Endocrine Abstracts

Introduction: Cushing’s disease is defined as hypercortisolism caused by excessive secretion of ACTH from a pituitary adenoma. Cushing’s disease is a rare entity in children

Observation: We report the case of 3 children with Cushing’s disease including 2 boys and 1 girl, the mean age at the diagnosis was 12.6 years. Clinically, weight gain was constant in all children, a delay in statural growth was noted in 2 children, signs of catabolism were found in one child. Biologically, the hormonal exploration reveal an ACTH-dependent hypercorticism Morphologically, the adenoma was visualised on MRI in one patient; however, the size of the adenoma was 4 mm requiring petrous sinus catheterisation. Petrous sinus catheterisation was used in all our children; this examination objectified a Centro peripheral gradient in 2 cases; but was non-contributory in the 3rd child. In terms of treatment: only one child with very severe cushing’s disease was operated by transphenoidal approach on 3 occasions with failure, requiring bilateral adrenalectomy complicated by Nelson’s syndrome, treated with radiotherapy and dostinex. The other 2 children are currently awaiting pituitary surgery and are on anticortisol therapy.

Discussion: Endogenous cushing’s syndrome in children is rare, the most common cause remains cushing’s disease. Often, in these children, the tumours are very small and cannot be detected on MRI. Despite the difficulty of this delicate procedure, petrosal sinus catheterisation was able to make the diagnosis in two out of three children.

Conclusion: Cushing’s disease in children is rare;. Its management represents a real challenge requiring the use of a specialised multidisciplinary team