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[Surgical disconnection of hypothalamic hamartomas].

AbstractBACKGROUND AND PURPOSE:
Surgical resection of hypothalamic hamartomas (HHs) associated with drug-resistant gelastic epilepsy carries a considerable risk of neurological and endocrine morbidity. Alternative surgical routes and techniques have therefore been proposed, especially for broadly attached lesions and for those with a third ventricular location.
METHODS:
We present an updated series of 43 patients (aged nine months to 34 years), operated on from 1998 through 2005 at our institution. The hamartoma was disconnected using a microsurgical pterional approach of those lesions extending from the hypothalamic floor downward into the interpeduncular cistern. When the HH presented as a paramedian mass, partly or exclusively bulging into the third ventricle, with a rather vertical plane of attachment, we chose a frameless stereotactic endoscopic technique to disconnect the lesion. In several of our patients, both methods were applied subsequently.
RESULTS:
Surgery-related morbidity was lower with the ventricular endoscopic technique. Twenty-one patients (50%) are seizure-free and two patients (5%) almost seizure-free, while in 17 patients (40%), there was a significant seizure reduction. Two patients (5%) had no postoperative improvement. According to the different topographic features of the HHs, for which we have recently proposed a classification into four subtypes, the intraventricularly located hamartoma had the best prognosis following endoscopic disconnection. Ten of the 12 patients (83%) with this HH location became seizure-free.
CONCLUSIONS:
Resection of epilepsy-related HHs can be replaced by disconnective procedures. Our results confirm their feasibility and acceptable morbidity, with particularly good seizure outcome in patients with intraventricularly located HHs.
AuthorsG Dorfmüller, M Fohlen, C Bulteau, O Delalande
JournalNeuro-Chirurgie (Neurochirurgie) Vol. 54 Issue 3 Pg. 315-9 (May 2008) ISSN: 0028-3770 [Print] France
Vernacular TitleDéconnexion chirurgicale des hamartomes hypothalamiques.
PMID18452954 (Publication Type: English Abstract, Journal Article)
Topics
  • Adolescent
  • Adult
  • Cerebral Ventricles (pathology)
  • Child
  • Child, Preschool
  • Endoscopy
  • Epilepsy (classification, etiology)
  • Female
  • Hamartoma (complications, pathology, surgery)
  • Humans
  • Hypothalamic Diseases (complications, pathology, surgery)
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Neurosurgical Procedures (adverse effects)
  • Postoperative Complications (epidemiology)
  • Radiosurgery
  • Seizures (surgery)
  • Treatment Outcome

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