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Multiple treatment options are available for patients with hypothalamic hamartoma (HH) including the frontotemporal approaches, the anterior transcallosal transseptal interforniceal approach, the transventricular endoscopic approach, and stereotactic radiosurgery. Relatively large patient series of the transcallosal resection/disconnection from Royal Children's Hospital in Melbourne and the Barrow Neurological Institute in Phoenix, AZ, show, respectively, that 52% to 54% are 100% seizure free, and 24% to 35% have >90% seizure reduction. However, there appears to be an 8% to 14% risk of persisting memory problems. The surgery should ideally be performed in the early years of childhood before secondary generalized epilepsy develops and developmental delay and behavioral problems are established. Radiosurgery may be a preferable option for higher-functioning adolescent or adult patients with HH. The choice of treatment must be individualized depending on the age and clinical circumstances of the patient and the size and anatomic relationships of the hamartoma. The transcallosal resection of HH is an effective and safe treatment, but there is a small risk of short-term memory impairment. The endoscopic approach is an alternative to the transcallosal approach for smaller HH.

Introduction: There is increasing evidence that removal or disconnection of hypothalamic hamartomas can lead to seizure control and improvement in behavior. The purpose of this study is to report our experiences with endoscopic removal of these hamartomas., Methods: This is a prospective review of the early outcome of 44 patients undergoing endoscopic resection utilizing frameless stereotaxis and a micromanipulator., Results: All patients had normal-sized ventricles. Complete removal was possible in 14 patients, 13 of whom were seizure free at follow-up. Four patients suffered complications that have persisted for more than 3 months: One had significant hemiparesis and three had persistent difficulties with short-term memory. Postoperatively, no patient suffered a permanent hormonal deficiency. In one patient who had undergone a previous attempt at transcallosal resection, the procedure was abandoned because of the lack of internal anatomic landmarks. In a second patient, insufficient mass could be removed due to the size of the lesion and the distorted anatomy. Consequently, 1 week later, the patient underwent a transcallosal approach., Conclusion: In appropriately selected patients, sessile hypothalamic hamartomas can be removed completely or they can be completely disconnected from the hypothalamus with an acceptable risk. At least a year after surgery is needed to evaluate the efficacy of the procedure in terms of control of seizures and improvements in behavior and emotional ability.

Purpose: To present the results of transcallosal surgical resection of hypothalamic hamartoma (HH) in 26 patients with refractory epilepsy in a prospective outcome study., Methods: Patients with refractory epilepsy symptomatic to HH were referred for surgical resection of their HH (mean age, 10.0 years; range, 2.1-24.2 years). A transcallosal, interforniceal approach was used to remove and/or disconnect the hamartoma. Volumetry was obtained on pre- and postoperative brain MRI scans to determine percentage of resection. Outcome assessment included determination of postoperative seizure frequencies in comparison to baseline and the incidence of postoperative complications. Postoperative changes in cognitive and behavioral functioning, in comparison to baseline, were elicited by parental report., Results: The average postoperative follow-up interval was 20.3 months (range, 13-28 months). Fourteen (54%) patients were completely seizure free, and nine (35%) had at least a 90% improvement in total seizure frequency. Parents reported postoperative improvement in behavior in 23 (88%) patients and in cognition in 17 (65%) patients. Transient postoperative memory disturbance was seen in 15 (58%) patients, but persisted in only two (8%). Two (8%) patients had persisting endocrine disturbance requiring hormone replacement therapy (diabetes insipidus and hypothyroidism in one each). With univariate analysis, the likelihood of a seizure-free outcome correlated with younger age, shorter lifetime duration of epilepsy, smaller preoperative HH volume, and 100% HH resection., Conclusions: Refractory epilepsy associated with HH can be safely and effectively treated with surgical resection by a transcallosal, interforniceal approach. Short-term memory deficits appear to be transient for most patients, and family perception of the impact of surgery on cognitive and behavioral domains is favorable. Complete resection yields the best result.

Objective: We present a novel variable aspiration tissue resector for use with neuroendoscopy., Methods: Two patients, 4 and 14 years old, respectively, presented with intractable gelastic seizures refractory to maximal medical therapies. Magnetic resonance imaging showed mass lesions of the third ventricle consistent with hypothalamic hamartoma., Results: The patients underwent magnetic resonance imaging wand-guided endoscopic resection of the tumor with the Suros novel variable aspiration tissue resector. There were no device-associated complications or adverse events. The hamartoma was disconnected in one patient, and gross total resection was achieved in the other., Conclusion: Endoscopy for tumor resection is still frustrated by the lack of surgical tools, such as ultrasonic aspirators, comparable with those available for use during open procedures. The variable-aspiration tissue resector reported here can be used to resect tumor tissue safely. These two cases demonstrate that gross total resection of small hypothalamic hamartomas is feasible with minimal morbidity through an endoscopic approach.

Object: The authors provide evidence that direct resection of hypothalamic hamartomas (HHs) can improve associated gelastic and nongelastic seizures., Methods: Ten children younger than 17 years of age underwent resection of HHs (nine sessile and one pedunculated) that were causing refractory epilepsy. Lesions were approached from above transventricularly through a transcallosal anterior interforniceal approach in six cases, endoscopically through the foramen of Monro in one, and from below with a frontotemporal craniotomy including an orbitozygomatic osteotomy in three. Medical charts were reviewed retrospectively, and follow-up data were obtained through office records and phone calls. Follow-up periods ranged between 12 and 84 months (mean 16.8 months). All patients in whom the approach was from above had sessile HHs. Five were free from seizures at follow up and two had a reduction in seizures of at least 95%. The transventricular route allowed excellent exposure and visualization of the local structures during resection. Among the three patients in whom the approach was from below, one became free of seizure after two procedures and one had a 75% reduction in epilepsy; the latter two had sessile HHs. The exposure was inadequate, and critical tissue borders were not readily apparent. Although the HH was adequately exposed and resected, the epilepsy persisted in the third patient, who had a pedunculated lesion. The overall rate of major permanent hypothalamic complications appeared to be slightly lower for the orbitozygomatic osteotomy group., Conclusions: Sessile lesions are best approached from above. Approaches from below adequately expose pedunculated hamartomas. The likelihood of curing seizures seems to be higher when lesions are approached from above rather than from below.