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The risk of long-segment Hirschsprung's disease recurring in further sibs of a patient is well known.Ehrenpreis (1970) estimated the incidence to be 121 %.The extreme variety of this condition in which the entire large and small bowel is aganglionic seems to constitute a separate group. This is a fatal condition and fortunately is rare. The patient described here is a further affected sib in the family reported by Ahmed et al. in 1971. The second child born in the family was a normal healthy female. Case reportThe male third child of healthy unrelated white British parents was born at 38 weeks' gestation by normal delivery at home. When feeding was begun he vomited bile-stained fluid. He was admitted as an emergency to hospital late on the third day of life, by which time he had not passed meconium; he was not dehydrated and weighed 3 02 kg. The abdomen was distended but soft. Rectal examination suggested the presence of a microcolon. The full details of the family history were not then available and the possibility of total intestinal aganglionosis was not considered. It was only later that the case notes of the eldest sib were made available, showing that she had died at the age of 20 days from total intestinal aganglionosis.Hb was 16 6 g/dl, WBC 5 7x 109/1. Serum electrolytes were normal. X-ray of abdomen showed several distended intestinal loops with fluid levels, while in the right lower quadrant there was the characteristic stippled appearance of meconium. A diagnosis was made of small bowel obstruction possibly due to a complicated meconium ileus. At laparotomy the proximal third of the small bowel was grossly distended with a serosal tear already present. The terminal ileum was full of putty-like meconium and was of narrow calibre, as was the colon. The contents of the proximal small bowel were more fluid than commonly found in meconium ileus. The jejunum was deflated by aspiration and the serosal tear repaired, and a caecostomy was established through which the distal ileum was emptied.The following day, after reviewing the case notes of the eldest sib, the diagnosis of total intestinal aganglionosis was considered. This was confirmed firstly by rectal biopsy, then at a further laparotomy the extent of the aganglionic segment was shown to include the entire small bowel. The child died 14 days after admission and post-mortem studies showed that there were ganglion cells present only as far as the pylorus. Discussion

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