title: [Marfan Syndrome: new diagnostic criteria, same anesthesia care? Case report and review].
abstract: BACKGROUND
  Marfan's Syndrome (MFS) is a disorder of connective tissue, mainly involving the cardiovascular, musculoskeletal, and ocular systems. The most severe problems include aortic root dilatation and dissection. Anesthetic management is vital for the improvement on perioperative morbidity.
  
  
  METHODS
  61-year-old male with MFS, presenting mainly with pectus carinatum, scoliosis, ectopia lens, previous spontaneous pneumothorax and aortal aneurysm and dissection submitted to thoracoabdominal aortic prosthesis placement. Underwent routine laparoscopic cholecystectomy due to lithiasis. Important findings on preoperative examination were thoracolumbar kyphoscoliosis, metallic murmur on cardiac exam. Chest radiograph revealed Cobb angle of 70°. Echocardiogram showed evidence of aortic mechanical prosthesis with no deficits.
  
  
  CONCLUSIONS
  Preoperative evaluation should focus on cardiopulmonary abnormalities. The anesthesiologist should be prepared for a potentially difficult intubation. Proper positioning and limb support prior to induction is crucial in order to avoid joint injuries. Consider antibiotic prophylaxis for subacute bacterial endocarditis. The patient should be carefully positioned to avoid joint injuries. Intraoperatively cardiovascular monitoring is mandatory: avoid maneuvers that can lead to tachycardia or hypertension, control airway pressure to prevent pneumothorax and maintain an adequate volemia to decrease chances of prolapse, especially if considering laparoscopic surgery. No single intraoperative anesthetic agent or technique has demonstrated superiority. Adequate postoperative pain management is vitally important to avoid the detrimental effects of hypertension and tachycardia.
authors.affiliation: Departamento de Anestesiologia, Hospital Santa Maria, Lisboa, Portugal. Electronic address: m.rita.araujo@gmail.com.
authors.forename: Maria Rita
authors.initials: MR
authors.lastname: Araújo
authors.affiliation: Departamento de Anestesiologia, Hospital Santa Maria, Lisboa, Portugal.
authors.forename: Céline
authors.initials: C
authors.lastname: Marques
authors.affiliation: Departamento de Anestesiologia, Hospital Santa Maria, Lisboa, Portugal.
authors.forename: Sara
authors.initials: S
authors.lastname: Freitas
authors.affiliation: Departamento de Anestesiologia, Hospital Santa Maria, Lisboa, Portugal.
authors.forename: Rita
authors.initials: R
authors.lastname: Santa-Bárbara
authors.affiliation: Departamento de Anestesiologia, Hospital Santa Maria, Lisboa, Portugal.
authors.forename: Joana
authors.initials: J
authors.lastname: Alves
authors.affiliation: Departamento de Anestesiologia, Hospital Santa Maria, Lisboa, Portugal.
authors.forename: Célia
authors.initials: C
authors.lastname: Xavier
keywords: Anestesia geral; Cuidados pré‐operatórios; General anesthesia; Marfan Syndrome; Perioperative care; Síndrome de Marfan