Vaginoplasty through Mayer technique in Rokitansky-Hauser-Kuster Syndrome. Apropos of a case

Abstract

The Rokitansky-Hauser-Kuster Syndrome is a serious congenital abnormality of the female reproductive tract. It has an embryologic etiology by a failure of fusion of the mullerian ducts. It may coincide with other kidney and skeletal malformations, mainly of the spine and ribs, and others in the abdominal cavity. The karyotype is 46xx, therefore, the patient may or may not have uterus, fallopian tubes, ovaries, normal hair distribution, or may also present agenesis of the genital organs. There is a familial appearance and an autosomal dominant mutation linked to sex. Its diagnosis is presumptive in childhood and adolescence. The clinical, diagnostic and therapeutic characteristics of a patient with vaginal agenesis are presented, as well as the creation of an artificial vagina.

Keywords

• anomalías congénitas/etiología
• conductos de müller
• procedimientos quirúrgicos ginecológicos