Pediatric urologists are frequently called upon to perform vaginoplasty for congenital or acquired female genitourinary anomalies at any age. There is a wide spectrum of disorders that might potentially be treated with a vaginoplasty.
Congenital female anomalies can be classified by which perineal structures are affected with the vagina. They include:
Isolated vaginal anomalies (figure 1) can exist with or without affecting the uterus and include partial or complete vaginal duplication, agenesis, or obstruction. A relatively common example associated with unilateral renal agenesis that presents with primary amenorrhea due to complete vaginal and uterine agenesis is the Mayer-Rokitansky-Küster-Hauser Syndrome.
Urogenital sinus anomalies (figure 2) can exist with or without external genital ambiguity in infancy and consist of internal fusion of the vagina and urethra. Urogenital sinus is most commonly due to congenital adrenal hyperplasia. The vagina and urethra drain onto the perineum via one shared orifice and the anorectum is normal.
Cloacal anomalies (figure 3) are the most severe neonatal genitourinary fusion anomalies wherein there is internal fusion of the vagina, urethra, and anorectum. The vagina, urethra, and anorectum drain onto the perineum via one shared orifice.
Acquired female genitourinary anomalies are secondary to other treatments. Examples include:
- vaginectomy (total or partial) due to cancer
- stenosis or complete loss of bowel or flap vaginoplasty
- postoperative vaginal stenosis (figure 4).
The commonality shared by all these conditions is the need for some form of vaginal reconstruction surgery. This article discusses current surgical options, with a focus on autologous buccal mucosa vaginoplasty.