EAP 2019 Congress and MasterCourse

Craniopharyngioma in a Child Revealed by Psychomotor Regression: A Case Report

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1Departement of Pediatrics, University Hospital Farhat Hached Sousse, Yousra Mesbahi, Tunisia
2Departement of pediatrics, university hospital fattouma bourguiba monastir, besbes habib, Tunisia

Introduction: Craniopharyngioma are rare histologically benign brain tumors that develop in the pituitary–hypothalamic area. With an overall incidence of 0.5-2 new cases per million population per year, 30-50% of all cases occur in childhood. They may invade nearby anatomical structures causing significant rates of neurological, neurocognitive, and endocrinological complications including remarkable hypothalamic damage. The diagnosis of craniopharyngioma is often made late, sometimes years after the initial appearance of symptoms.

Observation: In this report, we report the case of a 20-month-old boy presenting a craniopharyngioma revealed by visual troubles. He was hospitalized in our service for vomiting occurring in a context of psychomotor regression made of a loss of ability to sit and walk properly for two months, as well as convergent strabismus. The clinical examination showed a pyramidal syndrome and low vision behavior. Magnetic resonance imaging (MRI) revealed well-defined lobulated T1-hypointense and T2-heterogenous hyperintense solid cystic lesion in suprasellar location measuring 7.2 cm × 4.7 cm compressing the optic chiasma and V3 with significant hydrocephalus. The patient underwent a cyst drainage to relieve pressure and improve vision, followed by a surgical resection. Actually, he kept a visual loss and a hypothalamic dysfunction.

Conclusion: The survival rate for childhood craniopharyngioma has been improving, with more long-term survivors. Unfortunately it is rare for the patient to be normal, either from the disease itself or from the effects of treatment. Long-term survivors of childhood craniopharyngioma suffer from a number of impairments, which include visual loss, endocrinopathy, hypothalamic dysfunction, cerebrovascular problems, neurologic and neurocognitive dysfunction.









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