To describe a patient with Simpson-Golabi-Behmel syndrome and small bowel obstruction due to ileocaecal herniation into an unusual right paracaval peritoneal recess (not described in the medical literature to our knowledge). To discuss the potential for unusual bowel internal herniation into right paracaval recess in patients with malrotation and visceroptosis.

Simpson-Golabi-Behmel syndrome Simpson-Golabi-Behmel syndrome (SGBS) is a rare, partially recessive X-linked syndrome which classically features pre- and post-natal macrosomia including macrocephaly, coarse facial features, organomegaly and multiple congenital abnormalities [1, 2]. Relevant to this case, patients with SGBS have been described with intestinal malrotation, pyloric rings, Meckel's diverticulum, hypotonic visceroptosis (prolapse of abdominal viscera away from normal position) in addition to umbilical, inguinal and diaphragmatic hernias [3], all of which may predispose to gastrointestinal obstruction. Whilst there are over 300 articles on SGBS within the...

24 hours after admission the patient developed increasingly severe right upper quadrant pain, associated with nausea and vomiting. Examination findings included abdominal distension and reduced bowel sounds. Chest and abdominal x-rays at this time (Day 3) demonstrated extensive dilated small bowel, with relative paucity of colonic gas suggestive of small bowel obstruction. [Fig 12] Subsequent CT abdomen and pelvis post IV contrast later that day demonstrated distended oesophagus and stomach, and dilated jejunum and ileum. The cause of the small bowel obstruction was confirmed to...

This unusual case may be termed internal 'right paracaval' hernia and demonstrates the potential for unusual intestinal internal herniation in a patient with SGBS, visceroptosis and intestinal malrotation. We propose that this is consequent on developmental anomalies of peritoneal recesses and ligaments.

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