There have been a few reports of familial anorectal malformations extending over more than one generation. We experienced a case of a family with 3 members spanning 2 generations affected with isolated low type anorectal malformations. They had same low type of anorectal malformations. In all 3 patients, a perianal anoplasty was performed.

A case of congenital ectopic scrotum in neonatal period is described. The ectopic scrotum was located in the right inguinal area and the left hemiscrotum was found in normal location and each hemi-scrotum contained their testis. The neonate also had imperforate anus as low anorectal malformation with spinal abnormalities (hemi-sacrum and hemi-pelvis), right knee flexion contracture and right club foot. The embryological explanation in the literature of ectopic scrotum and its associated anomalies is discussed.

Currarino syndrome is a hereditary syndrome characterized by the triad of a sacral bony defect, presacral mass and anorectal malformation. We retrospectively reviewed 13 Currarino syndrome patients who were treated in our center between 1997 and 2010. Demographic data, initial symptoms, initial diagnosis, pathologic diagnosis of presacral mass, associated anomalies and managements were analyzed. There were 8 boys and 5 girls. Four patients were diagnosed as Currarino syndrome immediately after birth with failure of passage of meconium and abdominal distension. Four patients underwent surgery for imperforate anus immediately after birth and were diagnosed as Currarino syndrome later and underwent reoperation. Three patients were diagnosed during work-up and management with of the tentative diagnosis of Hirschsprung's disease. Diagnosis of the remaining two patients was at the age of 26 months and 9 years and anorectal malformation was not associated. Twelve patients showed hemi-sacrum and one patient showed bilateral sacral subtotal agenesis. Two patients without anorectal malformation underwent presacral mass excision, untethering of spinal cord and repair of myelomeningocele. Six out of 8 patients, excluding 3 that expired or were lost to follow up, with anorectal malformation underwent colostomy, presacral mass excision, untethering of spinal cord, repair of myelomeningocele, posterior sagittal anorectoplasty and colostomy repair. One patient underwent only posterior sagittal anorectoplasty after colostomy. One waits the scheduled operation only with Hegar dilatation. Pathologic examation of presacral masses showed myelomeningoceles in 4 patients, lipomyelomeningoceles in 3 patients and dermoid cyst in one patient. Teratoma was combined in 2 patients. Eight patients needed neurosurgical operation for spinal cord problems. Seven patients had urologic anomalies and two of them underwent operation. Currarino syndrome should be considered as a differential diagnosis in pediatric patients with abdominal distension, constipation and anorectal malformation. For proper evaluation and treatment, a multi-disciplinary approach is recommended.

Anorectal malformations comprise a spectrum of disease and the majority of patients have one or more abnormalities that affect other systems. In evaluating a newborn with anorectal malformation, the decision regarding the need for a colostomy and detection and management of any life threatening associated anomalies are thetwo most important considerations. Perineal inspection provides the clue to the surgical approach in about 80-90% of male and 90% of female newborn baby. The remaining patients who do not show any clinical evidence need radiologic evaluation to decide whether a colostomy should be performed. In most cases the decision to make a colostomy should not be made until the baby is 20 to 24 hours old and evaluation to rule out the presence of associated anomalies completed. A divided colostomy at the junction of the descending and sigmoid colon is recommended for anorectal malformations.

Artificial classification of anorectal malformation (ARM) is difficult because it is a spectrum of embryologic maldevelopment. However, the establishment of an ideal classification of ARM should be established in order to choose the adequate treatment and evaluate the prognosis. The classification system of ARM has followed the development of medicine, and the pediatric surgeons, who manage the ARM, should understand the change of this classification system.

History, incidence and associated anormalies of the anorectal malformations were reviewed.

Postoperative management of anorectal malformation consists of colostomy management and then management after definitive surgery. Colostomy management requires attention to certain details, i. e. complete decompression of the distal limb to avoid secondary megarectosigmoid and prevention and treatment of urinary tract infections in patients with rectourethral fistula. Management after definitive surgery requires the care of catheters placed in the rectourethral fistula or cloacal defect. Prevention and treatment of various complications after definitive operation, i. e. wound infection, anal stenosis or stricture, anal mucosal prolapse, and management of functional disorders, i.e. constipation, fecal incontinence and urinary incontinence are also necessary. In this review, recent trends for the prevention and treatment of the above mentioned problems after operation for anorectal malformation are presented.

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 aim of this study was to evaluate the posterior sagittal anorectoplasty (PSARP) as a re-do operation in patients who failed initial repair of anorectal malformation. Nine patients (4 boys and 5 girls) who had previous failed surgery for anorectal malformation underwent secondary operations through posterior sagittal approach. The main reasons of surgery were constipation (n=3) and persistent anatomical derangement in spite of previous correction surgery (n=6). In addition to constipation, the former group (n=3) had various anatomical defects, and the latter group (n=6), of course, had constipation in some degrees. Patients ranged in age from 2 to 19 years (median 3 years) with only one over the age of 6 years. The primary procedures included PSARP (n=8) and anoplasty (n=1). The rectum was mobilized from surrounding structures through posterior sagittal approach and anatomical defects were corrected. The rectum underwent reconstruction, which involved relocation of the rectum and anus within the limits of the intact muscle complex. Patients underwent follow-up for periods ranging from 6 to 77 months (mean 37 months) after surgery. Anatomical corrections of all the defects were successfully fulfilled in 9 patients. All the patients were satisfied with the functional results after redo-PSARP compared with the preoperative defecatory function. This study suggests that (1) some of the patients with troublesome constipation may have anatomical defects, prominent or hidden, (2) surgeons should suspect the possibility of anatomical defect as the cause of incontinence and (3) preoperative thorough investigation to reveal the anatomical defects should be included in estimating patients with severe incontinence after previous surgery and planning the correction for failed previous surgery as well.

Persistent cloaca in a female newborn is one of the most complex and challenging developmental malformations. The incidence is about 10% of all anorectal anomalies. Treatment of cloacal malformations has evolved during the past 40 years; however, it still remains one of the most difficult operations. In 1997, Pe a reported that a new technical variant called "Total urogenital mobilization" We also repaired cloacal anomaly using “Total urogenital mobilization” in 3 patients. The operations were performed between age 15- and 28-month. The length of the common channels was 2.5-3.0 cm. Two cases had double vaginas and one of them also had double uterus. The operation time was 4-5 hours and no major complications occurred. A satisfactory functional and cosmetic results were obtained.

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

Evaluation of the sacrum in anorectal malformations (ARMs) is important because of the frequent association with ARMs and functional outcome after correction of ARMs. Sacral defects are not easily detected because of immaturity of sacrum in children and overlooking of pediatric surgeons. The authors utilized the sacral ratio in normal children and patients with ARMs. In normal children, the mean true sacral ratio and mean sacrococcygeal ratio were 0.60±0.08 and 0.72±0.13 respectively. Sacral ratio was not correlated with age and did not changed with age in same patient. However, true sacral ratio and sacrococcygeal ratio were significantly lower in patients with high type ARMs than those of normal children (p<0.001). There was no difference between patients with low type ARMs and normal children. These results suggest that abnormal sacrums are more frequently encountered in patients with high type ARMs than in normal children, and that true sacral ratio and sacrococcygeal ratio can be used in the evaluation of the abnormal sacrum.