Waardenburg Sendromlu Hastanın Anestezi Yönetimi ve Literatür İncelemesi

Objective & Hypothesis:Waardenburg syndrome is a rare autosomal dominant disease that may cause hearing loss, pigmentary abnormalities, neurocristopathy and partial albinism.

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Key Findings & Data:Incidence is estimated as 2%–3% among the cases of congenital deafness and 1/42,000 of the general population.

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Conclusion & Impact:We aimed to report anaesthetic management of a child with Waardenburg syndrome who underwent surgery for cochlear implantation.

🔬Design:Research Article

📍Scope:Comprehensive Sample

⚙️Variables:Domain Parameters

Abstract

Waardenburg syndrome is a rare autosomal dominant disease that may cause hearing loss, pigmentary abnormalities, neurocristopathy and partial albinism. Incidence is estimated as 2%–3% among the cases of congenital deafness and 1/42,000 of the general population. Children with Waardenburg syndrome usually require anaesthesia for the cochlear implant operation in early age. The features of the syndrome that may bear importance for anaesthetic management are laryngomalacia, multiple muscle contractures, limited neck movements, cyanotic cardiopathy and electrolyte imbalance. Patients with Waardenburg syndrome stand for difficult airway. We aimed to report anaesthetic management of a child with Waardenburg syndrome who underwent surgery for cochlear implantation.

Özet

Waardenburg sendromu, işitme kaybına, pigment anormalliklerine, nörokristopatiye ve parsiyel albinizme neden olabilen nadir bir otozomal dominant hastalıktır. İnsidansı, kalıtsal işitme kaybı olguları arasında %2-3 ve genel popülasyonda 1/42.000 olarak tahmin edilmektedir. Waardenburg sendromlu çocuklar sıklıkla erken dönemde koklear implantasyon için anesteziye ihtiyaç duyarlar. Sendromun anestezi yönetimi için önem arz eden belirtileri laringomalazi, multipl kas kontraksiyonları, kısıtlı boyun hareketleri, siyanotik kardiyopati ve elektrolit dengesizliğidir. Waardenburg sendromlu hastalar zor havayolu için adaydırlar. Koklear implantasyon için cerrahi geçiren Waardenburg sendromlu bir çocuğun anestezi yönetimini bildirmeyi amaçladık.

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Cite This Article

PEKER, K., ERGİL, J., ÖZTÜRK, İ. (2015). Waardenburg Sendromlu Hastanın Anestezi Yönetimi ve Literatür İncelemesi. Turkish Journal of Anaesthesiology and Reanimation, Cilt: 43 - Sayı: 5, 80–82. https://ajindex.com/articles/370349

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