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 needed to suppress adrenal androgen production, may lead to signs of Cushing syndrome. Incompletely suppressed androgen levels commonly lead to premature closure of growth centers, acne, virilization, precocious puberty, irregular or absent menses, and decreased fertility in female CAH patients. A newly proposed therapy for CAH patients is bilateral adrenalectomy. Three Caucasian female patients with 21-hydroxylase deficiency were treated with bilateral adrenalectomy. Two of the three procedures were accomplished laparoscopically. In each patient, medical management alone was unsuccessful. Two patients had salt-losing 21-hydroxylase deficiency. The third patient had uncontrolled hyperandrogenism complicated by obesity and glucose intolerance. All patients had low height percentiles with respect to their normalized percentiles for weight. Bone age was advanced in one patient. Androgen and renin levels were well controlled in two patients, whereas the third patient had persistent hyperandrogenism. Bilateral adrenalectomy was performed at the ages of 14, 19, and 30 years with follow-up, to date, of 25 months, 10 months, and 26 months, respectively. Postoperatively, all patients were free from hyperandrogenism. One patient experienced one episode of urosepsis precipitating an addisonian crisis. Bilateral adrenalectomy may successfully address the problems of increasing steroid requirements and hyperandrogenism in patients with severe CAH. The ability to perform this operation laparoscopically coupled with the overall metabolic benefits make bilateral adrenalectomy a reasonable alternative to lifelong androgen suppression in select patients.
Warinner
S A
SA
Department of Surgery, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA.
Zimmerman
D
D
Thompson
G B
GB
Grant
C S
CS
D000312:Adrenal Hyperplasia, Congenital / Q000175:diagnosis* / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods*; D002657:Child Development / Q000502:physiology*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D010535:Laparoscopy / Q000379:methods; D008991:Monitoring, Physiologic; D011379:Prognosis; D016896:Treatment Outcome