J Neurosurg Pediatr. 2026 Apr 10:1-12. doi: 10.3171/2025.11.PEDS25377. Online ahead of print.
ABSTRACT
OBJECTIVE: Surgical management of hypothalamic hamartomas (HHs) presents substantial challenges due to their deep-seated location and proximity to critical neurovascular structures. Less invasive techniques, such as endoscopic disconnection and laser interstitial thermal therapy (LITT), have become preferred over traditional microsurgery to enhance seizure control while minimizing complications. Notably, LITT has steadily gained popularity in recent years. The aim of this study was to assess seizure, neurocognitive, and endocrine outcomes following endoscopic disconnection in patients with HH-related epilepsy, and to determine whether endoscopy remains a viable treatment option in the management of HH.
METHODS: This retrospective analysis included patients with HH-related epilepsy who underwent robotic-assisted endoscopic disconnection between 2011 and 2023 at a single institution. All patients received comprehensive presurgical evaluation, and formal assessments of global cognitive and neuropsychological function were conducted preoperatively and postoperatively in eligible patients.
RESULTS: Twenty-nine patients who underwent 37 procedures were included in this analysis. All patients experienced gelastic seizures, and most patients (51.7%) had type II HH according to the Delalande classification system. Twenty-two patients (75.9%) underwent a single intervention. The procedure was repeated 2 times in 6 patients and 3 times in 1 patient. Endoscopy proved effective in providing direct visualization, real-time monitoring, and histopathological sampling during procedures. Permanent postoperative complications occurred after 4 procedures (10.8%); these included mild CN VI deficit (n = 1), hypothalamic obesity (n = 1), and hypothyroidism (n = 2) after second procedure. Over a mean follow-up of 6.9 years, Engel class I seizure freedom was achieved in 65.5% of patients, with the best outcomes seen for those with type II HH. Among the 22 patients who underwent a single procedure, the long-term endocrinological status remained unchanged compared with the preoperative condition in 17 (77.3%), improved in 4 (18.2%), and worsened in 1 (4.5%). Among the 20 patients with available comparative data, cognitive outcomes remained stable or improved for most patients, although some exhibited decline. Statistical analysis revealed a moderate correlation between the HH type and postoperative Engel class outcome. Comparison between patients who underwent single versus multiple procedures revealed a significantly poorer seizure outcome in the repeat surgery group, while the complication rates were comparable. The outcome was significantly associated with the number of procedures.
CONCLUSIONS: Endoscopic disconnection remains a viable and minimally invasive surgical option for the treatment of HH-related epilepsy, particularly for newly diagnosed or residual HHs with intraventricular involvement.
PMID:41962169 | DOI:10.3171/2025.11.PEDS25377
