Między napadami a sygnałami: Chirurgia kręgosłupa w zespole Draveta - Opis przypadku
Between Seizures and Signals: Spine Surgery in Dravet Syndrome : A Case Report
W skrócie
[Preprint - wstępne wyniki] Zespół Draveta to rzadka choroba neurologiczna, w której pacjenci są bardzo podatni na napady padaczkowe, co utrudnia przeprowadzenie dużych operacji chirurgicznych. W opisanym przypadku 18-latka z potwierdzonym genetycznie zespołem Draveta poddano operacji kręgosłupa, modyfikując standardowe metody znieczulenia i monitorowania, aby nie wywoływać napadów. Operacja przebiegła pomyślnie bez powikłań, co pokazuje, że chirurgia dużego zakresu u pacjentów z zespołem Draveta jest możliwa, jeśli odpowiednio dostosuje się metody leczenia bólu i monitorowania.
Oryginalny abstract (angielski)
Abstract Background Dravet syndrome is a rare developmental and epileptic encephalopathy associated with a high susceptibility to seizures and specific perioperative challenges. Major spinal surgery requires effective analgesia and reliable neurophysiological monitoring, both of which may require adaptation in patients with Dravet syndrome. Case presentation An 18-year-old man with genetically confirmed SCN1A -related Dravet syndrome and pharmacoresistant epilepsy underwent posterior spinal fusion for progressive neuromuscular scoliosis. Because of concerns regarding sodium-channel blockade, surgical-site infiltration with local anaesthetics was omitted and intrathecal morphine was used as the main component of postoperative analgesia. Intraoperative spinal cord monitoring was also modified: somatosensory evoked potentials were maintained, whereas transcranial motor evoked potentials were omitted following multidisciplinary discussion because of concern that transcranial electrical stimulation could precipitate seizures. Total intravenous anaesthesia with propofol and remifentanil was used. Despite substantial blood loss and delayed emergence, no perioperative seizures or new neurological deficits occurred, and postoperative analgesia was satisfactory. Conclusions Major spinal surgery in patients with Dravet syndrome may require individualized adaptation of both analgesic and neurophysiological monitoring strategies. This case illustrates the importance of multidisciplinary assessment when the benefits of standard perioperative techniques must be balanced against seizure susceptibility.