AYŞE BELİN ÖZER, Ömer Lütfi ERHAN, Mehmet Akif YAŞAR, Hasan ARIK

Goldenhar's sendromlu hastada genel anestezi uygulaması

Goldenhar's sendromu birinci ve ikinci brankiyal arkdan gelişen yapıların konjenital defektidir. İnguinal herni nedeniyle herniografi planlanan Goldenhar's sendromlu 18 aylık erkek hasta preoperatif olarak değerlendirildi. Fizik muayenede fasiyal asimetri, sağ kulak atrezisi, preauriküler appendiks, mikrognati ve duyma defekti olduğu tespit edildi. Hastada fasial asimetriye bağlı zor entübasyon olabileceği düşünüldü. Çeşitli boyutlarda airway, endotrakeal tüp, bleyd ve LMA hazırlandı ve gerektiğinde trakeostomi açılabilmesi için aileden izin alındı. Anestezi indüksiyonu %50 O2- %50 N2O içerisinde sevofluran ile sağlandı ve maske ventilasyonunda sıkıntı yaşanmadı. Laringoskopi uygulaması 1 numara Miller bleyd ile denendi, bleydin büyük olduğu düşünülerek 0 numaralı bleyd ile laringoskopi tekrarlandı. ve 3,5 numaralı kafsız tüple entübe edildi. Anestezi idamesi %50 O2- %50 N2O içerisinde sevofluran ile sağlandı. Elli dakika süren operasyon süresince ve sonrasında komplikasyon yaşanmadı. Bu hastalarda gerek entübasyon gerek ek patolojiler yönünden tam bir preoperatif değerlendirme yapılması ve olası zor entübasyona karşı hazırlıklı olunması gerektiği düşüncesindeyiz.

General anaesthesia in an infant with goldenhar's syndrome

Goldenhar's syndrome is a congenital developmental defect of structures of the first and second branchial arches. We aim to present our anaesthetic management of an 18 month-old male patient with Goldenhar's syndrome scheduled for herniography. Facial asymmetry, right ear atresia, preauricular appendix, micrognathy and aural defect were identified in the physical examination. Difficult intubation was presumed in patient due to facial asymmetry. Airways, endotracheal tubes, blades and LMA in different sizes were prepared and permission was obtained from the family to create a tracheostomy if necessary. Sevoflurane in %50 O2- %50 N2O was used for anaesthetic induction and mask ventilation was uneventful. Laryngoscopy was tried using no. 1 sized Miller blade, and but as the blade was considered large, laryngoscopy was repeated with no. 0 sized blade. The patient was intubated with (ID) 3,5mm uncuffed tube. Maintenance of anaesthesia was managed by administered of sevoflurane in %50 O2- %50 N2O. No complications were observed during the operation and after the recovery period which lasted fifty minutes. We believe that, in these patients, a full preoperative evaluation should be administered in terms of intubation or additional pathologies and get prepared for a possible difficult intubation.

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1) Goldenhar's syndrome. http://www.whonamedit.com/synd.cfm/2300.html.

2) Gorlin RJ, Cohen MM, Hennekam RCM. Oculo-auriculo-vertebral spectrum (hemifacial microsomia, Goldenhar syndrome). Syndromes of the Head and Neck forth edition. Oxford, New York 2001; 790-849.

3) Kulkarni VV, Shah MD, Parikh AA. Goldenhar syndrome (a case report). J Postgrad Med. 1985; 31: 177-179.

4) Nijhawan N, Morad Y, Seigel-Bartelt J, Levin AV. Caruncle abnormalities in the oculo-auriculo-vertebral spectrum. Am J Med Genet. 2002; 113: 320-325.

5) Ottaviano G, Calzolari F, Martini A. Goldenhar syndrome in association with agenesia of the internal carotid artery. Int J Pe-diatr Otorhinolaryngol. 2007; 71: 509-512.

6) Ermiş B, Öztaş N, Yıldırım M, Hoşnuter M, Yavuz T. Pulmonary Hypoplasia in a Newborn with Goldenhar Syndrome. J Pediatr. 2010; 156: 161.

7) Kourelis K, Gouma P, Naxakis S, Kalogeropoulou C, Goumas P. Oculoauriculovertebral complex with an atypical cause of obstructive sleep apnea. Int J Pediatr Otorhinolaryngol. 2009; 73: 481-485.

8) Kara İ, Özmen S, Kocaoğlu C. Goldenhar-Gorlin Sendromlu Vakada Hava Yolu Yönetimi. Selçuk Tıp Derg 2009; 25: 87-89.

9) Know which child with a genetic syndrome may be a difficult intubation. http://www.wrongdiagnosis.com/i/imprinted_chromosome_conditions/book-diseases-19a.htm

10) Kabalak A, Pehlivan VF, Akçay F, Ortak T, Baydar M, Göğüş N. Goldenhar sendromlu olguda zor entübasyon: olgu sunumu. T Klin J Anest Reanim 2004; 2: 83-86.

11) Özlü O, Şimşek S, Alaçakır H, Yiğitkanlı K. Goldenhar syndrome and intubation with the fiberoptic broncoscope. Paediatr Anaesth. 2008; 18: 789-807.

12) Khalil S, Vinh B. Successful intubation of a child with Goldenhar syndrome, who previously failed intubation, using an Airtraq. Paediatr Anaesth. 2010; 20: 195-207.

13) Sukhupragarn W, Rosenblatt WH. Airway management in a patient with Goldenhar syndrome: a case report. J Clin Anesth 2008; 20: 214-217.

14) Milne AD, Dower AM, Hackmann T. Airway management using the pediatric GlideScope in a child with Goldenhar syndrome and atypical plasma cholinesterase. Paediatr Anaesth. 2007; 17: 484-487.

15) Grewal A, Bhat D, Sood D, Garg S, Singh A, Bharti R, Garg M. Goldenhar Syndrome: Anaesthetic and Airway Management. J Anaesth Clin Pharmacol 2005; 21: 313-316.