Endoscopic Robot-Guided Disconnection of Hypothalamic Hamartomas: Results in 18 Cases and Review of the Literature

Purpose: To review our experience on the endoscopic disconnection of drug-resistant hypothalamic hamartomas (HHs) and perform a meta-analysis over published data to define the best surgical approach for hypothalamic hamartoma. Method: Our series with a minimum follow-up of 18 months includes 18 patients: 13 males, 5 females; mean age at diagnosis of epilepsy was 2,1 years and 9,5years at first disconnection. Each patient received a complete presurgical assessment, including neurological, endocrinological and neuropsychological evaluation, scalp and video-electroencephalography, and brain MRI. HHs were defined according to the Delalande' classification [type II: 9 (50%); type III: 7 (38%); type IV: 2 (11%)]. In all surgeries, the disconnection was performed by using monopolar (10 cases, 55%) or laser thulium (Revolix) (8 cases, 44%) coagulation. We then review all published article from January 2000 to December 2016 and identified 51 article with 480 patients in which post-surgical outcome for hypothalamic hamarthoma was reported. Results: Postoperative evaluation was performed every 6 months after surgery. No permanent deficits were registered. At a mean follow-up period of 10 months, the Engel class (I–IV) was: I in 11/18 patients (61%); II in 3/18 patients (16%); III in 2/18 patients (11%); IV in 2/18 patients (11%). No significant variations of the neuropsychological status were reported. Six patients underwent a second surgery, with a postoperative improvement of the Engel score. The overall seizure free rate of patient reported in the literature was 45% (218/480). Conclusion: Endoscopic transventricular disconnection is an effective mini-invasive treatment of HHs, with a positive impact on epilepsy and neuropsychological outcome. Neuronavigation stereotactic systems, in association with robotic technology and laser coagulation, allowed to safely manage more difficult cases (e.g. tight ventricles or small lesions). We will discuss this results in relation to the meta-analysis of published patients.