Deferring surgical treatment of ambiguous genitalia into adolescence in girls with 21-hydroxylase deficiency: a feasibility study - Inserm - Institut national de la santé et de la recherche médicale Access content directly
Journal Articles International Journal of Pediatric Endocrinology Year : 2017

Deferring surgical treatment of ambiguous genitalia into adolescence in girls with 21-hydroxylase deficiency: a feasibility study

Abstract

AbstractBackgroundGenital surgery in Disorders of Sex Development (DSD) has been an area of debate over the past 20 years. Emerging scientific evidence in the late 1990s defied the then routine practice to surgically align genitalia to the sex of rearing, as early as possible. However, despite multitude of data showing detrimental effects to genital sensation and sexuality, few patients born with ambiguous genitalia have remained unoperated into adolescence.MethodsWe followed up girls with 21 hydroxylase deficiency (21- OHD) in genital morphology during childhood and acceptability among patients and parents of such an approach.ResultsPreliminary results from 7 children, aged 1–8 years (median 4.5 years), suggest that it is acceptable among patients and families to defer genital operation in 21-OHD. All patients had a Prader stage III and above. Median clitoral length at birth was 24 mm (20-28 mm) and had diminished to a median of 9 mm (5-15 mm) at their last visit. Height and weight have remained strictly normal in all patients. So far girls and their parents have not expressed significant concerns regarding genital ambiguity.ConclusionsWith this encouraging data at hand, we propose to formally address levels of anxiety, adaptation and quality of life during childhood, with an ultimate goal to assess long term satisfaction and effects on sexuality through deferring genital surgery for adolescence.
Fichier principal
Vignette du fichier
13633_2016_Article_40.pdf (913.71 Ko) Télécharger le fichier
Origin : Publication funded by an institution
Loading...

Dates and versions

inserm-01448493 , version 1 (28-01-2017)

Identifiers

Cite

Pierre Bougnères, Claire Bouvattier, Maryse Cartigny, Lina Michala. Deferring surgical treatment of ambiguous genitalia into adolescence in girls with 21-hydroxylase deficiency: a feasibility study. International Journal of Pediatric Endocrinology, 2017, 2017 (1), pp.3. ⟨10.1186/s13633-016-0040-8⟩. ⟨inserm-01448493⟩
189 View
130 Download

Altmetric

Share

Gmail Facebook X LinkedIn More