Читать книгу Ridley's The Vulva - Группа авторов - Страница 41

The Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is the commonest form of Müllerian agenesis, occurring in about 1 in 4500 newborn females. The uterus, cervix, and upper vagina are absent in otherwise phenotypically normal 46,XX females. It presents at puberty when amenorrhoea is investigated. The vulva appears normal, but a short, blind‐ending vagina is found. MRKH type 1 affects only the upper reproductive tract, but type 2 is associated with renal (40‐60%), skeletal and, rarely, cardiac and hearing abnormalities [42,43]. Most cases appear to be sporadic but familial cases are described, and various associated genetic abnormalities have been studied [44,45].

Management of these patients requires a specialist multidisciplinary approach with expert psychological counselling and management advice [46]. The long‐standing technique described by Frank [47] of regular dilatation can create a neovagina in 95% of cases [48]. Several surgical techniques for vaginoplasty have been used with varying success, but sexual satisfaction is better with a non‐surgical approach.