Clinical Vignette Abstracts NASPGHAN Annual Meeting October 10-12, 2013
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Clinical Vignette Abstracts NASPGHAN Annual Meeting October 10-12, 2013 Poster Session I Eosphagus Stomach 11 Successful Use of Biliary Ducts Balloon Dilator in Repairing Post-Surgical Esophageal Stricture in premature infant. I. Absah, Department of Pediatirc Gastroenterology and Hepatology, Mayo Clinic college of Medicien, Rochester, Minnesota, UNITED STATES. Absah, M.B. Beg, Department of Pediatirc Gastroenterology and Hepatology, SUNY Upstate Medical University, Syracuse, New York, UNITED STATES. Background: Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common type of gastrointestinal atresia with occurrence rate of I in 3,000 to 5,000 births. There are 5 major varieties of EA. The most common variant of this anomaly consists of a blind esophageal pouch with a fistula between the trachea and the distal esophagus, which occurs in 84% of the time. Treatment is by extra-pleural surgical repair of the esophageal atresia and closure of the tracheoesophageal fistula. Complications may include leakage to the mediastinum, fistula recurrence, GERD, and stricture formation at the anastomosis site. Benign stricture occurs in 40% of the cases after surgical repair. These benign post-surgical strictures cause feeding difficulties and require treatment. Esophageal dilation is 90% effective, but strictures that do not respond to dilation must be resected surgically. Case report: A 52 days old premature baby that was born at 35 weeks presented with feeding difficulty, due to benign esophageal stricture 44 days after surgical repair of EA and TEF. The luminal diameter of the stricture was ≤ 4mm, for that regular balloon esophageal dilators (smallest = 6mm) couldn’t be used. Then a 4mm biliary balloon dilator over 0.035 inch guidewire was successfully used without complications. Dilation was repeated using the biliary balloon dilators 3 times, then regular esophageal dilators were used until feeding difficulty and the stricture completely resolved. Conclusion: Biliary duct balloon dilators can be safely used to dilate narrow esophageal strictures with diameter of 6mm or less. This safe and effective method should be considered prior to seeking other complex alternatives, which may involve retrograde esophageal access or surgical repair 12 Eosinophilic Esophagitis and Esophageal Achalasia in a Teenager with Chronic Dysphagia. E. Barfield, A. Kesavan, T. Ciecierega, Pediatric Gastroenterology and Nutrition, New York Presbyterian-Weill Cornell , New York, New York, UNITED STATES. Introduction: Dysphagia is defined as swallowing difficulty. It can present with gagging, coughing or painful swallowing. The differential diagnosis in children is broad and includes anatomical anomalies, eosinophilic esophagitis (EoE), psychogenic causes and esophageal dysmotility. Co-occurrence of multiple pathologies is rare. Prompt diagnosis and treatment is crucial in these patients. We present the case of a 15 year old male with chronic dysphagia caused by co-existing etiologies. Case Description: A 15 year old African American male with repaired congenital esophageal atresia presented to our practice with the complaint of chronic dysphagia since birth. Nissen fundoplication was performed at three years of age for severe gastro-esophageal reflux symptoms not amenable to pharmacotherapy. Recently he complained of worsening dysphagia, odynophagia, burning in the esophagus and postprandial nausea. Barium esophagogram showed distal esophageal narrowing with pooling of contrast and significantly delayed emptying of the esophagus. Upper endoscopy showed active esophagitis in the proximal and mid esophagus with up to 30 eosinophils (eos)/high power field (hpf) suggestive of EoE. He was treated pharmacologically with Fluticasone and acid suppression and subsequently underwent esophageal dilatation of the stenotic area. Because of persistence of dysphagia despite appropriate treatment of EoE (less then 15 eos/hpf) he underwent esophageal manometry testing which demonstrated total esophageal aperistalsis and poorly relaxing lower esophageal sphincter consistent with esophageal achalasia. A repeat esophageal manometry was performed a few months later and remained consistent with esophageal achalasia. Discussion: While EoE is a common diagnosis in children with dysphagia, esophageal achalasia is not. Their co-occurrence is rare and has only been reported on a few occasions. It is important to consider rare causes of dysphagia including esophageal achalasia in children with chronic dysphagia in the setting of appropriately treated EoE. Esophageal manometry should be considered in these patients to exclude concomitant motility disorders. 13 Collagenous Gastritis in a young adolescent male. A. Chan, J. Screws, D. Tolosa, D. Laman, Pediatrics, UT Chattanooga, Chattanooga, Tennessee, UNITED STATES. Collagenous Gastritis was first described by Coletti and Trainer in 1989 and remains extremely rare. Treatment is empirical without defined treatment guidelines. Upon review of the literature, there appears to be two generalized patterns for presentation of Collagenous Gastritis: a pediatric type associated with anemia and abdominal pain and an adult type associated with collagenous colitis and watery diarrhea. We describe a 16-year- old male who presented with intractable postprandial emesis and periumbilical / epigastric pain. Laboratory studies included complete blood count, metabolic panel, urinanalysis, H. pylori, celiac panel, and quantitative immunoglobulins – all normal except for an elevated IgE. Radiographic studies included a normal gallbladder ultrasound and normal CT scan. Initial EGD revealed a hiatal hernia and a moderately severe gastritis with concern for NSAID gastropathy, with biopsies showing markedly thickened subepithelial collagen and a conspicuous amount of eosinophils in the fundus with increased intraepithelial lymphocytes in the duodenum. There was erythema of the esophagus as well as erosions and medium sized polyps in the stomach on a repeat EGD three weeks later, with pathology showing moderate chronic gastritis with eosinophils and subepithelial collagen. Colonoscopy had a normal appearance, but biopsies showed increased eosinophils in the colon with a normal terminal ileum. He was tried on multiple medications initially without success, including sucralfate, PPIs, oral budesonide, as well as scheduled antiemetics such as ondansetron and benadryl. IV methylprednisone was given for several days but stopped due to severe side effects. Nitazoxanide / fish oil was trialed but the medicine was not tolerated due to emesis. Biofeedback was initiated for pain control. Prednisolone was initiated and he achieved both symptomatic improvement and histopathologic remission. There is a need for further investigation of this rare and poorly understood disease. Our patient does not fit clearly into either of the previously seen patterns of collagenous gastritis, and further cases and/or case series are needed to help establish treatment guidelines. 14 A 15 year old female with Plummer Vinson Syndrome. S. Edwards, Gastroenterology, Children's Mercy Hospital, Kansas City, Missouri, UNITED STATESM. Manalang, Hematology, Children's Mercy Hospital, Kansas City, Missouri, UNITED STATES. Background Plummer Vinson Syndrome (PVS) is the triad of iron deficiency anemia, dysphagia and esophageal webs. It is typically a disease of middle age to elderly adults. There are few reports of PVS in the pediatric literature. The pathogenesis is not clearly understood. It is important to identify these patients early because there has been evidence, from the adult population, that there is an increased risk for the development of esophageal squamous cell carcinoma. Therefore, it is recommended that patients with PVS be scoped yearly. Case Presentation A 15 year old female with a lifelong history of anemia, thought to be due to alpha thalassemia presented to the hematology department for profound fatigue. She was anemic with a hemoglobin 9.3 g/dL, MCV 58 fL and RDW 17.8%. Ferritin was low at 9 ng/mL. She was diagnosed with iron deficiency anemia and was started on 65 mg of elemental iron by mouth three times daily. Repeat labs, one month later, showed a hemoglobin of 8.7 g/dL with an MCV of 58 fL and reticulocyte percent of 1.5. Given her lack of response while compliant with oral iron therapy, an investigation for occult chronic disease was performed and was negative. Follow up five months later revealed no change in her hemoglobin and persistently low ferritin levels with continued compliance with oral iron therapy. She subsequently failed an iron challenge performed in clinic. Since she was unable to absorb oral iron, a celiac panel was obtained and was negative. She was also referred to gastroenterology for further investigation of possible gastrointestinal malabsorption. She was seen in gastroenterology where she denied any abdominal pain or weight problems. She did report a history of choking and vomiting as a child, which improved as she aged, but continued to have trouble with pill swallowing. An esophagogastroduodenoscopy (EGD) was performed and showed an upper esophageal web. The web was disrupted with the scope and did not warrant any further intervention. Given her iron deficiency anemia, dysphagia and esophageal web, she was diagnosed with PVS. Eventually she was diagnosed with iron resistant iron deficiency anemia (IRIDA) and treated with IV iron. She has severely impaired intestinal iron uptake without evidence of a chronic inflammatory