Orbitozygomatic resection for hypothalamic hamartoma and epilepsy: patient selection and outcome

Abstract

Purpose: This study aims to examine the outcomes of ten patients after orbitozygomatic (OZ) pterional surgery in cases of refractory epilepsy caused by hypothalamic hamartomas (HH).

Methods: Ten patients with HH and treatment-resistant epilepsy (mean age 18.3 years, range 0.7 to 42.7) underwent HH resection with an OZ approach (n = 8) or an OZ approach combined with a transventricular endoscopic approach (n = 2). Follow-up for the patients ranged from 0.5 to 6.2 years (mean 3.1). Outcomes were prospectively monitored with the use of a proprietary database.

Results: Four patients (40%) are seizure-free, and four (40%) have had greater than 50% reduction in seizures. One patient had no significant change in seizure frequency, and one patient died unexpectedly 2.8 years after surgery. Six patients had total or near-total HH resection (98-100% of HH lesion volume). Of these, four of six (66%) were seizure-free, and two had at least greater than 50% reduction in seizures. Residual complications include diabetes insipidus (n = 1), poikilothermia (n = 1), visual field deficit (n = 1), and hemiparesis (n = 1). Eight families (80%) reported improved quality of life.

Conclusions: Patients with treatment-resistant epilepsy and tumors with an inferior or horizontal plane of attachment to the hypothalamus should continue to be approached from below. Those with both intrahypothalamic and parahypothalamic components may require approaches from above and below, either simultaneously or staged. For appropriately selected patients, the success of controlling seizures with an OZ is comparable to results utilizing transcallosal or transventricular approaches. The likelihood of controlling seizures appears to correlate with extent of resection.