at 4‐months‐of‐age, coinciding with addition of fruit to her lactose‐based formula diet. ‐Liquid medicines were also invariably vomited and an extra bottle was routinely prescribed for re‐dosing. ‐In time she developed aversion to fruit, beans, tomato, soda, candy and juice. With dietary transgression, she has generalized abdominal pain, nausea and malaise but not vomiting or diarrhea. ‐On exam, she was slightly built (BMI 11th centile), Tanner III and did not have HSM. A CBC, chemistry and liver panels, INR, AFP and a UA were normal. ‐Based on the diet history and vomiting of liquid medications (which frequently contain sucrose, fructose or sorbitol as excipients), ALDOB sequencing was obtained to support the clinical impression of HFI. ‐She is a compound heterozygote for two mutations described in patients with HFI, c.448G>C (p.A150P) and c.113‐1_115delGGTA (p.G38del). 96 KWASHIORKOR IN A STUBBORN TODDLER. Bitton, Samuel 1; Weinstein, Toba A1; Levine, Jeremiah J1; Pettei, Michael J1, 1. Pediatric Gastroenterology and Nutrition, Cohen Children's Medical Center, New Hyde Park, NY, United States. Kwashiorkor is commonly observed in developing regions as a consequence of inadequate protein intake and characterized by dermatitis, edema and abdominal distension. In the US predominant protein malnutrition without a predisposing condition is rare. Kwashiorkor has been reported in the US in infants inappropriately placed on restrictive diets. We describe a case of kwashiorkor in a 3.5 y.o. Caucasian boy from suburban Long Island, NY. Case History: Following episodes of infection at about 1 y.o. he began to take mainly cow’s milk with few solids. About 8 months prior to presentation, his milk intake was restricted to encourage solid intake, but to no avail. He then refused the re‐introduction of milk and would only consume orange juice and banana purée, a very low protein diet. Behavioral therapy and supplementation were attempted. After 3 months of a worsening rash unresponsive to topical steroids, the child presented at 3.5 y.o. with swollen ankles and difficulty walking. On exam, the BMI was <3% and he was irritable, with a rounded face, protuberant abdomen without hepatomegaly, and an erythematous, pruritic, ‘flaking paint’ rash over most of his body. There was also pitting edema of his ankles. His laboratory studies were significant for albumin 2.9 g/dL (nl 3.3‐5), prealbumin 10 mg/dL (20‐40), thyroid binding globulin 11 mcg/ml (12‐26), and transferrin 70 mg/dL (200‐360). Linoleic acid, selenium, zinc, and copper levels were also low. The patient was aggressively treated with a standard nutritionally complete formula via NG tube. Within the first 48 hours there was marked improvement of both his rash and disposition. Conclusion: Kwashiorkor is a rarely‐seen, often delayed diagnosis in American children. This case of kwashiorkor resulted from a self‐selection of protein deficient foods in a toddler with a behavioral feeding disorder, despite conventional pediatric care. Treatment includes correction of deficiencies with attention to avoidance of refeeding syndrome. Prevention requires aggressive nutritional support when known nutritional inadequacies are present. 97 PANCYTOPENIA FROM SEVERE VITAMIN B12 DEFICIENCY IN JOHANSON‐BLIZZARD SYNDROME. Rashid, Mohsin 1; Aubrey, Stephanie 1, 1. Dalhousie University, Halifax, NS, Canada. BACKGROUND: Johanson‐Blizzard syndrome is a rare disorder characterized by hypoplastic nasal alae, visual and hearing impairment and exocrine pancreatic insufficiency. Therapy includes pancreatic enzyme replacement and fat soluble vitamins. Vitamin B12 (cobalamin) deficiency can occur rarely in pancreatic insufficient patients although the precise mechanisms remain unclear. Two distinct cobalamin‐binding proteins heptocorrin (R binder) and intrinsic factor (IF) compete for free vitamin B12 released in the stomach. In the acidic environment, haptocorrin binds free vitamin B12 with much greater affinity than IF. Pancreatic enzymes degrade the haptocorrin‐vitamin B12 complex in the small intestine, releasing free vitamin B12 to bind to IF for its eventual absorption in the terminal ileum. Vitamin B12 deficiency has not been reported in Johanson‐Blizzard syndrome. CASE: A 19 year‐old male adolescent with Johanson‐Blizzard syndrome presented with severe fatigue, anorexia and weight loss. He had been followed at the GI clinic since infancy and had remained generally well with oral pancreatic enzymes and fat‐soluble vitamin supplements. Examination was significant for marked pallor. The hemoglobin was 55 (g/L), WBC 1.8 and platelet count 53. The MCV was high at 106.3fl. Bone marrow revealed hypercellularity but no malignancy. Ferritin, folate and vitamin E levels were normal. Vitamin B12 level was low at 81 pmol/L. Serum homocystine and methylmalonic acid were markedly elevated. Patient required a blood transfusion and was given parenteral vitamin B12 daily for a week. Within a few days, the pancytopenia started to improve. He was discharged on oral vitamin B12 at 1 mg daily. At follow‐up a month later, the symptoms had resolved, the blood counts and vitamin B12 level were normal. CONCLUSION: Severe vitamin B12 deficiency can occur in patients with Johanson‐Blizzard syndrome and pancreatic insufficiency. Routine monitoring of vitamin B12 status should be considered in these patients for early detection of deficiency and to avert serious complications. 98 PLASTICIZED GLUTEN IN A DENTAL RETAINER OF A CHILD WITH CELIAC DISEASE. Memon, Zebunnissa 1; Hashmi, Humaira 1; Baker, Susan 1; Baker, Robert 1; Gelfond, Daniel 1, 1. University of Buffalo, Buffalo, NY, United States. Objectives: Case report of a persistent elevation of celiac serology in a child with celiac disease on a strict gluten free diet attributed to plasticized gluten in a dental retainer. Background: Celiac disease is a gluten enteropathy that is treated with dietary elimination of gluten. There is evidence to demonstrate that as little as 50mg of gluten ingestion, which is 1/40th of a slice of bread, in individuals with celiac disease can lead to clinical symptoms, persistence of celiac serology and histological changes consistent with disease. However non‐dietary sources of gluten which are used in the manufacture of products like plastics, dental equipment and cosmetics might trigger or exacerbate celiac disease. Case presentation: A 9 year old child who presented with non specific abdominal discomfort and elevated celiac serology was diagnosed with Celiac disease after duodenal biopsies revealed Marsh 3B histopathology. Despite strict dietary elimination of gluten, she continued to have elevated tissue transglutaminase and marginal improvement of her symptoms. When the focus was turned toward potential non‐dietary sources, it was discovered that the child’s orthodontic retainer contained a plasticized methacrylate polymer, in which gluten is a common additive. She discontinued its use and demonstrated complete normalization of her serology as well as improvement of her symptoms. Conclusion: We present a case of a child with celiac disease, persistence of clinical symptoms and elevated tissue transglutaminase while on strict gluten free diet, triggered by non dietary plasticized gluten (methylmethacrylate) in the dental prosthesis. Increased utilization of gluten in the material manufacturing industry arises from improved technical properties as well as an increased demand to create biodegradable materials. Healthcare providers need to be vigilant when providing dietary advice and guidance to patients with celiac disease. HEPATOBILIARY/TRANSPLANT 115 ACUTE HEPATITIS C (HCV) INFECTION WITH NEGATIVE SEROLOGY MIMICKING AUTOIMMUNE HEPATITIS. Paul, Adam 1; Safta, Anca 1, 1. Pediatrics, University of Maryland Children's Hospital, Baltimore, MD, United States. Autoimmune hepatitis is a chronic inflammatory disorder of the liver. In more than 50% of AIH cases there can be a similar clinical and histological presentation to acute viral hepatitis. As part of the initial workup, hepatitis antibodies, together with a liver biopsy, are obtained to rule‐out viral causes. A 17 year old female presented with a six day history of abdominal pain, decreased PO intake, jaundice, vomiting and severe transaminitis. Hepatitis A, B, and C, adenovirus, EBV, CMV, HIV antibodies, serum ceruloplasmin, serum copper, thyroid, A1AT level and phenotype, tissue tranglutaminase antibodies and toxicology screen were negative on admission. Anti‐smooth muscle antibody, liver‐kidney microsomal antibody, and ANA were normal. The initial liver biopsy showed marked acute hepatitis consistent with autoimmune hepatitis (AIH). Therapy with prednisone, azathioprine (AZA), and ursodiol was initiated. As the transaminitis resolved, steroids were weaned and she continued AZA. Soon after discontinuation of steroids the patient had recurrence of abdominal pain, fatigue, jaundice and vomiting. Transaminases later increased with the concern of superimposed infection, flare of the presumed AIH, or AZA induced hepatitis. A repeat infectious work up yielded a positive HCV antibody followed by positive HCV RNA (RT‐PCR). All immunesuppresants were discontinued and HCV therapy was contraindicated secondary to the patient's severe depression and substance abuse history. Transaminitis resolved and HCV RNA decreased by one log and was not detectable at one month and two month follow‐up, respectively. The initial presentation is most likely due to acute HCV infection with negative HCV serology and histology mimicking AIH. Obtaining HCV RNA at the initial visit despite negative HCV serology would have made a timely diagnosis. Since HCV antibody cannot be reliable for the diagnosis of infection in the very early stage, we suggest obtaining HCV RNA early in the course with equivocal histology so that diagnosis is not confused with that of AIH. 116 A 7 MONTH OLD INFANT WITH CHOLELITHIASIS REQUIRING CHOLECYSTECTOMY. Hyunh, Sandy 1; Badalyan, Vahe 1; Enav, Ben 1, 1. Inova Fairfax Hospital for Children, Falls Church, VA, United States. A 7 month old African American boy presented with one day of vomiting (~ 10 episodes) after feeds. Initially, he vomited gastric contents, but progressed to have bilious vomiting. Between episodes the baby was comfortable. Parents denied any fever, diarrhea, or stool changes. There were no sick family contacts. The infant was born full‐term. He had a sinus infection two months before, which was treated with amoxicillin. His growth and development were on target. His family history was negative for gastrointestinal problems or hemolytic disease. There was no pet exposure or recent travel. On physical exam the patient had normal vital signs. He had tears, but tacky oral mucosa. Auscultation of his heart revealed no murmurs. His abdomen was soft, non‐distended, and non‐tender. He had no hepatosplenomegaly on palpation. His skin was dry with capillary refill of 3 seconds. His CBC, metabolic panel, liver panel, lipase, CRP, and urinalysis were within normal limits. His abdominal x‐ray showed no obstruction, but his abdominal ultrasound revealed gallstones, gallbladder wall thickening, and pericholecystic fluid. His pancreas was normal, and his biliary tree was not dilated. The infant was admitted for re‐hydration and PO challenge. He continued to have bilious emesis after feeds. He underwent laparoscopic cholecystectomy, during which multiple irregular black calculi ranging from <0.1cm to 0.4cm were retrieved. Stone analysis showed a 100% carbonate apatite content. Pathology report showed chronic cholecystitis with cholelithiasis. Further workup showed normal hemoglobin electrophoresis and osmotic fragility tests. Patient was discharged within 36 hours after the surgery, feeding well and with no further vomiting. Cholecystitis and cholelithiasis are rare entities in otherwise healthy children. Black pigment stones are usually complications of chronic hemolytic anemias. Our patient makes for an unusual presentation of this rare pediatric condition, in that he was well appearing, had normal lab values, and negative personal and family history of hemolytic disease. 117 UNUSUAL CASE OF BRUCELLOSIS AND WILSON'S DISEASE, FOLLOWING A TRIP TO INDIA. Prasad, Deepa 1; Bal, Aswine 2; Soroush, Azam 1, 1. Pediatrics, Jersey Shore University Medical Center, Neptune City, NJ, United States. 2. Pediatrics, Robert Wood Johnson Medical school, Neptune, NJ, United States. Case summary: A previously healthy, 11 year old boy presented with intermittent vomiting, fever and jaundice for 3 weeks. He had a history of recent travel to India, where he developed diarrhea and jaundice. After initial workup, he was diagnosed with nonspecific hepatitis. Physical exam was significant for stable vital signs, scratch marks on the thighs, icterus, pallor and splenomegaly. Lab work showed anemia, reticulocytosis, hypoalbuminemia, AST 76 U/L (5‐40), ALT 16 U/L (6‐36), ALP L10 U/ (80‐250), ESR 67mm/hr, INR 2.1 (0.8‐1.2), total bilirubin 11.5 mg/dl (0‐1.5), direct bilirubin 3.9 mg/dl (0‐0.5) and negative coombs test. Thick and thin smears for malaria were negative. Laboratory work up for autoimmune and infectious etiologies of hepatitis was negative. Brucella IgM antibody by ELISA was positive and febrile agglutinin test was positive for B. Abortus. CT of the abdomen revealed micro nodular liver suggestive of cirrhosis, splenomegaly, mild ascites, and extensive adenopathy in the mesentery. He was treated with doxycyline and rifampin for Brucellosis. Due to the presence of liver cirrhosis, hemolysis and very low ALP, further work up was done to evaluate for underlying chronic liver disease. It showed serum ceruloplasmin of 4.7 mg/dl (16‐36) and urinary copper of 258 mcg/day (3‐75). Liver biopsy done a month later showed cirrhosis with focal copper accumulation in hepatocytes, consistent with Wilson’s disease (WD). Conclusion: In this case report, we describe a child presenting with acute Brucella hepatitis, which unmasked WD. Upon review of literature, there are no case reports of concurrent diagnosis of Brucellosis and WD. Brucellosis is very rare in United States but it is important to consider it in a patient with fever and jaundice, with a history of animal exposure or travel to an endemic area. 118 LIVER TOXICITY OF ANABOLIC ANDROGENIC STEROID USE IN AN ADOLESCENT WITH NONALCOHOLIC FATTY LIVER DISEASE. Yu, Elizabeth Louise1, 2; Awai, Hannah Idiong1, 2; Schwimmer, Jeffrey B1, 2, 1. Department of Pediatric Gastroenterology, Hepatology and Nutrition, University of California, San Diego, San Diego, CA, United States. 2. Rady Children's Hospital, San Diego, CA, United States. Introduction: Nonalcoholic fatty liver disease (NAFLD) is particularly common in adolescent males. Moreover, anabolic androgenic steroid (AAS) use is also increasing in adolescentS males. AA use is often unregulated and potentially hepatotoxic. Case: 18 year‐old male with NAFLD presented with 3 days of scleral icterus, intermittent emesis and difficulty with concentration and increased irritability. Labs demonstrated cholestasis and elevated aminotransferases (direct bilirubin 10.4 mg/dL, AST 163 U/L, ALT 229 U/L). Patient reported taking 40 mg of an anabolic steroid (Beastdrol) for 2 weeks prior to presentation to increase muscle tone while weight lifting. Physical exam demonstrated BMI 34 kg/m2, scleral icterus, jaundice and hepatomegaly. Other etiologies were investigated and ruled out including acute viral hepatitis (HAV, HBV, HCV, HIV, EBV, CMV, adenovirus, enterovirus)d an autoimmune hepatitis. A liver biopsy demonstrated cholestatic hepatitis and an inflammatory infiltrate of eosinophils and neutrophils suggestive of immunoallergic drug‐induced hepatotoxicity – consistent with injury secondary to AAS use. Patient was treated with ursodiol, lactulose, anti‐pruritic therapies and cessation of AAS. Direct bilirubin peaked at 32.6 mg/dL at one month and slowly decreased over the following 2 months. The patient suffered from intense pruritus with associated sleep disturbance. We speculate that pre‐existing liver disease, NAFLD, makes adolescents more vulnerable to the hepatotoxicity of AAS. Given the prevalence of NAFLD and the increasing use of AAS, there is a need for enhanced diagnosis, education, and awareness. The counseling of patients with NAFLD, on avoidance of hepatotoxins, should include discussion of supplements and anabolic steroids. 119 HEPATOCELLULAR CARCINOMA (HCC) IN CHRONIC HEPATITIS B: A CAUSE FOR MODIFYING CURRENT SCREENING CRITERIA? Prince, Esther 1; Xu, Jiliu 1; Rabinowitz, Simon 1; Schwarz, Steven 1, 1. Peds GI, SUNY Downstate, Brooklyn, NY, United States. HCC is a relatively rare complication of chronic hepatitis B infection (HB) in children and particularly in non‐cirrhotic HB. HBV genotype C, especially in the presence of basal core promoter (BCP) mutation , has been associated with an increased risk for HCC. Although early detection of this life‐threatening neoplasm is essential, currently approved guidelines do not suggest screening for HCC in children unless there is presence of cirrhosis or a family history of HCC. We report an adolescent male with asymptomatic, biochemically indolent HB, who developed HCC; and, suggest a modification in current screening approaches. Case Report: A 16 yr old mainland Chinese male with perinatally acquired, asymptomatic HB, presented for evaluation. Initial laboratory studies showed: AST 41 U/L (normal 10‐ 35), ALT 75 U/L (normal 9‐40) and AFP 206 ng/ml (normal <40). Viral studies included: HBeAg (‐), HBeAb (+), HBV DNA >10^9 copies/mL, genotype C with precore and BCP mutations. In 2 mos, AFP = 1400 ng/ml, abdominal U/S and follow‐up MRI showed a solitary 2.1x1.7x1.5 cm arterial enhancing mass within the left hepatic lobe with heterogeneous enhancement and rapid washout, consistent with HCC. Guided liver biopsy confirmed HCC, and the patient was started on anti‐HBV treatment and underwent a successful left lobectomy. Conclusion: 1. This report suggests that individuals with HB emigrating from “high‐risk” geographic regions should undergo screening, regardless of age or evidence of active liver disease, for HCC‐associated viral genotypes and BCP mutations. 2. Evidence of HBV genotype C and/or BCP mutations should prompt frequent biochemical/radiologic evaluation for HCC. Speculation: We recommend that the current AASLD Guidelines for HCC screening be modified to include these younger age, high‐risk subjects. 120 TYROSINEMIA IS NOT JUST LIVER!! Alnsour, Reem 1; Altamimi, Eyad 1, 1. Pediatric Department, Mu'tah University, Alkarak, alkarak, Jordan. 7 year‐old‐girl with 4 years history of painful plantar and palmar hyperkeratotic lesions, worsen with eating meat, dairy product and poultry. Referred to our pediatric Gastrointestinal Clinic to role out food allergy. The lesions were so painful, preventing the child from walking, requiring multiple ER visit for pain control. Her younger brother started to have similar lesions. Her tongue showed hyperkeratosis also. The lesions suggested tyrosinemia type 2. She had learning disabilities but her neurological exam was normal. Ophthalmological evaluation was normal. Her Amino Acid Chromatography showed very high levels of Tyrosine (urine amino acid not done) confirming the diagnosis. She was started on low protein diet and supported with low Phenylalanine and Tyrosine Formula. Her symptoms improved. 121 SECONDARY SYPHILITIC HEPATITIS IN A 17‐YEAR‐OLD GIRL. Lin, Henry 1; Russo, Piere 2; Rook, Michelle 1, 1. Division of Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA, United States. 2. Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA, United States. We present an unusual presentation of syphilis associated cholestatic hepatitis. A 17 year‐old African American female presented with a painless skin rash on her neck and abdomen. In 2 weeks, the rash spread to her hands and feet. She developed scleral icterus and jaundice. Physical exam was otherwise unremarkable with no hepatosplenomegaly. The patient was sexually active. Initial labs revealed a total bilirubin 7.5 mg/dL, conjugated bilirubin 5.4 mg/dL, alanine aminotransferase (ALT) 2048 U/L, aspartate aminotransferase (AST) 1551 U/L, gamma‐glutamyl transferase (GGT) 116 U/L, and alkaline phosphatase 138 U/L. Assays for Hepatitis A, B, and C, Cytomegalovirus, Epstein‐Barr virus, Wilson disease, alpha‐1 antitrypsin deficiency, and celiac disease were negative. Anti‐Nuclear Antibody was positive (1:80 titer) as was Anti‐Smooth Muscle Antibody (1:160 titer). Abdominal ultrasound with doppler flow was normal. Given the palmer rash, the patient was evaluated for syphilis and had a reactive RPR (1:64 titer) and a reactive Fluorescent Treponemal Antibody. The patient was treated for secondary syphilis with 2.4 million units of Penicillin G Benzathine. The patient's hepatitis worsened with total bilirubin 21.9 mg/dL, conjugated bilirubin 13.3 mg/dL, ALT 3826 U/L, and AST 3448 U/L. She became coagulopathic with INR of 1.66. Liver biopsy showed hepatitis characterized by marked hepatocellular swelling and multinucleation of hepatocytes, marked lobular changes, and a mixed portal inflammatory infiltrate with polymorphonuclear leukocytes around the proliferating ducts. Warthin‐Starry stain was negative. Differential diagnosis included syphilitic hepatitis with a possible autoimmune component. The patient was given a second dose of Penicillin G and in 6 weeks the patient had clinically improved with resolution of her jaundice, hepatitis and scleral icterus. Serum transaminases and autoimmune markers returned to normal. 122 AN UNUSUAL PRESENTATION OF WILSON DISEASE IN AN 8 YEAR‐OLD BOY. Lin, Henry C1; Lee, Dale Y1; Rook, Michelle T1; Ryan, Matthew Joseph1; Rand, Elizabeth 1, 1. Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, United States. Wilson disease is a rare autosomal recessive disorder of copper metabolism characterized by excessive deposition of copper. Hepatic involvement in a young child typically occurs as an insidious elevation of transaminases. Here we report an unusual case of Wilson disease in a previously healthy 8‐year‐old boy who presented with anemia, subacute liver failure and atypical liver biopsy. The patient presented with two weeks of headache, malaise, and fatigue. Physical exam was notable for hepatosplenomegaly with a firm liver, and mild facial and pedal edema. Initial labs were significant for albumin 2.8 g/dL, alanine aminotransferase 141 U/L, aspartate aminotransferase 324 U/L, total bilirubin 1.2 mg/dL, hemoglobin 9.6 g/dL, international normalized ratio 1.8. Abdominal ultrasound confirmed marked hepatosplenomegaly and ascites. Assays for Hepatitis A, B, and C, Cytomegalovirus, Epstein‐Barr virus, alpha‐1 antitrypsin deficiency, and celiac disease were negative. Cytoplasmic antineutrophil cytoplasmic antibody was positive (1:40 titer). Ceruloplasmin (21 mg/dL) was normal, but the 24‐hour urine copper was elevated at 211 ug/day (normal 3‐50 ug/day). Liver biopsy was non‐diagnostic, demonstrating a marked diffuse neutrophilic infiltrate, hepatocellular necrosis, mild sinusoidal fibrosis and no steatosis. The diagnosis was ultimately made on Trientene challenge after the 24‐hour urine copper level increased to 912 ug/day. Quantitative liver copper was elevated (798 mcg/g dry weight) and subsequent copper stain of liver tissue was highly positive. Ophthalmologic exam showed no Kayser‐Fleisher rings. Genetic testing revealed two missense mutations in ATP7B gene: c.2305A>G (p.M769V) and c.2762G>A (p.S921N). Despite treatment with Trientene and Vitamin E, the patient worsened and required a liver transplant one month after starting treatment. This case is unusual in the atypical liver biopsy findings as well as the subacute development of liver failure, demonstrating the variable hepatic presentations of Wilson disease. 123 ESOPHAGEAL CAPSULE ENDOSCOPY FOR EVALUATION OF PORTOSYSTEMIC SHUNT PATENCY. Isola, Kimberly 1; Lee, Samantha 1; Miloh, Tamir 1; Iyer, Kishore 1; Potack, Jonathan 2; Arnon, Ronen 1, 1. RMTI, Mount Sinai, New York, NY, United States. 2. Gastroenterology, Mount Sinai, New York, NY, United States. Background: Portosystemic shunt malfunction is usually assessed by imaging studies or by endoscopy. Aim: To describe the use of esophageal capsule endoscopy (CE) to assess portosystemic shunt function. A 19 year old Pakistani girl was referred for evaluation and treatment of chronic intractable ascites. Initially, she was treated for presumed tuberculosis peritonitis requiring frequent large volume paracentesis. On physical examination she appeared cachectic with a distended abdomen. Paracentesis revealed clear sterile ascitic fluid negative for tuberculosis. Liver synthetic functions were normal; liver biopsy showed hepatocellular cholestasis without fibrosis. CT angiography revealed complete splanchnic venous thrombosis and stenosis of the hepatic veins. Endoscopy revealed grade IV esophageal varices with red wale. Hypercoagulable workup revealed elevated levels of Lipoprotein A. After a failed meso‐ caval shunt, she underwent mesoatrial shunting with full anti‐coagulation using a ringed synthetic graft. Peritoneal biopsy revealed fibrosis without granulomas. The ascites initially improved but soon reaccumulated. Venography revealed shunt stenosis which was dilated by angioplasty. It was decided to survey her varices as a surrogate marker of shunt patency. CE revealed moderate‐large size esophageal varices. Follow up venography demonstrated shunt re‐stenosis which was successfully dilated. CE two months later again revealed moderate‐large size esophageal varices prompting another angioplasty. Two months later, repeat CE demonstrated small‐medium size varices. The patient tolerated the capsule ingestions without adverse effects. Discussion: Stenosis/obstruction is a known complication of portosystemic shunt. In this patient we have demonstrated CE is a safe and effective modality to monitor portosystemic shunt function reducing the need for sedation and radiation. Additional study of this application of CE is warranted. 124 GANGRENOUS NECROSIS OF THE GALLBLADDER IN A SIX WEEK OLD INFANT WITH ACALCULOUS CHOLECYSTITIS. Lukacik, Marek 1; Pipkin, Walter L2, 1. Pediatric Gastroenterology, Georgia Health Sciences University, Augusta, GA, United States. 2. Pediatric Surgery, Georgia Health Sciences University, Augusta, GA, United States. Introduction: Acalculous cholecystitis is a rare condition that has been previously described in all pediatric age groups. We present a case of a 6 week old with right upper quadrant mass who on subsequent cholecystectomy was shown to have gangrenous necrosis of the gallbladder. Case report: A previously healthy 6 week old infant presented to a local emergency room with a 1 to 2 day history of decreased p.o. intake and decreased urine output. On admission he was noted to be dehydrated with delayed capillary refill as well as metabolic acidosis and an elevated white blood cell count with a left shift. He received a full septic workup as well as fluid resuscitation and broad spectrum antibiotics. On palpation a 2‐3 cm movable soft mass was noted ine th right upper quadrant. Ultrasound of the area demonstrated gallbladder wall thickening and sludge. The patient clinically improved and was discharged home. One week later patient was readmitted with severe dehydration, necessitating multiple fluid boluses. Significant leukocytosis and left shift were seen. Again the mass was palpable in the right upper quadrant. A HIDA scan showed non‐visualization of the gallbladder. The patient underwent exploratory laparotomy for further investigation of the mass where an inflamed gallbladder with areas of frank necrosis was seen. The patient then underwent a cholecystectomy and recovered uneventfully. Several months later the patient was diagnosed with renal tubular acidosis. Discussion: Gallbladder diseases are rare in the pediatric age group and acalculous cholecystitis is quite rare in this age group as well, even though it has previously been described. Acalculous cholecystitis leading to gangrenous changes in the gallbladder is extremely rare. Early recognition and treatment is crucial to treat this condition to minimize the significant morbidity associated with it. 125 HEPATITIS E: AN IMPORTANT CAUSE OF LIVER FAILURE, EVEN IN THE US . Tanpowpong, Pornthep 1; Sabharwal, Sabina 1; Shah , Uzma 1, 1. Pediatrics , Hepatobiliary and Pancreatic Program, Massachusetts General Hospital for Children, Harvard Medical School, Boston, MA, United States. Whilst Hepatitis E virus is recognized as a cause of acute hepatitis and liver failure among travelers to endemic area, disease due to the virus is rare in the US. We present a teenager with acute liver failure who was found to have concomitant hepatitis E and Ebstein‐Barr virus infections. An 18‐year‐old male presented with a two‐day history of fever, chills, jaundice and altered mental status. Laboratory and radiologic studies are shown in Table. He was admitted to the intensive care unit with acute liver failure. Evidence of hemolysis led to testing for hepatitis E with strongly positive IgM titers demonstrated on two occasions. His transaminases, bilirubin, hemoglobin and liver synthetic function returned to normal over the next six months. We would like to recommend testing for Hepatitis E as a causative agent for acute liver failure, particularly with associated hemolysis. Although infection with hepatitis E is rare in the US and mainly sporadic, it needs to be considered in the evaluation of acute liver decompensation particularly where the severity may not be explained by a single etiology. References 1. Teshale EH, Hu DJ, Holmberg SD. The two faces of hepatitis E virus. Clin Infect Dis 2010;51(3):328‐34. 126 TRIANGULAR CORD SIGN IN ALAGILLE SYNDROME WITH A NOVEL MUTATION IN JAG1 . Mohanty, Prita 1; Gabel, Megan 1; Brown, Marilyn 1, 1. Pediatric Gastroenterology, , Rochester, NY, United States. Introduction Alagille Syndrome(AGS) is a genetically heterogeneous disorder caused by either a mutation in JAG1 (seen in 94% of clinically diagnosed probands) or Notch 2 (seen in 0.8%). Our patient was found to be heterozygous for a duplication of 3 nucleotides in exon 7, a mutation in this region was determined to be consistent with producing a clinical phenotype of Alagille syndrome. To our knowledge, this is the first reported case of AGS associated with the triangular cord sign. Case description A 3 month old boy was diagnosed to have AGS based on the presence of characteristic facies, peripheral pulmonic stenosis, posterior embryotoxon, cholestasis, growth retardation and developmental delay. Liver biopsy showed no bile duct paucity or proliferation. Genetic testing was consistent with the diagnosis of AGS. It revealed a c.962_964dupAGT mutation in JAG1 gene in exon 7, which results in replacement of cysteine at codon 322 with a premature stop codon. This is predicted to cause loss of normal protein function either through protein truncation or nonsense mediated mRNA decay. His evaluation at 11 months of age due to worsening cholestasis showed no visible gall bladder and a triangular cord sign(TCS) on ultrasound which was confirmed by magnetic resonance cholangiopancreatography. HIDA scan showed excretion of contrast. Discussion ‐TCS was first reported in 1996 by Choi et al to describe the obliterated fibrous ductal remnant in the porta hepatis in biliary atresia. ‐TCS is defined as a thickness of the echogenic anterior wall of the right portal vein of more than 4 mm. ‐TCS has 80% sensitivity, 98% specificity, 94% PPV and 94% accuracy in the diagnosis of Biliary Atresia. ‐Erroneous diagnosis can result in interventions deleterious to the patient’s outcome. ‐Any abnormal condition causing periportal infiltration, including periportal edema, fibrosis, or tumorous condition can appear as a TCS. Conclusion:This is the first report of TCS in a patient with genetically confirmed Alagille syndrome. 127 WILSON DISEASE PRESENTING WITH BILIARY DISEASE. Wadera, Sheetal 1; Magid, Margret 2; McOmber, Mark 3; Carpentieri, David 3; Miloh, Tamir 3, 1. Department of Pediatrics, Phoenix Children's Hospital, Phoenix, AZ, United States. 2. Department of Pathology, Mount Sinai Medical Center, New York, NY, United States. 3. Department of Pediatric Gastroenterology, Phoenix Children's Hospital, Phoenix, AZ, United States. A 15 yr old female, previously on the human Chorionic Gonadaotropin (hCG) diet, presented with a 3 month history of edema and fatigue, and acute onset of fever, cholestatis, coagulopathy, anemia, and acute kidney injury (Table 1). Liver and biliary system imaging were normal. Liver biopsy revealed pronounced bile ductular reaction and periductular neutrophilic infiltrate, bridging fibrosis, and widespread hepatocytic anisocytosis with large eosinophilic hepatocytes. She responded to IV antibiotics, Vit K and blood transfusions and was discharged on oral antibiotics, but experienced relapse of symptoms upon discontinuation of antibiotics. Diagnosis of Wilson disease was made based on the low serum ceruloplasmin, increased urinary and hepatic copper, and presence of Kayser Fleischer rings. Diagnosing Wilson disease requires a high level of suspicion, especially in patients with cholestasis, low ALP, Coombs negative hemolytic anemia and AKI. However, the clinical presentation of cholangitis and reversible coagulopathy is uncommon, and may result from concurrent acute cholangitis and/or the hCG diet. 128 CONGENITAL DISORDER OF GLYCOSYLATION TYPE IA AS A CAUSE OF FAILURE TO THRIVE. Ciciora, Steven 1; Perez, Maria E1; Russo, John 1, 1. Division of Gastroenterology, Nationwide Children's Hospital/Ohio State University, Columbus, OH, United States. BACKGROUND: Congenital disorders of glycosylation (CDGs) are a rare, diverse group of metabolic disorders with multi system involvement due to abnormal synthesis or processing of N‐glycoproteins. CDG‐Ia (a defect in the PMM2 gene, OMIM 212065) is the most common with over 600 known cases worldwide. We present the case of an infant with symptom onset and diagnosis among the youngest in the literature. CASE: A one‐month‐old, full‐term female presented to the GI clinic with emesis, loose stools, failure to regain birth weight and a failed newborn hearing screen. Weight and length were less than the 3rd percentile. Physical examination was significant for a thin, microcephalic child with lack of ocular fixation and no hepatomegaly. Initial evaluation was notable for a failed repeat hearing screen, transaminitis, hypoproteinemia, hypoalbuminemia, cerebellar atrophy on MRI, abnormal EEG without abnormal motor activity, and optic nerve hypoplasia with pigmentary retinopathy. Hospital course was complicated by anemia, persistent hypoglycemia necessitating continuous feeds and dextrose‐containing IV fluids, and coagulopathy. She required transfer to the PICU on two separate occasions, the second of which was due to presumed Parainfluenza‐induced liver failure. Ultimately, carbohydrate‐deficient transferrin (mass spectrometry) testing was found to be abnormal and genetic testing confirmed a mutation in the phosphomannomutase‐II (PMM2) gene consistent with a diagnosis of CDG‐Ia. DISCUSSION: Although CDGs are rare, the multisystem involvement in the setting of failure to thrive and this particular constellation of symptoms led to the diagnosis. In particular, persistent hepatic dysfunction, cerebellar atrophy, and ophthalmologic findings are characteristic of CDG‐1a. Long‐term multidisciplinary follow‐up care is needed for these patients including the involvement of therapy services. Associated phenomena for which this child will need monitoring include stroke‐like episodes, coagulopathy, cardiomyopathy, and orthopedic complications such as scoliosis. 129 PEDIATRIC AUTOIMMUNE HEPATITIS: A LINK TO HYPER IGM SYNDROME. Flass, Thomas 1; Mack, Cara 1; Hauk, Pia 2; Sundaram, Shikha S1, 1. Digestive Health Institute, Children's Hospital Colorado, Aurora, CO, United States. 2. Pediatric Allergy and Immunology, National Jewish Health, Denver, CO, United States. Hyper IgM syndromes (HIGM), immunoglobulin class switching disorders, cause recurrent respiratory and gastrointestinal infections. The association between secondary sclerosing cholangitis and HIGM is known, but occurrence inn childre of autoimmune hepatitis (AIH) is poorly defined. We describe 2 cases of AIH related to HIGM. Case 1, a 4 yr old Hispanic male, had a history of anemia, chronic diarrhea and recurrent otitis media. During workup for a septic joint he was diagnosed with HIGM and treated with monthly IVIG. Years later he presented with abdominal distention, firm hepatomegaly and elevated liver tests. ANA, anti‐smooth muscle (ASMA) and anti‐liver kidney microsomal (LKM) antibodies were negative. Liver histology revealed periportal lymphocytic infiltrate with interface hepatitis, consistent with AIH. MRCP was inconclusive for sclerosing cholangitis. Liver tests normalized on prednisone, and he remains on low dose prednisone maintenance therapy. Case 2 is a 6 year old Hispanic female with a history of immune thrombocytopenic purpura (ITP) at 6 months of age, treated successfully with prednisone and IVIG. ITP recurred 4 years later, requiring RiTUXimab to induce remission. One year later she presented with fever, abdominal pain, jaundice, and hepatosplenomegaly. Aminotransferases and bilirubin were elevated; ANA/LKM were negative but ASMA was positive. She was deficient in serum IgA and IgG, with marked elevation of IgM. Liver histology showed panacinar hepatitis with mixed inflammatory infiltrate, bile ductular proliferation, and mild portal fibrosis consistent with AIH. MRCP was unremarkable. Liver tests normalized on prednisone therapy, and she is maintained on low dose prednisone. In conclusion, patients with HIGM should be monitored for development of hepatic complications, including AIH, and in the appropriate clinical context, HIGM should be considered in pediatric patients with AIH. PANCREAS/CYSTIC FIBROSIS 136 A CASE OF PEDIATRIC ZOLLINGER‐ELLISON WITH SEVERE DISACCHARIDASE DEFICIENCY. Huang, Andrew S1; Huang, Clifton Steve2; Muinoz, William 1, 1. Pediatric Gastroenterology, Miami Children's Hospital, Miami, FL, United States. 2. Department of Pediatric Gastroenterology, Emory University , Atlanta, GA, United States. A. Huang MD, C. Huang MD*, W. Muinoz MD, Miami Children’s Hospital, Department of Pediatric Gastroenterology. *Emory University, Department of Pediatric Gastroenterology. Zollinger‐Ellison syndrome (ZES) is a rare disease characterized by gastric acid hypersecretion secondary to an autonomous elevated secretion of Gastrin usually due to a neuroendocrine tumor in the pancreas or in the small intestine (SI) called Gastrinoma. This case is remarkable as ZES is a rare disease, commonly developed in older population and it has never been reported concomitantly with Disaccharidase Deficiency (DD). This patient started with clinical symptoms at the age 15 years, diagnosed at 18 years of age with progressive worsening of DD, demonstrated by decreased enzyme activity in subsequent biopsies, concomitantly with progression of the intestinal mucosal inflammation. ZES itself remains a diagnostic challenge, especially in the pediatric population were less than 60 cases have been reported worldwide. Knowing that Gastrinomas represents about 2% of all causes of Peptic Ulcer Disease, it is important to consider this pathology in the differential diagnosis. This case illustrates how DD with subsequent malapsorption can be the result of SI mucosal inflammation and diarrhea which are part of the clinical spectrum of ZES. 137 RECURRENT PANCREATITIS AND MEDIASTINAL PSEUDOCYST PRESENTING WITH CHEST PAIN IN A 5 YEAR OLD CHILD. Gurram, Bhaskar 1; Lerner, Diana 1; Thomas, Ciecierega 1; Richard, Noel 1; Steven, Werlin 1, 1. Gastroenterology, Medical College of Wisconsin, Milwaukee, WI, United States. Background: The incidence of pancreatic pseudocyst formation in a child following pancreatitis is unknown. In adults the incidence of pseudocyst formation in acute pancreatitis is estimated to be nearly 20%. Pseudocyst formation in chronic pancreatitis may be even higher. Pancreatic pseudocysts are usually located adjacent to the pancreas in the retroperitoneum. Although extension into the mediastinum is well described in adults, only 10 cases of pancreatic mediastinal pseudocysts have been reported in children. We report a pediatric patient with a chronic pancreatitis presenting with chest pain due to a large mediastinal pancreatic pseudocyst. Case Report: A 5 year old girl who was diagnosed with recurrent pancreatitis with mediastinal pseudocyst at 3 ½ years of age. Her initial episode was presumed to be secondary to Mycoplasma infection. She had numerous episodes of abdominal pain and vomiting for 18 months. Episodes were managed with NPO, TPN and pain medications. Attempts to feed enterally by NG and NJ tube were unsuccessful because of worsening abdominal pain. She had resolution of her pseudocyst documented on an US abdomen after her initial few episodes. She was referred to our institution for evaluation of new onset chest pain and increasing size and complexity of her mediastinal pseudocyst. MRCP revealed a large dumbbell shaped peripancreatic pseudocyst extending from the tail of the pancreas through diaphragmatic hiatus in to the left paraesophageal region measuring 9.7 x 4.4 x 5.4 cm. Endoscopic transgastric drainage was unsuccessful. She ultimately needed a surgical cystogastrostomy. Work‐up revealed a heterozygous CFTR mutation. Conclusion: Mediastinal pancreatic pseudocyst is a rare complication of pancreatitis. It should be one of the differentials in children with pancreatitis presenting with chest pain. 138 ACUTE PANCREATITIS SECONDARY TO LISINOPRIL IN PEDIATRIC PATIENT. Ashai‐Khan, Farhat N1, 2; Frazin, Dana 1, 2; Canessa, Leonard 1, 2, 1. Gastroenterology, Children's Medical Center, Dayton, OH, United States. 2. Department of Pediatrics, Wright State Unviersity Boonshoft School of Medicine, Dayton, OH, United States. Introduction: Multiple medications are associated with drug induced pancreatitis and the list of medication implicated to cause drug induced pancreatitis continues to grow. Herein, we report a case of 10 year old female who developed acute pancreatitis after starting Lisinopril. Case Report: Ten year old Caucasian female presented to emergency department with complaint of significant epigastric abdominal pain, chest pain and vomiting for several hours. Past medical history was significant for obesity, BMI 30.4, recently diagnosed with hypertension and known asthmatic. Current medications included Lisinopril 5 mg which she started taking 2 months ago. Family history insignificant. Laboratory data was suggestive of pancreatitis, hemoconcentration and metabolic acidosis. CT scan abdomen showed acute, severe pancreatitis grade E, CTSI 10. Patient was admitted to ICU since findings were suggestive of severe pancreatitis. Lipid panels, including triglycerides, immunoglobulin subclasses, ANA, viral studies, blood cultures were obtained and were unrevealing. Pancreatitis gene testing was negative. Abdominal ultrasound was negative for biliary disease. Lisinopril was eliminated.e Sh progressed to multiorgan failure and responded to supportive care and early nasojejunal feeds. She was transferred to medical floor from ICU on day 8 of her illness and discharged home a week later. CT scan abdomen prior to discharge revealed pseudocyst. We conclude that her severe pancreatitis was drug induced since all other causes of pancreatitis were negative. Conclusion: With growing epidemic of obesity and co‐morbidities associated with that, increased number of pediatric patients are screened and treated for hypertension. This article emphasizes the risk of pancreatitis associated with Lisinopril not reported previously in pediatric literature. 139 PANCREATIC INSUFFICIENCY AS A SEQUELAE OF KAWASAKI DISEASE: A UNIQUE CASE PRESENTATION . Malandra, Michael 1; Tolosa, Drago 1; Laman, Douglas 1, 1. University of Tennessee College of Medicine ‐ Chattanooga Unit, Chattanooga, TN, United States. Pancreatic insufficiency as a sequelae of Kawasaki Disease has never been reported in the medical literature. This report describes a 3 year old patient with normal pancreatic function until she was hospitalized with fever, mouth pain and rash with a subsequent diagnosis of Kawasaki Disease. She was treated with Acetaminophen for pain and after cardiology evaluation she was placed on aspirin and was given IVIG. During the following 4‐6 weeks she developed foul smelling and floating stools that were orange in color with some visible oil. She was stooling 6‐8 times a day. Despite a ravenous appetite she failed to gain any weight. After a complete evaluation she was documented to have pancreatic insufficiency via a pancreatic stimulation study. After a thorough evaluation no other reason for the pancreatic insufficiency was found. She has been managed with daily enzyme replacement therapy and has done well with normal growth parameters. 140 PANCREATIC NEUROENDOCRINE TUMORS IN PEDIATRIC PATIENTS WITH TUBEROUS SCLEROSIS. Tanpowpong, Pornthep 1; Shah, Uzma 1; Larson , Anna 2; Thiele , Elizabeth A 2, 1. Hepatobiliary Program, Massachusetts General Hospital, Boston, MA, United States. 2. Herscot Center, Neurology , Massachusetts General Hospital for Children, Harvard Medical School, Boston , MA, United States. Pancreatic neuroendocrine tumors (PNETs) are an important form of pancreatic neoplasia. They represent 1‐2% of pancreatic cancer in all age groups, with an incidence of 1 in 100,000 individuals. These rare tumors occur sporadically or with multiple endocrine neoplasia type 1, von Hipple Lindau, and tuberous sclerosis complex (TSC). Functional PNETs have the potential to secrete insulin, gastrin, somatostatin and ACTH. TSC is a multisystem disorder, characterized by the development of hamartomas in numerous organs. It is caused by mutations in TSC1 on chromosome 9q34 and TSC2 on chromosome 16p13.3 encoding for hamartin and tuberin. Methods: A literature review was done of all TSC‐associated PNET cases published between 1959 and 2009. TSC genotypic and phenotypic data were compiled along with the clinical history for each case. A retrospective review of all TSC‐associated PNET cases at MGH was also done. Radiologic, surgical, and pathologic data for each case was reported along with history and genetic studies. Results: Twelve case reports of TSC‐associated PNET were identified on literature review with an additional six from the MGH records. Five patients were pediatric (0‐18 years) with 2 from MGH(Table). In both these patients, the PNETs were identified incidentally, were biopsied on endoscopic ultrasound and benign on pathology. One patient had a tumor resection while the other awaits surgery. Conclusion: Although pancreatic tumors in pediatrics are rare, PNETs may occur in TSC and are identified on imaging. We suggest that these pancreatic lesions are biopsied to identify pathology and plan treatment. 141 LEVETIRACETAM ASSOCIATED ACUTE PANCREATITIS IN AN ADOLESCENT WITH AUTISM. Oliveira, Stephanie B1; Almeida, Daniel M2; Jean, M. Raphaelle1; Monahan, Ellen 1; Monteiro, Iona M1, 1. Pediatrics, UMDNJ‐ New Jersey Medical School, Newark, NJ, United States. 2. Psychiatry, UMDNJ‐New Jersey Medical School, Newark, NJ, United States. Epilepsy and Autism spectrum disorders (ASD) are two entities with a co‐occurrence rate of 30%. Cases of pancreatitis have been reported with the use of anti‐epileptic drugs. Pancreatitis is a rare side effect of levetiracetam as reported by FDA and the community, yet no cases exist in the literature. We report a case of an autistic child with epilepsy who developed pancreatitis probably related to the use of levetiracetam. Case report: 17 year old male with ASD diagnosed at age 2 (managed with chlorpromazine) and seizures diagnosed at age 16 presented to the Emergency Room (ER) with irritability, vomiting and abdominal pain for one day. Three months prior to presentation the patient developed his first seizure episode with a subsequent episode 47 days later. Phenytoin was started and discontinued after 18 days secondary to intermittent abdominal pain and complaints of bad taste. 15 days prior to presentation the patient was started on levetiracetam 250mg twice daily. After 11 days he presented to the ER with his third seizure episode, resulting in an increase in levetiracetam dosage to 500mg twice daily. Four days after adjustment of medications he returned to the ER with the above symptoms. Detailed characterization of his complaints was limited, secondary to his non‐verbal state. Serum amylase was 489 U/L and lipase was 1233 U/L. CT abdomen confirmed pancreatitis. Levetiracetam and chlorpromazine were discontinued, followed by normalization of amylase and lipase levels, as well as resolution of symptoms. Discussion: Clinical and epidemiological data led us to consider drug‐induced pancreatitis as our first hypothesis for the etiology of this case. The Naranjo algorithm was applied and the patient had a final score of 6, supporting the probability of an adverse drug reaction as the cause. Conclusion: Physicians should be aware of the possibility of pancreatitis secondary to antiepileptic drugs, including levetiracetam, specially in patients unable to verbalize their complaints, as in this autistic adolescent. 142 COLONIC NECROSIS ASSOCIATED WITH PANCREATIC PSEUDOCYST FORMATION IN A 4 YEAR OLD. Patton, Tiffany J1; Sentongo, Timothy 1; Waggoner, Darrel 3; Mak, Grace 2; Kahn, Stacy 1, 1. Pediatric Gastroenterology, University of Chicago, Chicago, IL, United States. 2. Pediatric Surgery, University of Chicago, Chicago, IL, United States. 3. Genetics, University of Chicago, Chicago, IL, United States. Colonic complications of acute pancreatitis and pseudocysts are uncommon in adults and children. Here we report the case of a 4‐year old boy who presented with colonic necrosis associated with a large pancreatic pseudocyst. On transfer to our institution, the patient presented with 5 days of nausea, vomiting, mild abdominal pain, a confirmed left retroperitoneal mass, and fasting trigylcerides of 7449 mg/dL. Vital signs were normal with a Tmax 39.4°C. Additionally, he had a 1‐year history of abdominal distension and intermittent pain. On exam the abdomen was soft, nontender with a large smooth palpable mass in the left upper quadrant. Admission laboratories revealed lipase 99 U/L, amylase 79 U/L, triglycerides 6060 mg/dL, and lipemic serum with hematocrit 16.1%. Due to concern for malignancy, the patient had a repeat chest/abdominal CT scan that showed a 10cm x 8.4cm left retroperitoneal mass. On HD #4, he underwent an exploratory laparotomy where a large necrotic mass with colonic invasion at the splenic flexure was identified with a short segment of necrotic bowel being resected and a transverse colostomy performed. On HD #6, a bone marrow biopsy revealed extensive fat necrosis with no tumor cells identified. Work‐up for infectious etiologies was negative. We determined the patient had hypertriglyceridemia that resulted in chronic pancreatitis with pseudocyst formation, which ultimately led to colonic necrosis due to autodigestion. The patient was managed with the placement of an external pseudocyst drain, total parenteral nutrition (TPN) and a very low‐fat enteral diet. He was discharged home on HD# 80 with subsequent pseudocyst resolution and drain removal with colonic reanastomosis 1 month later. Currently, the patient remains asymptomatic, off TPN, and on an extremely low fat diet. We believe this case emphasizes the importance of early recognition of hypertriglyceridemic pancreatitis and the prevention of severe colonic complications associated with pancreatitis. CLINICAL VIGNETTE POSTER SESSION II Friday October 22, 2011 12:15pm – 2:15pm ESOPHAGUS/STOMACH 178 EOSINOPHILIC ESOPHAGITIS PRESENTATION YEARS AFTER ESOPHAGEAL ATRESIA . Baez Socorro, Virginia M1; Kassabian, Sirvart 1; Splawski, Judy 1; Garcia, Reinaldo 1, 1. Peds Gastroenterology, University Hospitals, Cleveland, OH, United States. Esophageal atresia (EA) is defined as a discontinuity of the lumen of the esophagus repaired soon after birth (1). Dysphagia is a common symptom in these patients, usually related to stricture, dysmotility or peptic esophagitis (2). We present 4 cases of patients with EA who complained of dysphagia and the diagnosis of Eosinophilic esophagitis (EoE) was made, ages ranging from 9 to 16 years. Although our patients were on acid suppression years after their EA repair, they presented with acute worsening of dysphagia. Esophogastroduodenoscopy and/or barium swallow did not show stricture and biopsies revealed elevated eosinophil counts consistent with EoE. Two of 4 patients improved symptomatically with the topical steroids. It is important to note that all our patients have asthma and 3 out of 4 have tested positive for food allergies. One of our patients developed recurrent anastomotic strictures that improvede with th treatment of the EoE. A previous case report linked the recurrence of esophageal strictures in patients with EA repair with EoE (3). Once the EoE was treated the strictures resolved. On the other hand, based on our observation, EoE could be present in patients without recurrent anastomotic strictures. There appears to be a spectrum in the disease process. We are suggesting that EoE is a frequent concomitant problem in patients with history of congenital esophageal deformities, and for this reason any of these patient with refractory reflux symptoms or dysphagia (with or without anastomotic stricture) may benefit from an endoscopic evaluation with biopsies to rule out EoE. (1)Genevieve D et al. Genetic Factors in isolated and Syndromic Esophageal Atresia. J of Ped Gastro and Nutri. 2011; 52:S6‐S8. (2)De Lagausie P. GER is Oesophageal Atresia: Surgical options. J of Ped Gastro and Nutri. 2001; 52: S27‐S28. (3)Batres A, Liacouras C, et al. Case report: eosinophilic Esophagitis Associated with Anastomotic Strictures after Esophageal Atresia Repair. J of Ped Gastro and Nutri. 2002; 35: 224‐226. 179 COEXISTING CELIAC DISEASE AND EOSINOPHILIC ESOPHAGITIS: PATIENT OUTCOMES WITH A GLUTEN‐FREE DIET. Contreras, Emily 1; Newbury, Robert 1; Aceves, Seema 1; Dohil, Ranjan 1; Newton, Kimberly 1, 1. UCSD, San Diego, CA, United States. Background: Eosinophilic esophagitis (EoE) and celiac disease (CD) are hypersensitivity gastrointestinal disorders that respond to food elimination. However, CD is an autoimmune, Th1‐mediated disease, EoE is Th2‐mediated, and each have distinct clinical and histologic patterns. Previous reports of patients with coexisting CD/EoE show that both conditions will respond variably (0‐100%) to a gluten‐free diet (GFD). Method: Our Pediatric Gastroenterology database was queried to find patients with CD alone and with coexisting EoE. Patient characteristics and clinical presentation were analyzed. CD‐response was defined as Marsh 0 classification post‐treatment. EoE‐response was classified as reduction of peak eosinophil count to ≤15 eos/hpf. Baseline and post‐treatment EoE and CD endoscopy scores, EoE symptom scores, EoE histology scores, peak esophageal eosinophil counts and peripheral eosinophilia were recorded. Results: Of 116 patients with CD, 6 (5%) had coexisting EoE. These patients had family history of autoimmune disease, 5 had atopic/allergic diatheses and all presented with symptoms suggesting celiac disease (eg. diarrhea, poor weight gain, constipation, abdominal pain, osteoporosis). 5/6 patients had follow‐up endoscopies. GFD induced CD‐response in all 5 and EoE‐response in 1. GFD had no significant effect on EoE endoscopy scores, EoE symptom score, EoE histology score, peak esophageal eosinophil counts, or peripheral eosinophilia. All 3 patients who received GFD and specific EoE therapy had an EoE‐ response with decrease in EoE histology scores (6.3 to 0.67, p=0.036) and mean peak esophageal eosinophil counts (67 to 6.2eos/hpf, p=0.036). Conclusion: Our data suggests that EoE is more prevalent in CD patients than in the general population. In all patients, EoE was diagnosed incidentally during evaluationr fo CD and none presented with GERD‐like symptoms. The response rate of EoE in CD/EoE patients to GFD is low (20%). Whether EoE is an extension of CD in a subset of patients, or an etiologically distinct disease, remains to be evaluated. 180 ESOPHAGITIS DISSECANS SUPERFICIALIS‐ AN UNEXPECTED FINDING AT UPPER ENDOSCOPY IN A PEDIATRIC PATIENT. Vortia, Eugene 1; Mahajan, Lori 1; Plesec, Thomas 1; Steffen, Rita 1, 1. Cleveland Clinic Foundation, Cleveland, OH, United States. A 15 yr old female was evaluated for chronic abdominal pain and diarrhea. History was negative for dysphagia, nausea and emesis. Prior medications included cetirizine and omeprazole. She had no personal or family history of GI mucosal disease or painful skin lesions.m Exa was normal except for mild diffuse abdominal tenderness. Labs, including CBC, CMP, ESR, stool studies, IgA transglutaminase and endomysial antibodies were normal. Gliadin IgA was elevated at 40 units. EGD and colonoscopy were ordered. Cleanout for colonoscopy was bisacodyl 5 mg followed by 527 g of PEG 3350 mixed in 64 oz of a sports drink. At endoscopy, esophageal mucosa was noted to be sloughing in approximately 1 cm wide ribbon‐like vertical strands that extended the length of the esophagus, filling the lumen with a 'gift wrap ribbon' appearance. No gastric or duodenal mucosa abnormalities were identified. Esophageal biopsies showed variably necrotic fragments of superficial squamous mucosa with patchy active inflammation and parakeratosis with negative fungal stains, consistent with Esophagitis Dissecans Superficialis (EDS). Gastric and duodenal biopsies were normal. Colonoscopy with biopsy was also normal. Compliance with omeprazole was emphasized. On repeat endoscopy performed 1 month later, esophageal mucosa appeared entirely normal with no stricture and normal biopsies. EDS is a rare endoscopic finding characterized by sloughing of large fragments of the esophageal squamous mucosa. It has been associated with certain medications including biphosphonates and dermatologic conditions such as pemphigus vulgaris. Most cases, however, remain unexplained. This condition has never been previously reported in a pediatric patient. Although dramatic in appearance, this is typically a benign condition and resolves spontaneously as in our patient. A history of recurrent painful skin ulcers should prompt dermatologic referral. The medication list should also be carefully reviewed to identify potential causes. In our patient, the bowel preparation the day prior to initial procedure may have been etiologic 181 EOSINOPHILIC ESOPHAGITIS AFTER TRACHEOESOPHAGEAL FISTULA REPAIR: A REVIEW OF THREE CASES. Badalyan, Vahe 1; Leibowitz, Ian 1, 1. Pediatrics, Inova Fairfax Hospital for Children, Falls Church, VA, United States. We present three cases of eosinophilic esophagitis after tracheoesophageal fistula repair. Case 1: now 14 year old female with history of esophageal atresia, which was repaired in early infancy, had long history of reflux, and presented to GI clinic with symptoms of with dysphagia. Patient had normal pH probe and normal impedance study. Case 2: Now 5 year old male with history of tracheoesophageal fistula that was repaired shortly after birth, cleft lip and cleft palate that, history of asthma, as well as gastrointestinal reflux disease, presented with symptoms of dysphagia. Case 3: Now 3 year old male with history of esophageal atresia with tracheoesophageal fistula, presenting with symptoms of dysphagia. Upper endocsopy and biopsies in all three cases revealed eosinophilic esophagitis. Discussion: patients with history of TEF have high prevalence of GERD and other comorbidities, such as asthma. However, very few cases of EoE have been described in this group. Former TEF patients who have EoE may have higher rate of stricture formation and dysmotility compared to those who only have GERD. 182 TARGETED MITOMYCIN‐C APPLICATION FOR ESOPHAGEAL STRICTURE REFRACTORY TO DILATION. Warolin, Joshua 1; Moulton, Dedrick 1, 1. Pediatric Gastroenterology, Vanderbilt University, Nashville, TN, United States. Background: Esophageal strictures in children often require frequentdilation for symptomatic improvement. However, an inevitable sequela of dilation is scar formation through fibroblast proliferation and collagen deposition. Mitomycin‐C (MMC) is an antibiotic with an antiproliferative effect on fibroblasts. The first reportedC use of MM in decreasing scar formation in the esophageal was published in 2002. Despite subsequent case reports/series, a review of the literature reveals no consensus regarding ideal dosing and application technique. Case: Patient is a 2 year old female with dysphagia and history of tracheoesophageal fistula status‐post surgical repair as an infant. An esophagram demonstrated ~50% esophageal narrowing and we were unable to pass a pediatric endoscope at initial endoscopy. Patient required balloon dilation 4 times prior to initial MMC application by the pediatric surgeons. Temporary symptomatic relief seen, but required repeat dilation 3 weeks following MMC. After 5 subsequent dilations due to persistence of symptoms, a targeted application of MMC was accomplished via flexible endoscopy. Pledgets soaked in 0.4mg/mL MMC were front‐loaded into a cap used for band ligation and attached to an endoscope. The pledgets were then applied with biopsy forceps for 3 minutes to each of the 2 esophageal fissures formed during dilation. Following the second application, the patient remained asymptomatic for 2 months, with endoscopic confirmation. Discussion: Since the initial report of MMC use in pediatric esophageal strictures in 2002, there have been 9 papers published reporting on 27 total patients. A review of thee literatur shows: (1) patients receive an average of 17 dilations prior to MMC application, (2) require an average of 2 applications of MMC for symptomatic and endoscopic resolution, and (3) received MMC solutions with concentration ranging from 0.1‐1.0mg/mL and application duration ranging from 1‐4 minutes. Our patient achieved excellent symptomatic control following a targeted application of 0.4mg/mL soaked MMC pledgets directly to the mucosa tears formed during dilation. 183 EOSINOPHILIC ESOPHAGITIS PRESENTING AS AN ESOPHAGEAL MASS LESION. Maksimak, Martin 1; Maksimak, Emily 1, 1. Geisinger Clinic ‐ Janet Weis Children's Hospital, Danville, PA, United States. Martin Maksimak, MD; Emily Maksimak, DO Janet Weis Children's Hospital, Danville, PA. AO is a 16 year old girl, who presented to her pediatrician with a history of increasing dysphagia mainly for solids over the past year along with symptoms of occasional regurgitation and heartburn. No “red flag” symptoms such as weight loss or hematemesis were reported. UGI study from the referring hospital showed a distal esophageal mass lesion. Laboratory work‐up revealed a normal CBC and differential with no eosinophilia. A comprehensive chemistry panel and sedimentation rate were normal. Response to low dose PPI therapy was unremarkable. Upper endoscopy revealed erosive esophageal changes with circular ringing and vertical grooving along with multiple polyps in the distal esophagus, The polyps varied from small sessile polyps to multilobulated polyps up to 0.7 cm in size that were removed by either cold biopsy forceps or a hot snare depending of the size and shape of the particular polypoid lesion. Pathology revealed severe tissue eosinophilia up to 100 eos/hpf in the polyps and esophageal mucosa up to the mid esophagus. Treatment with twice daily PPI therapy was effective in relieving symptoms. A repeat endoscopy 3 months later showed improved appearance of the esophageal mucosa with no polyps present. The biopsies from mid and distal esophagus showed eosinophils from 0‐9/hpf and 10‐14/hpf respectively, while the proximal esophageal biopsies were normal. Endoscopic pictures and pathology specimens will be displayed. 184 CMV ASSOCIATED MENETRIER’S DISEASE OF CHILDHOOD. Mohanty, Prita 1, 2; Karjoo, Manoochehr 2; Beg, Mirza 2, 1. University of Rochester, Rochester, NY, United States. 2. Upstate Medical University, Syracuse, NY, United States. Introduction We describe a case of an adolescent with edema of the lower limbs, hypoalbuminemia and protein losing enteropathy associated with cytomegalovirus infection diagnosed to have Menetrier’s disease. Case description 18 year old girl presented with a 2 week history of bilateral lower extremity swelling and abdominal distension. On examination, the abdomen was distended,tympanic with shifting dullness.Pretibial pitting edema was noted. Significant lab values included hypoalbuminemia (albumin 2.5g/dl) and fecal alpha 1‐antitrypsin 246 mg/dl (normal < 2mg/dl). Upper GI series revealed grossly thickened gastric folds which were confirmed on esophagogastroduodenoscopy. Histology of the stomach showed foveolar hyperplasia and glandular dilatation. CMV was isolated from gastric mucosa. The patient was advised high protein diet. Follow up examination revealed resolving pedal edema and ascites. The albumin normalized with no further symptomatic recurrences. Discussion ‐ Menetrier’s disease is an uncommon disease in childhood, characterized by gastric hypertrophy involving predominantly the fundus and body and hypoalbuminemia secondary to protein loss through the gastric mucosa. ‐ Levels of TGF‐alpha are markedly increased in patients with Menetrier's disease. TGF‐alpha may exert its effect by binding to the epidermal growth factor receptor resulting in hyperplasia of gastric mucous cells. ‐ In contrast to adults, children usually have self limited disease, shorter duration and require only supportive care. ‐ Anti‐viral therapy is rarely required. ‐ Anticholinergics, prednisone, proton pump inhibitors and prostaglandins have been used in Menetrier's disease but none has proven to be beneficial. ‐ Studies have shown clinical and biochemical improvement after treatment with a monoclonal antibody directed against the epidermal growth factor receptor. ‐ No prospective randomized controlled trials have examined the effectiveness of ganciclovir in childhood Menetrier’s disease. Conclusion ‐ Childhood Menetrier’s disease remits spontaneously and has very good prognosis. ‐ Treatment is largely supportive. 185 RARE SUPRA‐ESOPHAGEAL COMPLICATION OF INLET PATCH OF THE ESOPHAGUS‐A CASE REPORT. Amarnath, Rathna P.1, 2; Geurkink, Deanne 1, 2; Manuat, Charissa 1, 2, 1. Palmetto health Children's Hospital, Columbia, SC, United States. 2. Department of Pediatrics, University of South Carolina School of Medicine, Columbia, SC, United States. 10 yr old Caucasian male presented with 6 month history of chocking and throat clearing due to foreign body sensation in the posterior pharynx. 2wks prior to Consultation He had an acute episode of 'Throat closing off' and stopped eating any solid foods since, He was seen by PCP and treated with Ranitidine 150 mg bid with no improvement. He also had shortness of breath associated with dysphagia to solids. He denied caustic or foreign body ingestion, fever, nor was he taking any medications. Physical Exam: Wt 34.5kg, BMI;16, No Tonsilar enlargement, Positive cervical lymphadenopathy, otherwise the exam was unremarkable. A Barium Swallow study was negative for stricture or spasm of the esophagus. Diglutition was normal. An upper endoscopy revealed a large irregular pharyngeal ulcer, esophagus showed a twin Inlet Patches (Heterotopic Gastic Mucosa) measuring 6×3cm just below the UES on opposing mucosal surface, remainder of the endoscopy was unremarkable Biopsies of the Patch and distal esophagus, duodenum and the Ulcer was obtained. Biopsy of the ulcer did not show any bacteria or viral changes. inlet patch cwas Gastri mucosa without dysplasia and others were normal. Patient was started on bland soft food and Lansaprazole 30mg bid. Symptoms improved over 4wks and resolved by 8wks. Last follow up 1yr later the patient is totally asymptomatic, gaining wt with BMI of 17. Esophageal inlet patches are not unusual on routine upper endoscopy. Previous complication associated with Inlet Patches were Ulceration of the Inlet Patch, Reactive air way disease and esophageal stricture. Our patient had a large Posterior pharyngeal Ulceration; a first report of this complication. In addition he also had twin patches which is also a rare finding. Conclusion: Close attention should be paid to the posterior pharyngeal area during endoscopy in patient presenting with dyspahgia, especially if an Inlet Patch is found. 186 EOSINOPHILIC GASTRITIS PRESENTING WITH GASTRIC OUTLET OBSTRUCTION IN AN INFANT. Sankararaman, Senthilkumar 1; Pant, Chaitanya 2; Sferra, Thomas J2; Altaf, Muhammad A2, 1. Pediatrics, Louisiana State University Health Sciences Center, Shreveport, LA, United States. 2. Pediatric Gastroenterology, University of Oklahoma Health Sciences Center, Oklahoma City, OK, United States. Eosinophil‐associated gastrointestinal disorders (EGIDs) are uncommon disorders of unclear etiology characterized by eosinophilic infiltration and inflammation of the gastrointestinal tract, mostly seen in the absence of recognized causes of eosinophilia. A one‐year‐old Hispanic female with an otherwise unremarkable medical history was admitted in our institution with a 3 month history of progressively worsening emesis after feeds. Her symptoms were worse with solids as compared to liquid diet. Physical examination and screening laboratory investigations did not reveal any abnormalities. Ultrasound did not support a diagnosis of hypertrophic pyloric stenosis. An upper gastrointestinal study was significant for delayed passage of contrast from the stomach. An esophagogastroduodenoscopy (EGD) was subsequently performed which revealed gastric outlet obstruction (GOO) with marked edema, erythema and narrowing of the pyloric outlet channel. Biopsy specimens from the gastric antrum were significant for a focal increase in eosinophils in the lamina propria with a maximum eosinophilic count of 72/hpf. A further work‐up revealed no evidence for extra‐intestinal disease or parasitic infection. A diagnosis of eosinophilic gastritis was made. She was restricted to elemental formula for few weeks without any significant response so was subsequently started on systemic corticosteroids. The patient demonstrated a rapid response to treatment with complete resolution of the symptoms. Eosinophilic gastritis is less‐ frequently encountered in clinical practice. However, as illustrated by this case, it has the potential to cause severe GOO, and therefore should be assessed for in the context of this condition. 187 CONGENITAL ACHALASIA OF THE LES PRECEDING EOSINOPHILIC ESOPHAGITIS IN INFANTS. Ciecierega, Thomas 1; Almadhoun, Osama 1; Shiel, Amy T1; Tipnis, Neelesh Ajit1, 1. Pediatric Gastroenterology, Medical College of Wisconsin, Milwaukee, WI, United States. Background: Congenital achalasia is a rare condition presenting in infancy with feeding intolerance. Eosinophilic esophagitis (EE) is a common cause of vomiting in toddlers and older children. While manometric abnormalities have been described in children with EE, association with achalasia of the lower esophageal sphincter (LES) has not been described in children. Moreover, achalasia of the LES preceding the presentation of EE has not been described in children or adults. Cases: 3 infants at 5‐9 months of age (3 full‐term, 1 male) presented with recurrent non‐bloody, non‐bilious emesis shortly after feeding in spite of adequate therapies for GERD. Upper GI series showed an aperistaltic dilated, bird’s beaked esophagus. HRM showed aperistalsis, pan‐esophageal pressurization events, high mean LES resting pressure (58 to 73 mmHg) and integration relaxation pressures. Esophagitis and other congenital anomalies were excluded. Laparoscopic Heller’s myotomy with partial fundoplication was performed and all quickly advanced to solids feeds post‐myotomy. At 13 months of age, all had a return of vomiting. Upper GI series showed esophageal aperistalsis. EGD revealed pan‐esophagitis with linear furrowing, distal esophageal ulceration and white plaque exudates. Biopsies from the proximal and distal esophagus were typical of EE (>20 eos/hpf). Vomiting ceased with viscous budesonide treatment. Repeat EGD showed remission of the EE, but HRM in 2 revealed continued aperistalsis with normal LES pressure. Discussion: We report the first 3 cases of congenital achalasia associated with EE in children. Manometric abnormalities were reported in 41% of children with EE, but none had primary esophageal motor disorders. In achalasia and EE, thickened LES musculature has been reported using ultrasonography. Eosinophilic infiltration of LES musculature hasn bee found in 52% of adults with achalasia. We propose that eosinophilic infiltration of the LES preceded mucosal infiltration in these infants, leading to the clinical findings of achalasia and delayed presentation of the EE. INTESTINE/COLON/IBD 212 VIDEO CAPSULE ENDOSCOPY IN THE DIAGNOSIS OF OBSCURE GASTROINTESTINAL BLEEDING IN A CHILD. Anani, Anthony 1; Hupertz, Vera 1; DiFiore, John 1; Mahajan, Lori 1, 1. Pediatrics, Cleveland Clinic Foundation, Cleveland, OH, United States. Obscure gastrointestinal bleeding (OGIB) is defined as recurrent iron deficiency anemia, positive fecal occult blood test or visible bleeding with no bleeding source found at original endoscopy. It can be a challenge to pediatric gastroenterologists despite advances in endoscopy and imaging. A previously healthy 7 year old female presented with a one day history of painless hematochezia. She had no constitutional symptoms, history of bleeding diathesis, recent travel or exposure to ill contacts. Her growth parameters were within normal limits and she was mildly tachycardic. On exam she was pale, afebrile, and anicteric. Abdominal exam was normal. Rectal exam revealed frank blood . Labs: Hb‐ 6.1g/dL; ESR, CRP, PT/APTT, Albumin – normal. She was transfused with 10cc/kg packed red blood cells; post transfusion Hb was 8.1g/dL. Meckel's scan, EGD and colonoscopy were normal. She was discharged home with no further hematochezia. Follow up in 2 weeks she was asymptomatic, stool occult negative, Hb 10.2g/dL. 4 weeks after initial presentation, she was readmitted for painless hematochezia; Hb was 7.6g/dL. Repeat Meckel’s scan negative after premedication with H2 blocker, repeat EGD/Colonoscopy were normal. Video capsule endoscope was deployed into the distal duodenum and revealed an ileal diverticulum with a large adjacent ulcer. Laparoscopic abdominal exploration revealed a 7 x 8 cm area of markedly dilated bowel containing the Meckel’s diverticulum. Diverticulum had a tan/yellow piece of tissue approximately 1.5 cm in size at the antimesenteric border grossly consistent with pancreatic tissue. Diverticulum and dilated bowel segment were resected and patient has been asymptomatic for 6 months. Extensive literature review shows only 5 prior reported cases of Meckels diverticulum diagnosed via video capsule endoscopy. Our case highlights the use of video capsule endospcopy in the diagnoses of OGIB. It should be considered early in the evaluation to avoid further exposure to blood products, radiation and more invasive tests such as angiography. 213 CELIAC DISEASE AND LARGE VESSEL VASCULITIS. A RARE COINCIDENCE OR POSSIBLE ASSOCIATION? Bornstein, Jeffery 1; Tatum, Elizabeth 1; Safder, Shaista 1, 1. Pediatric Gastroenterology, Arnold Palmer Childrens Hospital, Orlando, FL, United States. A 10 year old Caucasian female presented with a 4 month history of daily, periumbilical, abdominal pain that would frequently awake her from sleep. She had been experiencing weight loss and was noted to fall below the 3rd percentile 1 year prior to presentation. She had an elevated TTG IgA, elevated inflammatory markers and normal CBC with differential and iron studies. She underwent upper endoscopy which revealed intestinal villous blunting with increased intraepithelial lymphocytes consistent with celiac disease. She began a gluten free diet. At six week follow up, she was noted to have a worsening of her abdominal pain, additional 5 lb weight loss, nausea and anorexia. She presented ill appearing, tachycardic and severely hypertensive. She was admitted and during work‐up for hypertension, abdominal and pelvic CT with angiongraphy showed severe bilateral renal artery stenosis, proximal superior mesenteric artery occlusion with distal reconstitution and proximal celiac artery stenosis, suggestive of a large vessel vasculitis. Plan is for stent placement with tissue biopsy to confirm Takayasu's arteritis. Discussion: Celiac disease is an immune mediated enteropathy caused by sensitivity to dietary gluten. Patients with certain immunologic conditions, (ie type 1 diabetes mellitus and autoimmune thyoiditis) have an increased risk for celiac disease. Takayasu's arteritis is a rare large vessel vasculitis, characterized by granulomatous inflammation of vessels, leading to stenosis, thrombosis and aneursym formation. While the etiology of Takayasu's arteritis remains unknown, studies have shown an immunogenetic association. A Pub Med search has reported 4 other similar case reports of celiac disease and Takayasu's arteritis.To our knowledge, this is the first report of a child with concurrent Celiac disease and large vessel vasculitis. Unclear if chronic inflammation due to celiac disease is a trigger for the vasculitis. 214 NARROW BAND IMAGING WITH HIGH DEFINITION TELEVISION FOR THE ASSESSMENT OF GASTROINTESTINAL DISORDERS IN CHILDREN. Arias, Patricio 1; Gomara, Roberto 1; Muinos, William 1; Reeves‐Garcia, Jesse 1; Hernandez, Erick 1, 1. Pediatric Gastroenterology, Miami Children's hospital, Miami, FL, United States. Narrow Band Imaging (NBI) is a fairly new high resolution endoscopic technology. Its use is based in the concept that the depth of light penetration depends upon its wavelength. As opposed to white‐light endoscopy NBI utilizes 2 distinct wavelengths of light limiting the penetrance of light to the mucosal surface, highlighting the superficial capillary networks and the subepitelial vessels. NBI enhances the observation of capillaries in high contrast to the surrounding mucosa and emphasizes mucosal patterns in the lower gastrointestinal tract. NBI gives endoscopists the ability to identify lesions that may not be appreciated with conventional white‐light endoscopy. Targeted NBI may provide vascular and morphologic details that have not been noticed beforehand with standard endoscopy. There are very few studies done in pediatrics regarding the use of NBI for the detection and diagnosis of different gastrointestinal pathologies, otherwise difficult to distinguish with conventional white‐light endoscopy. We present the experience at our institution with the use of Narrow Band Imaging in the diagnosis and assessment of different gastrointestinal pathologies in the pediatric population. We have given particular emphasis in the importance that NBI has in the evaluation of children with portal hypertension and suspected esophageal varices. We have used NBI with high‐definition television (HDTV) for the assessment of common GI diseases as well as for unusual conditions in children 215 DUODENAL HEMATOMA COMPLICATING UPPER ENDOSCOPY WITH BIOPSY IN TWO PEDIATRIC PATIENTS WITH NOONAN’S SYNDROME: WHAT PEDIATRIC GASTROENTEROLOGISTS NEED TO KNOW. Vortia, Eugene 1; Mahajan, Lori 1; Kaplan, Barbara 1, 1. Cleveland Clinic Foundation, Cleveland, OH, United States. Noonan’s Syndrome (NS) is a common genetic disorder associated with distinctive facial features, short stature and congenital heart disease. Pediatric gastroenterologists are often consulted to manage reflux and poor feeding. Bleeding with surgical/endoscopic procedures can be significant and not correlate with labs (PT/APTT). We report two such cases. Patient 1 was a 19 mo old with NS referred for poor feeding and failure to thrive. Labs were normal except for mildly elevated gliadin IgG. EGD was uneventful with 4 duodenal biopsies taken. Persistent emesis developed several hours later. UGI and ultrasound were consistent with a large duodenal hematoma extending from the ligament of Treitz to the proximal jejunum. PT, PTT, INR were all normal. Hb decreased over 4 days from 11.4 to 7.2gm/dL. Patient was medically treated with NG suctioning and TPN with spontaneous resolution of the hematoma at 14 days post‐EGD. Patient 2, a 2 yr old with NS underwent EGD for symptoms of GER and weight loss. He had been circumcised without prolonged bleeding. Pre‐endoscopy labs were normal. EGD was normal with two biopsies taken from the distal duodenum. Persistent emesis developed 3 hours post‐EGD. UGI and ultrasound confirmed a large duodenal hematoma. PT, PTT, INR were normal. Hb decreased over 7 days from 11.4 to 6.5 gm/dL. This patient was non‐surgically managed with NG suctioning, TPN and transfusion. He was discharged on post‐EGD day 19. Hematologic evaluation later identified a platelet aggregation disorder. There have only been two prior cases of duodenal hematoma reported after EGD with biopsy in NS patients. Up to 65% of NS patients have a bleeding disorder. Our cases emphasize the fact that despite normal coagulation studies, no history of bleeding and/or prior tolerance of surgical procedures, patients with NS are potentially at increased risk of bleeding with endoscopy, specifically duodenal hematoma formation. Hematologic consultation is recommended in NS patients prior to elective endoscopic procedures. 216 PRIMARY SMALL BOWEL BEZOAR AND BLUNT FOREIGN BODIES CAUSING SMALL INTESTINAL OBSTRUCTION AND CLOSED PERFORATIONS IN AN AUTISTIC ADOLESCENT. Mohanty, Prita 1; Pegoli, Walter 1; Gabel, Megan 1; Brown, Marilyn 1, 1. Pediatric Gastroenterology, University of Rochester, Rochester, NY, United States. Introduction Bezoars and foreign bodies are frequently encountered in children with psychiatric disorders.The incidence of a primary small intestinal bezoar is rare.Small bowel obstruction and perforation are uncommon complications of a blunt bezoar.We describe the case of an autistic adolescent with intestinal obstruction due to multiple foreign bodies and a bizarre bezoar extending from jejunum to the ileum. Case description A 14 year old autistic boy presented with a 3 week history of decreasing oral intake and weight loss.The abdomen was soft and non tender.Abdominal CT showed signs of partial intestinal obstruction with foreign bodies,possibly bezoar.A radio opaque density was also noted in the pyloric region and transverse colon.A history of chewing on non food items was elicited.Attempt at nasogastric lavage using polyethylene glycol failed.The patient underwent laparotomy and a large mass of impacted foreign material was found extending from the proximal jejunum to the ileum with 10 scattered perforations.Segmental enterectomies and enteroenterostomy was performed.The resected pathological specimen contained a large synthetic bezoar composed of an aggregate of paper, cloth, coiled cylindrical material and foam.Histological sections demonstrated multiple perforations associated with transmural necrosis. Discussion ‐Primary small bowel bezoars without an associated gastric bezoar are uncommon. ‐Overall incidence of small bowel obstruction due to bezoars is low. ‐Small bowel perforation is a rare complication, induced mostly by sharp foreign bodies and less frequently by pressure necrosis of blunt bezoars. ‐Emergency laparotomy should be indicated to treat intestinal obstruction caused by bizarre bezoars. Conclusion ‐This case provides a reminder that bezoars should be suspected in cases of bowel obstructions in psychiatric and mentally retarded children. ‐An aggressive surgical approach to intestinal obstruction should be recommended in the pediatric neurologically impaired and psychiatric populations. 217 SWALLOWED MAGNETS ATTRACT TROUBLE IN TEEN. Abell, Rebecca 1; Morganstern, Jeffrey 1, 1. Stony Brook Long Island Children's Hospital, Stony Brook, NY, United States. A 14 year old female presented to the emergency room with 3 days of severe abdominal pain.Computed tomography of her abdomen demonstrated 2 circular foreign bodies in a single loop of proximal small bowel and a cluster of 3 foreign bodies in a distal small bowel loop.The patient revealed that she had accidentally swallowed magnets after trying to create the illusion of a tongue ring. She was admitted to the pediatric service and given polyethylene glycol via nasogastric tube to attempt forward progression of the magnets.After 5 days with no progression, pediatric gastroenterology was consulted.The patient was taken for an esophagogastroduodenoscopy(EGD)and colonoscopy.During the EGD magnets were not visualized,but upon removal of the endoscope, two magnets were found on the shaft of the instrument.A colonoscopy was performed without visualization of any magnets.Two hours post‐ procedure, the patient presented with a rigid abdomen and massive pneumoperitoneum on x‐ray.She was taken to surgery immediately.Jejunal perforation was found,for which she underwent resection and anastamosis.In addition, three magnets were found embedded in the cecal wall,requiring ileocecal resection with ileocolic anastomosis.The patient did well post‐operatively. Foreign body ingestion is common in the pediatric population.It is well known that the ingestion of multiple magnets may cause pressure necrosis,fistulas,obstruction,perforation,or volvulus.Ingestion of more than one magnet is an indication for immediate endoscopic intervention if the objects are located in an area accessible by endoscope.We propose that if more than one magnet is ingested,surgery rather than endoscopy should be considered as a first‐line treatment to reduce the risk of complications.To our knowledge,this is one of the first reported cases of a magnetic object being attracted to the endoscope resulting in its removal. Ingestion of a magnetic foreign body requires immediate consultation with a gastroenterologist who should be aware of the ferromagnetic properties of the endoscope. In the setting of multiple post‐pyloric magnets, surgical intervention may be the safest option. 218 UNEXPECTED ENDOSCOPY FINDING. Snyder, Brittani 2; Gugig, Roberto 1, 2, 1. Pediatric Gastroenterology, Children's Hospital UCSF Fresno, Madera, CA, United States. 2. Pediatric Gastroenterology, Kaweah Delta Healthcare District, Exeter, CA, United States. A 10 year‐old girl presented to the pediatric gastroenterologist with a 3‐year history of chronic intermittent periumbilical abdominal pain. The patient was an otherwise healthy youngd chil with no other associated symptoms. History and physical exam were unremarkable. Initial workup included normal CBC, CMP, amylase, lipase, stool H.pylori, UA, celiac testing, cryptosporidia and giardia. Abdominal and pelvic sonograms were normal. Breath testing for fructose and lactose were both positive. Symptoms mildly improved on restricted diet but did not resolve, therefore patient was referred for endoscopy. On colonoscopy there was an approximate 2 cm superficially raised lesion located approximately 3 cm from the rectum that on biopsy revealed presence of carcinoid tumor. Pelvic CT showed no nodal involvement. Patient was referred to surgery for transrectal resection. Comprising less than 0.2% of all pediatric cancers (1), carcinoid tumors are extremely rare in the pediatric population and their characterization has been mostly limited to case studies. Incidence of carcinoids increases with age in children and there is a 2/3 female predominance (2). Symptoms of abdominal pain are the most common presenting complaint in children and most of these tumors are incidentally found in the appendix after appendectomy (1,2). Pediatric carcinoid tumors have a good prognosis with 96% and 94.3% 5‐ and 10‐year disease free survival rates respectively (2). This is in part due to the fact that carcinoids rarely metastasize and over three fourths of all pediatric carcinoids are localized with less than 3% having distant metastasis (2). 1. Spunt, S. et al. A. Childhood Carcinoid Tumors: The St. Jude Children’s Research Hospital Experience. Journal of Pediatric Surgery, 2000 Sept, 35(9):1282‐6. 2. Yang, R. et al. Primary Solid tumors of the colon and rectum in the pediatric patient: A review of 270 cases. Journal of Surgical Research, 2010 Jun, 161(2):209‐216. 219 USE OF ENDOSCOPY AND GOLYTELY LAVAGE TO REMOVE PIECES OF INGESTED LIGHT BULB FROM AN 18 MONTH OLD CHILD. Ruskowski, Adam 1; Safder, Shaista 1, 1. Pediatric Gastroenterology, Arnold Palmer Childrens Hospital, Orlando, FL, United States. Background: Foreign Body ingestion in children is common. Management is dictated based on the type of foreign body and its location in the GI tract. Typically the management for sharp objects is endoscopic removal since the complication rate caused by a sharp‐pointed object passing through the gastrointestinal tract is as high as 35 percent. Sharp objects that pass beyond the reach of a flexible endoscope and then cause symptoms will require surgical intervention. Case Report: We present a case of an 18 month old child who ingested pieces of a broken light bulb. He initially had bleeding from oral cavity. Initial imaging revealed linear density in stomach concerning for lodged piece of glass in pylorus. Upon arrival to tertiary care facility repeat X ray revealed pulverized pieces of glass scattered through out small bowel. Bleeding from mouth stopped spontaneously. Pt underwent upper endoscopy for evaluation which revealed no active bleed or pieces of glass up to ligament of Treitz. Pt placed on NG Golytely lavage after initial endoscopy. Glass passed through small bowel and into caecum. However on serial imaging all the glass pieces seem to collect in the RLQ, with no progression over 24 hours with the lavage. Colonoscopy performed to visualize FB. Exam revealed pieces embedded in appendiceal orifice, Ileocaecal valve and caecum. Smaller pieces also scattered through out right and left colon. Use of endoscopic forceps, snare and flush employed to remove pieces of glass bulb piece meal out of appendiceal orifice and dropped in right colon. This technique successfully dislodged multiple pieces. Small pieces suctioned thru colonoscopy, larger pieces difficult to remove. Pt returned to floor with Golytely continued overnight. Pt passed multiple pieces of sharp glass per rectum overnight. Repeat imaging 24 hours later revealed complete evacuation of glass pieces. Pt tolerated the entire admission without any complications or symptoms. Conclusions: We describe a method to remove ingested pieces of glass from a light bulb which proved to be safe and effective. 220 COLOCOLIC INTUSSUSCEPTION WITH RECTAL PROLAPSE REDUCED LAPAROSCOPICALLY. Bhalla, Varun Kumar1; Warren, Jeremy A1; Hatley, Robyn M1; Howell, Charles G1; Pipkin, Walter L1, 1. Surgery, Georgia Health Sciences University, Augusta, GA, United States. INTRODUCTION: Intussusception is the most common cause of intestinal obstruction in children between the ages of 3 months and 6 years. Defined as the invagination of one segment of intestine into another, intussusception is most commonly ileocolic in location and idiopathic in etiology. However, those occurring in very young infants or older children are usually due to a lead point. We report a rare case of a colocolic intussusception presenting as a rectal prolapse in a six month‐old male, which was successfully treated laparoscopically. CASE REPORT: A 6 month‐old male presented with a one day history of non‐bilious emesis and a mass protruding from his rectum. The mother described his bowel movements as non‐bloody, soft, and well formed. On rectal exam, there was laxity of his anal sphincter with 8 cm of pink, viable mucosa visualized as a prolapsed rectal mass. The patient was taken to the operating room and after manual reduction, the intussusceptum was still palpable by digital exam. Proctoscopy revealed an intussusceptum in the rectum, which could not be reduced by pneumatic reduction. A laparoscopic procedure was then performed using a three‐port technique resulting in an uncomplicated, complete reduction. Post‐reduction proctoscopy demonstrated good dilation of the entire colon with no intraluminal lead point. The patient had no postop complications. DISCUSSION: Although the gold standard of treatment for intussusception is pneumatic or hydrostatic reduction, surgery plays an important role in those refractory to this treatment or complicated in their presentation. Although the role and indications for laparoscopy in intussusception are not clearly defined, we believe it provides a practical and valuable alternative to an open laparotomy. To our knowledge, this is the first reported case of a colocolic intussusception reduced laparoscopically, further highlighting the evaluable rol of laparoscopy in an atypical intussusception presentation. MOTILITY/FUNCTIONAL DISORDERS 255 MEGACYSTIS MICROCOLON INTESTINAL HYPOPERISTALSIS SYNDROME ASSOCIATED WITH MEGAESOPHAGUS .Vahabnezhad, Elaheh 1; Sicolo, Anita 1; Vargas, Jorge 1, 1. Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, UCLA, Los Angeles, CA, United States. Case Report: 4 mo girl born FT with prenatal US demonstrating megabladder, had bilious emesis and KUB postnatally showed air only in the stomach without distal bowel gas pattern. Further imaging confirmed a dilated bladder and bilateral hydronephrosis. A constrast study was significant for microcolon with limited visualization of contrast past the duodenal bulb. During an ex‐lap, the small bowel was described as short, approximately a third of the length as expected. No evidence of duodenal atresia or stricture. An ileal biopsy and colonic biopsy demonstrated ganglion cells. Unable to tolerate feeds, TPN was her only source of nutrition. Based on these findings, she was diagnosed with megacystis microcolon hypoperistalsis syndrome. At 3 mo of age, esophageal deviation and dilatation was identified incidentally on imaging. Previous radiographs at 3wks of age were read as pneumomediastinum. MRI of the chest and abdomen clearly identified the dilated, tortuous esophagus with evidence of air fluid levels and no distal obstruction. She developed aspiration pneumonia and required placement of a mid‐esophageal repogle to suction for decompression. Currently, surgical options are being explored. Discussion: Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a rare congenital disease and is part of a spectrum of intestinal motility disorders. The disease has high mortality rates and is characterized by hypoperistalsis or aperistalsis of the GI system, nonobstructive bladder distension, malrotation, dilated proximal ileum, and microcolon. The esophagus is rarely involved or described as a clinical feature. To date there are only two other published case reports of esophageal involvement with MMIHS. In our patient we noted a dilated esophagus prior to 3 weeks of life. This is unlikely due to a secondary cause such as obstruction given the early presentation. Esophageal findings with MMIHS may demonstrate a more global dysmotility disorder and should be more closely examined in this population. 256 ANAL WEB AS A MIMICKER OF HIRSCHSPRUNG DISEASE IN A CHILD WITH DOWN SYNDROME. Kundu, Neilendu 1; Alkhouri, Naim 1; Vortia, Eugene 1; Seifarth, Federico 1, 1. Cleveland Clinic, Cleveland, OH, United States. Constipation is a common problem in children with Down syndrome and can be caused by gastrointestinal anomalies including Hirschsprung disease (HD). Anal web (membrane) is part of the spectrum of anorectal malformation and may present in a similar fashion to HD. A 9‐month‐old boy who was diagnosed with Down syndrome at birth presented with feeding problems, constipation, and poor growth. Meconium was first passed five days after delivery. He had an upper gastrointestinal series that showed a meconium plug. He did well at home after his initial discharge but developed difficulty with bowel movements beginning at two months of age. He was noted to strain and cry during bowel movements, having around 3‐4 daily. He was placed on polyethylene glycol with partial improvement. His examination revealed a soft, mildly distended abdomen. A limited rectal exam was attempted, but was unsuccessful in traversing the anal canal. Barium enema revealed dilation of the rectum and sigmoid colon suggestive of HD. The patient was scheduled for an open rectal biopsy; however, visual inspection in the lithotomy position revealed a normally positioned anus with a circumferential web and a small central opening. A cotton‐tipped applicator was inserted without difficulty, but upon removal circumferential webbing was noted, limiting the anal lumen. The web was excised and the mucosa was approximated to the anoderm with interrupted Vicryl sutures. A rectal biopsy was also performed prior to the repair. Pathology revealed normal submucosal ganglion cells from the rectale biopsy. Th excised web was consistent with squamous epithelium. After the repair, our patient had substantial improvement in his appetite and activity with resolution of constipation. To our knowledge, this is the first report of anal web in a child with Down syndrome presenting with severe constipation. This diagnosis should be considered in the differential diagnosis of constipation in these patients and may have a delayed presentation when the web is partial allowing passage of feces. 257 ABDOMINAL CUTANEOUS NERVE ENTRAPMENT SYNDROME (ACNES): A COMMON AND UNDER‐RECOGNIZED CAUSE OF ABDOMINAL PAIN. Sandler, Richard H.1; Mayer, Alan N.1, 1. Pediatrics, Rush University, Chicago, IL, United States. Background: Abdominal cutaneous nerve entrapment syndrome (ACNES) has been described in the adult, but not the pediatric literature to our knowledge. It is thought to result from thoracic nerve entrapment within the lateral rectus foramen. The diagnosis is suggested by abdominal pain unrelated to bowel movements or eating, especially in a patient with an otherwise normal review of systems. Patients typically complain of a constant sharp or burning pain, which may radiate transversely in the upper abdomen and obliquely in the lower abdomen (following the thoracic nerve distributions). Physical examination shows several striking characteristics: 1)The tenderness overlies the expected thoracic nerve foramen on the lateral aspect of the rectus muscle; 2)There is one discrete point of tenderness that drops off just 1‐2 cm; 3)The tenderness is the same or increased with tensing of the abdominal musculature (as with straight leg raising). This latter finding is called “Carnett’s Sign.” It is present in all such patients, and helps to establish an abdominal wallvs. intra‐abdominal etiology. Treatment is with 1‐2 ml of 0.25% bupivacaine or 1‐2% lidocaine directed just anterior to the nerve foramen orifice. One injection usually results in complete and lasting relief, but several injections may be required. Complete resolution of the pain with injection also provides the final confirmation of the diagnosis. Cases: Our first 12 cases were reviewed. Nine were females (75%), with an average age of 16.1 (range 11‐21y). Duration of symptoms ranged from 2 wks to 1 yr. Eleven of 12 obtained complete relief with the initial injection. Five required one or more repeat injections (range 1‐3). Comment: ACNES is uncommonly considered in the differential diagnosis of abdominal pain in children. After diagnosing ACNES in an index case 8 months ago, we now diagnose and treat at least 1‐2 new cases each month, often to the amazement of the patient and their family. We suggest that ACNES should be considered in the differential diagnosis of atypical abdominal pain, especially in the female teenager. 258 SINGLE BALLOON ASSISTED COLONOSCOPY FOR PLACEMENT OF COLONIC MANOMETRY CATHETERS – INITIAL SINGLE CENTER EXPERIENCE IN CHILDREN USING A NOVEL TECHNIQUE. Sanghavi, Rinarani M1; Barth, Bradley 1, 1. UT Southwestern Medical Center, Dallas, TX, United States. BACKGROUND: Colonic manometry is being increasingly used in patients to assess colonic motility and to aid in surgical decisions. It involves placement of a manometry catheter in the colon from the anus to the ascending colon. It is imperative, for proper manometric interpretation, to place the catheter without loops in the colon. However, most patients with long standing constipation have a dilated and redundant sigmoid colon which makes proper placement of the catheter and the colonoscopy technically challenging. Once the cecum has been reached, subsequent placement of the manometry catheter is complicated due to looping of the catheter in the redundant sigmoid. Single balloon assisted colonoscopy has been successfully used in adults in cases of difficult colonoscopy. We report the first pediatric series of single balloon assisted colonoscopy in children. METHODS: All patients received NG golytely cleanout prior to the procedure. The Olympus S‐180 enteroscope was used. Single balloon technique was used to reach the cecum. Once the cecum was reached, the overtube was advanced beyond the scope. The overtube was then left in place, while the enteroscope was removed. This provided an easy path for placement of the colonic manometry catheter over a guide wire, without the usual issues of looping and inability to advance the catheter. RESULTS: Over 12 months, 19 patients underwent placement of colonic manometry catheter using this technique. Ages were 3years to 20 years.Weights ranged from 13.1kg to 72 kg.We were successful in placing the catheter in all cases. Procedure times varied from 119 min to 41 min. The terminal ileum was reached in all cases.There were no complications noted.CONCLUSIONS:This is the first reported series of single balloon assisted colonoscopy in children. Leaving the overtube in place to allow for passage of catheter is a novel method not previously described. It may have therapeutic applications in other patients for removal of polyps or placement of similar catheters. 259 HIGH AMPLITUDE ANTRAL CONTRACTIONS IN AN ADOLESCENT WITH CANNABIS ASSOCIATED HYPEREMESIS. Gomez, Roberto 1; Bentley, Elizabeth M1; Fortunato, John E1, 1. Pediatric Gastroenterology, Wake Forest University , Winston Salem, NC, United States. Introduction: Cannabis hyperemesis is a known side effect from chronic use of marijuana in adults and pediatrics. Cannabinoid receptors 1‐2 have been shown to decrease the frequency of transient relaxations of the lower esophageal sphincter (TRLES) without affecting gastric emptying. There is a paucity of studies showing the effects of cannabis on the antral motility. Case: A 16 year old male presented with a 1 year history of nausea and postprandial emesis. His symptoms were worse in the morning upon awakening resulting in decreased school attendance and quality of life. Emesis was non‐ bilious, non‐bloody, and non‐projectile improving throughout the day most notably after long, hot showers. He complained of heartburn, anxiety, and insomnia with a 15 lb weight loss over a year. He denied abdominal pain, diarrhea, constipation, hemathochezia, melena, diet restrictions, fever, malaise, headache, mouth sores, or joint pain. Medications including anti‐emetics, proton pump inhibitors and prokinetics did not relieve his symptoms. Laboratory assessment including CBC, CMP, TTG, IgA, TSH, and morning cortisol level were unremarkable. Anti‐nuclear antibody and Sci 70 were negative. An upper GI series showed normal anatomy and solid phase gastric emptying scintigraphy demonstrated late delayed gastric emptying. EGD biopsies revealed chronic esophagitis based on biopsies, but was otherwise normal. The patient tested positive for benzodiazepine and cannabis on the day of motility testing. Antroduodenal manometry showed antegrade duodenal phase III contractions with antral amplitudes reached 350‐400 mm Hg after erythromycin or meal stimulation without associated emesis. His symptoms improved after starting Dronabinol. Conclusion: This case is a strong example of cannabis hyperemesis in an adolescent, but is the first to demonstrate the presence of strong antral contractions, which may in part explain the severity of his symptoms. CLINICAL VIGNETTE POSTER SESSION III SATURDAY, OCTOBER 22, 2011 10:00am – 12:00pm ESOPHAGUS/STOMACH 297 H. PYLORI: A RARE CAUSE OF PROTEIN‐LOSING GASTROPATHY IN CHILDREN. Willis, Asha 1; Brief, James 2; Wetzler, Graciela 1, 1. Division of Pediatric Gastroenterology, Maimonides Infant's and Children's Hospital of Brooklyn, Brooklyn, NY, United States. 2. Pediatrics, Maimonides Infant's and Children's Hospital of Brooklyn, Brooklyn, NY, United States. Protein‐losing gastropathy in children has often been described in the setting of Menetrier’s disease. Typical findings are gastric hypertrophy and hypoalbuminemia due to protein loss through gastric mucosa. It has been associated with CMV infection and, less often, with H. pylori. We report a case of protein‐losing gastropathy and H. pylori gastritis, without evidence of gastric hypertrophy. A 3 y/o male presented with facial swelling, abdominal pain and abdominal distention for 5 days. He had poor appetite and no weight gain for 3 months. Physical exam revealed moderate abdominal distension, and facial and periorbital edema. His abdomen was non‐tender and there was no hepatosplenomegaly or pitting edema of the extremities. Laboratory values were significant for albumin of 1.5 g/dl and total protein of 2.7 g/dl. Complete blood count and liver function tests were normal and urinalysis was negative for protein. Abdominal CT showed small pleural effusion, minimal pelvic ascites and mesenteric edema. The stomach appeared normal with no thickening of the antrum. The small and large bowel appeared normal. EGD showed diffuse areas of ulceration in the body of the stomach, with normal appearing antrum. Gastric biopsies from body and antrum demonstrated moderate chronic gastritis without foveolar hyperplasia. Giemsa stains of these biopsies were positive for H. pylori, and immunohistochemistry stains were negative for CMV and HSV. Esophageal and duodenal biopsies were normal. Treatment with antibiotics and PPIs for H. pylori gastritis was successful. Hypoalbuminemia and edema improved with resolution of his symptoms. H. pylori should be considered as a cause of protein‐ losing gastropathy in children presenting with edema and hypoalbuminemia, even when signs of gastric hypertrophy are not present. 298 ENDOSCOPIC ULTRASOUND FOR DIAGNOSIS AND SURVEILLANCE OF GASTROINTESTINAL STROMAL TUMORS IN AN 11 YEAR OLD CHILD. Laroche, Greggy Denis1; Conway, Jason D2; Fortunato, John E1, 1. Pediatrics, Wake Forest University School of Medicine, Winston‐Salem, NC, United States. 2. Internal Medicine, Wake Forest University School of Medicine, Winston‐Salem, NC, United States. An 11 year old Caucasian female with a past history of chronic streptococcal pharyngitis presented with anemia and guaiac positive stool 1 month after tonsillectomy. She was seen by her pediatrician at this time for upper respiratory symptoms and due to complaints of paleness, nausea, lightheadedness, and abdominal discomfort, laboratory testing was performed. She had no symptoms of visible blood in her stools or heavy menses. Her hemoglobin and hematocrit were low, 6.9 dg/dL an 21%, respectively with a ferritin of 3 ng/mL. Her pediatrician started her on iron as her anemia was thought to be secondary to blood loss from her tonsillectomy. Her hemoglobin increased to 10.2 g/dL 4 weeks later, and decreased to 7.3 g/dL two weeks thereafter. For this reason she was admitted to pediatric hematology. GI consultation was obtained and esophagogastroduodenoscopy (EGD) was performed demonstrating 7‐8 large submucosal masses in the antrum and distal body with a deep ulceration noted in one of the masses. A CT of the chest, abdomen, and pelvis was performed defining multiple masses on both serosal and mucosal aspects of the stomach as well as liver enlargement. Endoscopic ultrasound (EUS) revealed multiple large well‐defined hypoechoic gastric masses arising from the muscularis propria. EUS guided fine needle aspiration revealed epithelioid gastrointestinal stromal tumor (GIST). Distal gastrectomy with Billroth I gastroduodenostomy was later performed. Annual EUS surveillance has been performed since surgery for 4 years with no evidence of recurrent GIST. This is the first case reported in children using EUS not only to define morphologic characteristics of a GIST, but also to obtain a specimen for pathological analysis, and allow periodic surveillance for recurrent disease. It underscores the feasibility and potential role of EUS in diagnosing and managing gastrointestinal tumors in children. 299 AN UNUSUAL CASE OF ABDOMINAL PAIN AND FLATULENCE IN A CHILD. Lopez, Heather Nicole1; Pranikoff, Thomas 1; Kirse, Daniel J.1; Hill, Ivor 1, 1. Pediatrics, Wake Forest Baptist Health, Winston Salem, NC, United States. Case Presentation: An 8 year old boy presented with chronic abdominal pain, distension and flatulence dating back to infancy. PMH was significant for three prior pneumonias and bilateral inguinal hernia repair. Numerous KUB studies showed air in the stomach and throughout the intestines. He had undergone three upper GI endoscopies with assays for pancreatic exocrine enzymes and intestinal disaccharidases, an abdominal ultrasound, a gastric emptying study and breath tests for lactose and fructose malabsorption that were all unremarkable. Treatments included pancreatic enzyme supplementation, beano with meals and a trial on a gluten‐free diet, all with no relief. Aerophagia was entertained as a diagnosis. Further history revealed his distension did not improve during sleep as expected. This, and the constant presence of air in the stomach, raised the possibility of a tracheoesophageal fistula (TEF). An esophogram identified a possible small fistulous connection between the trachea and esophagus in the cervical region. An H‐type TEF was confirmed on rigid bronchoscopy. The TEF was corrected surgically with complete resolution of his symptoms. Discussion: Abdominal pain in children is most often attributed to a functional disorder. When accompanied by excess flatulence, bowel distension is likely the cause of the pain. Excess intestinal gas is usually related to some form of carbohydrate malabsorption such as that of lactose or fructose. In such cases the gas is generally confined to the large intestine. Malabsorption syndromes were excluded on investigation. The presence of gas throughout the intestine and the stomach raised the possibility of aerophagia due to surreptitious air swallowing. Against this was the reported presence of abdominal distension and pain upon wakening each morning. This prompted concern for and subsequent investigation of a TEF. Small TEFs are difficult to identify on upper GI endoscopy and are best seen during bronchoscopy. Surgical correction if his H‐type TEF resulted in complete resolution of his symptoms. 300 DYSPLASTIC FUNDIC POLYP IN CHRONIC PPI USE ‐ NOT SO BENIGN. Aybar, Ahmet 1; Zheng, Hengqi B1; Twaddell, William S2, 1; Blanchard, Samra S1, 1. Pediatrics, University of Maryland, Baltimore, MD, United States. 2. Pathology, University of Maryland Medical Center, Baltimore, MD, United States. Chronic proton pump inhibitor (PPI)use has been linked to the development of sporadic fundic gland polyps (FGP). The underlying pathophysiology of PPI‐induced FGP continues to be investigated. Length ofy PPI therap was reported to be a determining factor in polyp development. Most cases report sporadic FGPs to follow a benign course in PPI use. Although reports of dysplastic changes in FPG do exist in familial adenomatous polyposis (FAP), dysplastic changes in sporadic FGP from PPI therapy remain rare. Although most of sporadic FGPs cases are reported as benign in adult PPI use, here we discuss a case report of a dysplastic FGP in a pediatric patient on PPI therapy. Patient is a 19 year old female with a history of autism, bipolar disorder, anxiety, seasonal allergies, hashimoto thyroiditis, constipation, heartburn and nutcracker esophagus. She had mild acid reflux in 24 hour pH study. The patient has been on acid suppression for a total of 35 months. The patient had been on lansoprazole 30 mg BID for 11 months and 30mg qday for 6 months for a total of 17 months. Thet patien had been on rabeprazole 20mg qday for 2 months, 20mg BID for 10 months, and 20mg TID for 6 months, for a total of 18 months. (her body weight varied between 69 to 72 kg) Prior to PPI therapy, her first EGD showed esophageal nodularity, diffuse nodular mucosal and submucosal hemorrhages in the stomach, with normal biopsies. Subsequent EGD performed due to refractory symptoms and showed nodular mucosa in the gastric fundus with multiple gastric polyps with biopsies consistent for FGP with high grade dysplasia. PPI therapy is discontinued. In this case, the patient had developed sporadic FGPs with high grade dysplastic changes within a 3 year time period with use of maximum dose of 1 mg/kg/day of PPI. To our knowledge, dysplastic fundic polyp is not reported in chronic use of PPI in children and adolescents in English literature. This case illustrates that perhaps we should perform follow‐up EGD on patients who are on chronic PPI for a long period of time. 301 CASE SERIES: SYMPTOMATIC GASTRIC INLET PATCHES ABLATED WITH ARGON PLASMA COAGULATION. Alberty, John Brannon1, 1. Gastroenterology, Our Lady of the Lake Children's Hospital, Baton Rouge, LA, United States. Introduction Gastric inlet patches (GIP) have been shown to occasionally cause symptoms in adults and respond to argon plasma coagulation (APC). No such data exists for children. We present 3 cases in children whose symptomatic GIP's were successfully ablated with long term symptomatic relief. Case 1 A 5 year old male presented with a 5 month history of globus and dysphagia to solids. He had no relief with twice daily lansoprazole. Endoscopy showed 2cm and 1.5cm GIP's. Ablation with a side‐fire APC probe at settings of 30 watts and 0.8 liters/min resulted in immediate relief of globus and after 1 week began to eat solids. Lansoprazole was discontinued. The patient was asymptomatic 1 year later. Case 2 A 7 year old female presented with a 2 month history of incessant cough, worse after eating and at night. She had no relief with twice daily Lansoprazole. Endoscopy showed a 2cm GIP. Ablation with a straight‐fire APC probe at the same settings resulted in immediate relief from the cough. Lansoprazole was discontinued. The patient was asymptomatic 1 year later. Case 3 A 9 year old male presented with a 4 month history of dysphagia to solids and a 7 pound weight loss. He had no relief with 3 months of daily Lansoprazole. Endoscopy showed a GIP that was ablated as in case 2. Within 1 week the patient was eating solids and regained his weight. At 4 months the patient remained asymptomatic. Discussion GIP's in children may cause symptoms such as dysphagia to solids, globus, and chronic cough. APC can be successfully applied to GIP's in children with symptom relief. We observed no post‐operative complications with APC in our small series. 302 IS ENDOSCOPIC INITIAL MICKEY GT PLACEMENT IN CHILDREN COST EFFECTIVE AND WHAT TO EXPECT. Yuwono, M. 1; Acierno, S. 2; Holland, R. 2; Lustig, D. 1, 1. Ped GI, MBCH, Tacoma, WA, United States. 2. Ped Surgery, MBCH, Tacoma, WA, United States. Introduction: Gastrostomy tube(GT) are useful to provide optimal nutrition support in children. Low profile GT placement is a common procedure and preferable to a conventional one. GT can be placed either surgically, endoscopically or radiologically. Since the availability of new Kimberly Clark Introducer Kit, we report the safety of initial Mickey GT placement endoscopically with surgical assistance vs open surgery or fluoroscopic technique and compare the cost using different methods. Methods: A retrospective study of children who require GT insertion was undertaken. Cost data was compared using an institutional activity‐based cost methodology. Low profile GT was placed in 22 patients (6 endoscopic, 2 surgical and 14 fluoroscopically). Results : Indications for GT tube placement were: FTT, dysphagia and seizure disorder. There were 6 females and 16 males with range of 4 months to 19 years old. Two patients had perforation of the bowel and one had significant pain after fluoroscopic insertion. All three required surgical repair. One patient had repeat GT placement after newly endoscopically‐ placed tube became dislodged. Another placement was abandoned when the stomach was noted to be abnormally positioned. One patient thad repea fluorospic placement after surgically placed GT tube was dislodged. Endoscopic and fluoroscopic placement were done without endotracheal intubation. The global average cost was about $2400 for endoscopic placement in GI suite, $2000 for fluoroscopic placement in IR and $ 4000 for surgical placement in OR. Summary : 1. Mickey GT can be placed not only under fluoroscopic but also using endoscopic approach, which can be done safely as an initial feeding tube in children. 2. Complications related to tube placement with endoscopic guidance are not significantly different than other methods of placement. Direct visualization in the stomach aids in placement position to void possible potential risk of perforation. 3. Given the significant cost differential between different methods, endoscopic approach is preferable for a non‐surgical candidate patients. 303 RETROGRADE DUODENOGASTRIC INTUSSUSCEPTION: A RARE COMPLICATION OF GASTROSTOMY TUBE. Patel, Nirav 1; Lewis, Allison 2; Marcus, Matthew3; Glasser, James 2, 1. Pediatric Gastroenterology, University of South Carolina School of Medicine, Columbia, SC, United States. 2. Pediatric Surgery, University of South Carolina School of Medicine, Columbia, SC, United States. 3. Radiology, University of South Carolina School of Medicine, Columbia, SC, United States. Complications associated with low‐profile gastrostomy buttons are infrequent occurrences; the most common, such as granulation tissue formation, tube dislodgement, and site infections are rarely serious. More significant complications include duodenal ulceration with upper GI bleeding, gastrocolic fistulae, and intraperitoneal leakage of feeds. We present the case of a 4 month old ex‐32 weeker baby boy who developed a retrograde duodenogastric intussusception, which is a rare complication of gastrostomy tube. The child had presented with fussiness and non‐bloody, non‐bilious emesis for 48 hours, particularly during gastrostomy tube feedings. On examination he was found to have a very tight‐ appearing, retracted gastrostomy button. The primary care provider (as well as the guardian) attempted to manually reposition and pull back on the gastrostomy tube. However the patient’s symptoms persisted. An upper GI series demonstrated a mass‐like filling defect in the gastric antrum, with no contrast emptying into the duodenum during the study. An abdominal sonogram revealed findings compatible with duodenogastric intussusception. Subsequently, endoscopic reduction was attempted, but unsuccessful. Therefore the intussusception was surgically reduced and a pyloromyotomy was performed. This case demonstrates that duodenogastric intussusception should be considered in the differential of any child with a gastrostomy tube who presents with symptoms of gastric outlet obstruction. It also serves as a reminder that the balloon of a tight‐appearing gastrostomy tube should be deflated prior to manipulation of the device. 304 RELATIONSHIP BETWEEN OBESITY AND GERD IN CHILDREN. Nathan, Radha 1; Matta, Sravan Reddy 1, 1. Pediatric Gastroenterology, Brookdale University Hospital and Medical Center, Brooklyn, NY, United States. BACKGROUND: GERD is one of the most common esophageal disorders encountered in pediatric gastroenterology practice. Research done across the world shows conflicting results concerning the relationship between GERD and obesity in children METHOD: We used the National Inpatient Sample (NIS) from the year 2007 to analyze the relation between GERD and obesity in a pediatric population between 5‐17 years in the US. NIS, which is the largest US all payer database is a part of the Healthcare Cost and Utilization Project (HCUP) sponsored by the Agency for Health Care Research and Quality (AHRQ) and contains information from a 20% stratified sample of hospitals extrapolated to show the entire national utilization in US. We used ICD 9 code (530.81) to extract all in‐hospital occurrence of GERD diagnosed by upper endoscopy and clinical symptoms. We further explored the association of GERD with overweight (278.02), obese NOS (278.00) and morbidly obese (278.01) population using ICD 9 codes. IBM PASW 18 was used for our statistical analysis. CONCLUSION: We found an increased prevalence of GERD in morbidly obese individuals when compared to overweight and obese children within the age group of 5‐17 yrs. This shows a positive correlation between GERD and increasing weight. These preliminary data shows the need for aggressive management of obesity to prevent high morbidity. More studies are needed to correlate GERD (endoscopic diagnosis) with obesity. 305 HYPERTROPHIC GASTROPATHY IN A CHILD WITH PERIORBITAL EDEMA: MENETRIER’S DISEASE. Ambati, Shashikanth Reddy 1; Lucia, Chantal 1; Reeves‐Garcia, Jesse 1; Muinos, William 1; Hernandez, Erick 1; Gomara, Roberto 1, 1. Pediatric Gastroenterology, Miami Children's Hospital, Miami, FL, United States. INTRODUCTION: Menetrier’s disease is a form of hypertrophic gastropathy involving mainly the fundus and the body and associated with hypoalbuminemia due to protein loss through the gastric mucosa. The disease is typically seen in adults and nearly 40‐50 casesn have bee reported so far in children. The onset and duration and the course of the disease in children varies widely than in adults. Typically children have an abrupt, self‐limited course lasting 5 to 8 weeks where as adults have an insidious onset of disease, which can go undiagnosed for years. CASE REPORT: A 4 year old boy with history of asthma and atopic dermatitis presented with 6 day history of worsening periorbital odema and no other symptoms. Physical exam is unremarkable except for bilateral periorbital odema and cervical lympadenopathy. Laboratory studies revealed a white count of 9.6 and a normal chemistry. Total protein and albumin are markedly decreased at 3.2 g/dL and 1.6 g/dL respectively. Liver enzymes are normal except for elevation of ALT 89 U/L. Urine analysis is normal except for traces of protein. Patient had an upper GI endoscopy which showed enlarged gastric folds involving the body and fundus but sparing the antrum. The histology of the biopsies revealed dense infiltrates of eosinophils in the lamina propia with elongated pits. During the hospitalization, the patient was placed on high protein diet and fluid restriction with strict monitoring of input and output and daily weights. On follow up after 2 weeks after the discharge, patient improved with no signs of periorbital edema and improved albumin levels. DISCUSSION: Although Menetrier’s disease of childhood is uncommon, it is important for emergency medicine physicians and pediatricians to consider protein‐losing gastroenteropathies when encountered with an edematous child. References: 1.Cytomegalovirus‐associated protein‐losing gastropathy in childhood. O. Megged Eur J Pediatr (2008) 167:1217–1220. 306 UPPER GI BLEED CAUSED BY GASTRIC PENETRATION OF TEFLON PLEDGETS USED FOR FUNDOPLICATION. Min, Steve B1; Chao, Catherine 2, 1. Pediatrics, Walter Reed Army Medical Center, Washington , DC, United States. 2. Pediatrics, Inova Hospital for Children, Fairfax, VA, United States. A 3 year old male with history of hypoplastic left heart syndrome, s/p Glenn and Fontan procedures, presented with hematemesis. He had a history of gastroesophageal reflux disease s/p Nissen fundoplication and gastrostomy tube placement 2.5 years ago. He had no abdominal pain, or preceding illness. During the upper endoscopy, a 2X2 cm, white solid material with sutures, was noted adherent to the fundus. The foreign body was encased in granulation tissue and fixed onto the fundic mucosa. Due to concern that the mass represented surgical material that had eroded through the stomach, pediatric surgery was consulted for evaluation. The surgeon performed an exploratory laparoscopy through the patient’s gastrostomy, and removed the foreign body without complications. There was no further bleeding post‐operatively. The foreign body mass was confirmed to be Teflon pledgets and suture material that were used for his fundoplication. The pledgets had eroded through the fundus, into the gastric lumen, becoming encased in granulation tissue. This led to mucosal irritation and gastric bleeding. Teflon pledgets are small flat pads that are used by some surgeons to help secure a fundoplication. Though uncommon, a few cases of gastroesophageal luminal penetration by pledgets have been described. Dally E, reported 11 adult patients who had symptomatic pledget erosion occurring after surgery (Am J Surg. 2004). Symptoms included dysphagia, recurrent gastroesophageal reflux, chest pain and melena. Similar pledget erosions occurring in children have not been reported in the literature. We report an unusual case of upper GI bleed caused by erosion of Teflon pledgets into the gastric lumen, 2.5 years after fundoplication. In a child who has had fundoplication using pledgets, with symptoms to include GI bleeding, recurrent gastroesophageal reflux, or dysphagia, evaluation to include endoscopy should be considered. INTESTINE/COLON/IBD 338 PRIMARY INTESTINAL LYMPHANGIECTASIA WITH EXTRA‐INTESTINAL FINDINGS. Paul, Adam 1; Zawahir, Shamila 1, 1. Pediatrics, University of Maryland Children's Hospital, Baltimore, MD, United States. Primary intestinal lymphangiectasia (PIL) is a rare cause of protein losing enteropathy. It is caused by ectasia of lacteals with impaired lymphatic drainage. It presents with diarrhea, vomiting and peripheral edema with hypoalbuminemia and lymphocytopenia. Congenital lymphangiomatosis is a rare disorder with multiple lymphangiomas in every organ system except CNS. These progressive and locally destructive lesions may cause interstitial lung disease, pleural and pericardial effusions, lytic bone lesions and splenomegaly. A 5 year old female presented with a two week history of abdominal pain and distention, decreased oral intake, emesis, and diarrhea. She had been diagnosed with peripheral lymphedema as an infant due to left upper and lower extremity edema. She now had tachypnea, marked anasarca, ascites, and a 3 cm subcutaneous mass on her back. CXR revealed bilateral pleural effusions, her serum albumin was 1.2 g/dL and she had a low lymphocyte count of 9% with elevated stool alpha‐1‐antitrypsin level. MRI revealed pleural effusions, multiple enlarged mesenteric lymph nodes and cystic lesions of her spleen consistent with lymphangiomas. Exploratory laparoscopy revealed chylous ascites and multiple enlarged lymph nodes within the small bowel mesentery and omentum. Lymph node and back mass biopsy revealed dilated lymphatic vessels. Her EGD and colonoscopy revealed marked lymphangiectasia in the duodenum and terminal ileum. She was diagnosed with congenital lymphangiomatosis with PIL. The patient was started on a high protein, low fat diet and had improvement in edema and serum albumin level. It should be noted that PIL may present with extra‐intestinal involvement in the form of congenital lymphangiomatosis and MR imaging is the most accurate for the diagnosis of lymphatic malformations. PIL may be managed with life‐long dietary therapy, but this is not the case with congenital lymphangiomatosis. Recognition and surveillance imaging of these lesions with their slow chronic progression will alert physicians to watch for acute deterioration particularly of the cardiopulmonary system. 339 COLLAGENOUS GASTRITIS AND COLITIS IN A PEDIATRIC PATIENT. Raizner, Aileen 1; Phatak, Uma Padhye1; Jain, Dhanpat 1; Pashankar, Dinesh S1, 1. Yale University, New Haven, CT, United States. Background: Collagenous gastritis is a rare disorder characterized by deposition of a subepithelial collagen band along with an inflammatory infiltrate in the stomach. In adults, it is accompanied with collagenous colitis. This combination has been reported recently in only two pediatric cases (Endoscopy 2009, Cases Journal 2009). Case Report: A 4 year old girl presented with a history of nonbloody diarrhea and intermittent abdominal pain following viral gastroenteritis. Endoscopic evaluation revealed revealed diffuse eosinophilia in the stomach, duodenum, TI, and colon along with collagenous bands in the colon. A dairy free and 6‐food elimination diet provided minimal relief of the symptoms. She continued to have intermittent diarrhea and slow weight gain. Blood tests revealed Hb 9.5 g/dL and albumin 2.8 g/dL. At 5 years of age, endoscopy revealed gastric and colonic nodularity. Histology showed eosinophilic inflitration in the stomach, duodenum, TI and colon along with gastric and colonic subepithelial collagen deposition of more than 10μm thickness (normal <5 μm). She was treated with lansoprazole and a steroid course. The diarrhea and growth improved and albumin increased to 3.1g/dL over time. Two years later, she presented with diarrhea and anemia without any obvious precipitating factors. Enodscopy showed collagenous gastritis and colitis with eosinophillia , but normal duodenum and ileum. Staining was negative for type IV collagen. She responded well to a steroid course and is currently doing well without therapy. During her clinical course, celiac antibody, allergy evaluation, stool cultures, and helicobacter studies were normal. Conclusions: We describe a rare case of a child with collagenous gastritis and colitis. We report a relapsing clinical course, response to steroid therapy, and an evolving spectrum of histological features of collagenous gastritis and colitis. 340 LACTOBACILLUS REUTERI ASSOCIATED PERITONITIS IN A CHRONICALLY ILL PEDIATRIC PATIENT. Ontaneda, A. 1; Huang, A. 1; Lenoir, A. 2; Muniz Crim, Alisa Jo‐El1, 1. Gastroenterology, Miami Children's Hospital, Miami, FL, United States. 2. Infectious Diseases, Miami Children's Hospital, Miami, FL, United States. Probiotic use has become an increasingly popular practice in chronically ill pediatric patients. Lactobacillus reuteri is a frequently used probiotic due to its reported safety profile. We describe a patient with Eagle Barrett Syndrome and end stage renal disease who received Lactobacillus reuteri for treatment and prevention of diarrhea secondary to multiple courses of antibiotics. The patient developed culture positive Lactobacillus reuteri peritonitis after several months of probiotic usage. The infection was rapidly identified and treated and the patient had an uncomplicated recovery. This report illustrates a case of probiotic‐associated peritonitis with the use of an agent frequently used in chronically ill and immunocompromised patients. This is the first reported case of peritonitis secondary to Lactobacillus reuteri. It is important to consider the ability of Lactobacillus reuteri to cause invasive disease. This case should not discourage appropriate therapeutic use of probiotics. 341 UNUSUAL PRESENTATION OF MULTIFOCAL LYMPHANGIOENDOTHELIOMATOSIS WITH THROMBOCYTOPENIA (MLT). Bitar, Anas 1; Altaf, Muhammad A1, 1. Pediatric Gastroenterology & Nutrition, Oklahoma University HSC, Oklahoma City, OK, United States. Background: MLT is a rare vascular disorder characterized by cutaneous and gastrointestinal (GI) lesions of lymphatic‐endothelial origin and intra‐lesional consumptive thrombocytopenia. All previously reported cases presented with cutaneous manifestations followed by GI bleeding during early. infancy Multiple drugs and endoscopic coagulation have been used with variable results. Case presentation: A three‐month‐old male presented with 6 weeks history of hematemesis and melena. Laboratory studies revealed severe anemia and thrombocytopenia. Bone marrow biopsy revealed slightly hypocellular marrow. Upper endoscopy showed multi‐focal, variable‐sized, erythematous lesions scattered throughout the stomach. Histological findings and immunostaining was consistent with MLT. He was managed with continuous octreotide drip, steroids and aminocaproic acid for first year of life. His clinical course has improved remarkably with decreased frequency of GI bleeding and successful discontinuation of steroids and octreotide. The patient has not developed a single skin lesion since diagnosis. Partially reversible radiographic metaphyseal changes in long bones, spontaneously resolving pneumatosis intestinalis (PI), worsening of bleeding with antibiotics and vaccinations were distinctive features. Discussion: This is the first reported case of MLT without any skin manifestations where the diagnosis was made on GI biopsies only. Bone lesions in association with MLT have been described previously in three cases. Bone changes are inconsistent feature of MLT. PI, not reported before, could be new feature of MLT secondary to the intestinal vascular lesions vs. steroids side effect. Exacerbation of GI bleeding in MLT with antibiotics and vaccinations has not been reported previously either. We recommend delaying vaccinations, minimal use of antibiotics and avoidance of any endoscopic or surgical intervention as symptoms improve after first year. Familiarity and vigilance to this disease is crucial in dealing with infants with GI bleeding. 342 MILK PROTEIN ALLERGY MIMICKING AUTOIMMUNE ENTEROPATHY. Srinath, Arvind 1; Ranganathan, Sarangarajan 2; Goyal, Alka 1, 1. Gastroenterology, Children's Hospital of Pittsburgh, Pittsburgh, PA, United States. 2. Pathology, Children's Hospital of Pittsburgh, Pittsburgh, PA, United States. Cow’s milk protein allergy (CMPA) presents with vomiting,diarrhea, and failure to thrive. It can mimic autoimmune enteropathy (AIE).Both can have intestinal inflammation and villous damage.CMPA is treated by milk protein elimination while AIE requires immunosuppression.A 2 month term male presented with diarrhea leading to hypovolemic shock.He was breastfed and had loose non‐bloody stools which worsened after an upper respiratory infection.His initial hemoglobin was 8 mg/dl.His diarrhea was refractory to elemental,carbohydrate‐free formula,NPO status,and octreotide.Duodenal biopsies showed villous blunting,crypt abscesses,and apoptosis.Sigmoid biopsies showed active colitis with mixed infiltrate of the crypts and epithelium.Stool infectious studies,upper gastrointestinal series,quantitative immunoglobulins,anti‐enterocyte antibodies,gastrin,VIP levels,lactate:pyruvate ratio,CPK,serum amino acids,transferrin isoelectric focusing,acylcarnitine profile,biotinidase deficiency profile,G6PD analysis,Cystic Fibrosis screen,comprehensive newborn screen,Galactose‐1‐phosphate uridyltransferase,and cortisol were normal.The patient was discharged on an advancing elemental diet but relapsed ten days later.Endoscopy and sigmoidoscopy showed neutrophilic microabscesses in the sigmoid and descending colonic crypts.The infiltrate in both areas had few eosinophils.Infectious etiology was ruled out. Duodenal intraepithelial lymphocytes were not increased.The pathology suggested AIE.The patient improved on intravenous steroids and advanced to a full elemental diet.Repeat endoscopy three months later showed improvement.He required steroids for the next six months.His diarrhea recurred when challenged with milk protein,but resolved on an elemental diet.He did not need further immunosuppression.The patient tolerated milk introduction by age 2,and is healthy one year later.In lieu of long‐term symptomatic improvement without need for continued immunosuppression,but recurrence onn milk protei challenge,we conclude the patient actually had CMPA rather than AIE. 343 AN UNUSUAL PRESENTATION OF A PEDIATRIC GIST. Moreau, Brigitte 1; Nguyen, Van‐hung 1; Sigman, Terry 1, 1. Mcgill University, Montreal, QC, Canada. Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors of the gastrointestinal tract which are rarely seen in children and the majority occurs in the stomach. We report the case of a 17 year old boy with a duodenal GIST. He presented with the sole symptom of acute onset fatigue and shortness of breath and on admission was found to have a severe microcytic anemia requiring a blood transfusion. An upper endoscopy was performed and a polypoid mass with an adherent clot was identified in the duodenum (figure 1). No obvious stalk was seen and a biopsy of the mass was taken. A unique polyploid mass at the level of D2‐D3 was confirmed by barium imaging and wireless capsule endoscopy. Histological findings of the biopsy revealed a spindle cell GIST positive for C‐KIT (CD117). Ten days after his admission, the tumor was resected and the surgical specimen showed a polypoid tumor (2.5 x 2.0 x 1.8 cm) arising from the muscle wall that was irregular in shape with an ulcerated surface. The mitotic rate was 9/ 50 HPF with negative margins which conferred an intermediate risk according to the NIH consensus. Staining for CD34, desmin, S‐100 and keratin AE1/AE3 were negative. C‐KIT positivity was confirmed and staining was also positive for vimentin and some focal staining for SMA and caldesmon. The PDGFRA mutation is pending. In conclusion, only 12 cases of small intestinal pediatric GIST have been reported in the literature and only 1 was diagnosed by endoscopic biopsy. On endoscopy, gastric GIST is usually described as a submucosal mass with smooth margins and a normal overlying mucosa and endoscopic biopsies are often reported as nondiagnostic. Our mass was polypoid and friable in appearance and was suspicious for a polyposis syndrome. This case substantiates the importance of including GIST in the differential diagnosis of a duodenal polypoid mass on endoscopy. Furthermore, it demonstrates the usefulness of endoscopic biopsy in confirming the diagnosis which can help in the surgical management of these patients. 344 JEJUNAL WEB: CLINICAL, RADIOLOGIC AND PATHOLOGIC DESCRIPTION. Rudolph, Bryan 1; Levin, Terry 3; Douglas, Lindsey 4; Ewart, Michelle 5; Borenstein, Steven 2; Jan, Dominique 2; Thompson, John 1, 1. Pediatric Gastroenterology, Children's Hospital at Montefiore, Bronx, NY, United States. 2. Pediatric Surgery, Children's Hospital at Montefiore, Bronx, NY, United States. 3. Pediatric Radiology, Children's Hospital at Montefiore, Bronx, NY, United States. 4. Pediatrics, Children's Hospital at Montefiore, Bronx, NY, United States. 5. Pathology, Children's Hospital at Montefiore, Bronx, NY, United States. Jejunal webs, or type I jejunolileal atresias, are rare congenital anomalies considered to be secondary to intrauterine ischemia. We report a 7 day old female presenting with post‐prandial bilious emesis. She was born full term, without complications, and passed meconium within 24 hours of birth. Initial lab work‐up was normal with the exception of mild dehydration (bicarbonate of 19 mEq/L). Physical exam revealed a non‐distended, non‐tender abdomen in a well appearing child. An x‐ray performed in the Emergency Department demonstrated a non‐specific bowel gas pattern and an upper GI (UGI) the following day was also interpreted as normal, without malrotation or evidence of obstruction. An abdominal ultrasound was normal. Bilious vomiting persisted and she continued to pass normal stools. A repeat UGI was performed on day of life 16 which demonstrated multiple mildly dilated proximal jejunal loops. The patient was brought to the operating room that day and a single, fenestrated, promixal jejunal web was found. The affected area of bowel was resected and a primary anastomosis was performed without complications. Gross pathology revealed mildly dilated proximal bowel and a narrow web which did not allow passage of a probe. Histology section revealede thre minute lumens with hyperplastic jejunal mucosa separated by lamina propria but without significant fibrosis. Circular and longitudinal muscle layers were intact. Neonatal bilious emesis without abdominal distension remains a critical situation in newborn. If normal rotation is confirmed, images must be carefully reviewed to exclude dilated loops of bowel suggesting a web, or images mimicking a bird's beak consistent with volvulus. Pathology suggests that this lesion was secondary to hyperplasia of intestinal mucosa rather than ischemia. 345 16 YEAR OLD IMMUNOCOMPETENT MALE WITH HERPES SIMPLEX VIRUS PROCTITIS. Lucia, Chantal 1; Muinos, William 2; Hernandez, Erick 2; Gomara, Roberto 2; Reeves‐Garcia, Jesse 2, 1. Pediatrics, Miami Children's Hospital, Miami, FL, United States. 2. Pediatric Gastroenterology, Miami Children's Hospital, Miami, FL, United States. Introduction: Herpes simplex virus (HSV) is a common sexually transmitted disease (STD), little has been published in pediatrics on the presentation and treatment of HSV proctitis. We report a case of a 16 year old male with hemorrhoids complaining of a one year history of intermittent painful rectal bleeding. The patient had lost ten pounds in the last month. Constipation, diarrhea, and alterations in bowel movements were denied. Additionally, the patient admits to anoreceptive sexual activity with men. Physical exam was significant for external hemorrhoids. Current human immunodeficiency virus (HIV) status was negative. Syphilis, Chlamydia, and Gonorrhea were negative. A computerized tomography (CT) scan of the abdomen showed mild thickening of the sigmoid and rectum with perirectal fat lymphadenopathy. Patient underwent a colonoscopy with biopsies. Grossly there were hemorrhoids, anal fissures, and ulcers with exudates in the rectum. Rectal biopsies showed inclusions and rare multinucleations with areas of necrosis. Immunohistochemical staining was positive for herpes nuclear stain. Rapid polymerase chain reaction (PCR) detected HSV II deoxyribonucleic acid (DNA), confirming the diagnosis of HSV associated proctitis. Treatment included five days of intravenous acyclovir (5 mg/kg three times a day) followed by five days of oral acyclovir (400 mg three times a day). Topical dibucaine 1% ointment was prescribed for pain. Resolution of symptoms occurred after completion of treatment. Conclusion: Although HSV proctitis is uncommon in the pediatric population, it is an important differential for rectal bleeding, especially in sexually active adolescents having anoreceptive intercourse. 346 POLYPOID MASS IN THE ASCENDING COLON; A RARE PRESENTATION OF AN INFLAMMATORY MYOFIBROBLASTIC TUM. Legg, Arthur 1; Tuchman, David 1; Walia, Ritu 1; Murthy, Kalpana 1; Dutta, Deepa 3; Williams, Holly 2; Wiley, Joseph4, 1. Pediatric Gastroenterology, The Herman and Walter Samuelson Children's hospital at Sinai, Baltimore, MD, United States. 2. Surgery, The Herman and Walter Samuelson Children's hospital at Sinai, Baltimore, MD, United States. 3. Pathology, The Herman and Walter Samuelson Children's hospital at Sinai, Baltimore, MD, United States. 4. Hematology/ Oncology, Then Herma and Walter Samuelson Children's hospital at Sinai, Baltimore, MD, United States. Inflammatory myofibroblastic tumors in the pediatric population have been rarely described. Common sites of presentation include lung, mesentery, liver and spleen; intestinal presentations are rare and the etiology remains obscure. We report the case of a 10‐year old female patient presenting with a three‐ week history of intermittent colicky abdominal pain, diarrhea with blood, weight loss, and anemia. Laboratory tests were significant for a normocytic anemia, normal BMP and ESR. A colonoscopy revealed a large obstructing 5.0 x 5.0 x 3.0‐cm, polyploid mass in the ascending colon. Resection of the mass with a right hemicolectomy was performed. Pathologic examination of the mass revealed spindle cell proliferation, ossification and focal necrosis involving the mesentery and the mucosa with ulceration and granulation tissue. Tumor markers, desmin, S100, synaptophysin, chromogranin, CD34 and ALK1 were negative.A diagnosis of inflammatory myofibroblastic tumor .was made The patient's symptoms resolved following surgery. Conclusion: The differential for a polypoid mass encountered during endoscopy should include inflammatory myofibroblastic tumors.Diagnosis is confirmed by histology.The majority of such tumors behave indolently and are adequately managed by surgical resection. A subset, however, may behave aggressively and thus close follow‐up to assess for recurrence of the lesion or possible metastasis is indicated. 347 ABDOMINAL PAIN: KEEP GI MALIGNANCY IN YOUR DIFFERENTIAL. Foglio, E. J.1; D'Cruz, C. 1; Sunaryo, F. 1, 1. Newark Beth Israel Medical Center, Newark, NJ, United States. A 17 y/o male presented with 4 mo history of epigastric pain, nausea, vomiting and 'voluntary' 80 lbs weight loss over the past year. There was no history of diarrhea, constipation or rectal bleeding. Family history was negative for any GI disorders. On exam his weight was 107 kg and height was 180 cm. There was pallor and epigastric tenderness. Rectal exam was unremarkable. Lab work showed Hgb 10.5, MCV 73, WBC 6600, platelets 509,000, and C‐RP 61.8. The serum transaminases, total protein and albumin were normal. EGD was done and showed nodularity in the antrum without any ulcer nor abnormal masses seen. The antral biopsy was positive for H pylori. Colonoscopy was also done and revealed an inflammatory mass with ulcerations and stricture in the proximal sigmoid. The colonoscope was unable to pass due to the narrowed sigmoid colon. Biopsy taken from the sigmoid showed no dysplasia or granuloma. Additional lab work showed an elevated CEA of 37.2. MRI was then done and showed a segmental concentric wall thickening and enhancement at the junction between sigmoid and descending colon with ascites and small bowel adhesions. Cytology done from paracentesis was negative for malignant cells. Exploratory laparotomy revealed diffuse peritoneal carcinomatosis. No bowel resection was done. Biopsies of the omentum and peritoneum revealed poorly differentiated adenocarcinoma with signet ring cells with mucinous feature. The primary site of the tumor was not determined as the immunohistochemistry study was inconclusive. Patient died 5 months after the initial presentation. GI malignancy is infrequent in children. The diagnosis is often delayed because of nonspecific clinical manifestations. Inflammatory bowel disease was initially suspected in this case. Due to the history of significant weight loss and sigmoid stricture, CEA was done which led to further investigations of neoplastic process. It is imperative to keep such an unusual diagnosis in the differential diagnosis of a seemingly common complaint in children. With this, there may be a better chance to catch these cases early and therefore lead to better management and prognosis. 348 MESENTERIC LYMPHANGIOMA: A RARE CAUSE OF VOLVULUS. Ahmad, Fareed 1, 1. Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Riley Hospital for Children, Indianapolis, IN, United States. A three year‐ old‐ Caucasian toddler was seen in the emergency room due to a two‐ day history of intermittent abdominal pain and vomiting. Vomiting was initially non bloody nor bilious. On physical exam, he was irritable and moderately dehydrated. His abdomen was tender in the right lower quadrant. Screening labs were significant for ESR of 30, and ketones in the urine. Abdomen US showed no evidence of intussusception, but a fecolith in the appendix. Abdomen CT scan showed a large fluid density mass arising from the intestinal mesentery, which measured 13.7 cm x 13.0 cm x 6.8 cm. This mass contained numerous thin internal septae as well as two coarse calcification. Swirling of the mesenteric root and the superior mesenteric vessels within the right hemiabdomen was noted as well, suggestive of mid gut volvulus.e Th patient developed bilious vomiting after obtaining the CT scan, so he was taking to the operative room immediately. Exploratory laparotomy showed mesenteric cyst, about 13 cm in the largest diameter, originating from the distal ileum; Volvulus, with a 270‐ degree rotation of the bowel loops; and an appendicolith was palpated in the appendix. No ischemia was seen. The mesenteric cyst was resected, and appendectomy was performed. Pathological examination of the of the obtained specimens revealed mesenteric lymphangioma, and a normal appendix. The post‐ operative course was without complications, and the patient was sent home shortly after 5 days. He is doing well now 18 months after his surgery. 349 FECAL BACTERIOTHERAPY AS TREATMENT FOR RECURRENT CLOSTRIDIUM DIFFICILE INFECTION IN A PEDIATRIC SOLID ORGAN TRANSPLANT PATIENT. Griffin, Jennifer 1, 2; McKenzie, Leanna 1, 2; Louie, Tom J3, 2, 1. Alberta Children's Hospital, Calgary, AB, Canada. 2. University of Calgary, Calgary, AB, Canada. 3. Foothills Medical Centre, Calgary, AB, Canada. Recurrent Clostridium difficile infection (CDI) is an emerging problem in the pediatric population. In particular, solid organ transplant patients are at a higher risk of developing recurrent CDI. Successful treatment of recurrent CDI can be challenging. Fecal bacteriotherapy is emerging as an effective therapy for adults with recurrent CDI, however few studies report the use of this therapy in the pediatric population. We present the first case of a pediatric solid organ transplant patient treated for recurrent CDI with fecal bacteriotherapy. This 6 year old boy had a cardiac transplant in the first year of life and was maintained on tacrolimus and mycophenolate mofetil. He presented with bloody diarrhea which was confirmed CDI by stool PCR. He initially responded to metronidazole, but relapsed 4 times despite treatment with further metronidazole, vancomycin, nitazoxamide and probiotics. After consideration of risks and benefits, fecal bacteriotherapy was instituted. The patient and the donor, his mother, were screened for HIV, EBV, CMV, HBV, HCV, and enteric infections. He remained on Vancomycin 125 mg po TID for 2 weeks prior to the procedure. A protocol described by Dr. TJ Louie was used. The day prior to the procedure, vancomycin was discontinued. The patient was given one dose of pico‐salax to cleanse the colon. The donor supplied a fresh stool sample which was emulsified using pre‐reduced PBS and filtered through a wire mesh. A volume of 220 mls of the slurry was introduced into the rectum using a pediatric enema bag and rectal tube. Instillation time was 15 minutes and the dwell time was 90 minutes. The patient suffered no adverse effects other than mild cramping. The patient has recovered from recurrent CDI following fecal bacteriotherapy. tHe has no required further antibiotics for CDI and PCR testing of the stool for CDI is negative. In this immunosuppressed patient, fecal bacteriotherapy was well tolerated and an effective treatment for recurrent CDI. 350 12YO WITH RESPIRATORY DISTRESS AND CHILAIDITI’S SYNDROME. Caicedo, Luis 1; Colombani, Paul 2; Karnsakul , Wikrom 1, 1. Pediatric Gastroenterology, Johns Hopkins Children's Center, Baltimore, MD, United States. 2. Surgery, Johns Hopkins Children's Center, Baltimore, MD, United States. Chilaiditi’s syndrome is a very infrequent disorder exceptionally found in children characterized by the Hepatodiaphragmatic interposition of the bowel. It can be describe as a radiological sign (Chilaiditi’s sign) or when associated with symptoms as part of the Chilaiditi’s syndrome. The Chilaiditi’s sign can be confused radiologically with critical entities such as pneumoperitoneum and subdiafragmatic abscess. Most of the time it is managed conservatively but few cases required surgical intervention. Here we report a case of a 9 yo male who was admitted to the PICU secondary to abdominal pain and severe respiratory distress. Initial blood work (cbc,cmp,crp) all within normal limits. Abdominal radiograph showed constipation and transverse colon interpose between the liver and the diaphragm. His symptoms did not improve after clean out protocol. The patient underwent laparoscopic colopexy and peritoneal abrasion of the diaphragm and liver. During this intervention it was found a quite redundant transverse colon and there was a relatively small right lobe of the liver with a big gap between the liver and the anterior chest wall and diaphragm. Respiratory distress completely disappeared. The patient describe here is to the best of our knowledge upon reviewing the available literature the 17th pediatric case published with Chilaiditis syndrome and 7th case with surgical treatment with good outcome. Our case illustrates that 1) Chilaiditi’s syndrome is a rare condition especially among the pediatric population. 2) It should be suspected when air is found under the diaphragm in order to avoid unnecessary surgeries. 3) All gastroenterologists should keep this diagnosis in mind when managing patient’s whit chronic liver disease and cirrhosis to avoid intestinal injury during percutaneous transhepatic procedures. 4) In the pediatric population both conservative and surgical approaches had satisfactory outcomes. 351 UNUSUAL PRESENTATION OF DUODENAL STENOSIS IN TRISOMY 21. Abbas, Mazen I1; Min, Steve 1; Goldman, Matthew 1; Safford, Shawn 1; Colombo, Jennifer 1, 1. Pediatrics, Walter Reed AMC, Washington, DC, United States. Duodenal atresia and stenosis have been well described as a cause of intestinal obstruction in these Trisomy 21 patients. Duodenal atresia is usually diagnosed prenatally or within the first few weeks of life. However, duodenal stenosis may be diagnosed later in life because of common symptoms to other disorders such as esophageal reflux, milk‐protein allergy or infant colic. We present two cases of duodenal stenosis in Trisomy 21. A 15 month old female with Trisomy 21 presented with a 3 month history of burping, halitosis and nocturnal fussiness. She had no history of emesis or significant esophageal reflux. She was eating normally and had been tracking appropriately on the Trisomy 21 growth chart. She had no other significant medical, social or family history and her exam was unremarkable. Another case involved a 4.5 year old male with Trisomy 21 who presented with increasing episodes of mild regurgitation and “wet burps” after eating. He had no vomiting, abdominal pain, or weight loss. He had a previous history of mild esophageal reflux which improved with the use of omeprazole; although during the past year he had been doing well without the need of any antacid. His exam was unremarkable. Both patients had an upper gastrointestinal series that noted an abnormally dilated duodenal bulb with significant narrowing of the duodenum. The second case also had an abdominal CT confirming the diagnosis. Surgical correction for duodenal stenosis with an open duodeno‐ duodenostomy was conducted successfully in both patients. Both patients had resolution of symptoms at follow‐up visits. Current Trisomy 21 health supervision guidelines include assessing for gastrointestinal anomalies prenatally and evaluating for duodenal atresia in the 1st month of life. However, these guidelines do not address duodenal stenosis. We propose that providers caring for patients with Trisomy 21 should consider duodenal stenosis as a possible etiology for gastrointestinal complaints regardless of age and should pursue appropriate diagnostic imaging. 352 PERSISTENT VOMITING: AN UNUSUAL ANATOMIC VARIANT. Walji‐Virani, Shabina 1, 2; Russo, Michael A1, 2, 1. Gastroenterology, Childrens Medical Center, Dallas, Dallas, TX, United States. 2. Pediatric Gastroenterology, University of Texas Southwestern, Dallas, TX, United States. A 15 year old male presented with sternal and epigastric abdominal pain that started in 2006. He was treated for gastro‐esophageal reflux (GERD)since his upper gastrointestinal series (UGI) was negative. He had some improvement on proton pump inhibitors and dietary modifications. However, emesis continued particularly when in a supine position. Laboratory testing and endoscopy pathology were unrevealing. Head magnetic resonance imaging was normal. Ultimately, computed tomography revealed “a cluster of proximal bowel loops situated between the stomach and pancreas”. Diagnostic laparoscopy and repair of the left duodenal hernia resulted in symptom resolution. An internal hernia is the protrusion of a viscus through a normal or abnormal aperture within the peritoneal cavity. Paraduodenal hernias, albeit rare, are the most common type of internal hernias. Clinical presentation can range from being asymptomatic to severe discomfort. Associated symptoms can include nausea and vomiting after meals and change in position may alter the intensity of their pain. The right paraduodenal hernia originates embryologically from an incomplete or defective rotation of the prearterial limb of the developing intestine, and the left originates from the incomplete fusion of the mesentery of the duodenum and the posterior abdominal wall. Paraduodenal hernias are 3 times more common in men and usually present between the 4th to 6th decades of life. Rare and difficult to diagnose in children, timely radiological testing and surgical treatment will alleviate the associated morbidity and mortality of this condition. Differential consideration of this entity should be included in the diagnostic evaluation for GERD. 353 A 16‐YEAR OLD MALE WITH A DUODENAL DIEULAFOY LESION. Fish, Samantha 1; Badalyan, Vahe 1; Lee, Peter 1, 1. Pediatrics, Inova Fairfax Hospital for Children, Falls Church, VA, United States. A 16 year old male presented with a 2‐day history of melanotic stools and bright red blood per rectum. For the past 2 weeks, he had intermittent abdominal pain. Review of systems was negative for fever, vomiting, weight loss, rashes, joint pains, or intake of NSAIDs. He denied alcohol intake or tobacco use. His past medical history was unremarkable. There was no family history of inflammatory bowel disease or ulcers. In the ER, abdominal CT was negative for colitis or obstruction. CBC showed low hemoglobin (9.9 g/dL) and hematocrit (27%), and normal white count (7900/mm3). He was discharged home, but returned to the ER the next day due to continuing symptoms. Repeat hemoglobin and hematocrit were significantly decreased (7.1 g/dL and 19.5%). On physical exam he was alert but fatigued. He was tachycardic, and was complaining of dizziness when standing up. His lips and oral mucosa were pale. Abdomen was soft with some epigastric tenderness, but no guarding, or rebound pain. No organomegaly was detected on palpation. His extremities were warm. The patient was transfused a total of 6 units of packed red blood cells for hemodynamic stabilization and procedures. Esophagogastroduedonoscopy (EGD) revealed old blood (dark, clotted) in the stomach but no source of bleeding. The patient continued to have melanotic and a new episode of hematemesis. Second EGD showed bright red blood in the stomach and duodenum. A Dieulafoy lesion was detected in the duodenum. It was cauterized,t bu continued to bleed. A hemoclip was placed, but got dislodged. Epinephrine was then injected at the base of the lesion, and it was recauterized. Dieulafoy lesion is a submucosal artery that aberrantly protrudes through a small defect in the mucosa, resulting in significant bleeding. Most Dieulafoy lesions are found ein th stomach and usually present with upper GI bleeding. Our patient, presenting with lower GI bleeding, made for an unusual presentation of this already rare condition, requiring a high index of suspicion and quick and aggressive measures to stop his bleeding. HEPATOBILIARY/TRANSPLANT 367 A CASE OF PATENT DUCTUS VENOSUS AND SUBSEQUENT HEPATIC MASS. Wyatt, Allyson Nelms1; Rakheja, Dinesh 2; Ziadie, Mandolin 2; Megison, Stephen 2; Sathe, Meghana 2, 1. Childrens Medical Center Dallas, Dallas, TX, United States. 2. University of Texas Southwestern Medical Center, Dallas, TX, United States. A full‐term, male infant with Down syndrome was born to a twenty‐four year old primigravida mother. He was born with cardiac anomalies (large atrial septal defect and patent ductus arteriosus) and an abdominal vascular malformation that had been identified on prenatal ultrasound. Post‐natal investigation by MRI and ultrasound showed a persistent patent ductus venosus bridging from the main portal vein to the intrahepatic inferior vena cava. Due to intermittently elevated levels of ammonia during the neonatal period m(ranging fro 26‐114) and risk of hyperammonemia secondary to the presence of this congenital portasystemic shunt, the patient was placed on lactulose and followed closely. During the second year of life, he had persistently elevated liver enzymes and was evaluated for other causes of liver disease including hepatitis A, B, and C, alpha‐1‐antitrypsin deficiency and Wilson’s disease. These tests were all negative. On routine follow‐up of his patent ductus venosus at four years of age, the patient was noted to have a focal mass in the left lobe of the liver on ultrasound, measuring 4.9 cm x 4.0 cm x 4.0 cm. Subsequent CT scan revealed two well‐defined, homogenous and predominantly exophytic masses within the liver. Alpha‐fetoprotein was within normal range at 5.7. Needle biopsy could not differentiate between focal nodular hyperplasia and adenoma. Due to the concern for an adenoma, complete resection of the masses was preformed. Pathologic evaluation determined that the masses best fit the category of focal nodular hyperplasia, although accompanied by some unusual features. It is speculated that the vascular malformation resulted in the formation of focal nodular hyperplasia. There are multiple reports of focal nodular hyperplasia in the presence of hereditary hemorrhagic telangiectasia. There is, however, only one other report of a patient with focal nodular hyperplasia and a patent ductus venosus. 368 EARLY PRESENTATION OF LOW PHOSPHOLIPID‐ASSOCIATED CHOLESTASIS SYNDROME. Bitar, Anas 1; Grunow, John 1; Steele, Marilyn 1; Sferra, Thomas J.1, 1. University of Oklahoma HSC, Oklahoma City, OK, United States. The spectrum of phenotypes associated with ABCB4 mutations is broad and encompasses multiple diseases including low phospholipid‐associated cholelithiasis (LPAC). LPAC is characterized by cholangiopathy related to cholestasis and cholesterol cholelithiasis resulting from low phospholipid bile with impaired cholesterol solubility.AC LP is associated with recurrent cholelithiasis, cholecystitis, cholangitis, and pancreatitis. It should be suspected with 2 or more of the following: symptomatic cholelithiasis at <40 years of age, intrahepatic sludge, microlithiasis, and cholesterol cholelithiasis in a first‐degree relative. Case: A 15‐year‐old boy presented with epigastric pain, nausea, and vomiting. Laboratory and radiologic findings were consistent with cholelithiasis, choledocholithiasis, and obstructive pancreatitis. Two days after admission, he underwent ERCP. Numerous small cholesterol stones were found in the common bile duct (CBD) and removed. A sphincterotomy was performed and a stent placed. A cholecystectomy was performed. Three weeks after the cholecystectomy, an MRCP revealed extrahepatic biliary dilatation with choledocholithiasis. A second ERCP demonstrated a dilated CBD with multiple stones. Mutational analysis of the ABCB4 gene revealed a heterozygous novel gene variant, c.1685C >T (p.A562V), predicted to be deleterious by the SIFT and PolyPhen algorithms. He was placed on ursodeoxycholic acid (UCDA) at 10 mg/Kg/day and increased to 15 mg/Kg/day due to abdominal pain. He has since remained asymptomatic without evidence of cholelithiasis for 5 months. Discussion: This is the youngest reported patient to be diagnosed with LPAC. Although this syndrome is an infrequent cause of cholelithiasis, it should be suspected in adolescents with symptomatic cholelithiasis and other features of the disorder. Diagnosis can be confirmed by ABCB4 genotyping. The disease course is progressive with recurrence of intra‐ and extrahepatic cholelithiasis. Cholecystectomy is indicated for symptomatic gallstones. UDCA appears to prevent recurrences, though higher doses (15‐20 mg/kg/day) than usual might be required. 369 LIVER PARENCHYMAL INJURY IN NEONATE WITH AN UMBILICAL VENOUS CATHETER (UVC). Chystsiakova, Anastasiya 1; Fofah, Onajovwe 1; Kothari, Neha 2; Monteiro, Iona M1, 1. Pediatrics, UMDNJ‐ New Jersey Medical School, Newark, NJ, United States. 2. Radiology, UMDNJ‐New Jersey Medical School, Newark, NJ, United States. 27 week gestation male weighing 890g had an UVC placed on day of life (DOL) 0. On DOL 2, total parenteral nutrition (TPN) was started through the UVC. On DOL 7, an echocardiogram noted a right upper quadrant cystic structure. Abdominal Ultrasound (US) revealed thick‐walled cystic lesion in the right hepatic lobe with mild internal echoes with no biliary involvement nor ascites. On DOL 9, the neonate developed abdominal distension. Repeat US showed persistence of the right hepatic lobe cystic lesion with echogenic septations within the cyst, as well as echogenic ascites around the liver and in the pelvis. Surgical drainage was performed and fluid aspirated from liver parenchyma and abdomen was consistent with TPN. The UVC was removed, and his condition improved rapidly. Repeat US showed interval resolution of ascites as well as hepatic cyst with residual calcification. UVC placement is a common bedside procedure in the neonatal intensive care unit (1). A complication associated with malposition of UVC is intraperitoneal or intrahepatic extravasation of TPN (2). Less than 10 reports have been published about hepatic parenchymal laceration secondary to an UVC. Common findings include abdominal distension, hepatomegaly, hepatic lesions, ascites and deterioration of vital signs (3). Our patient was asymptomatic and the liver lesion was found incidentally. He developed abdominal distension 2 days later. It is very important to suspect liver parenchymal injury in any neonate with an UVC and hepatic lesions in order ton begi management before clinical signs develop. Early diagnosis is key to preventing serious complications. 1. Nash P. Umbilical catheters, placement and complication management. J Infus Nurs.2006;29(6):346‐52 2. Coley BD et al. Neonatal total parenteral nutrition ascites from liver erosion by umbilical vein catheters. Pediatr Radiol 1998; 28:923‐7 3 Yigiter M et al. Hepatic laceration because of malpositioning of the umbilical vein catheter: case report and literature review. J. of Pediatric Surg. 2008;43, E39‐E41 370 AUXILIARY LIVER TRANSPLANTATION IN A PATIENT WITH AUTOIMMUNE HEPATITIS. Arias, Patricio 1; Reeves‐Garcia, Jesse 1; Muinos, William 1; Gomara, Roberto 1; Mercedes Martinez, Mercedes 2; Kato, Tomoaki 2; Lobritto, Steven 2; Hernandez, Erick 1, 1. Pediatric Gastroenterology, Miami Children's hospital, Miami, FL, United States. 2. Pediatric gastroenterology/hepatology, Columbia University, New York, NY, United States. A six year old male presented at 8 months of age with fulminant liver failure. Viral hepatitis was suspected as the cause of his liver failure with a liver biopsy showing significant necrosis of his liver. He received an auxiliary partial orthotropic liver transplant on 03/2006. After liver transplantation he developed episodes of acute cellular rejection that resolved with appropriate immunosuppression. Regeneration of his native liver was follow‐up by liver biopsy, imaging, and nuclear medicine. His immunosuppression was slowly tapered and discontinue three years after liver transplantation. After the withdrawal of his immunosuppression, he developed an abscess of his transplanted liver with a fistulous track formation at the level of his Roux‐en‐Y anastomosis, requiring drainage and resection of his transplanted liver. He was discharged home without any immunosuppression. He remained with normal liver enzymes, normal liver ultrasound and doppler for one year after stopping immunosuppression. He then developed elevation of his liver enzymes with a mild flow reversal of his native liver. His smooth muscle antibody was mildly increased with a liver biopsy indicating the presence of autoimmune hepatitis. Immunosuppression was started with prednisone and 6 mercaptupurine achieving complete remission of his autoimmune hepatitis. Conclusion: Auxiliary liver transplantation and immunosuppression withdrawal is feasible in pediatric patients with autoimmune hepatitis. However, a close follow‐up and long term immunosuppression will probably be required on most of these patients. 371 A CASE OF LIVER TRANSPLANT DUE TO ATYPICAL INFLAMMATORY MYOFIBROBLASTIC TUMOR. Ahmad, Fareed 1; Subbarao, Girish 1, 1. Riley Hospital for Children, Indianapolis, IN, United States. A five‐ year old, previously healthy, caucasian girl was referred with a 10‐day history of Jaundice. This was associated with dark colored urine, clay colored stool, itching, and fatigue. Physical exam was remarkable for icteric sclera, yellow skin, and a firm mass in the epigastric area. Liver enzymes were: ALT 48,AST 70, Total bilirubin 5.8, and direct bilirubin of 4.1. Alkaline phosphatase level was 750. The patient had negative hepatitis profile , normal AFP level, and normal uric acid level. Local abdominal ultrasound showed contracted gall bladder, with a heterogeneous hypo‐echoic mass in left lobe of liver. MRCP showed a 3.5 cm x 2.5 hypervascular left lobe infiltrating mass lesion, with central necrosis. The mass encased the common hepatic duct and causes marked intrahepatic biliary ductal dilation. Transcutaneous liver biopsy showed spindle shaped cells arranged in fascicles, which were present in a background of inflammatory infiltrate of lymphocytes, plasma cells and eosinophils. Many atypical, large 'rhabdoid' cells with abundant eosinophilic cytoplasm and prominent nucleoli were present. Nuclear pleomorphism including rare bizzare nuclei was seen. Mitotic figures including atypical forms were present. The tumor cells marked immunohistochemically for Anaplastic Lymphoma Kinase ( ALK1), smooth muscle actin and vimentin. The morphological features and immunohistochemical profile was consistent with inflammatory myofibroblastic tumor. The presence of nuclear pleomorphism and atypical mitotic figures renders it an atypical variant. Transcutaneous biliary drain was inserted by interventional radiology to drain the bile, and to decompress the dilated ducts. Surgical teamd deeme the tumor unrespectable. Due to the obstructive effect of the tumor, and its potential for local recurrence after resection, it was decided to proceed with Liver transplantation. The patient received a full orthotopic liver transplant. The operative and post‐operative course was uneventful. After nine months of liver transplant, the patient is doing very well, with normal liver enzymes and prograf level between 4‐5. 372 HYPERAMMONEMIA AFTER CHEMOTHERAPY FOR HEPATOCELLULAR CARCINOMA (HCC). Burgis, Jennifer Clare1; Enns, Gregory 1; Hurwitz, Melissa 1, 1. Pediatric GI, Stanford University, Palo Alto, CA, United States. An 8 y/o boy presented with a 4 month history of abdominal pain, non‐bilious emesis and poor weight gain. CT showed massive hepatomegaly with multi‐centric tumor without vascular invasion and innumerable pulmonary nodules. Initial lab studies revealed: elevated AST, ALT and GGT, low albumin, and INR of 1.3. AFP and βhcg were normal. Viral hepatitis serologies were negative. Liver biopsy was consistent with HCC. He received chemotherapy (doxorubicin and cisplatin) and developed irritability with visual hallucinations within 24hrs. Initial NH3 was 134 umol/L. Lactulose was started and then rifaximin without improvement. Workup revealed elevated urine orotic acid (2117 mmole/mole cr) and repeat ammonia of 170 umol/L, suggestive of ornithine transcarbamylase (OTC) deficiency. He received an ammonul loading dose (5.5gm/m2) followed by maintenance infusion (5.5gm/m2 over 24hrs). Citrulline 200mg/kg/day and sodium phenylbutyrate 10gram/m2/day were added. He received D10NS IV fluids and intralipid 2 gm/kg/day to avoid catabolism. Within 12 hrs, his NH3 declined to 71 umol/L and his mental status improved. Ammonul was discontinued after 48 hrs. Serum citrulline was elevated (273 nmol/L), not consistent with OTC deficiency, and supplementation was discontinued after 6 days. NH3 ranged 54‐169 umol/L with no repeat agitation. He received NG feeds of Pediasure and Prophee but advanced to regular diet at discharge. Sodium phenylbutyrate was continued. Second cycle of chemotherapy plus Sorafenib was given 3 weeks later. Initial NH3 was 85 umol/L and urine orotic acid was 555.9 mmole/mole cr. Serum glutamine was elevated with normal citrulline, consistent with hyperammonemia. He tolerated the second cycle without mental status changes and NH3 ranged from 64‐107 umol/L. After two months, OTC gene sequencing results revealed no mutations identified. There are only 2 case reports of hyperammonemia mimicking OTC deficiency after chemotherapy for HCC.(1,2) Hypotheses include down regulation of urea cycle enzymes in liver tumor cells.(3) 1 Chan et al. Southern Med J 2008 2 Winter et al. JPGN 1997 3 Liu et al. Am J Pathol 2011 373 NOVEL MUTATION IN THE LYSOSOMAL ACID LIPASE GENE ASSOCIATED WITH CHOLESTERYL ESTER STORAGE DISEASE. Jimenez, Jennifer 1; Conard, Katrina 1; Furuya, Katryn N1, 1. Pediatric gastroenterology , Alfred I. Dupont Hospital for Children, Wilmington, DE, United States. 5 yr old girl developed fever and vomiting; bloodwork showed AST 80 U/L, ALT 48 U/L, Alk Phos 163 U/L, albumin 3.8 g/dL. Her ultrasound showed hepatomegaly.The fever and vomiting resolved, but due to persistent hepatomegalyd an transaminitis, she was referred to our hospital.Physical exam revealed only a massively enlarged firm liver.Initial infectious etiologies were negative.Metabolic disease was suspected and screening tests were notable for markedly elevated cholesterol and triglycerides(TG). Liver biopsy revealed diffuse, microvesicular steatosis. Portal tracts were expanded by foamy macrophages. There was mild portal fibrosis. On EM, the cytoplasm contained lipid droplets and cholesterol crystals. These changes were in keeping with cholesterol ester storage disease (CESD) and a markedly low lysosomal acid lipase (LAL) confirmed this diagnosis. Subsequent sequence analysis identified two heterozygous mutations in the lysosomal acid lipase gene (LIPA). The first being a novel mutation and was a four‐nucleotide deletion, c.57_60delTGAG in exon 2. The second mutation was a c.894G>A change in exon 8. Discussion: CESD is an autosomal recessive, chronic liver disease caused by LAL deficiency. Its cognate gene is located on chromosome 10q23.3‐q23.3. LAL hydrolyzes cholesteryl esters (CEs) and TG’s that are delivered to the lysosomes by receptor‐mediated endocytosis and deficiency results in acumulation of both CE’s and TG’s in hepatocytes. Complete absence of LAL activity results in Wolman disease, whereas CESD is due to mutations that retain some enzyme activity. The novel mutation found in our patient is a four‐nucleotide deletion, c.57_60delTGAG in exon 2 of the LIPA gene. This change has not been previously reported but it is predicted to result in a premature translation stop downstream of the deletion (p.E20fs). Therefore, it is felt to be a disease‐causing mutation. The second is a previously noted disease causing mutation, c.894G>A change in exon 8 of the LIPA gene, which results in altered mRNA splicing and exon 8 skipping. 374 SYMPTOMATIC FOCAL NODULAR HYPERPLASIA IN A 10YO GIRL. RADANO, Marcella Calhoun2; Goldstein, Allan M1; Russell, George H2, 1. Pediatric Surgery, Massachusetts General Hospital, Boston, MA, United States. 2. Pediatric Gastroenterology, Massachusetts General Hospital, Boston, MA, United States. Focal Nodular Hyperplasia (FNH) is a benign liver tumor of unknown etiology. It is a relatively rare entity in pediatrics, especially in the pre‐pubertal age group. FNH is often incidentally found and asymptomatic. We report a case of a 10 year‐old girl with abdominal pain, persistent, intermittent, forceful nonbilious emesis and 'pencil thin' stools. She had a history of reflux and frequent sinus infections. Her symptoms regularly disrupted school and dance class. She was treated with PPI therapy for reflux and laxatives for constipation. Work‐up included normal celiac screen, liver function tests, amylase/lipase, abdominal radiography, upper GI series, anorectal manometry, upper endoscopy and colonoscopy. An abdominal ultrasound was initially reported as normal. HIDA scan showed a gallbladder ejection fraction of 19%. Based on ongoing symptoms and HIDA scan results, she underwent laparoscopic cholecystectomy and at the time of surgery was found to have a large, multi‐ lobulated, hypervascular hepatic lesion in the left lobe. Biopsy results suggested FNH. Cholecystectomy was not performed. Given the unexpected finding, further testing was performed. The patient had a normal chest CT. Abdominal CT was remarkable for a well‐circumscribed, arterially‐enhancing liver mass with a central scar. The mass displaced the gallbladder and the antrum of the stomach. Ca 19‐9 and HCG were normal. The patient underwent partial left hepatic lobectomy for resection of the mass, which measured. 8.6 x 7.0 x 5.8 cm. Final pathology was consistent with FNH. On follow‐up, the patient’s presenting symptoms completely resolved. She had no further episodes of emesis and discontinued PPI therapy. Her abdominal pain resolved. Stool caliber normalized. Foot swelling noted previously resolved and her shoe size decreased, likely from relief of IVC compression from the mass. Five months post‐resection her sinus infections decreased by half, presumably from resolution of the chronic emesis. She attends school regularly and is active in dance. 375 ALPER’S SYNDROME: AN UNUSUAL ETIOLOGY OF A COMMON PRESENTATION TO THE PEDIATRIC GASTROENTEROLOGIST. Mangalat, Nisha 1; Tatevian, Nina 2; Rhoads, J. Marc 1, 1. Pediatrics, University of Texas Health Science Center at Houston, Houston, TX, United States. 2. Pathology, University of Texas Health Science Center at Houston, Houston, TX, United States. CASE PRESENTATION: An 11 month old evaluated for a seizure that progressed to status epilepticus. She developed respiratory failure and was transferred to our pediatric intensive care unit. The patient’s past medical history was significant for FTT. Review of the growth chart indicated a decrease in weight percentiles from 25 % at 6 months to <3% by 11 months. Workup initiated by the primary care provider few weeks prior revealed elevated transaminases (AST 109, ALT 66). On review of systems, infant had episodes of fine “shaking” of her upper extremities that impaired her ability to bring food to the mouth. Both hepatomegaly and splenomegaly were present on examination. Initial workup demonstrated normal CT head. EEG suggested diffuse encephalopathy, but otherwise was non‐specific. Seizures were treated with valproic acid (VPA), phenytoin, and other anti‐convulsants. Workup for infection was negative. Abnormalities in liver function persisted: ALT 73, AST 100, total/conjugated bilirubin 1.5/1.1, and prothrombin time 17.5. Seizures remained refractory to anti‐epileptics. MRI of the brain revealed hyperintensities in the occipital regions and bilateral thalami. VPA was discontinued, but by hospital day 29, pt’s clinical status progressed to hepatic failure. Given the combination of weakness, refractory seizures, and hepatic failure, mitochondrial disease was suspected. Gene testing confirmed mutations in POLG on both copies of chromosome 15, consistent with Alper’s syndrome. CONCLUSION: We present this case to highlight the fact that a very uncommon pediatric disorder may present as a case of gastroesophageal reflux and failure to thrive. The presence of abnormal liver enzymes with failure to thrive, especially in the setting of seizures, should prompt a more thorough neurodevelopment workup and an evaluation for the presence of a mitochondrial disorder. 376 HEPATITIS C VIRUS INDUCED LIVER INJURY EXACERBATED BY ALPHA 1 ANTITRYPSIN HETEROZYGOSITY. Viswanathan, Preeti 1; Kogan‐Liberman, Debora 1; Schwartz, Daniel Schwartz 2; H Pan, Debra 1, 1. Pediatric Gastroenterology, Hepatology and Nutrition, Childrens Hospital at Montefiore, Bronx, NY, United States. 2. Pathology, Childrens Hospital at Montefiore, Bronx, NY, United States. Alpha 1 antitrypsin deficiency is a well known cause of significant liver disease in 10‐15% of the homozygous PIZZ population and is the most frequent genetic cause of liver disease in children.Despite the relatively common overall prevalence of the heterozygous phenotype(10%),there is no evidence that heterozygosity leads to chronic liver disease and its role in worsening liver injury sustained from other causes remains to be characterized. A Hispanic female with vertically transmitted chronic hepatitis C,genotype 1A presented at age 8 with mild elevation of transaminases.Liver histology showed minimal hepatitis without fibrosis that progressed rapidly to moderate active hepatitis and grade 4‐5/6 fibrosis by age 11.She failed treatment with pegylated interferon and ribavirin. At age 13,she had markedly elevated serum alphafetoprotein and an abdominal MRI discovered a cirrhotic liver with a focal lesion suspicious for hepatocellular carcinoma (HCC).CT guided radiofrequency ablation of the lesion with liver biopsy revealed a probable well differentiated HCC.This biopsy also revealed PAS positive diastase resistant globules in adjacent hepatocytes.She was found to have the heterozygous phenotype(PIMS). Liver injury from chronic hepatitis C infection in children usually progresses over many years.Our patient progressed from mild hepatitis to cirrhosis within 3 years and HCC within 5 years.PAS positive globules seen on her last biopsy were previously absent.Our case suggests that hepatitis C and alpha‐1 antitrypsin heterozygosity appeared to have a synergistic effect, with the hepatitis C induced liver injury unmasking alpha 1 antitrypsin deficiency, which in turn exacerbated the hepatitis C course by further increasing liver susceptibility to injury.Our case shows that children with known etiology of chronic liver diseases such as viral hepatitis should be screened for Alpha 1 antitrypsin heterozygosity,which could adversely affect the primary disease course and outcome. 377 SEVERE INDIRECT HYPERBILIRUBINEMIA IN A NEONATE HETEROZYGOUS FOR GILBERT SYNDROME. Abell, Rebecca 1; Ozturk, Berrin 1; Chawla, Anupama 1, 1. Pediatric Gastroenterology, Hepatology, and Nutrition, Long Island Children's Hospital, Stony Brook, NY, NY, United States. A 6 day old infant presented with indirect hyperbilirubinemia that peaked at 20.1/ 0.6. His hemoglobin dropped twice during his admission, requiring transfusion each time. A work‐up for a hemolytic process and metabolic disorder was negative. Crigler‐Najjar was suspected. He was trialed on Phenobarbital for 3 days with no change in his bilirubin level. Patient was discharged home after 20 days of phototherapy with a bilirubin of 3.1/0.4. Five months later, bilirubin was 0.2/0.1. Genetic testing was positive for a heterozygous mutation for Gilbert Syndrome. UGT1A1 gene was heterozygous for mutations in alleles *28 (TA 6/7), *60, and *93, consistent with a carrier state for indirect hyperbilirubinemia. This may be associated with mild to moderate hyperbilirubinemia. The promoter TA7 repeat polymorphism when linked with these two addition alterations, results in decreased glucuronidation capacity. The *60 allele is associated with reduced transcription of UGT1A1 and reduced activity of the UGT1A1 enzyme. The relevance of this haplotype of polymorphisms to hyperbilirubinemia in the neonate has not been established. Gilbert syndrome, the most common hereditary cause of hyperbilirubinemia typically presents after puberty and is associated with mild hyperbilirubinemia, around 3 mg/dL. In the homozygous state, diminished bilirubin glucuronidation is observed but it is questionable if the same degree of enzyme inactivity can be seen in the heterozygous state. Studies have suggested that the heterozygous TA 6/7 mutation is more common than previously believed and does not significantly contribute to unexplained prolonged hyperbilirubinemia. It has been proposed that when additional mutations exist in conjunction with a heterozygous state, neonatal hyperbilirubinemia is more pronounced as evidenced in our case. Conclusion: Unexplained prolonged indirect hyperbilirubinemia should raise the suspicion of a UGT1A1 gene mutation. Gilbert’s syndrome should be ein th differential of severe indirect hyperbilirubinemia in the neonate. 378 HEPATIC VENOUS OUTFLOW OBSTRUCTION AFTER LIVER TRANSPLANTATION. Ibrahim, Samar H.1; El‐Youssef, Mounif 1; Freese, Deborah K1, 1. Pediatric Gastroenterology and Hepatology , Mayo Clinic, Rochester, MN, United States. Segmental liver transplant has contributed to an increased incidence of hepatic venous outflow obstruction (HVOO), a known cause of graft & patient loss after pediatric liver transplantation. HVOO is a challenging diagnosis with an insidious onset. We report 2 patients who developed HVOO several years after liver transplant. Our first patient is a 12 year old male with orthotopic liver transplant at the age of 5 years for alpha‐1 antitrypsin deficiency. At the age of 11, he developed fatigue, peripheral edema & diarrhea. He was noted toe hav significant hypoalbuminemia, anemia, low‐grade immune deficiency, lymphopenia & elevated stool alpha 1 antitrypsin. Urine analysis was normal. Liver enzymes were mildly elevated. Patient had an extensive unremarkable gastrointestinal, infectious & cardiological evaluation at another institution. He was diagnosed with protein loosing enteropathy of unknown etiology. 6 months later, he presented to our institution & a venogram revealed retrograde flow in the IVC due to suprahepatic caval anastomotic stricture. Balloon angioplasty was successful. On follow up 1 year latter, the patient was asymptomatic, laboratory markers had normalized & caval anastomosis was widely patent on Doppler US. The second patient is a 6 year old female with left lobe split liver transplant at the age of 2 secondary to alpha‐1 antitrypsin deficiency. At the age of 3 years, she developed ascites. A venogram showed a possible kink in the hepatic vein without a pressure gradient. The vein was dilated by balloon angioplasty & the ascites improved. She did develop portal hypertension with small esophageal varices. Doppler US revealed patent hepatic & portal vessels. She presented to our institution at the age of 5 years with hematemesis & required variceal banding. Venogram revealed occlusion of the intrahepatic cava, which was not amenable to balloon dilation. She received a second liver transplant 1 year later. The diagnosis of HVOO requires a high index of suspicion, early screening with venography may identify a subset of patients responsive to interventional therapy. 379 HEPATIC VENOUS OUTFLOW OBSTRUCTION AFTER LIVER TRANSPLANTATION CAUSING PROTEIN‐LOSING ENTEROPATHY IN AN INFANT. Hourigan, Suchitra 1; Anders, Robert A2; Schwarz, Kathleen B1; Karnsakul, Wikrom 1, 1. Pediatric Gastroenterology and Nutrition, Johns Hopkins Hospital, Baltimore, MD, United States. 2. Pathology, Johns Hopkins Hospital, Baltimore, MD, United States. An eighteen month old female status post orthotopic liver transplant for biliary atresia presented nine months after transplant with severe diarrhea and intolerance of feeds. She was found to have a protein‐ losing enteropathy (PLE) as evidenced by a low serum albumin and a persistent elevation of fecal alpha‐ 1 antitrypsin, requiring parenteral nutrition to meet her protein requirements. Investigation eventually revealed that the cause of the PLE was a stricture at the anastomosis site between the hepatic vein and inferior vena cava, supported by resolution of the PLE after venoplasty of the stricture. The patient has subsequently required several repeat venoplasties for recurrence of her symptoms correlating with recurrence of the stricture. We believe this is the youngest reported case of this very rare presentation of hepatic venous outflow obstruction. Moreover normal duplex ultrasound imaging of liver vasculature and her unusual presentation led to a delay in her diagnosis highlighting the need for an increased index of suspicion. 380 A CASE OF PORTAL BILIOPATHY DUE TO EXTRAHEPATIC PORTAL VEIN OBSTRUCTION MISDIAGNOSED AS PRIMARY SCLEROSING CHOLANGITIS. Alkhouri, Naim 1; Keilman, Ashley 1; Uko, Victor 1; Gulati, Reema 1; Radhakrishnan, Kadakkal 1, 1. Pediatric Gastroenterology, Cleveland Clinic, Cleveland, OH, United States. Extrahepatic portal vein obstruction (EHPVO) is a common cause of portal hypertension in children and is idiopathic in the majority of cases. The most common presentation is hematemesis from esophageal varices; however, biliary changes can occur and result from compression by collateral veins. An 8‐year‐ old previously healthy female presented to an outside hospital with acute hematemesis and was found to have grade 3 esophageal varices on upper endoscopy that required banding. She had splenomegaly and thrombocytopenia with a platelet count of 103 k/uL. Her initial evaluation for A1AT deficiency, Wilson’s disease, autoimmune hepatitis, and viral hepatitis was negative. An MRI of the abdomen at the outside hospital revealed biliary ductular changes suggestive of primary sclerosing cholangitis (PSC). She was referred to our institution for further management and for consideration for liver transplantation. Due to the young age of our patient, acute onset of her symptoms, and absence of any known liver disease or inflammatory bowel disease, a decision was made to pursue a liver biopsy with pressure measurements for further evaluation. Liver biopsy showed minimal portal and lobular inflammation with no fibrosis and normal hepatic architecture and bile ducts. Hepatic venogram showed patent hepatic veins and the free hepatic vein pressure gradient was 3 mmHg with no evidence of intrahepatic portal hypertension. CT angiography of the abdomen revealed the presence of venous collaterals around the porta hepatis as well as moderate sized periesophageal collaterals and vascular varices around the gallbladder wall. A diagnosis of portal biliopathy was made and the patient is being considered for transjugular portosystemic shunt insertion or Rex bypass. This case highlights that EHPVO can present with biliary changes that resemble PSC on cholangiography. Differentiating these two entities is important because most patients with portal biliopathy have preserved liver function and respond to medical treatment or shunt placement.