Parental subfertility, fertility treatment, and the risk of congenital anorectal malformations.

BACKGROUND: Fertility treatment seems to play a role in the etiology of congenital anorectal malformations, but it is unclear whether the underlying parental subfertility, ovulation induction, or the treatment itself is involved. Therefore, we investigated the odds of anorectal malformations among children of subfertile parents who conceived with or without treatment compared with fertile parents. METHODS: We performed a case-control study among 380 cases with anorectal malformations treated at 3 departments of pediatric surgery in The Netherlands and 1973 population-based controls born between August 1988 and August 2012. Parental questionnaires were used to obtain information on fertility-related issues and potential confounders. RESULTS: In singletons, increased risks of anorectal malformations were observed for parents who underwent intracytoplasmic sperm injection (ICSI) or in vitro fertilization (IVF) treatment compared with fertile parents (odds ratio = 2.4 [95% confidence interval = 1.0-5.9] and 4.2 [1.9-8.9], respectively). For subfertile parents who conceived after IVF treatment, an elevated risk was also found when they were compared with subfertile parents who conceived without treatment (3.2 [1.4-7.2]). Among children of the latter category of parents, only the risk of anorectal malformations with other major congenital malformations was increased compared with fertile parents (2.0 [1.3-3.3]). No associations were found with intrauterine insemination or use of hormones for ovulation induction. CONCLUSIONS: We found evidence of a role of ICSI and IVF treatments in the etiology of anorectal malformations. However, subfertility without treatment increased only the risk of anorectal malformations with additional congenital malformations.

Early versus late reconstruction of cloacal malformations: the effects on postoperative complications and long-term colorectal outcome.

INTRODUCTION: Patients with a cloacal malformation generally undergo reconstructive surgery within the first years of life. However, the ideal age for surgery has rarely been mentioned. The aim of this study was to report differences in outcome between early (<6 months) and late repair of cloacal malformations. METHODS: Charts of patients with a cloacal malformation treated in 5 pediatric surgical centers between 1985 and 2009 were retrospectively studied for associated anomalies, postoperative complications, and colorectal and urological outcome. RESULTS: Forty-two patients were eligible for this study, giving a mean exposure of less than 1 patient yearly per center. Forty-five percent of the patients had a short common channel (>3 cm), and 14% had a long common channel. Length of common channel was missing in 41% of the patients. Median age of the cloacal reconstruction was 9 months (range 1-121 months). Twelve patients (29%) underwent an early surgical repair (within the first 6 months of age; median 3 months), and 30 (71%) patients underwent a late repair (after 6 months of age; median 14 months). Eighteen postoperative complications (<30 days) had been documented in 15 patients (35%), with significant more perineal wound dehiscences in patients with an early repair (42% vs. 10%, p=0.031). There were no differences in complication rate between patients with short and long common channels. Mean follow-up was 142 months (range 15-289). At the last follow-up, 10 patients (24%) had voluntary bowel movements. Fourteen patients (33%) had complaints of soiling, 25 (60%) were constipated, with no differences between the early and late repair groups. Patients in the late repair group as well as the group of patients with a short common channel were more frequently able to void spontaneously. CONCLUSIONS: Postoperative complications are common in patients with cloacal malformations. Early repair is associated with more wound dehiscences, however, without affecting long-term functional outcome. All centers had limited annual exposure of less than 1 patient. In these clinical settings, ideal age of cloacal reconstruction seems to be between 6 and 12 months. In general, centralized care for these complex malformations may be the crucial factor for reducing postoperative complications and better long-term outcome.

Transanal endorectal pull-through for classic segment Hirschsprung's disease: with or without laparoscopic mobilization of the rectosigmoid?

BACKGROUND: It has been suggested that the outcome of transanal endorectal pull-through for classic Hirschprung's disease can be improved by laparoscopically mobilizing the colon before the pullthrough. METHODS: Charts of 43 patients (2005-2009) with proven recto-sigmoid aganglionosis were retrospectively analyzed with respect to postoperative outcomes. Twenty-one had been treated with the transanal endorectal pull through (TERPT) and 22 with the laparoscopically assisted TERPT (LTERPT). RESULTS: Gender ratio, congenital anomalies, preoperative enterostomy, and follow up did not differ between the groups. More colon was resected in the TERPT group: median 25 cm vs. 15 cm in the L-TERPT group (p<0.001). The TERPT-procedure took less time: median 153 min. vs. L-TERPT 263 min (p<0.001). Postoperatively, three patients showed colonic torsions after TERPT (p=0.07). The long-term clinical outcomes did not differ significantly between both groups. There was a significant association between length of resection and obstructive symptoms (OR=0.92, p=0.01). CONCLUSION: Postoperative and clinical outcomes are similar using the TERPT or L-TERPT to correct classic segment Hirschsprung's disease. Prevention of colonic torsion should be the prime concern during the TERPT procedure. L-TERPT requires laparoscopic equipment and takes more operation time, whereas TERPT leaves no visible scars. The positive relation between the larger length of resection and obstructive symptoms requires additional research.