Does zonal aganglionosis really exist? Report of a rare variety of Hirschsprung's disease and review of the literature.

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.

Rhabdomyosarcoma of the biliary tree: the case for aggressive surgery.

Embryonal rhabdomyosarcoma of the bile ducts is associated with a poor prognosis because complete resection rarely is considered possible. We reviewed the literature and report our experience with the management of 3 consecutive patients. The patients were 1, 4, and 9 yr old at presentation, and had jaundice, cachexia, and an abdominal mass. The tumor arose in the common hepatic duct in 2 patients, and in the left hepatic duct in the other. All had tumor extension into both lobes of the liver and residual disease was noted after resection. Two patients were given tumor bed irradiation and all received multiple-drug chemotherapy. Two patients are alive, jaundice free, and with no evidence of disease at 9 mo and 14 yr follow-up. The other patient was a long-term survivor, but developed local recurrent tumor after cessation of chemotherapy and expired 33 mo after diagnosis. The traditional anatomic criteria of hepatic tumor resectability may not apply to botryoid sarcoma of the bile ducts. Aggressive surgery combined with the new adjuvant therapies appears to provide the best chance for a longer survival.