Mp50-07 Intravaginal Longitudinal Ridge: A Previously Undescribed Predictor of Ipsilateral Dysgenetic Testis in Patients With Disorders of Sex Development

You have accessJournal of UrologyPediatrics III (MP50)1 May 2024MP50-07 INTRAVAGINAL LONGITUDINAL RIDGE: A PREVIOUSLY UNDESCRIBED PREDICTOR OF IPSILATERAL DYSGENETIC TESTIS IN PATIENTS WITH DISORDERS OF SEX DEVELOPMENT Brett Teplitz, Esther Liu, Moira Dwyer, Pankaj Dangle, Miguel Reyes-Mugica, Svetlana Yatsenko, Selma Witchel, Omar Ayyash, and Francis Schneck Brett TeplitzBrett Teplitz , Esther LiuEsther Liu , Moira DwyerMoira Dwyer , Pankaj DanglePankaj Dangle , Miguel Reyes-MugicaMiguel Reyes-Mugica , Svetlana YatsenkoSvetlana Yatsenko , Selma WitchelSelma Witchel , Omar AyyashOmar Ayyash , and Francis SchneckFrancis Schneck View All Author Informationhttps://doi.org/10.1097/01.JU.0001008684.57262.97.07AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: In patients with disorders of sex development (DSD), endoscopy and diagnostic laparoscopy are often utilized to evaluate internal anatomy. When there exists a concern for dysgenetic testes, laparoscopic gonadectomy is performed to prevent potential development of gonadoblastoma. Here, we describe an anterolateral longitudinal vaginal ridge (VR) that we have consistently observed to be associated with an ipsilateral dysgenetic testis. This has not been previously described in the pediatric urologic literature. METHODS: 37 children with DSD underwent endoscopic evaluation for genital ambiguity by a single surgeon at our institution since 1995. Retrospective chart reviews were performed to obtain demographic, karyotype, anatomic, and pathologic diagnoses. Tissue biopsies of the observed VRs were not performed due to the poor risk-to-benefit ratio for the patients. Photographic and videographic footage was obtained for several representative patients (Figure 1). RESULTS: 8 children with DSD who underwent vaginoscopy have been identified as having longitudinal VRs. 7 of 8 patients were 46XY DSD and 1 of 8 was 46XX with a WT1 mutation. 3 of 8 patients had bilateral VRs. 5 of 8 patients had unilateral VRs identified extending from the cervical os to the introitus. In all 6 patients with both VRs and diagnostic laparoscopy, the side(s) with a VR have correlated with a dysgenetic testis, whereas the side(s) without a VR had an absent gonad (Table 1). 1 of 6 patients with available pathology had germ cell neoplasia in situ (16.7%). CONCLUSIONS: This is the first description of a longitudinal VR in patients with gonadal dysgenesis. All patients with gonadectomies or gonadal biopsies were found to have dysgenetic testicular tissue ipsilateral to the vaginal finding. Although the exact embryological nature of VRs is currently unknown and previously undescribed, given the lateral location of the Wolffian duct in relation to the Mullerian duct, we hypothesize these VRs are evidence of persistent Wolffian ducts. Download PPT Source of Funding: None © 2024 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 211Issue 5SMay 2024Page: e832 Advertisement Copyright & Permissions© 2024 by American Urological Association Education and Research, Inc.Metrics Author Information Brett Teplitz More articles by this author Esther Liu More articles by this author Moira Dwyer More articles by this author Pankaj Dangle More articles by this author Miguel Reyes-Mugica More articles by this author Svetlana Yatsenko More articles by this author Selma Witchel More articles by this author Omar Ayyash More articles by this author Francis Schneck More articles by this author Expand All Advertisement PDF downloadLoading ...