Anesthetic Management in Pediatric Patient with Moyamoya Disease in Orthopaedic Surgery: “Managing the Precarious Circulation”

A 12-year-old female, diagnosed case of Down phenotype with Moyamoya disease (MMD) was posted for internal fixation of compound fracture shaft of femur. Preanesthetic evaluation revealed history of acute URI, abnormal body movements, left hemiparesis and mental retardation. Patient was managed under general anesthesia with proseal LMA and caudal epidural was administered for adequate analgesia in the perioperative period. The goal of anaesthetic management in a case of MMD is to maintain the balance between cerebral metabolic oxygen consumption rate (CMRO2) and cerebral blood flow (CBF) in order to prevent any neurologic morbidity. Though there is no ideal anaesthetic agent in MMD, the anaesthetic technique should ensure normotension, normocapnia and normothermia in the perioperative period. To our knowledge regional analgesia has not been used in the pediatric orthopedic surgeries in patients diagnosed with Moya moya. The technique of general anaesthesia supplemented with caudal epidural analgesia ensures that all the anesthetic goals are met adequately and the precarious cerebral circulation in moya moya patient is not compromised.