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From question on page 343
The patient underwent capsule endoscopy which showed a single active bleeding vascular lesion of the small bowel (fig 2). The patient then proceeded to a segmental resection of the involved gut (fig 3A, 3B). Intraoperative enteroscopy was negative for further lesions. Postoperative recovery was rapid and uneventful.
Histological examination of the removed lesion disclosed cavernous haemangiomas compatible with blue rubber bleb nevus syndrome (BBRBNS). One month later haemoglobin concentration was 13.5 g/dl and is still maintained without oral iron supplements. A study of the small bowel performed with capsule endoscopy showed no further lesions.
BRBNS is characterised by haemangiomas in the skin, gastrointestinal tract, and other viscera. The most common mode of presentation of BRBNS is gastrointestinal bleeding. Lesions are most commonly found in the small intestine and distal large bowel and are typically discrete mucosal nodules with a central bluish nipple, although they may be flat, macular, or polypoid. BRBNS may affect several successive generations by autosomal dominant inheritance caused by a mutation on chromosome 9p.
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