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PATHOGENESIS OF
IMPERFORATE HYMEN
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The lumen of the vagina is separated from the cavity of the urogenital sinus
by the hymen until late in fetal life. The hymen usually ruptures in the
perinatal period and remains as a fold of mucous membrane around the entrance
of the vagina (1). Failure of this membrane to rupture results in an
imperforate hymen. Fetal cervical and vaginal secretions that are present
secondary to circulating maternal estrogens (2) accumulate because the
imperforate hymen results in an outflow of obstruction. This results in
hydrocolpos and hydrometrocolpos presenting as a pelvic mass.
- Persistent urogenital sinus
(due to virilization of female fetus / intersex anomaly / arrest of normal
vaginal development).
- Single exit chamber
for bladder and vagina.
- Separate orifice for
the anus.
- Associated with
ambiguous genitalia.
- Cloacal malformation (early
embryonic arrest).
- Single perineal
orifice for bladder, vagina and rectum.
- Associated with duplex
genital tract.
- Imperforate hymen, transverse
vaginal septum, segmental vaginal atresia, imperforate cervix, blind horn
of bicornuate uterus, Rokitansky-Hauser syndrome (agenesis of the uterus
and vagina with active uterine analge).
- Interlabial mass.
- May be associate with
imperforate anus, hydronephrosis, renal agenesis/dysplasia, polycystic
kidneys, duplication of vagina and uterus, sacral hypoplasia, esophageal
atresia.
- Moore KL. The urogenital
system. In: The Developing Human: clinically orientated embryology. 3rd
ed. Philadelphia: WB Saunders 1982:255-297.
- Spence HM. Congenital
hydrocolpos. JAMA 1962;180:1100-1105.