Emel GÜNDÜZ, Hakan KESKİN, Tülin AYDOĞDU TİTİZ

Perikardiyal Efüzyonu Olan Proteus Sendromlu Bir Hastada Anestezi Uygulaması

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

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

Keywords:

Proteus sendromu, Perikardiyal efüzyon, Sağ çift lümenli tüp,

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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.