H. Volkan ACAR, Hale UYSAL YARKAN, Serdar KÖSEOĞLU, Solmaz GÜNAL ERUYAR

Anesthesia for tracheostomy in an infant with Apert syndrome

Apert sendromu, irregüler kraniosinostoz ile yüz, eller ve ayaklarda malformasyonlarla karakterize otozomal dominant geçişli nadir görülen bir hastalıktır. Orofarengeal ve nazofarengeal kavitelerin hipoplazisi nedeniyle, sıklıkla solunumsal fonksiyonlar bozulur. Obstrüktif uyku apne sendromu, kor pulmonale ve ani ölüm sendromu Apert sendromunun komplikasyonları arasındadır. Havayollarına ait tüm bu anatomik ve fizyopatolojik bozukluklar, anestezi uygulaması sırasında dikkatli olmayı gerektirir. Bu hastalarda maske ventilasyonunda zorluk, zor entübasyon ve postoperatif havayolu obstrüksiyonu görülebilir. Bu olgu sunumunda, trakeostomi nedeniyle anestezi uygulanan ve zor entübasyonla karşılaşılan Apert sendromlu bir bebekteki anestezi deneyimimizi sunuyoruz.

Apert sendromlu bir bebekte trakeostomi için anestezi uygulaması

Apert syndrome is a rare, autosomal dominantly inherited disease characterized by irregular craniosynostosis and some malformations involving face, hands and feet. Respiratory functions are frequently deteriorated due to hypoplasia of oropharyngeal and nasopharyngeal cavities. Obstructive sleep apne syndrome, cor pulmonale and sudden death syndrome are among the complications of Apert syndrome. All of these anatomical and physiopathological disorders in the airways lead to a significant concern during anesthesia practice. Difficulty in mask ventilation, difficult intubation and postoperative airway obstruction may be seen in these patients. In this case report we present our anesthetic experience in an infant with Apert syndrome whom anesthesia was given for tracheostomy and difficult intubation was encountered.

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