Pantotenat Kinaz İlişkili Nörodejenerasyon (Hallervorden-Spatz) Hastasında Anestezik Yaklaşım ve Azalmış Anestezik Ajan İhtiyacı
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Tarih
2020
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info:eu-repo/semantics/openAccess
Özet
Genel anestezi altında tiroidektomi operasyonu yapılan Pantotenat Kinaz İlişkili Nörodejenerasyon (PKAN) hastasına anestezik yaklaşımımız sunulmuştur. Kırk altı yaşındaki kadın hastaya yutma güçlüğü ve takılma hissi nedeniyle başvurduğu genel cerrahi kliniği tarafından tiroidektomi operasyonu planlandı. Distonik, afonik, göz açıp kapama ile iletişim kurabilen hastanın yüksek kortikal fonksiyonları normaldi. Genel anestezi altında opere edilen hastada entübasyon zorluğu olmadı. İndüksiyonda tiyopental, rokuronyum, fentanil uygulandı. Anestezi idamesi Bispektral Indeks (BIS) monitörizasyonu altında sevofluran ve fentanil ile sağlandı. Hastanın 0,6 Minimum Alveolar Konsantrasyon (MAC) değerinde sevofluran ile BIS değerlerinin %38-40 seviyelerinde seyrettiği, anestezik ajan ihtiyacının azalmış olduğu gözlendi. Hastanın ekstübasyonunda sugammadeks kullanılarak postoperatif dönemde ekstübe halde yoğun bakım ünitesine takip ve tedavi amacıyla transfer edildi. Pantotenat Kinaz İlişkili Nörodejenerasyon (PKAN), eski adıyla Hallervorden- Spatz Hastalığı, otozomal ressesif geçişli, distoni, parkinsonizm ve beyinde demir birikimi ile karakterize bir sendromdur. Pantotenat kinaz 2 (PANK2) enzim mutasyonu nedeniyle gelişir. Anestezik yaklaşım, ağız açıklığı, atetoid postür, distonik hareketler ve bunların yol açtığı servikal patolojiler nedeniyle özellik göstermektedir. Entübasyon zorluğu gözlenmeyen olgumuzda anestezik ajan ihtiyacının oldukça azalmış olduğu dikkat çekmektedir. Önerimiz nöromonitorizasyon tekniklerinin, nörodejeneratif hastalıkların anestezik yönetiminde mutlak surette yer almasıdır.
We present our anesthetic approach to a patient with Pantotenat Kinase Associated Neurodegeneration (PKAN) (formerly called Haller-vorden-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 com-municate 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 sevoflu-rane 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.
We present our anesthetic approach to a patient with Pantotenat Kinase Associated Neurodegeneration (PKAN) (formerly called Haller-vorden-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 com-municate 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 sevoflu-rane 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.
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Kaynak
Cerrahpaşa Medical Journal
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Scopus Q Değeri
Cilt
44
Sayı
2
