ÎÎConsider bilateral adrenalectomy only in select cases that have failed
medical therapy, especially in rare cases of adult females with saltwasting CAH and infertility. Risk for noncompliance must be evaluated
prior to surgery (2|⊕).
CAH in Adulthood
ÎÎTreat adults with NCCAH with patient-important hyperandrogenism or
infertility (2|⊕).
ÎÎDo NOT prescribe daily GC substitution in adult males with NCCAH
(2|⊕).
ÎÎTreat adult patients with classic CAH with HC or long-acting GCs
(2|⊕).
ÎÎMonitoring of GC and mineralocorticoid (MC) treatment should
include at least annual physical examination and appropriate hormone
measurements.
ÎÎAll males with classic CAH should be periodically screened from
adolescence for testicular adrenal rest tumors by ultrasound (2|⊕).
ÎÎPediatric and adult endocrinologists, reproductive endocrinologists,
gynecologists, and urologists should have joint clinics for transfer of
patients with CAH to adult care (2|⊕).
Fertility and Pregnancy
ÎÎThe ES suggests a gynecological history and examination under
anesthesia in adolescent females with CAH.
ÎÎDo NOT routinely use pelvic ultrasound in CAH patients with regular
menstrual cycles (2|⊕).
ÎÎAdvise CAH patients with impaired fertility to consult a reproductive
endocrinologist and/or fertility specialist (2|⊕⊕).
ÎÎThe ES suggests pregnant women with CAH be followed jointly by
endocrinologists and obstetricians.
ÎÎPatients with CAH who become pregnant should continue their prepregnancy doses of HC/prednisolone and fludrocortisone therapy
(1|⊕⊕). GC doses should be adjusted if symptoms and signs of GC
insufficiency occur. Do NOT use GCs that traverse the placenta, such as
dexamethasone, for treatment of pregnant patients with CAH (1|⊕⊕).
Stress doses of GCs should be used during labor and delivery.
ÎÎOffer genetic counseling to parents at birth of a CAH child, and to
adolescents at the transition to adult care (1|⊕).
Mental Health
ÎÎRefer patients with CAH and psychosocial problems associated with
disorders of sexual development to mental health staff with specialized
expertise in managing such problems (2|⊕⊕).
