Bilateral adrenalectomy for severe hypertension in congenital adrenal hyperplasia due to 11β-hydroxylase deficiency: Long term follow-up

Abstract

Context Bilateral adrenalectomy has been recently proposed as a surgical treatment option for patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. There is however little documented data about the long-term efficiency and potential side effects of this treatment. Patients with 11β-hydroxylase deficiency (11βHD) have been also concerned by this new approach. Objective Our objective was to describe our experience with bilateral adrenalectomy as a treatment of severe hypertension in a patient with 11βHD deficiency and to report the long term follow-up (72 months) results after surgery. Patient and Intervention A 22-year-old genetically female patient with 11βHD deficiency was raised as a male because of severe pseudohermaphroditism. The patient has been managed by conventional steroid suppressive therapy and antihypertensive drugs with limited success; hypertension remained uncontrolled and led to severe complications. Bilateral adrenalectomy was offered to him. Results The intervention was followed by immediate blood pressure normalization and resulted in remarkable clinical improvement. Good compliance with glucocorticoid and androgen substitutive therapies was noted. However, a high 11-deoxycortisol, presumably due to non-ovarian ectopic adrenal rests was noted 24 months after surgery. Conclusion Bilateral adrenalectomy is a safe and efficient method of managing CAH with selected patients. Long-term clinical and biochemical follow-up of patients with CAH treated by bilateral adrenalectomy is needed to earlier detect ectopic adrenal rests.