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Endocrine Abstracts (2023) 95 OC8.1 | DOI: 10.1530/endoabs.95.OC8.1

BSPED2023 Oral Communications Oral Communications 8 (5 abstracts)

Is there reversal of virilisation after commencing medical replacement therapy for 46XX classical congenital adrenal hyperplasia (CAH)?

Supul Hennayake , Anju Goyal & Leena Patel


Royal Manchester Children’s Hospital, Manchester, United Kingdom


Genitalia appear larger and swollen in all neonates due to the circulating maternal hormones and this subsides spontaneously over few weeks or months. Additionally, general growth of the rest of the body outpaces any growth of the genitalia during childhood, resulting in apparent reduction in the size of the genitalia comparatively. There are high levels of circulating androgens in children with CAH until the commencement of medical replacement therapy (MRT). It is universally believed that the clitoris becomes smaller and virilisation reverses to some degree after starting MRT for CAH. Our aim was to assess changes in the clitoral length and virilisation parameters objectively.

Methods: Urogenital anatomy was prospectively studied in all consecutive 46XX CAH subjects by the first author from January 2003 to June 2023. The appearance of genitalia were described, and the stretched length of the clitoris was measured by the attending surgeon at the neonatal assessment. Subsequently, lengths of clitoris, common channel (CC), vagina and urethra were measured at an early cystoscopic assessment and later during feminising genitoplasty.

Results.: 38 patients had surgery during this period: 18% were Prader 5 and 76% were Prader 3 or 4. Eleven patients were excluded as they did not have cystoscopic assessments. There was no reduction in clitoral length or common channel length at any stage.

Table 1: Ultrasound Assessment of Thyroid Nodules
n=27Neonatal assessmentAt cystoscopy at median age of 10 months (range 3m-17m)At genitoplasty at median age of 20 months (range 4m-16 years)
Mean Clitoral length in mm (SD)17.15 (5.65)19.4 (6.22)28 (10.31)
Mean Common Channel length in mm (SD) 20 (6.14)25.8 (8.08)

Conclusion: Due to widely held belief regarding improvement in the appearance of genitalia after MRT, decision for genitoplasty is deferred till the extent of improvement is clear. However, serial assessment of clitoral and common channel lengths failed to show any actual reduction in size.

Volume 95

50th Annual Meeting of the British Society for Paediatric Endocrinology and Diabetes

Manchester, UK
08 Nov 2023 - 10 Nov 2023

British Society for Paediatric Endocrinology and Diabetes 

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