Congenital adrenal hyperplasia 11b-hydroxylase deficiency: two cases managed with bilateral adrenalectomy

John M, Menon SK, Shah NS, Menon P
Correspondence: Dr Mathew John, drmathewjohn@yahoo.com

ABSTRACT
This series describes two patients with congenital adrenal hyperplasia due to 11ß-hydroxylase deficiency. The first patient, a ten-year-old with XX genotype, reared as a male, presented with resistant hypokalaemia and hypertension. The second patient, a 23-year-old with XY genotype, presented with bilateral adrenal masses and resistant hypertension. Both the patients were offered bilateral adrenalectomy. These two patients are described with a discussion on the role of bilateral adrenalectomy in the management of difficult cases of congenital adrenal hyperplasia. The association of myelolipoma and testicular rests with this condition is also discussed.

Keywords: 11ß-hydroxylase deficiency, adrenal hyperplasia, adrenalectomy, congenital adrenal hyperplasia, myelolipoma, resistant hypertension, testicular rests
Singapore Med J 2009; 50(2): e68-e70