Giant hypothalamic hamartoma operated through subfrontal approach with orbitary rim osteotomy.

INTRODUCTION

Hypothalamic hamartomas are associated with precocious puberty, gelastic seizures and severe refractory epilepsy. Treatment options include surgical resection, radiofrequency and radiosurgery.

CASE REPORT

A 7-month-old girl presented with gelastic seizures and developmental delay related to a giant hypothalamic hamartoma. The patient was operated through a subfrontal approach. Intraoperatively the lesion appeared intimately adherent to the right internal carotid artery. Seizure control was improved after tumoral decompression.

CONCLUSIONS

Treatment of giant hypothalamic hamartomas should always include surgical resection, given the mass effect over surrounding vital structures. Subfrontal approach with orbitary rim osteotomy provides a wide exposure with minimal frontal lobe retraction. Close adherence of hypothalamic hamartoma to vascular structures may be present, requiring careful surgical manipulation.