Abstracts

Comparison of surgical outcomes in patients with hypothalamic hamartoma alone or with coexisting other potentially epileptogenic focal lesions: A single-center experience

Abstract number : 1010
Submission category : 9. Surgery / 9B. Pediatrics
Year : 2020
Submission ID : 2423343
Source : www.aesnet.org
Presentation date : 12/7/2020 1:26:24 PM
Published date : Nov 21, 2020, 02:24 AM

Authors :
Maureen Handoko, Baylor College of Medicine; Cemal Kerakas - Baylor College of Medicine; Nisha Gadgil - Baylor College of Medicine; Angus Wilfong - Barrow Neurological Institute at Phoenix Children’s Hospital; James Riviello - Baylor College of Medicine;


Rationale:
Hypothalamic hamartomas (HH) are developmental malformations associated with medically refractory gelastic/dacrystic seizures with and without secondary seizure types, for which surgical intervention is an effective treatment. Many patients with HH have additional coexisting potentially epileptogenic lesions such as focal cortical migrational defects. There is not an investigational study describing the effect of these additional potentially epileptogenic lesions on surgical outcomes in patients with HH.
Method:
We conducted a retrospective chart review study on 112 pediatric patients with HH who underwent Magnetic Resonance Imaging-guided Laser Interstitial Thermal Therapy (MRgLITT) targeted to the HH in a single tertiary care epilepsy center. We compared baseline seizure characteristics and surgical outcome after at least 6 months of postoperative follow-up(N=65) between patients with HH only (HH group) and with HH and additional potentially epileptogenic lesions (HH plus group) on brain Magnetic Resonance Imaging (MRI).
Results:
Out of 112 patients with HH, 16 (14%) had additional potentially epileptogenic lesions. These include focal cortical dysplasia, periventricular nodular heterotopia, and focal polymicrogyria. Sixty-five patients had at least 6 months of follow-up after their last surgery. The median duration of postoperative follow up was 13 months (interquartile range 11.6-20.5 months). There was no statistically significant difference in the proportion of patients having at least daily seizures in HH group (90.6%) compared to HH plus group (93.8%, P=1.000) at preoperative baseline. Likewise, the proportion of patients with other seizure types at baseline (in addition to gelastic/dacrystic seizures) in HH group (53.1%) compared to HH plus group (56.3%) was not statistically significantly different (P=1.000). Five out of 10 (50%) patients in HH plus additional focal lesions had Engel class 1 or 2 outcomes compared to 46 out of 55 (83.6%) patients with HH only (P=0.031). Four out of 10 (40%) patients with HH plus additional focal lesions became seizure-free after their last surgery, compared to 41 out of 55 (74.5%) patients with HH only (P=0.057).
Conclusion:
Surgical intervention remains an effective treatment option for patients with HH. However, the outcome of surgical procedures targeted to the HHis less favorable in HH patients with coexisting other potentially epileptogenic focal lesions such as focal cortical dysplasia, periventricular nodular heterotopia, and focal polymicrogyria. Thus, additional surgical workup may be warranted for HH patients with co-existing other pathologies who have failed surgical treatment for HH.
Funding:
:None
Surgery