As a teenager in 1982, our patient, now 52 years old, presented with intermittent colicky pain, constipation, and abdominal distension. He also had intermittent bouts of melena, during which his hemoglobin dropped to ~6 g/dL, requiring multiple blood transfusions. There was no history of any nonsteroidal anti-inflammatory drug (NSAID) intake. Multiple gastroduodenoscopies revealed duodenal ulcers, for which proton pump inhibitors were prescribed on regular basis.
These symptoms persisted for 7 years, but during an episode of small bowel obstruction and GIB he underwent a laparotomy at a local hospital where jejunal ulcerations were identified and resection anastomosis was done. Biopsy of the resected specimen showed nonspecific ulcers. After surgery, however, there was no symptomatic relief. He was extensively evaluated during this period. Gastroduodenoscopy, colonoscopy, abdominal computed tomography, and Meckel's scans were all normal.
For the next 10 years, the patient underwent several abdominal surgeries with no clinical improvement. He consulted several gastroenterologists. Barium studies revealed small bowel strictures and ulcerations. Small bowel tuberculosis (TB) was suspected, and antitubercular drugs were given empirically. As the patient didn't respond, Crohn's disease became the leading diagnosis. He received corticosteroids, mesalamine, and azathioprine for a prolonged period. His small bowel symptoms didn't improve, but he developed complications of steroids such as cataracts and uncontrolled diabetes. He also developed features of chronic diarrhea and hypoproteinemia.
Capsule endoscopy showed a stricture along with superficial ulceration of the mucosa in the mid-jejunal area (Figure 1). The capsule became lodged in the stricture and was retrieved using an enteroscope. Diagnostic laparotomy, adhesiolysis, on-table enteroscopy, and resection anastomosis of distal ileal segment were performed in 2005.The resected ileal segment showed multiple, superficial, circular and linear ulcers and sharply demarcated ulcers with normal surrounding mucosa. The microscopy showed nonspecific ulcerations and a few angioectasia (Figure 2). The ectasias on pathology did not show any evidence of obliteration or hypertrophy, which is inconsistent with diagnoses of Crohn's disease or TB.
Thus, from the clinical features, endoscopic pictures, and biopsy evaluation, a diagnosis of CMUSE was made. The patient was kept on a nonresidual diet, medium chain triglycerides, high protein, and dietary supplementations. His obstructive symptoms improved and hypoproteinemia was corrected. Periodic GIBs persisted, however, for which he received blood transfusions as before. Five years later, hormonal therapy consisting of ethinyl estradiol and norethisterone successfully addressed his angioectasias stopped his GIBs. For the past 7 years he has been on hormonal therapy and a nonresidual diet, and he is symptom-free. As a complication of hormonal therapy, however, he developed gynecomastia and loss in libido, so the therapy was stopped. The melena reappeared, and the hormonal therapy resumed.