Author/Authors :
Tambo, F. F. Mouafo Pediatric Sugery service - Gyneco Obstetric and Paediatric Hospital, Yaoundé, Cameroon , Dahoun, S. University Teaching Hospitals of Geneva, Geneva, Switzerland , Kamadjou, C. Pediatric Sugery service - Gyneco Obstetric and Paediatric Hospital, Yaoundé, Cameroon , Makon, A. S. Nwaha Pediatric Sugery service - Gyneco Obstetric and Paediatric Hospital, Yaoundé, Cameroon , Fossi, G. Pediatric Sugery service - Gyneco Obstetric and Paediatric Hospital, Yaoundé, Cameroon , Andze, O. G. Pediatric Sugery service - Gyneco Obstetric and Paediatric Hospital, Yaoundé, Cameroon , Sosso, M. A. Department of Surgery and Subspecialties - Faculty of Medicine and Biomedical Sciences - University of Yaoundé, Yaoundé, Cameroon , Mure, P. Y. Lyon Teaching Hospital, Lyon, France
Abstract :
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.
