Abstracts

Hypothalamic hamartomas (HHs) are rare developmental malformations of the hypothalamus that typically result in gelastic seizures followed by the development of other seizure types that are often refractory. Prior experience with surgical resection has shown limited efficacy and a significant rate of complications. We describe the successful resection of HHs in 36 patients with complete seizure control in 27. SURGICAL: The patients had their HHs removed either by a transcallosal-intraforniceal approach, endoscopic resection or orbito-zygomatic approach. NEUROLOGICAL: The baseline seizure types and frequencies were recorded prospectively pre and post-operatively. Other changes and/or complications were also recorded. Twenty-five patients underwent transcallosal resection, nine patients endoscopic resection and two patients had their HHs resected via an orbito-zygomatic approach (their HHs were pedunculated/parahypothalamic i.e. low-lying in position, unable to be accessed from a superior approach). There were no mortalities or significant complications. The 36 patients were followed up for an average of 6.5 months (range 1 to 13 months). One patient was lost to follow-up. Their average age was 11.9 years (range 2 to 55 years) and consisted of 24 males. Pre-operatively, all patients had at least weekly seizures, most with multiple, daily mixed seizure types. Four patients had Pallister-Hall syndrome and one patient, Mohr syndrome. Twenty-seven patients (77.1%) are currently seizure-free, five patients have had [gt]90% reduction, two patients [gt]50% reduction in seizure frequency and one patient with only slightly improved seizure control. Overall 91.4% (32 of 35 patients) achieved excellent seizure control. Most patients also report a dramatic improvement in behavior and/or developmental/learning ability. Neurosurgical resection of HH can be safe and effective treatment for patients with refractory epilepsy and should be considered in all HH patients with the often-devastating clinical scenario. The transcallosal approach and endoscopic resection appear to be superior to previously published surgical and other ablative techniques.