African Journal of Paediatric Surgery (2016-01-01)

Mixed gonadal dysgenesis in Yaoundé: A preliminary experience about three cases

  • F F Mouafo Tambo,
  • S Dahoun,
  • C Kamadjou,
  • A S Nwaha Makon,
  • G Fossi,
  • O G Andze,
  • M A Sosso,
  • P Y Mure

DOI
https://doi.org/10.4103/0189-6725.187822
Journal volume & issue
Vol. 13, no. 3
pp. 145 – 149

Abstract

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Mixed gonadal dysgenesis is characterised by unilateral chromosomal abnormality, which is probably the result of anaphase lag during mitosis. The 45, XO/46, XY karyotype is the most common form of mosaicism involving the Y chromosome. It is a rare clinical entity with a worldwide incidence of 1.5/10,000 live births. Its epidemiology in Sub-Saharan Africa is not known. This study reports experience in the management of 3 cases at the Yaounde Gynecologic-Obstetric and Paediatric Hospital. From November 2009 to November 2014, 3 cases were successfully managed at our institution. Results: All patients presented with asymmetrical gonadal differentiation. On one side of the body, a poorly-developed testicular gonad and on the other side a gonadal streak. A persistent Mόllerian remnant was equally found in the 3 cases. Management of mixed gonadal dysgenesis should be done in tertiary health care centres. A multidisciplinary team approach is recommended.

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