%193250
ICD+
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| VOLVULUS OF MIDGUT | ||||||||||||
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| INTESTINAL MALROTATION, FAMILIAL | ||||||||||||
| Clinical Synopsis | ||||||||||||
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| Smith (1972) documented 8 cases of midgut volvulus in 1 kindred. The propositus, his 2 sons and 3 daughters, and his 2 grandchildren demonstrated this midgut malrotation syndrome. The midgut volvulus caused great discomfort. Six of the affected had undergone a total of 24 operative procedures to alleviate problems caused by the malrotation of the midgut. The clinical course of the 5 sibs showed normal growth and development followed by the appearance of abdominal distention, pain, and constipation by the age of 6 years. The 2 affected grandchildren died at the age of 4 weeks, one of postoperative complications to repair the volvulus and the other from multiple congenital defects. This kindred also demonstrated a thick disc of fibromuscular tissue on the antrum of the stomach in 3 of the patients and major obstetrical abnormalities in 10 of 19 pregnancies produced by the 5 affected sibs. Carmi et al. (1981) reported father and daughter with congenital midgut volvulus and consequent intestinal obstruction, discovered soon after birth. The father's younger sister developed intestinal obstruction 2 days after birth and was found to have atresia of the ascending colon. The parents of these 2 sibs and a later-born son of the 'father' were normal by roentgenographic survey of the gastrointestinal tract. Budd and Powley (1988) reported the cases of 2 sibs with small bowel volvulus and malrotation. Stalker and Chitayat (1992) described 2 sisters with congenital midgut volvulus. Both had small intestinal malrotation. One had gangrene of the entire small intestine, suggesting that the intestinal volvulus was late in onset. In the second sister, the volvulus probably occurred at an earlier gestational age, causing intestinal atresia. Both sibs had an unusual facial appearance: a 'boxy' head, high forehead, frontal bossing, telecanthus, and long palpebral fissures. Both parents had normal barium meal roentgenograms, and they and 2 unaffected sibs had a different facial appearance. | ||||||||||||
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