PEDIATRIC ANESTHESIA AND CRITICAL CARE JOURNAL, cilt.2, sa.2, ss.122-125, 2014 (ESCI)
Pantothenatekinase-associated neurodegeneration is a rare progressive disorder characterized by dystonia, rigidity, choreoathetosis and mental deterioration. Patients requiring general anesthesia with this syndrome may have many anesthesia-relevant symptoms that influence the preanesthetic management, the induction of anesthesia and the postoperative care. In this case report, we present the anesthetic management of a 10-year-old male scheduled for bilateral Globus pallidus internus stimulation for pantothenatekinase-associated neurodegeneration under general anesthesia. The patient had to be sedated before the operation because of the extreme dystonia. Anesthesia was induced uneventfully by the use of modern anesthetic agents and a multidisciplinary approach.