Why the study?
Neonatal Cushing syndrome in McCune-Albright syndrome usually has an unfavorable outcome, and a unified approach to diagnosis, treatment, and follow-up is lacking.
Population
An 11-day-old girl with ACTH-independent Cushing syndrome due to McCune-Albright syndrome
Design
Case report
Follow-up
34 months
Key result
In a neonate with severe Cushing syndrome due to McCune-Albright syndrome, high-dose metyrapone failed to achieve remission, but right total and left three-quarters adrenalectomy successfully resolved hypercortisolism.
Authors
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Supports individualized management of neonatal MAS with CS; leaves open standardized protocols from larger studies.
Case Report (n=1)
No
Neonatal Cushing syndrome due to McCune-Albright syndrome can be successfully managed with adrenalectomy when medical therapy fails.
Ünsal et al. (2023) conducted a case report in McCune-Albright syndrome with neonatal Cushing syndrome (n=1). Metyrapone and partial adrenalectomy was evaluated on Clinical and biochemical remission of hypercortisolism. In a neonate with severe Cushing syndrome due to McCune-Albright syndrome, high-dose metyrapone failed to achieve remission, but right total and left three-quarters adrenalectomy successfully resolved hypercortisolism.