Transverse Vaginal Septum Resection: Technique, Timing, and the Utility of Dilation. A Scoping Review of the Literature.

STUDY OBJECTIVE: Transverse vaginal septae (TVS) are congenital, obstructive anomalies of the vagina typically presenting with abdominal pain and amenorrhea. Currently, the literature about surgical management is sparse. Postoperative complications are common. Vaginal dilation either pre- or postoperatively is sometimes recommended, the outcomes of which are poorly described. The aim of this scoping review was to guide surgical planning, namely, timing and postoperative care, to improve surgical outcomes. DESIGN, SETTING, PARTICIPANTS, AND MAIN OUTCOME MEASURES: This scoping review followed the Joanna Briggs Institute framework. The population of interest was patients 21 years of age or younger with a TVS. MEDLINE, OVID and CINAHL databases were searched using the subject headings listed herein. Literature written in English and French were included. Excluded were reports on patients with concurrent urogenital anomalies or severe developmental disabilities. The resultant articles were reviewed by 2 independent researchers. A third researcher was used in cases of disagreement. Using a descriptive analytical method, data were extracted from the included articles. RESULTS: We screened 1441 articles for eligibility; 41 articles met criteria, which described 152 cases of TVS. The most common management was simple vaginal excision. Five studies included outcomes from preoperative dilation, and 11 from postoperative dilation. There were 21 cases of postoperative vaginal stenosis; half (11/21) occurred without postoperative dilation. CONCLUSION: The goal of this review was to describe how TVS are managed and provide guidance for management options. Although the literature was sparse, the authors believe that management in a tertiary center, and surgical delay until the patient is ready to participate in vaginal dilation will improve outcomes for patients with TVS. Further prospective studies are needed to guide surgical planning.

Implications of Ehlers-Danlos Syndrome in a Patient With Mayer-Rokitansky-Küster-Hauser Syndrome.

BACKGROUND: Ehlers-Danlos syndromes (EDS) are a heterogenous group of connective tissue disorders characterized by defective collagen production. Patients with EDS have lax and fragile connective tissue in their joints, skin, blood vessels, and hollow organs. This can lead to, among other complications, joint hypermobility, aneurysms, organ prolapse, and musculoskeletal chronic pain. Given that patients with vaginal agenesis, which occurs with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, often require vaginal dilation as part of their treatment, tissue elasticity and fragility are important considerations. This case report aims to describe the intersection of MRKH and EDS and its impact on vaginal dilation. CASE: A 16-year-old girl with joint hypermobility and type III EDS presented with primary amenorrhea and a karyotype of 46 XX. Magnetic resonance imaging confirmed an absent uterus, cervix, and upper vagina. Physical examination showed Tanner V breasts and Tanner IV pubic hair, and an external genital examination revealed a blind-ending, 1-cm vaginal dimple. The patient was diagnosed with MRKH. Following her diagnosis, she received vaginal dilation instruction and returned for follow-up 2 months later, having quickly progressed to the largest vaginal dilator without symptoms of bleeding, pain, or dysuria. In that timeframe, her vaginal dimple had increased from 1 cm to 7-8 cm in depth, a rate much faster than is typically seen. Because of this rapid progress, a urogenital examination was performed. There was no evidence of urethral abnormality, perforation, or vaginal prolapse. SUMMARY AND CONCLUSION: Recognition of EDS in patients with Müllerian anomalies has important implications for safe and effective vaginal dilation. All patients using vaginal dilation to lengthen the vagina require education on the technique. This need is heightened in patients with EDS in order to prevent accidental dilation of the urethra due to their tissue elasticity, to avoid tissue prolapse, and to prevent the theoretical risk of vaginal perforation.