MES2008 Oral Communications (1) (7 abstracts)
Bilateral adrenalectomy in a patient with congenital adrenal hyperplasia
Owais Chaudhri 1 , Emma Hatfield 1 , Katie Wynne 2 , Fausto Palazzo 1 , Sanjeev Mehta 1 , Humera Shaikh 1 , Tricia Tan 1 , Niamh Martin 1 & Karim Meeran 1
1Endocrine Unit, Department of Investigative Medicine, Imperial College Healthcare NHS Trust, London, UK; 2Department of Endocrine Surgery, Imperial College Healthcare NHS Trust, London, UK.
We present the case of a 33-year-old woman diagnosed with congenital adrenal hyperplasia (CAH) due to classical 21-hydroxylase deficiency following a salt-losing crisis as a newborn. She had previously been controlled on dexamethasone 0.25 mg bd and fludrocortisone 100 mcg od, as evidenced by high-normal 17-hydroxyprogesterone (17-OHP) and low-normal testosterone levels (8.5 and 0.4 nmol/l respectively).
However, she had experienced significant symptoms of Cushing’s syndrome on this replacement regimen (body mass index 34.6 kg/m2, striae). A reduction in the dose of dexamethasone resulted in secondary amenorrhoea and distressing hirsutism refractory to treatment by conservative measures. Efforts to identify a steroid replacement dose that adequately suppressed adrenal androgen production without causing further progression of her iatrogenic Cushing’s syndrome were unsuccessful.
The patient underwent a long synacthen test to characterise the level of residual adrenal cortisol synthesis. Cortisol levels were undetectable throughout, in the presence of a significant rise in 17-OHP levels (peak levels 54.6 nmol/l), confirming complete blockade of the 21-hydroxylase enzyme. Bilateral adrenalectomy was therefore considered as a treatment option to permit the use of lower doses of exogenous steroid and removing the source of excess androgens.
She underwent an uncomplicated bilateral laparoscopic adrenalectomy in October 2007. One year later, she is maintained on hydrocortisone replacement (10 mg am, 5 mg lunchtime) and has gradually lost weight. Symptomatically, she has improved significantly.
Discussion will revolve around the use of adrenalectomy to overcome the difficult need to balance adequate suppression of adrenal androgen production vs weight gain in women with CAH.
Volume 18
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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