Goldenhar sendromlu (okulo-aurikulo-vertebral spektrum) bir olguda gözlenen havayolu problemi

Year 2018, , 68 - 70, 30.09.2018

Tuğba Güler , Atakan Sarıgül Nuri Alaçakır , Belkıs Aygün

https://doi.org/10.32322/jhsm.407828

Abstract

Çocuklarda,
özellikle 2 yaş altındaki hastalarda ve yenidoğanda, anatomik ve fizyolojik
farklılıklar nedeniyle endotrakeal entübasyon ve ventilasyonla ilgili güçlükler
daha sık görülmektedir. Özellikle yenidoğan döneminde dil ve oksiput büyük,
ağız küçük, epiglot hareketli ve boyun kısa olup bu faktörler laringoskopiyi ve
entübasyonu güçleştirebilmektedir. Goldenhar sendromu, etiyolojisi henüz
aydınlatılamamış, yaygın sistemik konjenital anomaliler ile karakterize, çoğu
kez sporadik nedenlerden kaynaklandığı gözlemlenen bir hastalıktır. Bu olgu
sunumunda yenidoğan döneminde yaşadığı solunum sıkıntısı nedeniyle entübe
edilmek istenen, ancak havayolu açıklığının sağlanmasında zorluk gelişen 8
aylık Goldenhar sendromlu hastamızın havayolu yönetiminde oluşabilecek
güçlükleri tartışmayı planladık.

Keywords

Goldenhar sendromu, infant, solunum sıkıntısı, entübasyon

Airline problem with a case of Goldenhar syndrome (oculo-auriculo-vertebral spectrum)

Abstract

In
children, especially under 2 year-old and newborn, problems about endotracheal
intubation and ventilation due to anatomic and physiologic differences are
frequently observed. Especially in newborn term, big tongue and occiput, small
mouth, mobile epiglottis and short neck may occur. These factors may complicate
laryngoscopy and intubation. Goldenhar syndrome, etiology is not clear yet, is
a disease which is characterized by common systemic congenital anomalies and it
is often observed to be caused by sporadic reasons. In this case report, we
aimed to discuss the complications during airway management of an 8 month-old
Goldenhar syndrome patient who wanted to be intubated due to respiratory
distress in infant term but had difficulty in providing airway. 

Keywords

Goldenhar syndrome, infant, respiratory distress, intubation

References

• 1. R A. Anesthesaia Secrets. Pediatric Anesthesia In Duke J. 2000:304.
• 2. Vagas E AA. Solunum Sistemi Fizyolojisi ve Çocuklardaki Farklar 2012; Available from: http://www.toraks.org.tr/uploadFiles/book/file/1772012111520-7783.pdf.
• 3. Pfleger A EE. Çocuklarda Akut Şiddetli Üst Solunum Yolu Obstrüksiyonunun Yönetimi. Selçuk Pediatri. 2013;1(2).
• 4. Nargozian C. The airway in patients with craniofacial abnormalities. Paediatric anaesthesia. 2004 Jan;14(1):53-9.
• 5. Gorlin RJ CM, Levin LS. Brachial Arch and Oro-acral Disorders. Syndromes of the Head and Neck. second ed. Newyork: Oxford University Press; 1990. p. 641- 9.
• 6. Feingold M, Baum J. Goldenhar's syndrome. American journal of diseases of children (1960). 1978 Feb;132(2):136-8.
• 7. Setzer ES, Ruiz-Castaneda N, Severn C, Ryden S, Frias JL. Etiologic heterogeneity in the oculoauriculovertebral syndrome. The Journal of pediatrics. 1981 Jan;98(1):88-90.
• 8. Gorlin RJ, Jue KL, Jacobsen U, Goldschmidt E. OCULOAURICULOVERTEBRAL DYSPLASIA. The Journal of pediatrics. 1963 Nov;63:991-9.
• 9. Lemmerling MM, Vanzieleghem BD, Mortier GR, Dhooge IJ, Kunnen MF. Unilateral semicircular canal aplasia in Goldenhar's syndrome. AJNR American journal of neuroradiology. 2000 Aug;21(7):1334-6.
• 10. Miura M, Sando I, Haginomori S-I, Takasaki K, Hirsch BE. Histopathologic Study of Temporal Bone and Eustachian Tube in Oculoauriculovertebral Spectrum. Annals of Otology, Rhinology & Laryngology. 2001;110(10):922-7.
• 11. Scholtz AW, Fish JH, 3rd, Kammen-Jolly K, Ichiki H, Hussl B, Kreczy A, et al. Goldenhar's syndrome: congenital hearing deficit of conductive or sensorineural origin? Temporal bone histopathologic study. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology. 2001 Jul;22(4):501-5.
• 12. Digilio MC, Calzolari F, Capolino R, Toscano A, Sarkozy A, de Zorzi A, et al. Congenital heart defects in patients with oculo-auriculo-vertebral spectrum (Goldenhar syndrome). American journal of medical genetics Part A. 2008 Jul 15;146a(14):1815-9.
• 13. Herwig MC, Gembruch U, Born M, Gevensleben H, Loeffler KU, Muller AM. Preterm diagnosis of choristoma and choroidal coloboma in Goldenhar's syndrome. Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society. 2011 Jul-Aug;14(4):322-6.
• 14. Schittkowski MP, Guthoff RF. Systemic and ophthalmological anomalies in congenital anophthalmic or microphthalmic patients. The British journal of ophthalmology. 2010 Apr;94(4):487-93.
• 15. Sedat Işıkay SS, Ayşe Demirçubuk, Evrim Kıray Baş, Ünal Sarıkabadayı. A syndrome extent to heart from the ear: Goldenhar’s Syndrome. Gaziantep Med J2013. p. 196-8.
• 16. Jena AK, Duggal R. Atypical goldenhar syndrome: a case report. The Journal of clinical pediatric dentistry. 2006 Winter;31(2):118-22.
• 17. Kim YL, Seo DM, Shim KS, Kim EJ, Lee JH, Lee SG, et al. Successful tracheal intubation using fiberoptic bronchoscope via an I-gel™ supraglottic airway in a pediatric patient with Goldenhar syndrome -A case report. Korean Journal of Anesthesiology. 2013 Jul;65(1):61-5.
• 18. Kim Y-L, Seo D-M, Shim K-S, Kim E-J, Lee J-H, Lee S-G, et al. Successful tracheal intubation using fiberoptic bronchoscope via an I-gel™ supraglottic airway in a pediatric patient with Goldenhar syndrome -A case report. Korean Journal of Anesthesiology. 2013 7/;65(1):61-5.
• 19. KABALAK A, PEHLİVAN VF, AKÇAY F, ORTAK T, BAYDAR M, GÖĞÜŞ N. Goldenhar sendromlu olguda zor entübasyon: olgu sunumu. Turkiye Klinikleri Journal of Anesthesiology Reanimation. 2004;2(2):83-6.
• 20. Sukhupragarn W, Rosenblatt WH. Airway management in a patient with Goldenhar syndrome: a case report. Journal of clinical anesthesia. 2008 May;20(3):214-7.
• 21. ÖZlü O, ŞImşek S, Alaçakir H, Yiğitkanli K. Goldenhar syndrome and intubation with the fiberoptic broncoscope. Pediatric Anesthesia. 2008;18(8):793-4.