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- What is the role of bilateral adrenalectomy in patients with CAH?
Bilateral adrenalectomy is a therapeutic option in patients with CAH who
are diffi cult to manage despite optimal medical therapy. Patients with severe
virilization and infertility and those who experience adverse effects on glu-
cocorticoid therapy are candidates for bilateral adrenalectomy. The benefi -
cial effects of bilateral adrenalectomy include reduction in glucocorticoid
doses, regression of features of virilization, improved quality of life, and
easier monitoring. There is reduction in doses of glucocorticoid as aim of
therapy after bilateral adrenalectomy is to provide replacement doses of
glucocorticoids rather than suppression of ACTH. Regression of features of
virilization and improvement in fertility outcome occur as the source of
adrenal androgens is eliminated. However, patients who are subjected to
bilateral adrenalectomy are at increased risk for adrenal crisis and develop-
ment of adrenal rest tumors. The reappearance of virilization in a female or
the presence of asymmetric testicular enlargement in a male after bilateral
adrenalectomy suggests the development of adrenal rest tumors in the ovary
(OART), or in the testis (TART), respectively.- What are the long-term risks associated with untreated simple virilizing CAH?
The long-term risks associated with untreated simple virilizing CAH include
short fi nal adult height, infertility, virilization and secondary polycystic ovarian
disease, bilateral adrenal nodular hyperplasia, and testicular/ovarian adrenal
rest tumors. Rarely, adrenocortical carcinoma, adrenal myelolipoma, and corti-
cotroph hyperplasia have been reported in these patients (Fig. 10.16 ).abFig. 10.16 CT abdomen showing ( a ) bilateral adrenal hyperplasia ( red arrows ) in a child with
classical CAH due to 21α-hydroxylase defi ciency. ( b ) Left adrenal myelolipoma ( red arrow ) in
another patient with CAH due to 21α-hydroxylase defi ciency
10 Congenital Adrenal Hyperplasia