• Advances in pediatric surgery
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• v.12 no.1
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• pp.86-90
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• 2006

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

• Advances in pediatric surgery
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• v.12 no.1
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• pp.99-106
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• 2006

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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.16 no.1
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• pp.28-33
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• 2013

Purpose: Anorectal malformations are often associated with other anomalies, reporting frequency with 40-70%. Gastrointestinal anomalies have been known to be relatively less common than associated anomalies of other organ system. This study was performed to assess a distinctive feature of cases associated with esophageal atresia. Methods: Clinical data (from January 2000 through December 2011) on the 196 subjects with anorectal malformations, managed in our Hospital, were reviewed. Total 14 neonates were identified with accompanying esophageal atresia and retrospective analysis was conducted. Results: The incidence was 7.1% and there were 8 male and 6 female subjects. Only 2 cases were associated with esophageal atresia without tracheoesophageal fistula. Although variable cases of anorectal malformation in female subjects, almost cases were anorectal malformations with rectourethral fistula in male. Other associated anomalies were identified in all cases, with more than 3 anomalies in 10 cases. There were 4 VACTERL (Vertebral abnormalities, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal and Limb anomalies) associations accounting for 28.6%, but could not identify chromosomal anomaly. Most cases were managed with staged procedure, usually primary repair of esophageal atresia and diverting colostomy. Overall mortality rate was 21.4%, mainly caused by heart problems. Conclusion: This study shows that early diagnosis and rational surgical approach with multidisciplinary plan are mandatory in managing anorectal malformations with esophageal atresia, when considering a high frequency of associated anomaly and a relative high mortality.

• Advances in pediatric surgery
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• v.12 no.1
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• pp.107-114
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• 2006

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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.4
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• pp.340-346
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• 2022

Purpose: The treatment and long term clinical outcomes of anorectal malformations (ARM) in children have always been the focus of pediatric surgeons. This study aimed at reporting our experience as far as long-term follow-up of ARM in children is concern. Methods: We enrolled patients treated between 1999 and 2019, and established selection criteria to choose appropriate subjects. A validated questionnaire was used to determine long-term quality of life outcomes. Results: Out of a total of 48 patients treated within the study period, 28 were enrolled in this study. Among the latter, more than 35% had at least one long-time complication, and more than 90% had a good lifestyle. Urinary and fecal continence was achieved in more than 95% of the patients using medical devices. Conclusion: This study aimed to bring up new concepts; taking into consideration all aspects of life in patients with ARM, from school life to sexuality, while evaluating fecal and urinary continence. This is essential for the improvement of the skills of the different specialists involved in the management of these patients, and for the implementation of strategies that can improve postoperative function. Most especially, it will also help improve communication between doctors to ensure an adequate transition of these children into adult life.

• Advances in pediatric surgery
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• v.6 no.2
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• pp.128-133
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• 2000

Persistent cloaca in a female newborn is one of the most complex and challenging anorectal malformation. 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, Pena reported a new procedure called "Total urogenital mobilization". We have repaired 3 cases of cloacal anomaly using "Total urogenital mobilization". The operations were performed between age 15 and 28 months. 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. Functional and cosmetic results were satisfactory.

• Advances in pediatric surgery
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• v.6 no.1
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• pp.32-39
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• 2000

Evaluation of the sacrum in anorectal malformations (ARMs) is important because there is an association between sacral abnormalities and poor functional outcome after corrective surgery for ARM. Sacral defects are not easily detected because of immaturity of sacrum in children and defects are often overlooked by pediatric surgeons. The authors utilized the sacral ratio in normal children (N=61) and patients with ARMs (N=26). In normal children, the mean true sacral ratio and mean sacrococcygeal ratio were $0.60{\pm}0.08$ and $0.72{\pm}0.13$ respectively. The sacral ratio was not correlated with age and did not changed with age in the 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.

• Advances in pediatric surgery
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• v.17 no.1
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• pp.81-87
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• 2011

This study was aimed to evaluate associated congenital anomalies in the patients with esophageal atresia with tracheoesophageal fistula (EA/TEF). Forty-two neonates with the diagnosis of EA/TEF treated over a 10 year period in a single institution were included in this study. The demography of EA/TEF was analyzed. Major associated anomalies including vertebral, anal, cardiac, renal, limb, neurologic and chromosome were reviewed and categorized. Males were slightly more dominant than females (1.47:1) and all patients had Gross type C EA/TEF. Only 19 % of the patients had solitary EA/TEF without associated anomalies. Cardiac anomalies were the most common associated congenital anomaly in patients with EA/TEF (73.8 %). But 47.6 % were cured spontaneously or did not affect patients' life. Atrial septal defect (ASD) was the most common cardiac anomaly followed by patent ductus arterious (PDA) and ventricular septal defect (VSD). Among gastrointestinal anomalies (23.8 %), anorectal malformations were the most frequent, 70 % Vertebral and limb abnormalities accounted for 11.9 % and urogenital malformations 9.5 % of the anomalies in patients with EA/TEF. VACTERL associated anomalies were 23.8 % and 1.8% had full VACTERL. Almost 12 % of EA/TEF had neurologic anomalies. Patients with EA/TEF require preoperative evaluation including neurologic evaluation to detect anomalies not related to VACTERL. Though associated cardiac anomaly occurred in 73.8 % of patients in our study, only 21.42 % needed surgical correction. The authors suggesrs further studies with large numbers of patients with EA/TEF.

• Advances in pediatric surgery
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• v.6 no.2
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• pp.106-123
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• 2000

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 high(H) 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.

• Advances in pediatric surgery
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• v.8 no.1
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• pp.11-15
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• 2002

This is a 20 year analysis of the problems associated with enterostomy formation, and closure. Forty-three stomas were established in 43 patients: 23 for anorectal malformations, 11 for Hirschsprung's diseases, 4 for necrotizing enterocolitis, 3 for multiple ileal atresias, 1 for volvulus neonatorum with perforation, and 1 for diaphragmatic hernia with colon perforation. Thirty boys and 13 girls were included (mean age 4.8 months). Stoma complications were encountered in 13 patients (30.2 %): stomal prolapse, stenosis, obstruction, paracolic hernia, retraction, dysfunction, and skin excoriation, Four patients (9.3 %) required stomal revision. Occurrence of complications was not related to age and primary disease, but sigmoid colostomy showed lower complication rate than transverse colostomy (20.0 % vs 42.9 %, p<0.05). There were five deaths but, only one (2.3 %) was directly related to the enterostomy complication. Twenty-one stomas were closed in our hospital and complications occurred in seven patients (33.3 %). The most common complication was wound sepsis in 5 children. In conclusion, because the significant morbidity of stomal formation still exists, refinements of the surgical technique seem to be required, Sigmoid loop colostomy is preferred whenever possible.