Case Communications Isolated Severe Gastropathy – an Unusual Presentation of Crohn’s Disease in a Child Roy Nattiv MD1, Gabriel Dinari MD1, Jacob Amir MD2 and Yaron Avitzur MD1,2 1Institute of Pediatric Gastroenterology and Nutrition and 2Department of Pediatrics C, Schneider Children’s Medical Center of Israel, Petah Tikva, and Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel Key words: gastropathy, stomach, Crohn’s disease, infliximab, children IMAJ 2008;10:322–324 Crohn’s disease may involve any part of and therapeutic implications of such a the gastrointestinal tract, but isolated presentation. Ultimately, the diagnosis was A involvement of the stomach is rare [1]. supported by suggestive endoscopic and In common presentations, the diagnosis histological findings and the presence of of Crohn’s disease is usually based on ASCA as a serological marker. Treatment a combination of typical clinical, labora- was refractory to steroids and treatment tory, endoscopic and pathological findings. with infliximab resulted in marked gross However, the diagnosis is difficult to clinical and endoscopic improvement. establish in cases of atypical presentation as in isolated gastroduodenal disease. In Patient Description such a scenario other possible etiologies A 13 year old girl was admitted with must be systematically ruled out in order symptoms of prominent epigastric pain, [A] Cobblestone appearance of the gastric body shown on gastroscopy. to establish the diagnosis. These may in- vomiting, bouts of both constipation and clude Helicobacter pylori infection, tuberculo- diarrhea, and anorexia with weight loss of sis, non-steroidal anti-inflammatory drugs, 5 kg. For 9 months prior to her admission eosinophilic gastritis, Menetrier’s disease, she was followed at an outside clinic for B gastrinoma, collagen vascular disease, and symptoms of recurrent epigastric pain and lymphoma. Additional diagnostic strategy constipation. During that period erythro- in atypical cases of inflammatory bowel cyte sedimentation rate was elevated (54 disease is the use of anti-Saccharomyces mm/hour) while hemoglobin and albumin cervisia antibody. This serological marker were within normal limits. Gastroscopy 2 can be a helpful adjunctive tool in the months prior to her admission showed diagnostic process despite the test’s erythema and edema of the gastric mu- limitations. cosa, a single gastric ulcer, pseudopolyps Treatment regimens for gastric Crohn’s and a cobblestone appearance of the [B] Gastroscopy showing antral nodularity disease have been poorly studied. The mucosa. Chronic gastritis had been noted and pseudopolyps. routine treatment of inflammatory gastritis on corresponding biopsies; H. pylori had in Crohn’s disease includes the concomi- not been identified. Although the etiology tant use of acid-suppressive drugs and of these findings was unclear, an empiric 0.0–0.5), and normal ESR, complete blood immunomodulators such as ASA products, trial with prednisone was initiated but count, ferritin, iron, and liver function or steroids. In recent years infliximab was unsuccessful. In addition, one month tests. The lack of a definitive diagnosis (anti-tumor necrosis factor-alpha) has prior to the child’s referral she underwent prompted further endoscopic evaluation, become an important addition to the an appendectomy due to acute appendi- which revealed mild esophageal edema therapeutic options in Crohn’s disease. citis. Pathological findings demonstrated and erythema, severe gastritis [Figure A], The effectiveness of infliximab in isolated appendicitis without granulomas. Her gastric pseudopolyps [Figure B], a single gastric Crohn’s disease is limited to only past medical history was otherwise antral ulcer and edema with erythema in a few case reports of adult patients and normal. the duodenal bulb. The second part of the the long-term outcome is unknown [2,3]. Physical examination on admission re- duodenum was normal. Colonoscopy was We present a child with isolated gastric vealed localized epigastric tenderness. The normal. Corresponding biopsies showed Crohn’s disease and discuss the diagnostic rest of the examination was normal. Initial chronic active gastritis with erosion and blood work showed mildly elevated C-re- ASCA = anti-Saccharomyces cervisia antibody active protein (2.230 mg/dl, normal value ESR = erythrocyte sedimentation rate 322 R. Nattiv et al. • Vol 10 • April 2008 Case Communications endoscopic findings, the exclusion of retrospective study of 230 pediatric Crohn’s C other possible etiologies, and a positive patients 30% of the children had lesions ASCA test. Treatment with omeprazole of the esophagus, stomach or duodenum, and methylprednisolone (60 mg/day) was but only three of them had Crohn’s dis- started but a 2 week course of intravenous ease isolated to the upper gastrointestinal steroids achieved only minimal clinical tract [1]. Although histological changes improvement. Second gastroscopy and in the stomach and duodenum may be biopsies following the 2 week treatment seen in 20–40% of patients with Crohn’s were essentially similar to the previous disease, clinically symptomatic proximal gastroscopy. Due to the lack of response disease is only seen in 4% of patients [4]. to steroids, a course of infliximab at 0, 2 Even in patients who initially presented [C] Histopathological stain of gastric mucosa showing moderate chronic active gastritis and 6 weeks was started with concomitant with isolated gastroduodenal disease, with regenerative changes (hematoxylin & administration of azathioprine. Six weeks the majority will develop distal disease eosin stain, original magnification x400) after the first dose the patient showed over time. In a series of 72 patients with marked clinical and endoscopic improve- proximal Crohn’s disease diagnosed on ment. Gastroscopy demonstrated only histopathology, all but one were eventu- marked regenerative changes; no granulo- mild stomach wall nodularity with antral ally diagnosed with distal disease as mas were noted [Figure C]. Non-specific pseudopolyps. No ulcers or erythema were well [5]. Similarly, in another study 56% acute and chronic inflammatory changes seen. Gastric biopsies showed ameliora- of patients who presented initially with were seen in the duodenal bulb. Colonic tion of the chronic gastritis compared to isolated gastroduodenal disease developed biopsies were normal. the previous biopsies. Laboratory values distal disease at a median follow-up of Single-contrast barium follow-through showed normal ESR, normal hemoglobin, 11.7 years. showed non-specific findings isolated to normal albumin and a C-reactive protein To date there are only a few the stomach, namely small protrusions of 0.01 mg/dl. One year after the initia- documented case reports of adults with and collaring in the lesser curvature of tion of infliximab treatment the disease isolated gastric Crohn’s disease and no the stomach that may represent small fis- has not progressed to the remaining reports in the pediatric population. In one sures or ulcers and early cobblestoning. intestine and the child has resumed her case series, four adult patients with non- The remaining small intestine including normal daily activities. However, repeated healing gastric ulcers refractory to conven- the terminal ileum was normal. Further infusions of infliximab were required due tional treatment were followed [2], and radiological investigation with computed to recurrent bouts of epigastric pain and in another report a 37 year old woman tomography of the abdomen showed appearance of extra-intestinal manifesta- with unusual gastric Crohn’s disease was widening of the stomach and thickened tions including erythema nodosum and described [3]. In all cases the diagnosis edematous mucosa in the region of monoarthritis of the right wrist. of Crohn’s disease was delayed for a long the pylorus without involvement of the time due to the non-specific presentation remaining gut. Comment of isolated gastropathy. The findings of non-specific gastropathy In most cases of Crohn’s disease the Normally, the diagnosis of Crohn’s without involvement of other parts of presentation, workup and diagnosis run disease is based on clinical presentation, the intestine and lack of inflammatory a familiar and substantiating course. radiological abnormalities of the small markers prompted a systematic workup to Sometimes, however, this disease can bowel, gastroscopy and colonoscopy find- rule out a number of possible etiologies manifest in an entirely non-specific and ings, and non-specific or typical patho- including H. pylori infection, tuberculosis, unusual manner. Uncommon presentations logical features. The abdominal pain and Zollinger-Ellison syndrome, collagen vas- of Crohn’s disease may manifest by a weight loss seen with our patient is by cular disease and lymphoma. Hence, the single symptom or sign, such as impair- far one of the most common modes of following tests were done and were found ment of linear growth, delayed puberty, presentation in Crohn’s patients, although to be within normal limits: Mantoux skin perianal disease, mouth ulcers, clubbing, it is non-specific. test, silver stain for H. pylori on gastric chronic iron deficiency anemia – or Radiology studies in gastroduodenal biopsies, serum gastrin levels, serology extra-intestinal manifestations preceding Crohn’s normally demonstrate similar for anti-neutrophil cytoplasmic antibody the gastrointestinal symptoms, mainly features to those found in more distal and antinuclear