Treatment
12
➤ In women with congenital adrenal hyperplasia who are pregnant, or trying
to become pregnant, ES recommends against using glucocorticoids that
traverse the placenta, such as dexamethasone. (1|⊕⊕
)
➤ In women with congenital adrenal hyperplasia who are pregnant, ES
advises that the birthing plan includes an obstetric specialist. (UGPS)
Surveillance for Long-Term Complications of Congenital Adrenal
Hyperplasia and Its Treatment
➤ For patients with congenital adrenal hyperplasia, ES suggests introducing
counseling regarding healthy lifestyle choices at an early age to maintain
body mass index within the normal range to avoid metabolic syndrome and
related sequelae. (2|⊕
)
➤ In adult patients with congenital adrenal hyperplasia, ES suggests
screening of bone mineral density in anyone subjected to a prolonged
period of higher-than-average glucocorticoid dosing, or who has suffered a
nontraumatic fracture. (2|⊕
)
➤ In adults with classic congenital adrenal hyperplasia, ES recommends
against routine adrenal imaging. (1|⊕
)
Technical remark: Reserve adrenal imaging for individuals with classic congenital
adrenal hyperplasia who have clinical evidence of an adrenal mass, poor disease control,
a lapse in treatment of several years, or lack of response to intensified therapy.
➤ In males with classic congenital adrenal hyperplasia, ES recommends
periodic testicular ultrasound to assess for the development of testicular
adrenal rest tumors. (1|⊕⊕
)
➤ In patients with congenital adrenal hyperplasia, ES recommends against
routine evaluation for cardiac and metabolic disease beyond that
recommended for the general population. (1|⊕⊕
)
Technical remark: Clinicians should use their own judgment for the above procedures.
Restoring Functional Anatomy By Surgery in Individuals
With Congenital Adrenal Hyperplasia
➤ In all pediatric patients with congenital adrenal hyperplasia, particularly
minimally virilized girls, ES advises that parents be informed about
surgical options, including delaying surgery and/or observation until the
child is older. (UGPS)
Technical remark: Surgeries should be performed only in centers with experienced
pediatric surgeons/urologists, pediatric endocrinologists, pediatric anesthesiologists,
behavioral/mental health professionals, and social work services. Extensive discussions
regarding risks and benefits, shared decision-making, review of potential complications,
and fully informed consent need to occur prior to surgery. The option to forgo surgery
should be considered.
