High-Intensity Focused Ultrasound for Treatment of Hypothalamic Hamartoma: A Case Report
Abstract number :
3.214
Submission category :
4. Clinical Epilepsy / 4C. Clinical Treatments
Year :
2023
Submission ID :
1201
Source :
www.aesnet.org
Presentation date :
12/4/2023 12:00:00 AM
Published date :
Authors :
Presenting Author: Sonia Ajmera, MD – University of Pennsylvania
Marissa DiGiovine, MD – Neurology – Children's Hospital of Philadelphia; Kathleen Galligan, MS, PA – Children's Hospital of Philadelphia; Casey Halpern, MD – University of Pennsylvania; Benjamin Kennedy, MD – Univerisity of Pennsylvania; Liming Qiu, MD – Neurosurgery – University of Pennsylvania
Rationale: Hypothalamic hamartomas are sporadic, non-progressive lesions that can result in debilitating symptoms, including seizures, cognitive deficits, and psychiatric changes. Open or endoscopic surgical resection, laser ablation, and Gamma Knife (GK) radiosurgery in this region can be associated with high morbidity. As such, magnetic resonance-guided high-intensity focused ultrasound (HIFU) can be considered an alternative treatment option.
Methods: We report a case of an 18-year-old highly functional male with intractable epilepsy, including classic gelastic seizures, secondary to a hypothalamic hamartoma. The patient was having 10 to 12 gelastic seizures per day, with new staring spells progressing to generalized tonic-clonic seizures. He had previously undergone partial surgical resection in 2013 with residual memory and visual deficits. Given the progression of epilepsy and his one remaining intact fornix and mammillary body complex wrapped around a very small residual hamartoma, HIFU was deemed the most favorable treatment option. Food and Drug Administration approval was obtained for single compassionate use of HIFU for this indication. Pre-operative MRI was obtained for ultrasound ablation target planning. The procedure was performed under general anesthesia.
Results: The patient was placed in a modified Leksell stereotactic frame (Elekta). The Exablate Model 4000 focused ultrasound system (Insightec) was used. Intra-procedural MRI T2 and thermometry were performed for treatment monitoring and eloquent structures such as the fornix and mammillary bodies were outlined for real-time temperature surveillance. To prevent damage to these structures, when temperatures exceeded consensus limits (46 degrees Celcius) sonication was halted. The patient was extubated and underwent post-procedure monitoring in the Intensive Care Unit without issue. He remained at his neurologic baseline with no new visual or memory deficits and was discharged on post-procedure day (PPD) two after physical and occupational therapy assessments. At PPD 10, he reported decreased gelastic seizure frequency at one to two seizures per day; he had no more generalized seizures. At PPD 30, he was having seizure free days with parents reporting improvement in memory and mood compared to pre-procedurally.
Conclusions: HIFU is a safe and viable minimally invasive, targeted, fast-acting treatment option for symptomatic hypothalamic hamartoma.
Funding: Not applicable
Clinical Epilepsy