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 11beta-hydroxylase deficiency (11betaHD) 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 11betaHD 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 11betaHD 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.