Carney complex - An unexpected finding during puerperium

Carney complex is an extremely rare, autosomal dominant, multi-system disor der characterized by multiple neoplasias and lentiginosis. The genetic defe ct responsible for this complex has been localized to the short arm of chro mosome 2 (2p16). The most prevalent clinical manifestations in patients wit h Carney complex are spotty skin pigmentation, skin and cardiac myxomas, Cu shing's syndrome and acromegaly, Here we report the case of a 31-year-old w oman with a spontaneous pregnancy. At 32 weeks of gestation, she was admitt ed to our Department of Obstetrics with hypertension and severe back pain. In addition, she had unusual pigmentation and typical cushingoid features. One day after admission, the pregnancy was terminated by emergency cesarian section because of preeclampsia and pathological CTG, During the postopera tive period the severe back pain persisted, and radiographic evaluation rev ealed a collapse of L-2/L-3 With severe osteopenia. A CT scan showed a mass in the right suprarenal area. Histopathological examination revealed a pri mary pigmented nodular adrenocortical disease. After biochemical confirmati on of the diagnosis of Gushing's syndrome, it was recognized that the patie nt met the diagnostic criteria for Carney complex. Copyright (C) 2001 S. Ka rger AG, Basel.