Bilateral adrenalectomy for severe hypertension in congenital adrenal hyperplasia due to 11beta-hydroxylase deficiency: long term follow-up.
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Kacem
M
M
Endocrinology Unit, Department of Internal Medicine and Endocrinology, Fattouma Bourguiba University Hospital, 5000 Monastir, Tunisia. kacem_maha@yahoo.fr
Moussa
A
A
Khochtali
I
I
Nabouli
R
R
Morel
Y
Y
Zakhama
A
A
D000311:Adrenal Glands / Q000473:pathology / Q000601:surgery; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications / Q000235:genetics* / Q000601:surgery*; D000315:Adrenalectomy; D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D001921:Brain / Q000473:pathology; D004247:DNA / Q000096:biosynthesis / Q000235:genetics; D005500:Follow-Up Studies; D006727:Hormone Antagonists / Q000627:therapeutic use; D006801:Humans; D006973:Hypertension / Q000209:etiology / Q000601:surgery*; D007030:Hypothalamo-Hypophyseal System / Q000473:pathology; D008297:Male; D012164:Retinal Diseases / Q000209:etiology; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012741:Sexual Maturation; D013252:Steroid 11-beta-Hydroxylase / Q000235:genetics*
D000959:Antihypertensive Agents; D006727:Hormone Antagonists; D004247:DNA; D013252:Steroid 11-beta-Hydroxylase