Gamma Knife surgery for hypothalamic hamartomas and epilepsy: patient selection and outcomes

Abstract

Object The authors present outcomes obtained in patients who underwent Gamma Knife surgery (GKS) at 1 institution as part of a multimodal treatment of refractory epilepsy caused by hypothalamic hamartomas (HHs). Methods Between 2003 and 2010, 19 patients with HH underwent GKS. Eight patients had follow-up for less than 1 year, and 1 patient was lost to follow-up. The 10 remaining patients (mean age 15.1 years, range 5.7–29.3 years) had a mean follow-up of 43 months (range 18–81 months) and are the focus of this report. Five patients had undergone a total of 6 prior surgeries: 1 transcallosal resection of the HH, 2 endoscopic transventricular resections of the HH, 2 temporal lobectomies, and 1 arachnoid cyst evacuation. In an institutional review board–approved study, postoperative complications and long-term outcome measures were monitored prospectively with the use of a proprietary database. Seven patients harbored Delalande Type II lesions; the remainder harbored Type III or IV lesions. Seizure frequency ranged from 1–2 monthly to as many as 100 gelastic seizures daily. The mean lesion volume was 695 mm3 (range 169–3000 mm3, median 265 mm3). The mean/median dose directed to the 50% isodose line was 18 Gy (range 16–20 Gy). The mean maximum point dose to the optic chiasm was 7.5 Gy (range 5–10 Gy). Three patients underwent additional resection 14.5, 21, and 32 months after GKS. Results Of the 10 patients included in this study, 6 are seizure free (2 after they underwent additional surgery), 1 has a 50%–90% reduction in seizure frequency, 2 have a 50% reduction in seizure frequency, and 1 has observed no change in seizure frequency. Overall quality of life, based on data obtained from follow-up telephone conversations and/or surveys, improved in 9 patients and was due to improvements in seizure control (9 patients), short-term memory loss (3 patients), and behavioral symptoms (5 patients); in 1 patient, quality of life remains minimally affected. Incidences of morbidity were all temporary and included poikilothermia (1 patient), increased depression (1 patient), weight gain/increased appetite (2 patients), and anxiety (1 patient) after GKS. Conclusions Of the approximately 150 patients at Barrow Neurological Institute who have undergone treatment for HH, the authors have reserved GKS for treatment of small HHs located distal from radiosensitive structures in patients with high cognitive function and a stable clinical picture, which allows time for the effects of radiosurgery to occur without further deterioration. The lack of significant morbidity and the clinical outcomes achieved in this study demonstrated a low risk of GKS for HH with results comparable to those of previous series.