[Androgen insensitivity syndrome--testicular feminization--Morris syndrome. A surgical procedure in cases when a testis is found during herniotomy in a girl]

Ugeskr Laeger. 1994 Nov 21;156(47):7057-8.
[Article in Danish]

Abstract

The androgen insensitivity syndrome is uncommon, but still the most frequent male pseudohermaphroditism. It is an X-linked recessive disorder characterized by androgen receptor defectiveness of various intensity. In the state of complete insensitivity, the male karyotype 46-XY develops a female gender with bilateral testes. Inguinal hernia is a common clinical presentation for a young child with complete androgen insensitivity. Thirty-five to forty percent of those with Morris syndrome come out with incarcerated hernias containing a testis, some even pass unnoticed at elective herniorraphy on apparently normal infant girls. It is recommended to perform orchiectomy when a testis is found during surgery. Subsequently the patient should be referred to major centres for further and thorough surveys.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Androgen-Insensitivity Syndrome* / diagnosis
  • Androgen-Insensitivity Syndrome* / genetics
  • Androgen-Insensitivity Syndrome* / surgery
  • Female
  • Hernia, Inguinal / diagnosis
  • Humans
  • Infant
  • Male
  • Orchiectomy