Background: Craniopharyngioma is low grade malignant tumour with high survival rate. Its incidence is 0.52.0 cases per million persons per year. 3050% of all cases occur in the childhood. The tumour location leads to numerous complications like deficits of pituitary function, impairment of vision, neuropsychological deficits and obesity. Excess body fat is observed in 4050% of craniopharyngioma patients.
Case presentation: We present two patients after neurosurgery carried out as treatment of craniopharyngioma with extreme obesity and liver cirrhosis secondary to NASH. The first patient is a 17-years-old girl and the second patient is now 27-years-old male who was under the care of the Department of Endocrinology in the period from 6 to 18 years of age. In the history both of them were in good condition till age of 6. Since then the symptoms of CNS tumour appeared. In case of the girl episodes of severe headache, vomiting were observed and the tumour around the optic chiasm and in the third ventricle was diagnosed. In case of the boy there were diabetes insipidus, weight loss and hypersomnia. CT showed cystic tumour of the suprasellar region. After total resection of craniopharyngioma symptoms of panhypopituitarism, diabetes insipidus, hyperphagia and steady weight gain were observed. In first six months after the operation the boy has put on weight by 35 kg. Attempts to modify the diet were unsuccessful. In a girl at 11 years of age the following examination revealed carbohydrate intolerance, hyperinsulinism and features of fatty liver. In the 8th year after the neurosurgery relapse of craniopharyngioma was diagnosed. Patient underwent endoscopic fenestration of tumour cyst.The rest of tumour has been identified, but her parents refused radiotherapy. Both of them at 1315 years of age began to show the symptoms of hepatopulmonary syndrome and liver failure. Based on liver biopsy diagnosed with NASH, after that they underwent liver transplantation. In the long term observation patients are still obese. A qualification for the bariatric surgery is considered.
Conclusions: The aim of our study is to highlight fatty liver disease as a significant problem in obese children with hypothalamic damage caused by tumour. Thorough, long-term observation of patients and performed examinations may allow for early detection of progress from simple steatosis to steatohepatitis and finally cirrhosis of the liver.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology