Apert sendromlu bir bebekte trakeostomi için anestezi uygulaması

Öz

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.

Anahtar Kelimeler

• Apert syndrome; infant; general anesthesia

Anesthesia for tracheostomy in an infant with Apert syndrome

Öz

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.

Anahtar Kelimeler

• Apert sendromu; bebek; genel anestezi

Kaynakça

1. 1. Butler MG, Hayes BG, Hathaway MM, Begleiter ML. Specific genetic diseases at risk for sedation/anesthesia complications
2. Anesth Analg 2000;91(4):837-55
3. 2. Tolarova MM, Harris JA, Ordway DE, Vargervik K. Birth prevalence, mutation rate, sex ratio, parents' age, and ethnicity in Apert syndrome. Am J Med Genet 1997;72(4):394-8
4. 3. Cohen MM, Kreiborg S. Visceral anomalies in the Apert syndrome. Am J Med Genet 1993;45(6):758-60
5. 4. Barnett S, Claire M, Robert B. Perioperative complications in children with Apert syndrome: A review of 509 anaesthetics
6. Paediatr Anaesth 2011;21(1):72-7
7. 5. Soliman D, Cladis FP, Davis PJ. The pediatric patient. In: Fleisher LA, (ed). Anesthesia and uncommon diseases. 6th ed
8. Elsevier Saunders, Philadelphia, PA, USA, 2012; 586-626

9. 6. Lo LJ, Chen YR. Airway obstruction in severe syndromic craniosynostosis. Ann Plast Surg 1999;43(3):258-64
10. 7. Başar H, Büyükkoçak Ü, Kaymak Ç, Akpınar S, Sert O, Vargel I. An intraoperative unexpected respiratory problem in a patient with Apert syndrome. Minerva Anestesiol 2007;73(11):603-6
11. 8. Elwood T, Sarathy PV, Geiduschek JM, Ulma GA, Karl HW
12. Respiratory complications during anaesthesia in Apert syndrome. Paediatr Anaesth 2001;11(6):701-3
13. 9. Atalay A, Doğan N, Yüksek Ş, Erdem AF. Anesthesia and airway management in two cases of Apert syndrome: case reports. Eurasian Med J 2008;40(2):91-3
14. 10. Hemmer KM, McAlister WH, Marsh JL. Cervical spine anomalies in the craniosynostosis syndromes. Cleft Palate Journal 1987;24(4):328-33
15. 11. Metodiev Y, Gavrilova N, Katzarov A. Anesthetic management of a child with Apert syndrome. Saudi J Anaesth 2011;5(1):87– 9