Anaesthetic Challenges in Neuronal Ceroid lipofuscinoses for Percutaneous Endoscopic Gastrostomy

Abstract

Neuronal ceroid lipofuscinoses are rare inherited lysosomal storage diseases with multisystem involvement. Here we describe the anesthetic management of a six-year-old female patient who presented with recurrent aspiration pneumonia, multidrug resistant seizures, progressive deterioration of milestones. She was mute with impaired vision and was posted for Percutaneous Endoscopic Gastrostomy. Presence of uncontrolled seizures, difficulty in communication, cognitive impairment, predisposition to bradycardia, hypothermia, risk of aspiration with persistent lung infection were few of the challenges we faced. Optimization of seizure therapy, antibiotics, chest physiotherapy, nebulization with broncho dilators helped in preoperative preparation. Use of glycopyrrolate helped prevent bradycardia and reduce secretions. Propofol and midazolam for induction of anaesthesia helped prevent seizures during the procedure which was done under intravenous sedation with monitored anaesthesia care. Patient was positioned slight head up to prevent aspiration and we were ready to intubate if necessary. Warmers and warm fluids helped prevent hypothermia. The procedure was completed uneventfully