Ülkü ÖZGÜL, Zekine BEGEÇ, Aytaç YÜCEL, M. Ali ERDOĞAN, Nizamettin BUCAK, M. Özcan ERSOY

Cornelia De lange sondromlu iki olguda anestezi yönetimimiz

Cornelia de Lange sendromu mezenkimin hipoplazisine sekonder geliştiği düşünülen gelişme geriliği, mental retardasyon, hirşutizm, kardiyak, gastrointestinal ve kas-iskelet sisteminin majör anomalileri ile dismorfik yüz görüntüsünün eşlik etti¤i nadir görülen bir sendromdur. Cornelia de Lange sendromu tanısı almış hastaların anestezi yönetiminde zor trakeal entübasyon, aspirasyon ve ilaçlara aşırı duyarlılık gibi problemlerle karşılaşılabilir. Biz bu olgu sunusunda Cornelia de Lange sendromunda anestezik yaklaşımı ve literatür bilgilerini gözden geçirmek istedik.

Anesthetic management of two patients with cornelia De lange syndrome

Cornelia de Lange syndrome is a rarely encountered disorder that is thought to progress secondary to hypoplasia of mesenchyma. Growth and mental retardation, hirsutism, major malformations of cardiac, gastrointestinal, and musculoskeletal systems with dysmorphic facial features are accompanied with this syndrome. Anesthetic management of these patients may encounter problems such as difficult tracheal intubation, aspiration and hypersensitivity to drugs. We want to review the anesthetic management and literature of the Cornelia de Lange Syndrome in this case presentation.

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