Perioperative management and outcome of patients with Rett syndrome undergoing scoliosis surgery: a retrospective review

Rett syndrome is a rare genetically inherited neuromuscular disorder exclusively affecting female patients. Progressive scoliosis is one of the main features of the disease and affected individuals are very likely to need spine correction surgery. We undertook a retrospective notes review of patients with Rett syndrome who had undergone spine surgery from 2005 to 2013. Patients were identified through the hospital's electronic records. The aim of the present study was to identify the anesthetic implications encountered and the perioperative adverse events, in an effort to improve perioperative management and reduce complications. We identified twenty-four children who had 29 procedures in total in this period. Frequent chest infections and poorly controlled epilepsy were the main preoperative findings. There were no adverse events during induction and intubation. Common anesthetic/analgesic drugs were used throughout. Postoperatively, gastrointestinal and respiratory tract complications were the most common. Mean intensive care unit stay was 8.1 days and mean time to discharge from hospital was 26.5 days. We had one in-hospital death. Our case series demonstrates a high incidence of complications in this subpopulation, mainly postoperative. Extreme postoperative vigilance is required and recovery in a high dependency unit is highly recommended.