Opšta anestezija kod adolescentkinje sa Marfanovim sindromom i dilatacijom aorte - prikaz slučaja (Marfanov sindrom - anesteziološka razmatranja)

• Published in: Serbian Journal of Anesthesia and Intensive Therapy Vol. 46; no. 3-4; pp. 61 - 67
• Main Authors: Krunić, Igor, Mandraš, Ana, Paunović, Zoran, Miličković, Stanko, Stevanović, Vesna, Vasiljević, Slađana
• Format: Journal Article
• Language: English
• Published: 2024
• DOI: 10.5937/sjait2404061K
• EISSN: 2466-488X
• ISSN: 2217-7744

Marfan syndrome is autosomal dominant connective tissue disorder. It becomes evident in childhood and persists through adulthood. Phenotype characteristics of disease are very different. Aortic dilatation stands out as one the most important. Rupture or dissection of aorta during anesthesia is a serious complication that increases the risk for perioperative complications and mortality rate. Case report: We described perioperative course in adolescent with borderline dilatation ascending aorta and aortic root as well as dilatation of sinuses of Valsalva during general anesthesia for spine deformity surgical correction. The aim of preoperative preparation was to identify all features of disease that increase risk for complications while chosen opioid based anesthesia technique aimed to preserve hemodynamic stability. Lung protective ventilation strategy prevented pneumothorax, atelectasis and other respiratory complications. Careful preparation for major blood losses using cell saver enabled saving half of intraoperative lost blood volume which was returned to patient. Whole perioperative course was uneventful. Conclusion: Patients with Marfan syndrome can be found in every age group. They often require different surgical procedures. Regardless of surgery, aim of every anesthesia and preoperative preparation is to prevent predictable complications