Saturday, June 15, 2024

Vaginal Atresia

Background

Vaginal atresia is a congenital defect resulting in uterovaginal outflow tract obstruction. It occurs when the caudal portion of the vagina, contributed by the urogenital sinus, fails to form. This caudal portion of the vagina is replaced with fibrous tissue. Vagina atresia and agenesis are congenital anomalies of the female genitourinary tract and may occur as an isolated developmental defect (extremely rare) or as part of a complex of anomalies (more common). Examples of some of these associations are as follows:

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome,

also referred to as  Mayer-Rokitansky syndrome, or Rokitansky-Küster-Hauser syndrome, or, simply, Rokitansky syndrome

Bardet-Biedl syndrome

Kaufman-McKusick syndrome

Fraser syndrome

Winters syndrome

In 1998, the American Society for Reproductive Medicine classified the uterine anatomic types as müllerian anomalies or vaginal anomalies. According to this classification system, vaginal atresia is an anomaly classified as type I, which refers to agenesis and hypoplasia of the uterus.
As a consequence, vaginal atresia is occasionally termed müllerian agenesis.

The most common clinical presentation of type I müllerian anomalies is vaginal or müllerian agenesis, which occurs in conjunction with an absent uterus (ie, MRKH syndrome). Renal anomalies, which may include unilateral agenesis of the kidney, ectopic kidneys, horseshoe kidney, and crossed-fused ectopia, occur in 30% of patients with MRKH syndrome. Associated skeletal anomalies may include anomalies found in Klippel-Feil syndrome (ie, aberrations of cervicothoracic somite development), which manifest as fused vertebrae or other variants. Anomalies of the ribs and limbs are also encountered.

Variants of vaginal atresia, formerly called partial vaginal agenesis, are more correctly classified as variants of a transverse vaginal septum. These variants and other developmental variants, such as obstructed duplications of the uterovaginal tract, occur on the basis of other pathophysiologic events and should be treated as separate entities.

Each examination of a newborn should include an inspection of the genitalia, as absence of the vagina or atresia could be detected during this simple evaluation. Delayed detection is common and can result in major risk to the female patient because of associated urinary tract anomalies. Early detection is possible if healthcare providers include a genital examination as part of their well-child examination.

Surgical management of vaginal atresia has been described in the European literature since the turn of the century. In the United States, Baldwin was the first to describe construction of a neovagina using an intestinal graft. As the technique became popular, the potential for clinically significant morbidity and mortality became evident. In 1940, Masson reported two deaths,
which provided the impetus to consider the skin-graft technique McIndoe and Counsellor first reported.

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