S68 Concurrent Esophageal Crohn’s Disease and Superior Mesenteric Artery Syndrome in a Pediatric Patient

Abstract

Case: A previously healthy 12-year-old female with 4-month history of malaise, dizziness, headaches, anorexia (20-pound weight loss). A month prior, she developed daily non-bilious, non-bloody emesis and early satiety. Denied abdominal pain, diarrhea, hematochezia, constipation, oral ulcers, rash or fever. Family history was negative for gastrointestinal illness. Physical exam showed thin stature, epigastric tenderness, weight 37.7 kg (27th percentile) and BMI 14.36 kg/m2 (2nd percentile). Initial labs: Hgb 13 g/dL, MCV 74.4 fL, albumin 2.5 g/dL, platelet 497,000/uL, CRP 16.6 mg/L, ESR 24 mm/hr and fecal calprotectin 2500 mcg/g with remainder unremarkable. Upper GI demonstrated mucosal irregularity/fold thickening and narrowing at the third portion of the duodenum. Superior mesenteric artery (SMA) ultrasound showed aortomesenteric angle EGD revealed esophagitis, gastritis and bulbar duodenitis. She received intravenous pantoprazole and NJ tube placed for enteral nutrition. Biopsy with chronic lymphocytic esophagitis, chronic active gastritis negative for Helicobacter Pylori or CMV, and chronic peptic duodenitis. MRE demonstrated a 1-2 cm segment of bowel wall thickening and hyperenhancement 10-20 cm proximal to the terminal ileum. Colonoscopy found terminal ileitis, unremarkable colon, and perianal disease consisting of a skin tag with small fissure. Biopsies revealed mixed inflammatory infiltrate consistent with chronic active ileitis. Discharged with NJ feeds for 4-6 weeks, and pantoprazole. Given first dose of adalimumab prior to discharge. Introduction: SMA syndrome is characterized by compression of the third portion of the duodenum between the SMA and the aorta. In pediatrics, the major cause is rapid weight loss leading to loss of the intervening mesenteric fat pad. Symptoms include postprandial epigastric pain and early satiety. Those with advanced obstructions may have severe nausea, bilious emesis and weight loss. The incidence ranges between 0.013 and 0.3%. Treatment is typically successful with enteral nutrition, with surgical intervention reserved for patient’s refractory to refeeding. Crohn’s disease (CD) is a chronic inflammatory disease that can affect any part of the GI tract. CD of the esophagus is less common, affecting 7.6-17.6% of pediatric patients. Most cases of esophageal CD present with symptoms similar to gastroesophageal reflux disease (GERD) with chest pain, heartburn and regurgitation and more advanced cases with odynophagia, vomiting, and weight loss. The mean time to diagnosis of CD and esophageal involvement is up to 3 years. Upper GI involvement of CD is more common in pediatric than adult population, but still less prevalent than lower GI involvement. Discussion: This is an atypical case of CD primarily involving the esophagus that led to the development of SMA syndrome. Though weight loss due to CD is common (57%) there is limited literature describing CD associated with SMA syndrome and only a few reported cases of SMA syndrome as the presenting sign of CD with nonspecific symptoms (vomiting, abdominal pain). There is an even smaller number of cases reported in the pediatric literature. Thus, it is important to highlight that esophageal CD, often presenting with nonspecific symptoms (vomiting, weight loss), is severe and can lead to SMA syndrome.