Goldenhar Syndrome and Airway Management

Introduction: Goldenhar syndrome is an oculoauriculovertebral spectrum attributed to the developmental anomalies of the first and second brachial arches. Its typical presentation in children with hemifacial microsomia poses a dual challenge for the anaesthesiologist on account of difficult airway often compounded with systemic abnormalities. Case report: We describe a case report wherein a 5 year female presented to the oculoplastic clinic of our hospital for surgical removal of limbal dermoid under general anaesthesia. Airway examination revealed classical facial asymmetry with underdevelopment of jaw bone coupled with protruding incisors. A predicted difficult airway, more so in a child led us to choose an anaesthesia technique with preservation of spontaneous breathing and planned use of supraglottic device in the form of an I Gel for airway management. Conclusion: The aim of this case report is to highlight the anaesthetic implications of this not so uncommon entity presenting to ophthalmology and ENT clinics. A thorough preoperative assessment, adequate preparedness and alternative plans are keys for successful airway management in such syndromic children. Keywords: Goldenhar syndrome, hemifacial microsomia, I Gel

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Airway management using the pediatric GlideScope® in a child with Goldenhar syndrome and atypical plasma cholinesterase

Pediatric Anesthesia ◽

10.1111/j.1460-9592.2006.02149.x ◽

2007 ◽

Vol 17 (5) ◽

pp. 484-487 ◽

Cited By ~ 47

Author(s):

ANDREW D. MILNE ◽

ANDREA M. DOWER ◽

THOMAS HACKMANN

Keyword(s):

Airway Management ◽

Plasma Cholinesterase ◽

Goldenhar Syndrome

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Airway Management for Rigid Bronchoscopy via a Freshly Performed Tracheostomy in a Child With Goldenhar Syndrome

Survey of Anesthesiology ◽

10.1097/01.sa.0000418860.23705.c2 ◽

2012 ◽

Vol 56 (5) ◽

pp. 220-221

Author(s):

Zulfiqar Ahmed ◽

Achir Alalami ◽

Michael Haupert ◽

Sankar Rajan ◽

Nasser Durgham ◽

...

Keyword(s):

Airway Management ◽

Rigid Bronchoscopy ◽

Goldenhar Syndrome

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Goldenhar Syndrome

10.1093/med/9780190685157.003.0058 ◽

2018 ◽

Author(s):

Amy Soleta ◽

Joelle Karlik

Keyword(s):

Airway Management ◽

Cervical Vertebrae ◽

Pulmonary Complications ◽

Goldenhar Syndrome ◽

Thoracic Insufficiency Syndrome ◽

Renal Malformations ◽

Vertebral Anomalies ◽

Difficult Ventilation ◽

Insufficiency Syndrome ◽

Degrees Of Severity

Goldenhar syndrome (also known as oculo-auriculo-vertebral spectrum, facio-auriculo-vertebral syndrome, and Goldenhar-Gorlin syndrome) is caused by fetal growth disturbances of the first two brachial clefts. Diagnostic criteria include eye, ear, mandibular, and/or vertebral anomalies. These patients may also have cardiac and renal malformations with varying degrees of severity. Airway management for Goldenhar patients may include difficult ventilation and intubation, which may become increasingly difficult with age. Vertebral anomalies including fused cervical vertebrae and/or cervical instability further complicate airway management. Pulmonary complications occur due to congenital malformations and scoliosis, which can lead to thoracic insufficiency syndrome. This chapter discusses genetics, presentation, and management of Goldenhar syndrome.

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Airway Management in a Child with Goldenhar Syndrome

Chinese Medical Journal ◽

10.4103/0366-6999.219146 ◽

2017 ◽

Vol 130 (23) ◽

pp. 2881-2882 ◽

Cited By ~ 1

Author(s):

Yan-Hua Sun ◽

Bo Zhu ◽

Bing-Yang Ji ◽

Xiu-Hua Zhang

Keyword(s):

Airway Management ◽

Goldenhar Syndrome

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