Perikardiyal Efüzyonu Olan Proteus Sendromlu Bir Hastada Anestezi Uygulaması

Yıl 2021, Cilt: 7 Sayı: 1, 168 - 172, 01.01.2021

Emel Gündüz Hakan Keskin Tülin Aydoğdu Titiz

Öz

Proteus syndrome PS is a rare congenital disease described by Cohen and Hayden, which is characterized by asymmetric and excess tissue growth in the body 1 . Various pathologies such as progressive skeletal deformities, tumors, vascular malformations, bullous pulmonary disease and skin lesions can be seen in these cases. There is a limited number of studies examining cardiac involvement

Anahtar Kelimeler

Proteus syndrome, Pericardial effusion, Right double-lumen tube

Anesthesia Application in A Proteus Syndrome Patient With Pericardial Effusion

Öz

Proteus sendromu PS , Cohen ve Hayden tarafından tanımlanan ve vücutta asimetrik ve aşırı doku büyümesi ile karakterize nadir görülen konjenital bir hastalıktır 1 . Bu olgularda ilerleyici iskelet deformiteleri, tümörler, vasküler malformasyonlar, büllöz akciğer hastalığı ve deri lezyonları gibi farklı patolojiler görülebilir. Kardiyak tutulumu inceleyen sınırlı sayıda çalışma vardır

Anahtar Kelimeler

Proteus sendromu, Perikardiyal efüzyon, Sağ çift lümenli tüp

Kaynakça

• Wiedemann HR, Burgio GR, Aldenhoff P, Kunze J, Kaufmann HJ, Schirg E. The Proteus syndrome. Partial gigantism of the hands and/or feet, nevi, hemihipertrophy, subcutaneous tumors, macrocephaly, skull anomalies and possible accelerated grovvth and visceral affections. Eur J Pediatr 1983; 140:5-12.
• Biesecker LG, Happle R, Mulliken JB, Weksberg R, Graham JM Jr, Viljoen DL, Cohen MM Jr. Proteus Syndrome: Diagnostic criteria, differential diagnosis and patient evaluation. Am J Med Genet 1999; 84(5):389-95.
• Pennat JH, Harris MF. Anaesthesia for Proteus Syndrome. Anaesthesia 1991; 46(2):126-8.
• Cantone E, Cavaliere M, Castagna G, Marino A, Del Vecchio L, lengo M. Operative managemet of OSAS in a complex case of proteus syndrome. Case Rep Otolaryngol 2015; 2015:137589.
• Shaw C, Bourke J, Dixon J. Proteus syndrome with cardiomyopathy and a myocardial mass. J Med Genet 1993; 46:145-8.
• Hannoush H, Sachdev V, Brofferio A, Arai AE, LaRocca G, Sapp J, Sidenko S, Brenneman C, Biesecker LG, Keppler-Noreuil KM. Myocardial fat overgrowth in Proteus syndrome. Am J Med Genet A 2015; 167A(1):103- 10.