Prolapse and sexual function 8 years after neovagina according to Shears: a study of 43 cases with Mayer-von Rokitansky-Küster-Hauser syndrome

Introduction and hypothesis: To investigate sexual and anatomical outcome after Shears neovagina in patients with Mayer-von Rokitansky-Kuster-Hauser syndrome (MRKH). Methods: Forty-three consecutive patients with MRKH syndrome underwent surgery creating a neovagina according to Shears. Follow-up was 8years. Sexual function was assessed using the Female Sexual Function Index (FSFI) as patient reported outcome; anatomical results were assessed measuring anterior, posterior and apical prolapse using the ICS Pelvic Organ Prolapse Score (ICS-POP Score). Pelvic floor contraction was measured applying the Oxford grading system. Results: Thirty-seven of the patients were regularly sexually active and filled in the FSFI; mean total FSFI was 27.2 (range 24-28). No significant prolapse of the neovagina was noted, 11 patients had a grade I cystocele, rectocele or apical descent that was asymptomatic. Oxford grading of the pelvic floor contraction was 4 in median (range 2-5). Conclusion: The neovagina according to Shears is a valuable surgical option with good sexual and anatomical outcome 8 years after therap

Isolated Langerhans cell histiocytosis of the vulva: a case report and review of the literature

Isolated Langerhans cell histiocytosis (LCH) of the female genital tract is very rare. A review of the literature revealed that only 15 cases of primary vulvar LCH have previously been published in the English literature. We describe an additional case of confined vulvar LCH. A 49-year-old woman presented with an ulcerous lesion that turned out to be LCH confined to the vulva only. After surgical excision, four recurrences followed, which were treated again by surgery. After the fourth recurrence, adjuvant radiotherapy was applied. When the fifth recurrence occurred, only surgical excision was performed, and the patient has now been disease-free for 51months. There are no standard treatment options for this rare disease. The most effective treatment options still remain elusive. In our case in the end surgery proved to be effectiv