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J Gynecol Obstet Biol Reprod (Paris) ; 43(6): 470-3, 2014 Jun.
Article in French | MEDLINE | ID: mdl-23490409

ABSTRACT

The androgen insensitivity syndrome (AIS) formerly known as testicular feminization is transmitted on a recessive X-linked. Its diagnosis is usually made at puberty if primary amenorrhea is present because the external genitalia are female. We report the case of two sisters aged 30 and 18 years, with testicular feminization syndrome discovered during the exploration of primary amenorrhea. Clinical examination found fairly well developed mammary glands, an hypertrophied clitoris, two formations in the labia majora corresponding to the testicles and agenesia of vagina. The uterus has not been seen on pelvic ultrasound and computed tomography scan of the pelvis. Cytogenetic analysis has objectified the chromosomal formula: 46 XY. We performed a vaginoplasty using peritoneum from the pouch of Douglas, a clitoroplasty and bilateral orchiectomy. The result was excellent. A neovagina with a depth of 6 cm was created. After 6 months of follow-up, the two sisters keep beautiful vaginal cavities with regular digital dilation. Peritoneum vaginoplasty is a simple and easy method for creating a neovagina with minimal complications. Its anatomical and functional results are satisfactory.


Subject(s)
Androgen-Insensitivity Syndrome/surgery , Clitoris/surgery , Plastic Surgery Procedures/methods , Surgically-Created Structures , Vagina/surgery , Adolescent , Adult , Androgen-Insensitivity Syndrome/genetics , Douglas' Pouch/surgery , Female , Humans , Male , Orchiectomy/methods , Siblings , Treatment Outcome
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