Hirschsprung's disease: an analysis of 104 cases

One hundred and four cases of Hirschsprung's disease in infants and children diagnosed at the National Taiwan University Hospital during the period of 1984 to 1989 were analysed for demographic patterns, clinical manifestations, accuracy of various diagnostic procedures, especially the reliability of suction biopsy, and postoperative course. The important symptom constellation included abdominal distention, delayed meconium passage, and tight anus in the biopsy-positive group. Complementary diagnostic procedures were mandatory for accurate diagnosis, since 26% of the patients (7 of them with ultrashort segment) were diagnosed over a period of more than two months. The first biopsy in 25% of the patients aged less than 1 month could not be interpreted, and in 27.6% of this subgroup the radiological pictures were inconclusive. Further scrutiny of clinical manifestations together with other elaborate study including repeated rectal biopsies may be required for establishing a definite diagnosis of the disease. The surgical mortality is low, but surgical morbidity remains high and needs further improvement.