Clinical characteristics and long-term outcome of surgery for hypothalamic hamartoma in children with refractory epilepsy

Abstract

Context: Hypothalamic hamartomas (HH) are ectopic masses of neuronal and glial tissue most commonly presenting with medically refractory gelastic seizures with evolution to other seizure types. They are also associated with cognitive and behavioral problems to varying extent. Surgery has been found to improve quality of life in more than 50% of patients. Aim: To evaluate the clinical characteristics and long-term outcome of surgery in children with HH and refractory epilepsy. Materials and Methods: A retrospective analysis of presurgical, surgical, and postsurgical data of six children who underwent surgery for HH and had at least 3 years follow-up was performed. Results: Six children (male: female = 5:1) aged 3-16 years (at the time of surgery) underwent surgical resection of HH for refractory epilepsy. At last follow-up (range 3-9 years), three children were in Engel′s class I, two in Class II, and one in class III outcome. Significant improvement in behavior, quality of life was noted in four children; while the change in intelligence quotient (IQ) was marginal. Conclusions: Medically refractory epilepsy associated with behavioral and cognitive dysfunction is the most common presentation of HH. Open surgical resection is safe with favorable outcome of epilepsy in 50% with significant improvement in behavior and marginal change in cognitive functions.

Keywords

• Gelastic seizures
• hypothalamic hamartoma
• refractory epilepsy
• surgery