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We present our anesthetic approach to a patient with Pantotenat Kinase Associated Neurodegeneration (PKAN) (formerly called Hallervorden-Spatz disease) who underwent thyroidectomy under general anesthesia. A 46-year-old female patient was admitted to the general surgery department because of dysphagia and swallowing sensation. The patient was dystonic, aphonic, and she was only able to communicate with eye movements. High cortical functions were normal. The patient was operated under general anesthesia. Thiopental, rocuronium, and fentanyl were used for induction and no intubation difficulty was observed. Anesthesia was maintained with sevoflurane and fentanyl under Bispectral Index (BIS) monitoring. It was observed that the need for anesthetic agents decreased, so BIS levels of 38%-40% were obtained with inhalation of 0.6 minimum alveolar concentration sevoflurane. In the postoperative period after extubation using sugammadex, the patient was transferred to the intensive care unit. PKAN is an autosomal recessive syndrome characterized by dystonia, Parkinson disease-like symptoms, and iron deposition in the brain, which is caused by panthotenate kinase 2 enzyme mutation. The anesthetic approach is characterized by reduced mouth opening, atetoid posture, dystonic movements, and cervical pathologies. In our case, endotracheal intubation was easy and the need for anesthetic agents was considerably reduced. Our absolute recommendation is to involve neuromonitoring techniques in the anesthetic management of neurodegenerative diseases.
Our anesthetic approach was presented to the patient with Pantotenat Kinaz-related neurodegeneration (PKAN) under general anesthesia under thyroidectomy surgery. Thirty-six-year-old woman was planned for thyroidectomy surgery by the general surgical clinic that she applied to the patient due to difficulty sweating and the feeling of clinging. The high cortical functions of the patient who was able to communicate with dystonic, afonic, eye open and closure were normal. The patient operated under general anesthesia had no difficulty in entubation. In the injection were applied tiopental, rokuronyum, fentanyl. Anesthesia was provided with sevofluran and fentanyl under BIS monitoring. The patient observed a reduced need for anesthetic agents, with sevofluran at the level of 0.6 Minimum Alveol Concentration (MAC) and BIS values at 38-40%. In the extubation of the patient, sugammadeks was transferred to the intensive care unit in the postoperative period for the purpose of follow-up and treatment. Pantotenate Kinaz Related Neurodegeneration (PKAN), formerly called Hallervorden-Spartz Disease, is a syndrome characterized by autosomal recessive transitional, dystonia, parkinsonism and iron accumulation in the brain. Pantotenate kinase 2 (PANK2) is developed due to the enzyme mutation. It is characterized by anesthetic approach, mouth openness, atetoid posture, distonic movements and cervical pathologies that they cause. The difficulty of entubation is not observed in our case that the need for an anesthetic agent has been significantly reduced. Our suggestion is that neuromonitorization techniques are absolutely involved in the anesthetic management of neurodegenerative diseases.
Alan : Sağlık Bilimleri
Dergi Türü : Uluslararası
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