Surgical Approaches to Hypothalamic Hamartomas
Surgical Approaches to Hypothalamic Hamartomas
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.
Hypothalamic hamartomas are rare CNS nonneoplastic lesions composed of hyperplastic neural tissue associated with 2 patterns of presentation. The Delalande classification classifies HHs based on size, and amount of and laterality of attachment to the hypothalamus. Children with HHs that are significantly attached to and involved with the hypothalamus become symptomatic with gelastic seizures (Delalande Types II, III, and IV). These lesions are usually resistant to medication and if left untreated devolve into more sinister epileptic patterns as the child grows. Furthermore, children with these lesions may develop problems with social interaction, including frequent rage attacks. In contrast, Delalande Type I HH can cause central precocious puberty and may be treated medically with excellent results. This type of HH seldom manifests with epilepsy or behavioral problems, and microsurgical excision tends to be curative.
The Barrow Hypothalamic Hamartoma Program was established late in 2003. Thus far, we have treated many patients with HH. The goal of this manuscript is to describe, in a single, free, online, easily accessible publication, the current methods of resection and to share pitfalls and pearls that we have learned during the process. The approaches used can be classified as endoscopic, transcallosal, or skull base. We also review data previously published by our group on the outcomes and complications associated with this challenging group of patients. This project is ongoing and continues to accrue data.
Abstract and Introduction
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.
Introduction
Hypothalamic hamartomas are rare CNS nonneoplastic lesions composed of hyperplastic neural tissue associated with 2 patterns of presentation. The Delalande classification classifies HHs based on size, and amount of and laterality of attachment to the hypothalamus. Children with HHs that are significantly attached to and involved with the hypothalamus become symptomatic with gelastic seizures (Delalande Types II, III, and IV). These lesions are usually resistant to medication and if left untreated devolve into more sinister epileptic patterns as the child grows. Furthermore, children with these lesions may develop problems with social interaction, including frequent rage attacks. In contrast, Delalande Type I HH can cause central precocious puberty and may be treated medically with excellent results. This type of HH seldom manifests with epilepsy or behavioral problems, and microsurgical excision tends to be curative.
The Barrow Hypothalamic Hamartoma Program was established late in 2003. Thus far, we have treated many patients with HH. The goal of this manuscript is to describe, in a single, free, online, easily accessible publication, the current methods of resection and to share pitfalls and pearls that we have learned during the process. The approaches used can be classified as endoscopic, transcallosal, or skull base. We also review data previously published by our group on the outcomes and complications associated with this challenging group of patients. This project is ongoing and continues to accrue data.
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