Introduction: Cushings syndrome is rare in the paediatric population, affecting 0.89 per million children between the ages of 0 and 20 years. The causes of Cushings syndrome of endogenous origin are dominated by pituitary causes in 70-95%, with a peripubertal revelation. We report the case of a child followed for Cushings disease in the department of Endocrinology-Diabetology-Nutrition of Mohammed-VI University Hospital Center of Oujda, in the eastern of Morocco.
Observation: It is a 14-year-old female child, without any particular pathological history, referred to our department for secondary amenorrhea, weight gain and hirsutism. The clinical examination found an obese child with a BMI of 35.5 kg/m², large purple stretch marks and a puffy face. Biological assessment revealed ACTH-dependent biological hypercortisolism. Hypothalamic-pituitary MRI revealed a pituitary microadenoma measuring 4 mm. The case was discussed in a multidisciplinary meeting including endocrinologists, neurosurgeons, radiotherapists, and oncologists, then the patient benefited from a selective microadenectomy with complete post-operative remission.
Discussion-Conclusion: Cushings disease in children is a diagnostic and therapeutic emergency. Early diagnosis remains a challenge for the clinician in order to prevent the consequences of hypercortisolism especially on growth and puberty. It requires a rigorous evaluation as well as a codified management as soon as possible, following a multidisciplinary meeting. Transphenoïdal surgery remains the only curative treatment.
Key-words: Cushings disease children
22 Sep 2021 - 26 Sep 2021