Corpus Callosotomy in LIS1 Lissencephaly: Postoperative Ketogenic Diet-Related Metabolic Derangements and Feeding...

Classic lissencephaly from PAFAH1B1 (LIS1) haploinsufficiency presents with early, drug-resistant epilepsy; palliative strategies such as corpus callosotomy (CC) and vagus nerve stimulation (VNS) can be effective but are challenging to coordinate in children maintained on ketogenic dietary therapy (KDT). We report the case of a 6-year-old girl with de novo PAFAH1B1 deletion who underwent total CC with VNS generator replacement for medically refractory epileptic spasms and focal seizures. Her post-operative course featured early breakthrough seizures responsive to benzodiazepine rescue, then a pediatric ICU (PICU) readmission for emesis, somnolence, edema, and nitrite-positive urinalysis; empiric ceftriaxone was administered while a pretreated catheterized urine culture returned commensal flora/mixed growth, a common interpretive challenge in neurogenic bladder. KDT-associated derangements included β-hydroxybutyrate 8.52 mmol/L, bicarbonate 9-16 mmol/L, and hypoglycemia to 48 mg/dL, managed with acetate-containing IV fluids, protocolized glucose rescue, and ratio adjustment. After a negative anatomic evaluation, erythromycin 75 mg QID improved tolerance to continuous peptide feeds, with near-resolution of emesis by discharge. This case highlights integrated CC+VNS care within KDT using proactive metabolic surveillance, stepwise dysmotility management, and antimicrobial stewardship in neurogenic bladder.