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Friedreich Ataksisi Olan Hastaların Skolyoz Cerrahisinde Anestezi Yönetimi: Dört Olgu Sunumu

Year 2022, , 789 - 792, 31.12.2022
https://doi.org/10.54005/geneltip.1164943

Abstract

Friedreich ataksisi (FRDA), kardiyak, endokrin ve pulmoner komorbiditelerin yanısıra skolyozun da eşlik edebileceği otozomal resesif geçişli nörodejeneratif bir hastalıktır. Bu raporda Friedreich ataksili dört hastada, skolyoz cerrahisinde total intravenöz anestezi (TIVA) yöntemi ile anestezi deneyimlerimizi sunmayı amaçladık. Hastalar, santral venöz basınç (CVP), bispektral indeks (BIS), invaziv kan basıncı ve near infrared spectroscopy (NIRS) ile izlendi. Friedreich ataksisi olan hastalarda malign hipertermi ve zor hava yolu riski yüksektir. Bu nedenle zor hava yolu için gerekli ekipman hazırlığı ve malign hipertermi riski için dantrolen sağladık. Bir olguda anestezi indüksiyonunda kas gevşemesi sağlayabilmek için kas gevşetici kullandık, diğer üç olguda ise kas gevşetici kullanmadık. Friedreich ataksisi olan hastalarda kalpte hipertrofik kardiyomiyopati sıktır. Bu nedenle Friedreich ataksili hastalarda hemodinamik stabilite için detaylı preoperatif değerlendirme, intraoperatif yakın kanama takibi, kan gazı analizi, hemodinamik izleme ve postoperatif multimodal analjezi önemlidir. Tıbbi literatür rehberliğinde, skolyoz cerrahisi geçiren Friedreich ataksili dört hastadaki anestezi yönetimi deneyimimizi sunmayı amaçladık.

References

  • 1-Pandolfo M: Friedreich Ataxia: the clinical Picture. J Neurol 2009; 256:3–8.
  • 2- Mancuso M, Orsucci D, Choub A, Siciliano G. Current and emerging treatment options in the management of Friedreich ataxia. Neuropsychiatr Dis Treat. 2010;6:491-9.
  • 3- Kurul SH, Yiş U, Güzel AI, Kasap H, Başak N, Dirik E. Evaluation of the Cases with Friedreich Ataxia. Gulhane Med J. 2013; 55: 123-7.
  • 4- Morgan GE, Mikhail MS, Murray MJ, Larson CP (Editor). 3rd edition, United States of America: Lange Medical Books. 2004; 9: 178-98.
  • 5-Corben LA, Lynch D, Pandolfo M, Schulz JB, Delatycki MB; Clinical Management Guidelines Writing Group. Consensus clinical management guidelines for Friedreich ataxia. Orphanet J Rare Dis. 2014;9:184.
  • 6- Scott B.K, Baranov D. Neurologic Diseases. In: Fleisher L.A, eds. Anesthesia and Uncommon Diseases, 6th edn. Philadelphia: W.B. Saunders,2012; 275-6.
  • 7- Delatycki MB, Corben LA. Clinical features of Friedreich ataxia. J Child Neurol 2012;27:1133-7.
  • 8- Schmitt HJ, Wick S, Münster T. Rocuronium for muscle relaxation in two children with Friedreich’s ataxia, Br J Anaesth 2004;92:592-6.
  • 9- Romero A, Joshi GP. Neuromuscular disease and anesthesia. Muscle Nerve. 2013;48:451-60. 10- Pancaro C, Renz D. Anesthetic management in Friedreich’s ataxia, Pediatric Anesthesia 2005;15:433-4.

Anesthesia Management in Scoliosis Surgery of Patients with Friedreich’s Ataxia: A Report of Four Cases

Year 2022, , 789 - 792, 31.12.2022
https://doi.org/10.54005/geneltip.1164943

Abstract

Friedreich's ataxia (FRDA) is an autosomal recessive neurodegenerative disease, which may be accompanied by scoliosis, cardiac, endocrine and pulmonary comorbidities. We present our anesthesia experiences using total intravenous anesthesia (TIVA) method in scoliosis surgery of four patients with Friedreich ataxia. Patients were monitored with central venous pressure (CVP), bispectral index (BIS),invasive blood pressure and near infrared spectroscopy (NIRS). The risk of malignant hyperthermia and difficult airway are increased in patients with Friedreich's ataxia. We were prepared for difficult airway and provided dantrolene preoperatively. Neuromuscular blocker was used for anesthesia induction in one case, we did not use it in the other cases. Hypertrophic cardiomyopathy may accompany patients with Friedreich's ataxia. Therefore, detailed preoperative examination, intraoperative close bleeding, blood gas analysis, hemodynamic monitoring and postoperative multimodal analgesia for hemodynamic stability in patients with Friedreich's ataxia were performed. We aimed to discuss our anesthesia management with four patients with Friedreich's ataxia who underwent scoliosis surgery.

References

  • 1-Pandolfo M: Friedreich Ataxia: the clinical Picture. J Neurol 2009; 256:3–8.
  • 2- Mancuso M, Orsucci D, Choub A, Siciliano G. Current and emerging treatment options in the management of Friedreich ataxia. Neuropsychiatr Dis Treat. 2010;6:491-9.
  • 3- Kurul SH, Yiş U, Güzel AI, Kasap H, Başak N, Dirik E. Evaluation of the Cases with Friedreich Ataxia. Gulhane Med J. 2013; 55: 123-7.
  • 4- Morgan GE, Mikhail MS, Murray MJ, Larson CP (Editor). 3rd edition, United States of America: Lange Medical Books. 2004; 9: 178-98.
  • 5-Corben LA, Lynch D, Pandolfo M, Schulz JB, Delatycki MB; Clinical Management Guidelines Writing Group. Consensus clinical management guidelines for Friedreich ataxia. Orphanet J Rare Dis. 2014;9:184.
  • 6- Scott B.K, Baranov D. Neurologic Diseases. In: Fleisher L.A, eds. Anesthesia and Uncommon Diseases, 6th edn. Philadelphia: W.B. Saunders,2012; 275-6.
  • 7- Delatycki MB, Corben LA. Clinical features of Friedreich ataxia. J Child Neurol 2012;27:1133-7.
  • 8- Schmitt HJ, Wick S, Münster T. Rocuronium for muscle relaxation in two children with Friedreich’s ataxia, Br J Anaesth 2004;92:592-6.
  • 9- Romero A, Joshi GP. Neuromuscular disease and anesthesia. Muscle Nerve. 2013;48:451-60. 10- Pancaro C, Renz D. Anesthetic management in Friedreich’s ataxia, Pediatric Anesthesia 2005;15:433-4.
There are 9 citations in total.

Details

Primary Language English
Subjects Clinical Sciences
Journal Section Case Report
Authors

Şemsi Mustafa Aksoy 0000-0003-3197-5655

Ayşe Lafçı 0000-0002-3215-4114

Ercan Bal 0000-0003-0138-2090

Muhammed Köse 0000-0003-0475-9586

Publication Date December 31, 2022
Submission Date August 22, 2022
Published in Issue Year 2022

Cite

Vancouver Aksoy ŞM, Lafçı A, Bal E, Köse M. Anesthesia Management in Scoliosis Surgery of Patients with Friedreich’s Ataxia: A Report of Four Cases. Genel Tıp Derg. 2022;32(6):789-92.