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Management of Partial Vaginal Atresia in Infancy: Early Experience

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Management of Partial Vaginal Atresia in Infancy: Early Experience Annals of Pediatric Surgery, Vol 2, No 3-4, July- October 2006, PP 174-179 Original Article Management of Partial Vaginal Atresia in Infancy: Early Experience Omar Mansour*, Hani Morsi** Departments of General Surgery* and Urology**, Cairo University Children's Specialized Hospital, Faculty of Medicine, Cairo University, Egypt Background/ Purpose: Transverse vaginal septum or partial vaginal agenesis is a subtype of vaginal atresia characterized by uterovaginal obstruction. This study was carried out to highlight the various patterns of clinical presentations, management options and outcome of this rare congenital anomaly. Materials & Methods: Eleven female patients presenting with manifestations related to uterovaginal obstruction during infancy were studied. Proper clinical evaluation and imaging studies including ultrasonography and computerized tomography were done. Cystovaginoscopy and laparoscopy were used for better delineation of the genitalia, adnexa and the uterus besides guiding for drainage and incision of the vaginal septum. Each patient was evaluated as regard to the age at presentation, presenting symptoms, associated malformations, imagining studies, operative details, and outcome Results: The main presenting symptoms were related to genitourinary obstruction. Endoscopic guided laparoscopic assisted division of the septum was done in 6 patients. Preliminary vaginostomy followed by vaginal pullthrough was done in 3 patients, and only endoscopic incision of the septum was done in 2 patients. No reported major complications were encountered during 1 year follow up. Conclusion: Diagnosis of transverse vaginal septum is still a challenge during early infancy .Division of the septum together with vaginal pull-through was found to facilitate marsupialization of the incised septum to the edges of the introitus. Incision of the septum guided by laparoscopy and vaginoendoscopy can minimize accidental injuries to the bladder, rectum and urethra. vaginostomy and staged repair is advisable in confusing cases. Index Word: Vaginal atresia, hydrometrocolpos, septum, marsupialization. INTRODUCTION aginal atresia is an uncommon congenital Consequently, vaginal atresia occasionally is termed V anomaly resulting in uterovaginal tract Müllerian agenesis. 1 obstruction. According to the classification of Between the sixth and eighth weeks of fetal uterovaginal anomalies by the American Fertility development, the caudal aspects of the bilateral Society, complete vaginal atresia is categorized under Müllerian ducts fuse at the midline. A single midline agenesis or dysgenesis of the Müllerian ductal system. tubular structure forms as a consequence of cell proliferation (i.e., uterovaginal canal). This midline 88 Correspondence to: Omar Mansour, MD, Department of General Surgery, Cairo University children's Specialized Hospital, Faculty of Medicine, Cairo University, E mail: [email protected] Mansour O& Morsi H structure extends to the Müllerian tubercles, where it PATIENTS AND METHODS encounters the urogenital sinus. Bilateral endodermal In this study, we present 11 female infants that invaginations (i.e., sinovaginal bulbs) form as the presented with symptoms related to uterovaginal Müllerian tubercles regress. Canalization of the tract obstruction, upper or lower urinary tract uterovaginal canal is believed to occur from the obstruction (LUTS). Clinical examination, endoscopic caudal to the cephalic aspect, with an epithelial lining cystovaginoscopy and radiological investigations derived from the urogenital sinus. Failures at the revealed that they all had partial vaginal atresia. Data vaginal plate level may explain the transverse vaginal collected from patients records included the age at septum variants. 2 presentation, presenting symptoms, and associated Vaginal atresia is estimated to occur in 1 in 4000- malformations. 5000 live female births. Often, the anomaly is The clinical records and radiological findings undetected until adolescence, when primary together with laboratory findings were reviewed. amenorrhea or abdominal pain from an obstructed Structural anatomy was determined by clinical uterovaginal tract prompts a diagnostic evaluation. 3 examination, ultrasonography, and computerized Transverse vaginal septum, or partial vaginal tomography. Cystovaginoscopy and laparoscopy was agenesis, is less common, with a reported incidence of used to further delineate the genitalia, adnexa and 1 in 70,000 females.4 Variants of vaginal atresia, uterus and facilitate the operative technique. Timing formerly termed partial vaginal agenesis, currently and type of surgical intervention were studied. are classified more correctly as variants of transverse Findings during a period of one-year follow up were vaginal septum. So, failure of vaginal canalization at discussed. various levels of the vaginal plate results in the transverse septum. The most common location of the transverse septum is the upper vagina, followed by RESULTS the middle, with the least common location being the Age at presentation ranged between 2 weeks and lower third. 3 4 years but the majority were below 1 year. Mean age The most common clinical presentation of vaginal was 7.5 months. Presenting symptoms were mainly atresia occurs in conjunction with an absent uterus, related to genitourinary obstruction. Urinary outlet which is termed Rokitansky-Küster-Maier-Hauser obstruction was reported in 6 patients (54.5%), syndrome (RMKH) syndrome. Uterovaginal atresia in recurrent lower abdominal pain in 5 patients (45.5%), those patients with RMKH syndrome is best pelviabdominal mass in 5 patients (45.5%) and 3 explained by the failure of the caudal development of infants presented by upper urinary tract obstruction the Müllerian ducts. Vaginal atresia occurs also in (hydroureteronephrosis) secondary to compression of conjunction with renal anomalies, such as (ectopic both ureters by the pelviabdominal mass, with kidney(s), horseshoe kidney, and crossed-fused elevated serum creatinine level (Table.1). ectopia), occur in 30% of these patients. 5 Classic findings using pelvic US and CT revealed Obstructed uterovaginal anomalies typically the presence of a well-defined cystic round structure present either in the neonatal period or at menarche. posterior to the bladder and anterior to the rectum, Hydrometrocolpos is the accumulation of secretions compressing both of these structures . There was rim in the vagina and uterus caused by the combination of enhancement and displacement of the uterus intrauterine stimulation of the infant’s mucous glands superiorly. The uterus was felt to be unremarkable in by circulating maternal estrogen and an obstruction of size, but fluid was noted in the endometrial cavity. the genital tract by an intact hymen, vaginal septum The diagnosis of hydrometrocolpos was suggested in or vaginal atresia. most of the cases. If the accumulation of secretions is limited to the The selection of operative interventional technique vagina, the condition is termed hydrocolpos. The was dictated by the clinical presentation of the typical presentation of these conditions in a newborn patient, genitourinary obstruction and depth of the is as a lower midline abdominal mass or a bulging vaginal canal. perineal mass. Dysuria or urinary retention also may be present. 6 175 Vol 2, No 3-4, July - October 2006 Mansour O& Morsi H Operative details: In 6 patients (55.5%), the vaginal septum was found to be in the upper third of the vagina with distal Laparoscopic assisted endoscopic guided division hypoplastic vagina. Incision of the vaginal septum of the septum was done in the last 6 patients in this was initially done followed by mobilization of the study. The procedure started by endoscopic proximal part of the vagina down to the introitus. examination of the urethra and vagina. Then This pullthrough procedure was facilitated by laparoscopic exploration was followed by incision transabdominal or transperineal dissection both and evacuation of the distended proximal vagina. A anterior and posterior to the distended proximal dilator was inserted inside the proximal vagina to vagina. guide the site of endoscopic guided incision of the vaginal septum avoiding injury to the urethra and rectum in particular. No patients with uterine or adenexal agenesis were detected in this study. Table 1. Patient's Data Age at Presenting symptom Associated anomalies Management Presentation 1 6 weeks Pelviabdominal mass Bilateral Endoscopic incision of the hydrouretronephrosis vaginal septum. 2 8 weeks Genitourinary Congenital heart disease, VSD Vaginostomy, Later obstruction, UT transperineal vaginal infection pullthrough mobilization. 3 23 weeks Pelvi abdominal mass. Congenital heart disease, Vaginostomy, Later ASD, Haemangima. transperineal vaginal pullthrough mobilization. 4 21 weeks Genitourinary Left vesicoureteric reflux Endoscopic incision of the obstruction, Dysuria, disease. vaginal septum. UT infection. 5 14 weeks Genitourinary Left hydronephrosis, Down’s Laparosopically assisted obstruction Syndrome. endoscopic incision of the septum.+Marsupialization. 6 14 months Genitourinary Vesicoureteric reflux. Bilateral Vaginostomy, Later obstruction hydronephrosis. transperineal vaginal pullthrough 7 23 months Pelvi abdominal mass, Left vesicoureteric reflux. Laparoscopic Assisted, UT infection Endoscopic incision of the septum+ Marsupialization. 8 2 years Genitourinary - Laparosopically assisted obstruction, endoscopic incision of the Pelviabdominal mass. septum +Marsupialization.
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