ESPE Abstracts (2014) 82 P-D-2-3-503

Survey of Opinion on the Antenatal and Surgical Management of Disorders of Sex Development and Congenital Adrenal Hyperplasia

Mary Whitea & Jacqueline Hewitta,b

aMurdoch Children’s Research Institute at The Royal Children’s Hospital, Melbourne, Victoria, Australia; bUniversity of Melbourne, Melbourne, Victoria, Australia

Background: Congenital adrenal hyperplasia (CAH) is characterised by elevated adrenal androgens which can lead to virilisation of affected females.

Objective and hypotheses: To outline clinical opinion on two controversial aspects of CAH management (antenatal dexamethasone and genital surgery of affected females) which was hypothesised would differ between regional centres.

Methods: A survey was conducted via the Australian Paediatric Endocrine Group (APEG).

Results: There were 52 participants comprising consultant endocrinologists (75%), endocrinology trainees (11.5%), nurse specialists (1.9%), and others (11.5%) including geneticists, surgeons, and gynaecologists. Respondents were practicing in all regions of Australia (86.5%), New Zealand (7.7%), and a small group in international locations (5.8%). A multidisciplinary group for the management of disorders of sex development (DSD) was in practice for 75% of the participants; however only 14% experienced a specialised clinic for DSD. Forty per cent had personal experience of prescribing antenatal dexamethasone, 33% were confident that the risks of this treatment were adequately conveyed to the parents, 90% had concerns with the prescription of antenatal dexamethasone, and 4% thought the standard dosing is appropriate. Most respondents thought that antenatal dexamethasone should be provided only as part of prospective research trial (59.6%) or long-term follow-up registry (11.5%). Regarding genital surgery, most respondents thought that surgery at any age is only indicated in Prader stages III–V virilisation (69.2%), with 17.3% recommending surgery only at Prader stage IV or V. When early surgery was considered, the most popular recommendation was for surgery to occur at 6–12 months (46%), followed by 12–18 months (22%). The majority of respondents supported universal neonatal screening for CAH (88.5%).

Conclusion: These results show that although opinions were broadly within the scope of international consensus, significant differences remain regarding critical aspects of care. A comparison with international opinion will follow.

Article tools

My recent searches

No recent searches.