Diagnosis and treatment of hypothalamic hamartoma in children

Objective To investigate the diagnosis and microsurgical treatment of hypothalamic hamartoma in children.Methods A retrospective analysis was conducted on five cases with hypothalamic hamartoma in children from January 1998 to December 2006.Five children(two boys and three girls)were treated for precocious puberty and gelastic seizures secondary to hypothalamic hamartoma.The age ranged from 20 months to 9 years with a mean of 44.4 months.Two cases mainly displayed precocious puberry,1 with gelastic seizures and the other 2 cases with both of precocious puberty and gelastic secures.The sex hormone level of four cases was about 5 to several dozen times more than nolTnal before operation.Magnetic resonance imaging clearly demonstrated an isointense mass in the interpeduncular cistern(5 cases)and suprasellar cistem(2 cases)ranged from 13.9 to 21.0mm in diameter without enhanced pictures after injected Gd-DTPA.The hamartomas were totally removed via a right frontotemporal approach under microscope.Results All hypothalamic hamartomas were completely resected under microscope and confirmed by pathological examination.The symptoms and signs of precocious puberty completely regressed postoperatively,and sex hormone level descended to prepubertal level in all patients.The symptoms of gelastic seizures completely disappeared in two patients and obviously reduced from ten times per day before operation to one time per fortnight in another one patient.There was no any complication during 1 year follow-up in all patients.Conclusions Microsurgery can be deserved the first choice of treatment for hypothalamic hamartoma in children. Key words: Hamartoma; Epilepsy; Microsurgery