True hermaphroditism in southern Africa: the clinical picture.
This is an 18-year (1985-2001) retrospective review of 85 patients with true hermaphroditism, with the aim of facilitating early recognition of this condition. Presentation of neonates and infants 6 months or younger, constituting 54% of this cohort, were different from the older children. The presentation, clinical features and investigative results of all patients diagnosed with true hermaphroditism at a single South African paediatric surgical unit were reviewed. This paper highlights the previously reported high incidence (51%) of this condition, as well as some of the unusual features of true hermaphroditism in this region. Diagnosis of true hermaphroditism requires a high index of suspicion for subtle anomalies of the genitalia. Although there were no pathognomonic clinical features, the true hermaphrodite presents as a patient of either gender with a congenital anomaly of the genitalia. The child is likely to have a normal male phallus, bifid labio-scrotal folds, a perineal hypospadias and in 53% of patients there was a palpable gonad. The method of investigation, together with the results and some of the management dilemmas associated with true hermaphroditism in a Third World population are presented.
Wiersma
R
R
Department of Paediatric Surgery, University of Natal, Private Bag 7, 4013 Congella Durban, South Africa. wiersma@ukzm.ac.za
D000293:Adolescent; D044383:Black People / Q000706:statistics & numerical data; D002648:Child; D002675:Child, Preschool; D012734:Disorders of Sex Development / Q000097:blood / Q000175:diagnosis* / Q000453:epidemiology; D005260:Female; D005836:Genitalia, Female / Q000002:abnormalities; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010413:Penis / Q000002:abnormalities; D012189:Retrospective Studies; D013019:South Africa / Q000453:epidemiology; D014521:Urethra / Q000002:abnormalities