Comparison of Surgical Outcomes in Individuals With Hypothalamic Hamartoma Alone or With Other Potentially Epileptogenic Focal Lesions

Pediatr Neurol. 2022 Jun:131:13-19. doi: 10.1016/j.pediatrneurol.2022.03.004. Epub 2022 Apr 9.

Abstract

Background: Hypothalamic hamartoma is a rare condition associated with refractory seizures. It can occur in isolation or with additional epileptogenic lesions. The aim of this study was to investigate the effects of additional potentially epileptogenic lesions on surgical outcomes in individuals with hypothalamic hamartoma.

Methods: We conducted a chart review of 112 patients with hypothalamic hamartoma who underwent magnetic resonance imaging (MRI)-guided laser interstitial thermal therapy targeted to the hypothalamic hamartoma. We compared surgical outcomes after at least six months of postoperative follow-up (N = 65) between patients with hypothalamic hamartoma alone and those with hypothalamic hamartoma plus additional potentially epileptogenic lesions.

Results: Sixteen out of 112 (14%) patients had additional epileptogenic lesions, including focal cortical dysplasia, gray matter heterotopia, and polymicrogyria. Ten out of 16 patients with additional lesions and 55 out of 96 patients with hypothalamic hamartoma alone had more than six months of follow-up and are included in the outcome analysis. Mean follow-up in these patients is 21.5 ± 17.3 months (standard deviation, range: 7.3-76.8 months) for patients with hypothalamic hamartoma alone and 16.1 ± 15.0 months (standard deviation, range: 6.6-58.2 months) for those with hypothalamic hamartoma plus additional epileptogenic lesions. Fewer patients with hypothalamic hamartoma plus other lesions had Engel class I/II outcomes than patients with hypothalamic hamartoma alone (5/10 [50%] vs 46/55 [83.6%]; P = 0.031).

Conclusions: MRI-guided laser interstitial thermal therapy remains an effective treatment option for patients with hypothalamic hamartoma. However, the outcome of surgical procedures targeted to the hypothalamic hamartoma may be less favorable in patients who have hypothalamic hamartoma coexisting with other potentially epileptogenic focal lesions. Thus, an additional surgical workup is warranted for these patients who have failed surgical treatment of hypothalamic hamartoma.

Keywords: Coexisting epileptogenic lesion; Gelastic seizure; Hypothalamic hamartoma; Laser ablation.

MeSH terms

  • Hamartoma* / complications
  • Hamartoma* / diagnostic imaging
  • Hamartoma* / surgery
  • Humans
  • Hypothalamic Diseases* / complications
  • Hypothalamic Diseases* / diagnostic imaging
  • Hypothalamic Diseases* / surgery
  • Magnetic Resonance Imaging / methods
  • Treatment Outcome

Supplementary concepts

  • Hypothalamic hamartomas