HIRSCHSPRUNGS-DISEASE IN AN ADULT PATIENT WITH FAMILIAL OCCURRENCE - REPORT OF A CASE

Hirschsprung's disease is almost always associated with newborns or in fants; however, we report herein the unusual case of a 46-year-old wom an in whom the symptoms of Hirschsprung's disease emerged late in adul t life, The involved rectosigmoid region was successfully removed by p erforming Duhamel's operation with a diverting colostomy, After the co lostomy was closed, she regained normal defecatory function. She had o ne male child affected by Hirschsprung's disease of the total colon ty pe who was operated on as a 1%-month-old baby. The genetic predisposit ion of Hirschsprung's disease has been reported, but its mode of inher itance has not yet been clarified, Moreover, most papers on the famili al occurrence of this disease have reported that siblings were affecte d. Our patient was unique for the definite occurrence of the disease i n successive generations. The features of Hirschsprung's disease in ad ults and the familial occurrence are discussed with a review of the li terature.