Study of three patients with congenital adrenal hyperplasia treated by bilateral adrenalectomy

Medical management of congenital adrenal hyperplasia (CAH) patients has led to suboptimal results in most cases. High glucocorticoid doses, often need ed to suppress adrenal androgen production, may lead to signs of Gushing sy ndrome. Incompletely suppressed androgen levels commonly lead to premature closure of growth centers, acne, virilization, precocious puberty, irregula r or absent menses, and decreased fertility in female CAH patients. A newly proposed therapy for CAH patients is bilateral adrenalectomy. Three Caucas ian female patients with 21-hydroxylase deficiency were treated with bilate ral adrenalectomy. Two of the three procedures were accomplished laparoscop ically. In each patient, medical management alone was unsuccessful. Two pat ients had salt-losing 21-hydroxylase deficiency. The third patient had unco ntrolled hyperandrogenism complicated by obesity and glucose intolerance. A ll patients had low height percentiles with respect to their normalized per centiles for weight. Bone age was advanced in one patient. Androgen and ren in levels were well controlled in two patients, whereas the third patient h ad persistent hyperandrogenism. Bilateral adrenalectomy was performed at th e ages of 14, 19, and 30 years with follow-up, to date, of 25 months, 10 mo nths, and 26 months, respectively. Postoperatively, all patients were free from hyperandrogenism. One patient experienced one episode of urosepsis pre cipitating an addisonian crisis. Bilateral adrenalectomy may successfully a ddress the problems of increasing steroid requirements and hyperandrogenism in patients with severe CAH. The ability to perform this operation laparos copically coupled with the overall metabolic benefits make bilateral adrena lectomy a reasonable alternative to lifelong androgen suppression in select patients.