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True hermaphroditism in southern Africa: the clinical picture

R Wiersma1

  • 1Department of Paediatric Surgery, University of Natal, Private Bag 7, 4013 Congella Durban, South Africa. wiersma@ukzm.ac.za

Pediatric Surgery International
|May 28, 2004
PubMed

Insights

Early recognition of true hermaphroditism is crucial. This study highlights subtle genital anomalies and varied presentations in infants, emphasizing the need for high suspicion in diagnosing this rare condition.

Area of Science:

  • Pediatric Surgery
  • Endocrinology
  • Genetics

Background:

  • True hermaphroditism is a rare disorder of sexual development.
  • Early diagnosis is essential for appropriate management and psychological well-being.
  • Previous studies have reported varying incidences and presentations.

Purpose of the Study:

  • To review cases of true hermaphroditism over 18 years.
  • To identify key clinical features aiding early recognition.
  • To discuss diagnostic and management challenges in a South African pediatric surgical unit.

Main Methods:

  • Retrospective review of 85 patients diagnosed with true hermaphroditism (1985-2001).
  • Analysis of patient presentations, clinical features, and investigative results.
  • Focus on neonates and infants younger than 6 months.

Main Results:

  • True hermaphroditism presented differently in neonates/infants compared to older children.
  • A high incidence (51%) was noted, with unusual regional features.
  • Common findings included ambiguous genitalia, bifid labio-scrotal folds, perineal hypospadias, and palpable gonads in 53% of cases.

Conclusions:

  • Diagnosis requires a high index of suspicion for subtle genital anomalies.
  • No pathognomonic features exist; presentation is typically a child with ambiguous genitalia.
  • Management dilemmas in a Third World setting are presented.

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