Female Children with Ambiguous Genitalia in Awareness- Poor Subregion

Osarumwense D Osifo, Taiwo I Amusan

Abstract

Congenital aberrations of female children’s external genitalia are common worldwide with varied mode of presentation especially in regions with poor awareness. This prospective experience between July 2004 and June 2008 at two Nigerian healthcare facilities is on the mode of presentation and challenges of management of female children with ambiguous genitalia. Congenital adrenal hyperplasia (CAH) 19 (47.5%), female pseudohermaphroditism 20 (50%) and vaginal atresia 1 (2.5%) manifested as aberrations of external genitalia of 40 female children who presented between the ages of 3 months and 16 years (average 9 years). Cultural influence, lack of awareness, inadequate examination of external genitalia at birth and lack of diagnostic facilities resulted in late presentation and diagnosis with all the cases of CAH and pseudohermaphroditism raised as males. Five cases who developed female secondary sexual characteristics at puberty attempted suicide before presentation. Gender reassignment and feminizing genitoplasty were major challenges, but outcomes were encouraging (Afr J Reprod Health 2009; 13[4]:129-136).

 

RĖSUMĖ

 

Les filles qui ont des organes génitaux ambigus dans une sous-région peu sensibilisée. Les aberrations congénitales des organes génitaux externes chez les filles sont communes partout dans le monde, ayant des divers modes de présentation dans les régions qui sont peu sensibilisées.  Cette expérience prospective entre juillet 2004 et juin 2008 dans  deux établissements de santé est basée sur le mode de présentation et les défis du traitement des filles qui ont des organes génitaux ambigus.  L’hyperplasie surrénale congénitale (HSC) 19 (47,5%) le pseudohermaphrodisme 20 (50%) et l’atrésie vaginale 1 (2,5%) se sont manifestées comme des aberrations des organes génitaux externes des 40 filles qui se sont présentées entre les ages de 3 mois et 16 ans (âge moyen – 9 ans).  L’influence culturelle, le manque de conscience, l’insuffisance de l’examen des organes génitaux à la naissance et le manque des établissements d’analyses médicales ont abouti à la présentation et au diagnostic tardifs, ce qui fait que tous les cas de HSC et du pseudohermaphrodisme ont été élevé comme des mâles.  Cinq cas qui ont développé des caractéristiques sexuelles secondaires féminines ayant atteint l’âge de puberté ont tenté de se suicider avant de se présenter.  La réattribution de sexes et la génioplastie féminisante ont été des défis principaux, mais les résultats ont été encouragements (Afr J Reprod Health 2009; 13[4]: 129-136).

 

 

KEYWORDS: Congenital aberration, Ambiguous genitalia, Female children 

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