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Endoscopic resection of hypothalamic hamartomas for refractory symptomatic epilepsy

From the Division of Pediatric Neurology (Y-T.N., J.F.K.), Division of Neuroradiology (E.C.P.), Division of Neurology (N.C.W., S.S.C.), Division of Pediatric Endocrinology (R.E.J.), and Division of Neurological Surgery (H.L.R., I.F.-E., M.R.V.), Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ.

Address correspondence and reprint requests to Dr. Yu-tze Ng, c/o Neuroscience Publications, Barrow Neurological Institute, 350 W. Thomas Rd., Phoenix, AZ 85013 y2ng{at}chw.edu

Background: Hypothalamic hamartomas (HHs), rare developmental abnormalities of the inferior hypothalamus, often cause refractory, symptomatic, mixed epilepsy, including gelastic seizures. We present 37 patients with HH who underwent transcortical transventricular endoscopic resection.

Methods: Between October 2003 and April 2005, 42 consecutive patients with refractory epilepsy who underwent endoscopic resection of HH were studied prospectively. The endoscope was held by an articulated pneumatic arm and tracked with a frameless stereotactic neuronavigation system. Data collection and follow-up were performed by personal interview. Five patients were excluded. The remaining 37 patients (22 males, 15 females; median age 11.8 years; range 8 months to 55 years) had frequent and usually multiple types of seizures.

Results: Postoperative MRI confirmed 100% resection of the HH from the hypothalamus in 12 patients. At last follow-up (median 21 months; range 13–28 months), 18 (48.6%) patients were seizure free. Seizures were reduced more than 90% in 26 patients (70.3%) and by 50% to 90% in 8 patients (21.6%). Overall, the mean postoperative stay was shorter in the endoscopic patients compared with our previously reported patients who underwent transcallosal resection (mean 4.1 days vs 7.7 days, respectively; p = 0.0006). The main complications were permanent short-term memory loss in 3 patients and small thalamic infarcts in 11 patients (asymptomatic in 9).

Conclusions: Endoscopic resection of hypothalamic hamartoma (HH) is a safe and effective treatment for seizures. Its efficacy seems to be comparable to that of transcallosal resection of HH, but postoperative recovery time is significantly shorter.

Abbreviations: AED = antiepileptic drug; DDAVP = desmopressin acetate; GTC = generalized tonic-clonic; HH = hypothalamic hamartoma; OR = odds ratio.

Supplemental data at www.neurology.org

Disclosure: The authors report no conflicts of interest.

Received June 16, 2007. Accepted in final form January 2, 2008.

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