Background: Surgery represents a last resort therapy for epilepsy. Anesthesia perioperative care is essential to outcome.

Case Report: We report the clinical cases of two girls with refractary epilepsy associated with Rasmunssen syndrome (RS), proposed for functional cerebral hemisferectomy with neuronavigation due to rapid uncontrolled clinical progression. Anti-epileptic drug therapy was optimized and mainstained in the preoperative period. 

CASE A: 10-year old ASA II girl without additional comorbidities. Anesthesia was mainstained on a remifentanil/propofol target-controlled infusion total intravenous anesthesia along with a rocuronium infusion. Midazolam was administered on induction. Intraoperative convulsion profilaxis with Levetiracetam. An ultrasound-guided central venous catheter (CVC) was positioned on the internal jugular vein. Monitorization with ASA-standard (ASAs) parameters, invasive blood pressure and bispectral index. The procedure was uneventfull and the patient was transfered to the intensive care unit (ICU) under sedation and mechanical ventilation.

CASE B: 7-year old ASA III girl with personal history of early puberty, hemiparesis and obesity. Anesthesia was induced and maintained with a remifentanil/propofol infusions and rocuronium bolus. Valproic acid was chosen for intraopertive convulsion profilaxis. We opted for landmark-guided CVC. The patient was monitored with ASAs parameters, invasive blood pressure and bisepectral index. Surgery went on without mishappenings. The patient was transfered to the ICU under sedation and mechanical ventilation.

Discussion: RS is a progressive disease characterized by drug-resistant focal epilepsy associated with progressive cortical atrophy in more advanced cases, usually affecting one brain hemisphere. Despite its functional consequences, surgery is the most effective method to ensure complete disconnection of the affected hemisphere.

The anesthetic management of these patients includes concern about intraoperative blood loss, as the surgical approach is via a large craniotomy, duration of surgery and delayed recovery, often requiring postoperative ventilatory support and intensive care unit surveillance.

References: Varadkar et al., "Rasmussen's encephalitus: clinical features, phatobiology, and treatment advances", Lacent Neurol. 2014 February; 13(2): 195-205.

Learning points: A multidisciplinary approach to these patients is essential to ensure safety and reduce the risk of complications.

09 / Anesthesia for cerebral hemispherectomy in Rasmunssen Syndrome - Two clinical cases