A 14 year old girl was referred with a diagnosis, made at birth, of congenital adrenal hyperplasia. She had undergone clitoral reduction and vulvovaginoplasty at age 2. Her height had been on 10th-25 th centile, and, at presentation to us, she was taking prednisolone (7.5milligrams in divided doses) and fludrocortisone 0.05 milligrams. At age 14 her height dropped to the 3 rd centile. She was hirsute, obese and had abdominal striae. A variety of steroid regimes only led to an increase in her cushingoid features.
At age 17 she had primary amenorrhoea and marked masculine features. Her blood markers were rising with a free androgen index (FAI) of 65 (normal<6), testosterone of 12.4 nanomoles per litre (normal <2.8) and 17-hydroxyprogesterone (17-OHP) of 407 nanomoles per litre (normal <12). We proceeded to laparoscopic bilateral adrenalectomy because of difficulty in controlling her symptoms and signs medically, and because of our unit's extensive experience of bilateral adrenalectomy, for other conditions, with previously reported low morbidity and mortality.
There were no complications and she was discharged on day 4 post-operatively on maintenance glucocorticoids and mineralocorticoids. Serum Testosterone, FAI and 17- OHP levels quickly normalized. At 18 months post-operatively her 17-OHP level was slightly raised at 15. Menarche began 6 months post-operatively and she has a regular cycle. She is not cushingoid, is less hirsute but remains overweight.
The literature reports 18 cases of bilateral adrenalectomy for congenital adrenal hyperplasia. No operative complications occurred and 6 out of 7 female patients of childbearing age had more regular menstrual cycles; the 7 th was found to have an absent uterus. Obesity remains a problem in some.
Since outcomes after adrenalectomy in experienced regional centres are good, we suggest that bilateral adrenalectomy be considered more often as a treatment for this difficult group of patients.
22 - 24 Mar 2004
British Endocrine Societies