التظاهرات الغدية للورم القحفي البلعومي و علاقتها بنسبة المراضة و الوفيات عند الأطفال

Abstract EN

Background and Objective: Childhood craniopharyngioma is a rare embryogenic malformation of the sellar area.

It constitutes up to 6-10% of childhood brain tumors.

Due to the proximity of craniopharyngiomas to the hypothalamus and pituitary gland most children presenting with these tumors will exhibit significant endocrine dysfunction.

The purpose of this study is to recognize the endocrines disturbances seen in children with craniopharyngioma and their roles in morbidity and mortality.

Materials and Methods: This descriptive prospective study involved 20 cases with craniopharyngioma admitted to Damascus University Children Hospital over 3 years period.

There were 20 confirmed cases of autopsy.

The ages ranged from 7 months to 13.58 years and median age 6,23 ± 3.96 years with a tendency to age under 10 years in 70% of cases.

Female accounted for 60% of cases.

The symptoms of intracranial hypertension as headaches and vomiting were seen in 70% of cases.

The symptoms of endocrine dysfunctions were seen in the majority of cases where the diabetes insipidus was seen in 50% of the cases before surgery and the majority of the cases after the surgery, while the weight was deficient in 65% of the cases.

Hypothyroidism was seen in 47% of cases before surgery, while adrenal failure was seen in 38% of cases before surgery and increased to 67% of cases thereafter.

Growth hormone deficiency was seen in 62% of cases before surgery.

The symptoms of hypothalamic dysfunctions such as obesity, appetite and sleep disorders have developed in most cases after surgery.

A statistically significant association was observed between the severity of underweight and hypothyroidism and mortality.

Conclusion: Post operation endocrine deficiencies are one of the leading causes of morbidity and mortality especially adrenal deficiency seen in most cases.

endocrine deficiencies play a majeure role in morbidity an mortality before and after surgery of craniopharyngioma in children and they need to be closely monitored.