Major advances in the management of anorectal malformations have been achieved during the last 20 years. Alberto Pena introduced the posterior sagittal anorectoplasty (PSARP) in 1982. He divided all the sphincteric muscles at the exact posterior midline and fully exposed the crucial anatomy. He was able to manage the associated fistula under direct vision with minimal urinary tract injury. The rectum could be placed in the middle of the sphincteric muscle complex. Even with PSARP by Alberto Pena himself, only 37.5% of all cases were considered totally continent. Anorectal malformation is still acontinuing challenge for the pediatric surgeons.

The purpose of the survey was to clarifY the clinical features and management of the anorectal malformations (ARM) in Korea. Twenty-seven members of the Korean Association of Pediatric Surgeons were surveyed. The members completed a case registration form for each patient during the two years period of 1996-1997, and a questionnaire that contained their management preferences for primary and complicated patients. Twenty-four members of the 22 institutions registered 295 cases of the ARM, and 27 out of 34 members (77.1 %) responded to the questionnaire. The patients were classified by the Wingspread classification of ARM(1984). The case registration form was a modification of Wingspread workshop for the postoperative assessment and case registration. The male to female ratio was 1.7: 1. The average number of patients per surgeon was 6.1 cases per year. Prematurity(> 36 weeks) was present in 1.9 % of cases and low birth weight (< 2.6 Kg) in 12.1 %. Among 187 male patients, 62 were higheR) type, 29 intermediate(l) type, 88 low(L), and 8 unclassified. In female, there were 2 cases of cloacal anomalies(C), 4 H type, 30 I type, 66 L type and 6 unclass ified. For male, 87.9 % of H and I type were operated by the Pena procedure, but only 7.9 % in L types. In female, all of C, H and I types, and 40.9 % of L type were done by the Pena procedure. One case expired (MR; 0.003%) as a result of surgical complication. Over-all complication rate was 12.5 %. Among 6 cases of reoperation, 4 were failed Pena procedures. Among 140 colostomies sigmoid colon was utilized in 75.7%, and loop colostomy was commonly used. Ten surgeons prefered primary maturation of the stoma. In conclusion, posterior sagittal anorectoplasty is popular for high types of ARM in this country, but considerable number of patients are still suffering from failed operations. For better understanding and analysis, necessity of prospective study by new classification was discussed

The association of congenital colonic atresia with imperforate anus is extremely rare. There are only three cases reported in the English literatures. The authors have recently managed one case in female infant.

Colonic atresia is the least common form of intestinal atresia. Colonic atresia, like jejunoileal atresia, mostly occurs as a result of an intrauterine vascular accident. According to Sutton's classification, type 1 colonic atresia exibits external continuity with an intraluminal diaphragm(colonic web) which may be imperforate or perforate. Authors experienced a case of colonic web associated with imperforate anus. The patient was treated under the impression of the high type imperforate anus. Posterior sagittal anorectoplasty at the age of 9 months revealed normal anorectal canal. At the age of 12 months, colostomy was repaired. Thereafter abdominal distension was developed. Barium enema noted a membranous obstruction of the colon on the level of the rectosigmoid junction. After exicision of the colonic web, the patient was discharged with no problem.

Diphallus is a rare congenital anomaly and is frequently associated with duplication of the urinary tract and rectosigmoid, and commonly associated with vertebral anomalies. Remzi reported less than 100 cases of duplication of all or a portion of the penis, but about 10 cases of complete diphallus with exstrophy of cloaca was reported, and a case of complete diphallus associated with hingut duplication was reported, and complete diphallus with displacement of bladder associated hindgut duplication and imperforate anus was not reported in Korea. We experienced a case of the complete diphallus associated with displacement of bladder, hindgut duplication, and imperforate anus as a variant of cloacal exstrophy. A review of published cases suggests that this may be the first example of a complete dip hall us with displacement of bladder coexisting with the hindgut duplication and imperforate anus.