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J.Jpn. Surg. Soc.. 115(6): 312-316, 2014

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President, Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi, Japan

Masahiro Fukuzawa

Hirschsprung's disease (HD) is a relatively common cause of intestinal obstruction in the newborn. The disease is named after Harald Hirschsprung, who first described two infants with the condition in 1888. It is characterized by the absence of ganglionic cells in the distal bowel beginning at the internal sphincter and extending proximally for varying distances. The aganglionosis is confined to the rectosigmoid in 80% of patients. HD occurs in about one in 5,000 Japanese children. The most accepted theory of the cause of HD is a defect in the craniocaudal migration of neuroblasts originating from the neural crest which occurs during the first 10 weeks of gestation, as proposed by Okamoto in 1967. The RET protooncogene accounts for the highest proportion of both familial and sporadic cases. Diagnostic techniques involve anorectal manometry, barium enema, and rectal biopsy. The treatment of HD consists of surgical removal (resection) of the abnormal section of the colon, followed by reanastomosis. Definitive surgery for HD has been performed for many years using one of the techniques developed by Swenson, Duhamel, or Soave. Recently, minimally invasive laparoscopic procedures have been introduced for the treatment of HD.

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