A Case of Collagenous Enterocolitis in an Infant

Abstract

Purpose: Collagenous colitis is where a thick subepithelial collagen layer in the colon is seen on histology. Few cases are reported in children. To date, there have been no cases of collagenous enterocolitis reported in children. We present a case of collagenous enterocolitis diagnosed in a male infant with an unknown neuromuscular disorder who was evaluated for protein losing enteropathy after presenting with hypoalbuminemia and ascites. He had a history of failure to thrive for which an NG tube was used to provide calories. He resided in a nursing facility when he was transferred to our institution with worsening abdominal distention over 1 week. He had been experiencing voluminous, foul-smelling, mucoid stools. On examination, he had a soft, moderately distended abdomen and severe hypoalbuminemia. Imaging studies showed irregular mural thickening of small bowel loops and ascites. His work-up was negative for infectious or renal causes prompting endoscopy. Gross endoscopic findings were duodenal edema with exudative lesions and a nodular rectum. Histology showed thickened subepithelial collagen in the duodenum, sigmoid and rectum. He was trialed on Prednisone 1 mg/kg/day and allowed to continue NG feeds. A few days later, he suffered respiratory insufficiency requiring intubation. Because of his acute decompensation, he was placed on stress-dose Hydrocortisone, feeds were discontinued, and he started parenteral nutrition (PN). Despite being on steroids and having optimal protein in his PN, hypoalbuminemia persisted, requiring daily albumin infusions; his fecal alpha-1-antitrypsin and reducing substances were positive. It was determined that he would require long-term PN. The steroids appeared to provide no benefit so they were subsequently tapered off over a month. He was trialed on Sulfasalazine 40 mg/kg/day while further work-up was ongoing. After a 2 month hospitalization, he eventually stabilized and was transferred back to the nursing facility where he remained NPO and on Sulfasalazine. After a month, a feeding trial was attempted but he developed diarrhea, guaiac positive stools, and hypoalbuminemia, so feeds were discontinued. A trial was reattempted another month later with the same results. Review of the literature shows that common variable immune deficiency, autoimmune enteropathy, and celiac disease may present with subepithelial collagen but work-up has thus far been negative. Presently, the patient remains NPO with PN. Sulfasalazine was discontinued after he was readmitted with acute respiratory failure. A repeat endoscopy was conducted at the time of this abstract submission. Biopsy samples were sent for electron microscopy and further testing is ongoing.