Gastrointestinal: Rectal sarcoidosis due to paralytic ileus resembling adult‐onset Hirschsprung disease

Abstract

A 37-year-old woman was referred to our hospital with refractory paralytic ileus. Previous abdominal CT scans from two months previously were normal, but the oral contrast medium remained in the bowel when the test was repeated. No organic obstruction of the small intestine was found by gastrograffin small bowel study. We tentatively diagnosed chronic idiopathic intestinal pseudo-obstruction (CIIP). However, gastroduodenal manometry revealed heterogeneous phase III activity from the second to third parts of the duodenum (visceral myopathy type), which made CIIP unlikely. Colonoscopy revealed no organic obstruction of the colon but there was rectal dilatation and extensive retention of stools. Biopsy specimens from the terminal ileum, ascending colon and the rectum were normal with no inflammation or dysplasia. Because of the finding of rectal dilation and weak colonic contraction a barium enema was performed for suspected adult-onset Hirschsprung disease. This revealed narrowing of the distal rectum (Fig. 1 arrow). Rectal manometry showed that the patient had no reflex contraction of the rectum despite anal dilatation with a balloon at 30 mmHg. These findings were compatible with Hirschsprung disease. To confirm the diagnosis, biopsy of all layers of the rectal wall was performed. Histological examination revealed numerous epithelioid cell granulomas (Fig. 2) involving the peripheral nerves and nerve plexuses of the muscular and the submucosal layers. Hyperplasia of acetylcholinesterase-positive fibers was not found in the lamina propria. Therefore, she was diagnosed as having rectal sarcoidosis with secondary paralytic ileus. There was no hilar lymphadenopathy, granular changes, or opacification on chest X-ray and CT scan. Angiotensin-converting enzyme, a marker of sarcoidosis, was normal at 10.5 (8.3–21.4) IU/L, but serum soluble IL-2receptor antibody was very high at 1,901 (135–483) pg/ml. Tuberculin skin test was negative (0 × 0/6 × 6 mm). Bronchoscopy revealed a mucosal reticular network that was compatible with sarcoidosis. The CD4/CD8 cell ratio was high (4.76) in the bronchial lavage fluid, but no granulomas were detected by transbronchial lung biopsy. We treated her for neurosarcoidosis with prednisolone at 60 mg/day. Her symptoms improved gradually, and she was discharged on oral prednisolone (30 mg/day). At that time, the mucosal reticular network had resolved on bronchoscopy. Narrow rectum (arrow) on barium enema with proximal recto-sigmoid dilatation. Epithelioid cell granulomas involving preipheral nerves and nerve plexuses of the muscular and submucosal layers (HE stain, magnification × 20). Sarcoidosis is a systemic disease that causes the formation of epithelioid granulomas. The incidence of symptomatic bowel obstruction due to sarcoidosis is 0.6%. However, all previous reported cases of bowel obstruction were from mechanical obstruction caused by sarcoid granulomas. A Pubmed search using the key words ‘sarcoidosis’ and ‘paralytic ileus’ from 1949 to 2009 did not reveal any previous publications on paralytic ileus induced by intramuscular sarcoid granulomas resembling adult-onset Hirschsprung disease. Contributed by