Major Özelliği Yüz Ekstremite Defektleri Olan Sendromlar Ve Anestezi Yönetimi

Yıl 2016, Cilt: 26 Sayı: 1, 28 - 33, 01.01.2016

Ali Özgül Saltalı Betül Kozanhan

Öz

2Kadınhanı Refik Saime Koyuncu Devlet Hastanesi, Kadınhanı, Konya. Kraniyofasiyal iskelet ile üst ekstremitenin embriyolojik dönemde gelişim zamanlarının örtüşmesi ve belirli gen mutasyonlarının her iki sistemin gelişiminde de etkili olması sebebiyle bir çok ekstremite defekti, konjenital kraniyofasiyal anomaliler ile birlikte seyreden bir sendromun parçası olabilir. Apert, Saethre-Chotzen, Pfeiffer, and Carpenter sendromları üst ekstremite defektleri ile birliktelik gösteren en sık kraniyofasiyal sendromlardır. Bu sendromlar, hava yolu yönetimi, intravenöz damar yolu açılması ve hemodinamik kontrol açısından gerçek bir sorun olabilir. Başarının anahtarı etkin hazırlık, zor pediatrik havayolu yönetimi ve intravenöz kanü- lasyonda uzmanlığa sahip personel varlığı, düzenli eğitim ve takım çalışmasıdır. Bu derlemede kraniyofasiyal ve ekstremite gelişim anomalileri içeren çocuk hastalarda güncel bilgiler ve uygulamalar ışığında anestezik yaklaşım sunulmuştur

Anahtar Kelimeler

Ekstremiteler, kafa yüz anormallikleri, anestezi, çocuk

Syndromes with facial - extremity defects as major feature and anesthesia management

Öz

Syndromes with facial - extremity defects as major feature and anesthesia management Many of extremity defects could be part of a syndrome associated with congenital craniofacial abnormalities due to overlapp of embr- yological development period of upper limb and craniofacial skeleton and specific gene mutations which is effective in development of both systems. The most common craniofacial syndromes coexistence of upper limb defects are Apert, Saethre -Chotzen, Pfeiffer and Carpenter syndrome. These syndroms can be a real challenge in terms of airway management, intravenous cannulation and hemodynamic control. The key to success is effective preparation, presence of personnel with expertise in difficult pediatric airway management and intravenous cannulation, regular training, and teamwork. In this review we presented anesthetic managent with current knowledge and practice of craniofacial anomalies associated with extremity malformations in children

Anahtar Kelimeler

Extremities, craniofacial abnormalities, anesthesia, child

Kaynakça

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