Surgical approaches to hypothalamic hamartomas
Scott D. Wait, M.D., Adib A. Abla, M.D., Brendan D. Killory, M.D., Peter Nakaji, M.D., and Harold L. Rekate, M.D., Neurosurg Focus 30(2) E2 2011 Journal of Neurosurgery
ABSTRACT:
Object: Hypothalamic hamartomas (HHs) are devastating lesions causing refractory epilepsy, rage attacks, social ineptitude, and precocious puberty. Microsurgical and/or endoscopic resection offers an excellent risk/benefit profile for cure or improvement of epilepsy.
Methods: The authors reviewed a prospective database maintained during the first 7 years of the Barrow Hypothalamic Hamartoma program. They describe and illustrate their surgical methods, and they review data from several previous publications regarding surgical outcome.
Results: To date, the authors have performed surgery in 165 patients for symptomatic HHs. Patients underwent an endoscopic, transcallosal, or skull base approach, or multiple approaches. Twenty-six patients (15.8%) required more than 1 treatment for their HH.
Conclusions: Microsurgical and endoscopic resection of symptomatic HHs are technically demanding but can be performed safely with excellent results and an acceptable risk profile. Meticulous attention to the subtleties of surgical management helps optimize outcomes.
Repeat surgery for hypothalamic hamartoma in refractory epilepsy
Sandipan Pati, M.D., Adib A. Abla, M.D., Harold L. Rekate, M.D., and Yu-Tze Ng, M.D, Neurosurg Focus 30(2) E3 2011 Journal of Neurosurgery
ABSTRACT:
Object: Hypothalamic hamartomas (HHs) often cause pharmacoresistent epilepsy, incapacitating behavioral abnormalities, and cognitive decline. Surgical intervention offers the patient the best opportunity of seizure resolution, which occurs in approximately 50%–60% of patients, and improvement in both cognitive and behavioral difficulties. For those in whom the initial operation has failed, further medical treatment options remain quite limited, whereas, in some cases, a second surgery may improve seizure outcome. The authors retrospectively reviewed their surgical cases to document the success rate and complications of reoperations in patients with HHs.
Methods: Data were obtained from the HH epilepsy surgery database at the Barrow Neurological Institute between 2003 and 2010. Surgical treatment consisted of open and endoscopic procedures, as well as radiosurgery. Demographic details, seizure history, presurgical evaluation, and postoperative follow-up data were evaluated.
Results: In the last 7 years, 21 (13%) of 157 patients underwent reoperation after an initial epilepsy operation. The initial surgical approach in the 21 patients included: endoscopic (8 patients [38%]), transcallosal (8 patients [38%]), orbitozygomatic (3 patients [14%]), and radiosurgery (2 patients [10%]). Of the 8 patients who initially underwent endoscopic resection, repeat procedures included: radiosurgery in 4 (50%), an orbitozygomatic approach in 2 (25%), repeat endoscopy in 1 (12.5%), and a transcallosal approach in 1 (12.5%). Repeat procedures after an initial transcallosal resection included: endoscopic resection in 2 (25%); radiosurgery in 1 (12%); an orbitozygomatic approach in 2 (25%), and repeat transcallosal surgery in 3 (38%). Predominant seizure types that recurred after the first surgery were gelastic seizures, complex partial seizures, and tonic-clonic seizures. Magnetic resonance imaging in all patients prior to reoperation demonstrated either residual HH and/or connection with the mammillary bodies. Review of patients with more than 6 months of follow-up since the last surgery showed greater than 90% reduction in seizures in 4 patients (19%) and by 50%–90% in 10 patients (48%). Two patients were seizure free, and in 5 patients (24%) there was no change in seizure frequency. Following reoperation, none of the patients had any worsened behavioral issues such as increased rage attacks or disruptive violent behavior. New postoperative complications after reoperation included hemiparesis, thalamic stroke (asymptomatic and symptomatic), hyperphagia, and panhypopituitarism.
Conclusions: Reoperation should be considered in selected patients with HH in whom initial epilepsy surgery fails because more than half the patients have significant reductions in seizure.
Long-term outcome after emergency resection of hypothalamic hamartomas for status gelasticus
Sandipan Pati, M.D., Reena G Rastogi, M.D., Adib A. Abla, M.D., Harold L. Rekate, M.D., and Yu-Tze Ng, M.D Neurosurg Focus 30(2) E5 2011 Journal of Neurosurgery
ABSTRACT:
Object: Gelastic seizures are epileptic events characterized by bouts of laughter. They are rare and mostly associated with hypothalamic hamartomas (HHs). Status gelasticus, a rare form of status epilepticus, is defined as a prolonged cluster of gelastic seizures (> 20–30 minutes) without necessarily involving loss of awareness between seizures. Emergency resection of the hamartoma is highly effective in these situations and should be considered as early as possible. The authors retrospectively reviewed their surgical cases to document the success, complications, and long-term follow-up after emergency resection of HHs for status gelasticus.
Methods: The authors report on a retrospective case series from a single tertiary care center. Three patients who presented with status gelasticus underwent emergency resection of HHs. Demographic details, seizure history, medical treatment, and postoperative follow-up data were evaluated. Long-term follow-up (minimum 2 years) data were obtained either from the last clinic visit notes or via telephone and e-mail contacts. The institutional review board at St. Joseph’s Hospital approved this study.
Results: In the last 7 years, of 157 patients who underwent HH resection, the resection was performed on an emergency basis for status gelasticus in 3 cases. At emergency surgery, these 3 patients ranged in age from 9 months to 3.5 years. All of the patients were boys. Delalande and Fohlen Type II, III, and IV lesions were present in the 3 patients. Surgical approaches for resection of HH included an orbitozygomatic, transcallosal anterior interforniceal approach and endoscopic resection. Status gelasticus was terminated following emergency surgery in all cases, and 1 patient was seizure free. Postsurgical complications included, in 1 case, a small right thalamic infarct with mild transient left hemiparesis, which completely resolved within 2 days. Within 2 years of their original surgery, 2 patients underwent further elective surgeries (endoscopic resection and radiosurgery for persistent symptomatic seizures). Follow-up since their most recent surgery ranged from 8 months to 2 years. Two patients were seizure free and 1 patient had greater than 50% reduction in seizures.
Conclusions: Status gelasticus associated with HHs can be successfully terminated by emergency resection of the HH. Long-term follow-up in the present series suggests good seizure freedom results or at least greater than 50% reduction in seizures, although repeat operations were necessary.
ABSTRACT:
Surgical Management of Hypothalamic Hamartoma in Patients with Gelastic Epilepsy
Addas and others, Neurosurg. Focus 25(3) E8 2008 ©
ABSTRACT: Gelastic epilepsy (GE) associated with hypothalamic hamartomas (HHs) is now a well-characterized clinical syndrome consisting of gelastic seizures starting in infancy, medically refractory seizures with or without the development of multiple seizure types, and behavioral and cognitive decline. It has been postulated that the development of the HHGE syndrome is a result of a progressive epileptic encephalopathy or secondary epileptogenesis, which is potentially reversible with treatment of the HH. A variety of surgical options for the treatment of HHs exist, including open and endoscopic procedures, radiosurgery, interstitial radiotherapy, and stereotactic radiofrequency thermocoagulation. Surgical treatment can result in seizure freedom in up to 50% of patients and can be accompanied by significant improvements in behavior, cognition, and quality of life. Partial treatment of HHs may be sufficient to reduce seizure frequency and improve behavior and quality of life with less risk. A component of reversible cognitive dysfunction may be present in some patients with an HH-GE syndrome.
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Radiosurgery for Hypothalamic Hamartoma
Romanelli, Striano and others published Neurosurg. Focus 24 (5): E9 2008 ©
ABSTRACT: Radiosurgery plays an important role in the treatment of refractory seizures induced by hypothalamic hamartomas (HHs). These lesions, deeply located and surrounded by a delicate vascular and neuronal network, are often associated with catastrophic epilepsy leading to progressive cognitive and behavioral deterioration. Surgical approaches include microsurgical resection, endoscopic resection or disconnection, radiofrequency lesioning, and interstitial brachytherapy. Radiosurgery is an emerging treatment modality for HHs, which provides excellent seizure outcomes and no lasting complications to date.
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Assessment of cognitive functions before and after stereotactic
interstitial radiosurgery of hypothalamic hamartomas
in patients with gelastic seizures
A. Quiske, J. Unterrainer, K. Wagner, L. Frings, T. Breyer, U. Halsband,
C. Ostertag, C.E. Elger, A. Ebner, I. Tuxhorn, J.-P. Ernst, B.J. Steinhoff,
T. Mayer, A. Schulze-Bonhage, Epilepsy & Behavior 10:328-332 © 2007 Elsevier Inc.
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Gamma Knife Surgery for Epilepsy Related to Hypothalamic Hamartomas
Jean Regis et al, Semin Pediatr Neurol 14:73-79 © 2007 Elsevier Inc.
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Treatment Options for Gelastic Epilepsy Due to Hypothalamic Hamartoma: Interstitial Radiosurgery
Adreas Schulze-Bonhage et al, Semin Pediatr Neurol 14:80-87 © 2007 Elsevier Inc.
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Hypothalamic Hamartoma Treatment: Surgical Resection With the Transcallosal Approach
Jeffrey Rosenfeld et al, Semin Pediatr Neurol 14:88-98 © 2007 Elsevier Inc.
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Endoscopic Resection of Hypothalamic Hamartoma for Refractory Epilepsy: Prelimnary Report
Yu-tze Ng and Harold Rekate, Semin Pediatr Neurol 14:99-105 © 2007 Elsevier Inc.
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Transcallosal Resection of Hypothalamic Hamartomas in Patients with Intractable Epilepsy
Simon Harvey et al, Epileptic Disorders, 2003 5: 257-65
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Hypothalamic Hamartoma with Refractory Epilepsy: Surgical Procedures and Results in 18 Patients
Fohlen, Delalande, Epileptic Disorders, 2003 5: 267-73
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Stereotactic Surgical Approach to Hypothalamic Hamartomas.
Kuzniecky and Guthrie, Epileptic Disorders 2003 5: 275-80 © 2003 Epileptic Disorders.
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Transcallosal Resection of Hypothalamic Hamartoma with Control of Seizures in Children with Gelastic Epilepsy
Rosenfeld et al, Neurosurgery Vol 48 No.1 Jan 2001 p108 – 118 © Lippincott, Williams and Wilkins
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