Endocrine Disorder in Childhood Craniopharyngioma

Introduction: Craniopharyngioma is a benign congenital tumor but it remains a severe tumor due its close position to neighboring anatomical structures (hypothalamus, chiasma and optic nerves). It represent 10% of the childhood tumors. There are usually combinations of visual impairment, endocrinopathy, hypothalamic dysfunction, cerebrovascular disease, neurologic dysfunction, and neurocognitive dysfunction.

Endocrine disorders may be indicative of the tumor. They settle several months or years before the visual and neurological manifestations.

Methods: This was a retrospective study over a period of 18 years and 7 months ( june 2000-February 2019) of 11 observations of children with craniopharyngiomas admitted to the pediatric ward of Sahloul University Hospital in Sousse.

Results: We collected 11 cases of children with craniopharyngioma. There were 3 boys and 8 girls. The age of the patients was between 6 and 15 years old. Inbreeding was found in 3 cases. The main reason for consultation was headache in all cases, associated with gastrointestinal manifestations in 8 cases, visual disturbances in 3 cases and behavioral disturbance in 2 cases. Clinical examination revealed signs of dehydration with polyuria in 5 cases, abnormal weight-loss in 3 cases, pubertal delay in 1 case and amblyopia in 3 cases. CT showed an intrasellar process in all patients. Cerebral MRI was performed in only 36% of cases. Endocrine exploration showed adrenal insufficiency in all cases, hypothyroidism was found in 4 cases, damage to the somatotropic axis in 2 cases, and diabetes insipidus was found in 5 cases. Surgical treatment was conducted in all cases. Postoperatively, panhypopituitatrism was observed in 4 postoperative cases. All patients were put on hormone replacement therapy for life.

Conclusion: Craniopharyngioma is a serious pathology in children because of its endocrine consequences before and after surgery. Collaboration between neurosurgeons, ophthalmologists and endocrine-pediatricians is necessary for a global and optimal care.