The congenital anomaly of female genital organ is one of the causes of primary amenorrhea. Among these, Rokitansky-Kuster-Hauser Syndrome is not uncommon, occuring ones in every 4,000~5,000 births. It involves the conginital absence of vagina,
cervix
and corpus uteri, and/or proximal fallopian tubes. In addition to the mullerian agenesis, skeketal and renal anomalies have been noted in affected young women.
And congenital adrenal hyperplasia is a main cause of female hermaphroditism and most affected individuals have a failure of 21-hydroxylation in enzymatic steps of steroidogenesis, and so lead to excessive production of adrenal androgens causing
virilization.
We report two vaginoplasties in patients of congenital anomaly of vagina, one is McIndoe operation in patient of Rokitansky-Kuster-Hauser Syndrome, and the other is vaginoplasty using perineal skin flap in patient of congenital adrenal
hyperplasia
including a review of the literature.
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