This case highlights the rare presentation of isolated micronodular adrenocortical disease in a young child presenting with Cushing's syndrome.
Alerts clinicians to consider micronodular adrenocortical disease in pediatric Cushing's syndrome; leaves open optimal management pending larger series.
Cushing's syndrome (CS) is uncommon in childhood. CS may be either dependent or independent of adrenocorticotrophic hormone (ACTH). ACTH independent micronodular adrenocortical (MAD) disease may present in the second to third decade of life or between ages 2-3 years. It may occur in isolation, or as a part of the Carney complex and it represents an elusive entity to diagnose. We present a 3 year 7 month old boy with isolated MAD (iMAD). Abdominal CT revealed prominent mildly lobulated anteromedial margin of adrenals with nodular appearance. Cardiac echo, thyroid and testicular ultrasounds performed as a work up for Carney complex were normal. Bilateral adrenalectomy confirmed MAD as the cause of CS.We present the history and identification of a unique case of iMAD.
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Henry et al. (2010) studied this question.
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