HISTORY AND CLINICAL FINDINGS: In a 31-year-old patient a conventional X-ray was performed due to persistent pain at the lumbar spine level after a cesarean section. It revealed compression fractures of L2 and L3. Besides very clear clinical signs of hypercortisolism, multiple hyperpigmentations and naevi in the patient's face including the lips and the conjunctiva of the right eye were visible, suggesting a Carney complex. INVESTIGATIONS: Insuppressible cortisol levels confirmed an adrenal origin of hypercortisolism. A selective catheterization of adrenal veins supported the presence of bilateral adrenal cortisol production. The computed tomography showed nodular lesions in the right and a hyperplasia of the left adrenal gland. TREATMENT AND COURSE: A bilateral adrenalectomy was performed and a primary pigmented nodular adrenal hyperplasia was confirmed histologically. Clinical signs of hypercortisolism rapidly resolved after adrenalectomy. CONCLUSIONS: The diagnosis of Cushing's syndrome as a part of Carney complex was diagnosed at the end of a pregnancy although signs of hypercortisolism were present a long time before. The rare diagnosis of Carney complex should be considered in patients exhibiting symptoms of hypercortisolim and the typical clinical signs (hyperpigmentations).
HISTORY AND CLINICAL FINDINGS: In a 31-year-old patient a conventional X-ray was performed due to persistent pain at the lumbar spine level after a cesarean section. It revealed compression fractures of L2 and L3. Besides very clear clinical signs of hypercortisolism, multiple hyperpigmentations and naevi in the patient's face including the lips and the conjunctiva of the right eye were visible, suggesting a Carney complex. INVESTIGATIONS: Insuppressible cortisol levels confirmed an adrenal origin of hypercortisolism. A selective catheterization of adrenal veins supported the presence of bilateral adrenal cortisol production. The computed tomography showed nodular lesions in the right and a hyperplasia of the left adrenal gland. TREATMENT AND COURSE: A bilateral adrenalectomy was performed and a primary pigmented nodular adrenal hyperplasia was confirmed histologically. Clinical signs of hypercortisolism rapidly resolved after adrenalectomy. CONCLUSIONS: The diagnosis of Cushing's syndrome as a part of Carney complex was diagnosed at the end of a pregnancy although signs of hypercortisolism were present a long time before. The rare diagnosis of Carney complex should be considered in patients exhibiting symptoms of hypercortisolim and the typical clinical signs (hyperpigmentations).
Authors: Damian J Ralser; Brigitte Strizek; Patrick Kupczyk; Birgit Stoffel-Wagner; Julia Altengarten; Andreas Müller; Joachim Woelfle; Ulrich Gembruch; Dietrich Klingmueller; Waltraut M Merz; Anke Paschkowiak-Christes Journal: Front Endocrinol (Lausanne) Date: 2020-05-08 Impact factor: 5.555