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

IntroductionRecently, there has been a paradigm shift in the management of hypothalamic hamartoma (HH) from traditional microsurgical techniques to less invasive alternatives such as stereotactic radiosurgery and laser interstitial thermal therapy. However, large and extensive HH may fail to respond to minimally invasive therapies, ultimately necessitating microsurgery.MethodsAll patients who underwent microsurgical resection of a complex HH by the 2 senior authors (D.J.L., H.L.R.) in 2011-2017 were included. Charts were retrospectively reviewed and demographic, clinical, imaging, and outcome data were recorded.Results8 patients, 7 children and 1 adult, with a mean age of 7 years (10 months-27 years), were included. Of those, 2 had failed previous treatments. All 7 children presented with pharmacoresistant gelastic seizures and cognitive dysfunction, 6 exhibited central precocious puberty, and 3 had behavioral problems. Other seizure types affected 6/8 patients. Mean lesion size was 21.6 mm (14-31), all with interpeduncular extension and 5 with intraventricular extension (Delalande type I: 3, type III: 4, type IV: 1). A frontotemporal orbitozygomatic (FTOZ) approach with optic nerve decompression was used in all patients, supplemented by another approach in 3 (endoscopic transventricular: 3, transcallosal: 1). Gross total resection was achieved in 6 patients and subtotal resection in 2. Transient complications occurred in 3 patients (37.5%): self-limited sodium imbalance (n=3), subdural hygroma (n=2). Permanent complications occurred in 2 patients (25%): perforator infarct (n=1), short-term memory loss (n=1). All patients experienced seizure resolution postoperatively with preserved hypothalamic-pituitary axis function. After a mean follow-up of 41 months (2-66), 7 patients remain seizure-free, while 1 has rare recurrent seizures. Cognitive and behavioral symptoms improved significantly in all patients.ConclusionFor large HH with interpeduncular extension, microsurgery via the FTOZ approach is a safe and highly effective treatment modality.