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Gastrointestinal: Ischemic enteritis and sickle cell disease

A man, aged 45 years, with known sickle cell disease (hemoglobin SS) was admitted to hospital with abdominal pain and abdominal distension that had persisted for 2 days. He did not describe diarrhea or rectal bleeding. On examination, there was generalized abdominal tenderness but normal bowel sounds. Upper gastrointestinal endoscopy revealed elevated lesions, 1–2 cm in diameter, with central ulceration in the second and third parts of the duodenum. A contrast-enhanced computed tomography scan showed diffuse thickening of the wall of the small intestine (arrows) creating a target-like appearance (Fig. 1). Additional findings were a small amount of fluid in the peritoneal cavity and thickening of the peritoneal reflection. Histological evaluation of biopsies from the ulcerative lesions showed florid vascular proliferation, acute and chronic inflammatory cells, focal areas of necrosis and occlusion of some vascular channels by thrombi. Congested vascular channels (large arrow) with sickled red blood cells (small arrows) are shown in Figure 2 (H&E ¥400). A diagnosis of ischemic enteritis was made and the patient improved with analgesics, fluid replacement, oxygen and antibiotics. Sickle cell disease is an inherited disorder of hemoglobin structure that results in chronic hemolytic anemia and recurrent episodes of vascular occlusion with ischemia and tissue infarction. A relatively common manifestation is that of abdominal pain, either generalized pain or pain in the right upper quadrant. This pain may be difficult to distinguish from other causes of pain such as intestinal obstruction, acute cholecystitis, acute pancreatitis and acute appendicitis. A particular problem is pain in the right upper quadrant as most of these patients have an elevated bilirubin level and there is a high frequency of gallbladder stones. In patients with generalized abdominal pain, mesenteric ischemia may well be an important mechanism for pain although it is rare to document ischemic syndromes such as ischemic colitis or, in this case, ischemic enteritis. In sickle cell disease, the pain associated with vasoocclusive crises is usually relieved by rehydration and oxygen within 48 h.