Shirozu et al., undertook a study to validate the feasibility and safety of stereotactic radiofrequency thermocoagulation (SRT) for the surgical treatment of giant hypothalamic hamartoma (HH).
Of the 109 patients who underwent SRT for hypothalamic hamartoma (HH) at the authors' institution between 1997 and 2013, 16 patients (9 female, 7 male) had giant HHs (maximum diameter ≥ 30 mm). The clinical records of these 16 patients were retrospectively reviewed.
The patients' age at first SRT ranged from 1 to 22 years (median 5 years). The maximum diameter of their HHs was 30-80 mm (mean 38.5 mm). Eleven HHs had bilateral attachments to the hypothalamus. All patients had gelastic seizures (GS), and 12 had types of seizures other than GS. Some of these patients also had mental retardation (n = 10, 62.5%), behavioral disorders (n = 8, 50.0%), and precocious puberty (n = 11, 68.8%). A total of 22 SRT procedures were performed; 5 patients underwent repeat SRT procedures. There was no mortality or permanent morbidity. After 17 of the 22 procedures, the patients experienced transient complications, including high fever (n = 7), hyperphagia (n = 3), hyponatremia (n = 6), disturbance of consciousness (n = 1), cyst enlargement (n = 1), and epidural hematoma (n = 1). Thirteen patients (81.3%) achieved freedom from GS after the final SRT procedure during a follow-up period ranging from 6 to 60 months (mean 23 months). Twelve patients had nongelastic seizures in addition to GS, and 7 (58.3%) of these 12 patients experienced freedom from their nongelastic seizures.
SRT provided minimal invasiveness and excellent seizure outcomes even in patients with giant HHs. Repeat SRT is safe for residual GS. SRT is a feasible single surgical strategy for HH regardless of the tumor's size or shape 1).
The authors examined the clinical records of 100 consecutive patients (66 male and 34 female) with intractable gelastic seizures (GS) caused by HH, who underwent SRT as a sole surgical treatment between 1997 and 2013. The median duration of follow-up was 3 years (range 1-17 years). Seventy cases involved pediatric patients. Ninety percent of patients also had other types of seizures (non-GS). The maximum diameter of the HHs ranged from 5 to 80 mm (median 15 mm), and 15 of the tumors were giant HHs with a diameter of 30 mm or more. Comorbidities included precocious puberty (33.0%), behavioral disorder (49.0%), and mental retardation (50.0%). RESULTS A total of 140 SRT procedures were performed. There was no adaptive restriction for the giant or the subtype of HH, regardless of any prior history of surgical treatment or comorbidities. Patients in this case series exhibited delayed precocious puberty (9.0%), pituitary dysfunction (2.0%), and weight gain (7.0%), besides the transient hypothalamic symptoms after SRT. Freedom from GS was achieved in 86.0% of patients, freedom from other types of seizures in 78.9%, and freedom from all seizures in 71.0%. Repeat surgeries were not effective for non-GS. Seizure freedom led to disappearance of behavioral disorders and to intellectual improvement. CONCLUSIONS The present SRT procedure is a minimally invasive and highly effective surgical procedure without adaptive limitations. SRT involves only a single surgical procedure appropriate for all forms of epileptogenic HH and should be considered in patients with an early history of GS 2).
A total of five patients with hypothalamic hamartoma were treated by stereotactic thermocoagulation during the period October 1997 through February 2004. In all patients, the hamartoma was less than 10mm in diameter and was located on the floor of the third ventricle with sessile attachment to the wall. To identify ictal onset, chronic intracranial electroencephalography was performed in three patients with the use of a depth electrode implanted in the hamartoma. Attempts were made to induce gelastic seizure by electrical stimulation of the hamartoma in three patients. After magnetic resonance imaging-guided targeting, radiofrequency thermocoagulation of the boundary between the hamartoma and normal hypothalamus was performed to achieve disconnection effects. Marked reductions in seizure frequency were obtained in all cases, with three patients becoming seizure-free after the procedure. No intraoperative complications occurred except in one patient who experienced acute and transient panidrosis with hot flushes during coagulation. Our results suggest that stereotactic thermocoagulation of hypothalamic hamartoma is an acceptable treatment option for patients with intractable gelastic seizures 3).
Adult-onset refractory epilepsy with hypothalamic hamartoma and no gelastic seizures successfully treated by stereotactic thermocoagulation: A case report 4).
A 22-year-old man presented with intractable gelastic seizures and focal seizures refractory to medical treatment. Magnetic resonance imaging showed a 6 mm x 6 mm x 7 mm sessile intraventricular HH. Under local anesthesia, four intra-hamartoma lesions were made via stereotactic radiofrequency thermocoagulation using a depth electrode for recording and stimulation.
Transient central hyperthermia, hypertension, and tachycardia were observed during the coagulation procedure. Intra-hamartoma spikes and slow waves were identified on depth electrode recordings. No gelastic seizure was induced by deep stimulation. The patient was seizure-free during the 12-month follow up and no permanent surgical complications occurred.
Stereotactic radiofrequency thermocoagulation may be an effective and safe treatment option in selected cases of hypothalamic hamartoma with gelastic seizures 5)