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.

Microsurgical Management of Complex Hypothalamic Hamartomas in the Era of Minimally Invasive Therapy: A Case Series and Narrative Review

“…4 Pterional skull base approaches have yielded some success for treatment of hypothalamic hamartomas, however, access to the hypothalamus can be limited through this approach. 11,27,15 Noteably, Drees, et al, reported a mean resection of 64% in 10 patients treated with transcallosal or endoscopic-pterional/endoscopic-FTOZ approaches; it was noted that giant HH led to signi cant di culty in obtaining a complete resection. No patients experienced seizure freedom.…”

The diagnosis and management of hypothalamic hamartomas in children

“…Furthermore, surgical resection is safe with a favorable outcome of epilepsy in 50 % with significant improvement in behavior and marginal change in cognitive functions. The reasons for thinking that HH with GS play a major role on development [36] and behavior lie in the following considerations. These children with HH usually have no history of retarded or abnormal development before the onset of GS.…”

“…Refractory epilepsy related to behavioral and cognitive dysfunction may be the most common presentation of HH. Open surgical resection is safe with a favorable outcome of epilepsy in 50 % with significant improvement in behavior and marginal change in cognitive functions [36].…”

Forms of clinical presentation of hypothalamic hamartoma

“…It is estimated that it achieves seizure control in 50% of cases, with improvement in behaviour and cognition. 1,5 Several studies have demonstrated the efficacy of stereotactic radiosurgery (gamma-knife surgery), with outcomes similar to those of conventional surgery and decreased morbidity and mortality, 1,6 with the downside that its effects show later (6 months post-surgery) and that sometimes several interventions are required to achieve adequate symptom control. …”