Читать книгу Ridley's The Vulva - Группа авторов - Страница 49

If the sub‐umbilical portion of the anterior abdominal wall fails to meet in the midline above the genital tubercles, the anterior bladder wall is partially or completely absent, leading to incomplete or total bladder exstrophy. It is always associated with epispadias and other abnormalities of the external genitalia. The clitoral bodies are bifid, and the mons pubis and labia protrude anteriorly. The vaginal introitus is situated anteriorly and is frequently stenosed. Magnetic resonance imaging can be very helpful in identifying the anatomical abnormalities and is used prior to surgery, which is usually done in stages [70,71]. Most patients require vaginoplasty in the teenage years.

A more severe form of this structural defect is cloacal exstrophy, in which there are defects of the urorectal structures, bladder, and terminal gastrointestinal tract [72]. Advances in neonatal surgery can now result in survival rates of up to 100%, and guidelines exist for management which should be undertaken by specialist multidisciplinary teams [73].