No abstract

The aganglionic segment of intestine in Hirschsprung's disease begins at the anus and extends proximally for a distance that varies from case to case. Occasional reports describe patients in whom the aganglionosis is segmental, with normal distal innervation or a skip area of normal innervation within an area of aganglionosis. This paper describes 4 patients with Hirschsprung's disease wherein a segment of normally innervated colon was found in an otherwise aganglionic colon. Two of these patients were siblings with different fathers. Problems encountered in the management of these patients are detailed. In a critical review of the literature, 2 additional male patients with well-documented zonal aganglionosis were identified. Although variations from the usual morphology or Hirschsprung's disease do exist, they are so rare that they merit clinical consideration only when the anatomic record and the clinical course are in obvious disagreement. Rectal biopsy remains the best method for the diagnosis of Hirschsprung's disease.

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THE LENGTH of the small intestine in infants and children has received little attention in the literature. Our interest was aroused by an error in radiographic diagnosis. A film of the abdomen of a 1-day-old infant was interpreted as atresia of the jejunum because only two gasfilled intestinal loops were seen. At operation, the atresia was almost at the ileocecal valve, but the total bowel length was only 50 cm, thereby accounting for the misinterpretation.Standards of small intestinal length in normal infants are few. Benson 1 mentions 248 cm as the average length for the term newborn infant and a 230 cm length of jejunum and ileum in a single 3 lb 11 oz (1,673 gm) premature infant. Benson et al2 state that the proportionately shortened length of small intestines in the premature infant varies from 160-240 cm. Potts 3 states only that the infant's intestine is 305 cm in length. Abnormally short bowels have been described in association with atresia.2 If prenatal bowel injury or vascular accident is responsible for atresia as proposed in recent studies.2·4·5 avas¬ allar necrosis of segments of varying lengths would also occur and result in bowel shortening.Our review did not uncover any reports of short bowel without atresia. No reports of excessively long bowel were found. In order to study bowel lengths further, the autopsy records of 389 unselected patients in which the intestine had been measured were