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AIM: To evaluate postoperative urinary continence in patients with congenital adrenal hyperplasia (CAH) with intermediate (IT) and high urogenital sinus (UGS) who underwent a UGS mobilization maneuver. METHODS: We called IT to those that although needing an aggressive dissection to get to the vagina, still have enough urethra proximal to the vaginal confluence. Very low variants are excluded from this analysis. Dissection always started in the posterior wall of the UGS with an aggressive separation from the anterior rectal wall. If the wide portion of the vagina was reached dissection stopped and the UGS opened ventrally widening to the introitus. Nineteen patients were treated using this maneuver (Group 1). When more dissection was required the anterior wall of the UGS was dissected and carefully freed from the low retropubic space. Then the UGS was opened either ventrally or dorsally. Thirty three patients required this approach (Group 2). Combined procedures were used in three patients with high UGS (Group 3). RESULTS: Mean age at the time of the repair and length of the UGS were 12.2 years (4 months-18 years) and 3.75 cm (3-8 cm) for G1; 8 years (5 months-17 years) and 6.34 cm (4-12 cm) in G2 and 8.3 years (2-14 years) and 11.5 cm (11-12 cm) in G3. All patients had been regularly followed. Mean age at last follow up was 14.3, 17, and 9.9 years for Groups 1, 2, and 3, respectively. All patients continue to void normally and are continent. All patients have two separate visible orifices in the vulva. Only three are sexually active. CONCLUSION: Urogenital sinus mobilization for vaginoplasty in girls with CAH does not compromise voiding function or urinary continence.
RESUMO
INTRODUCTION: Rectovaginal fistulas are a rare variety of anorectal malformations. Eight patients with this anomaly among 420 children with anorectal malformations were treated in our center. We used a laparoscopic approach in 5 of the children. The aim of this study is to define the feasibility and technical details of laparoscopy in the treatment of these patients as compared with those treated by posterior sagittal anorectoplasty (PSARP) and to evaluate the preliminary results. METHODS: Three patients were treated with a PSARP starting February 2000 (group 1 [GI]). The laparoscopic approach (laparoscopic treatment [LT]) was used in the next 5 girls starting in November 2002 (group 2 [G2]). All patients were operated on by the authors. In LT cases, we used 3 ports. The rectum was dissected laparoscopically, and the fistula was transected. An assisted anorectal pull-through was then performed using a minimal perineal incision. Clinical features, age at operation, associated anomalies, ratio/sacrum (sacral ratio [SR]), distal colostogram, operative findings, complications, urinary continence, voluntary bowel movements, constipation, soiling, and requirements of bowel management program were analyzed. Patients older than 3 years and 6 months and with more than 3 months after colostomy closure were included in the analysis of functional results. RESULTS: The mean age at the time of operation was 21.8 months. Urogenital anomalies were seen in 6 (75%) of the 8 girls. Two patients had SRs less than 0.6. Only 3 patients in each group were considered for evaluation of functional results. Mean age was 67 months in 3 (G1) and 64 months in 3 (G2) patients. All 6 patients were continent of urine. Only 1 in G1 had an SR less than 0.6. Voluntary bowel movements were present in 1 patient in G1 and 2 in G2. One patient in G1 had severe constipation and grade 2 soiling requiring bowel management program (SR <0.6), and 1 patient in G2 had occasional soiling. CONCLUSIONS: Laparoscopy allowed an optimal view of the pelvis and helped to achieve a low dissection of the fistula. Although the number of patients in this study is few, functional results with LT seem to be comparable to the open PSARP approach when done by experienced pediatric surgeons. The laparoscopic approach may be considered as an option for the treatment of this rare anomaly.
