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  Table of Contents 
Year : 2019  |  Volume : 20  |  Issue : 1  |  Page : 39-41

Difficult airway challenge in a pediatric patient with Goldenhar syndrome and atrial septal defect for bone anchored hearing aid surgery

1 Department of Anaesthesiology, Seth G.S. Medical College and King Edward VII Memorial Hospital, Mumbai, Maharashtra, India
2 Department of Anaesthesiology, Tata Memorial Hospital, Mumbai, Maharashtra, India

Date of Submission12-Nov-2018
Date of Acceptance21-Feb-2019
Date of Web Publication6-May-2019

Correspondence Address:
Dr. Shamala Dinkar Yashod
Dhavalgiri 7, Veer Savarkar Nagar, Naughar, Vasai Road (West), Palghar, Vasai - 401 202, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/TheIAForum.TheIAForum_61_18

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Goldenhar syndrome is ocular-auriculo-vertebral dysplasia with cardiac, renal, visceral, and facial abnormalities. Presence of craniofacial and vertebral anomalies increases the risk of difficult airway. Here, we present a case report of a child with Goldenhar syndrome and atrial septal defect (ASD) posted for bone anchored hearing aid surgery. The case report highlights a successful awake retrograde intubation under conscious sedation and topical airway anesthesia, in an 11-year-old child with Goldenhar syndrome and ASD.

Keywords: Atrial septal defect, bone anchored hearing aid, Goldenhar syndrome, retrograde intubation

How to cite this article:
Yashod SD, Oak SP, Kalkundre RS, Chincholi IH. Difficult airway challenge in a pediatric patient with Goldenhar syndrome and atrial septal defect for bone anchored hearing aid surgery. Indian Anaesth Forum 2019;20:39-41

How to cite this URL:
Yashod SD, Oak SP, Kalkundre RS, Chincholi IH. Difficult airway challenge in a pediatric patient with Goldenhar syndrome and atrial septal defect for bone anchored hearing aid surgery. Indian Anaesth Forum [serial online] 2019 [cited 2020 Jul 11];20:39-41. Available from: http://www.theiaforum.org/text.asp?2019/20/1/39/257684

  Introduction Top

Goldenhar syndrome was first described by Goldenhar in 1952.[1] The incidence is 1:3500–1:5600 births, and the M:F is 3:2.[2] The etiopathology is multifactorial. The cause is maldevelopment of first and second branchial arches during the first trimester of pregnancy resulting in hemifacial hypoplasia, macrognathia, and large forehead with other systemic affection. The abnormalities of the ear are associated with upper airway abnormalities due to their common embryonic origin.[3]

  Case Report Top

An 11-year-old child with Goldenhar syndrome was planned for bone anchored hearing aid (BAHA) surgery. He had B/L microtia with atrial septal defect, ectopic kidney, torticollis, vertebral defects, and cervicothoracic scoliosis. The patient had restricted mouth opening, restricted neck movement, facial asymmetry, web neck, torticollis and airway examination revealed Mallampati classification grade IV [Figure 1], [Figure 2], [Figure 3]. Thyromental and mentosternal distances were 6 cm and 9 cm, respectively. His Wilson's score was 5 (weight: 0, head and neck movement: 1, jaw movement 2 [interincisor distance <5 cm, and slux = 0], receding mandible: 1, buck teeth: 1). There was systolic murmur in pulmonary area. Chest X-ray showed cervicothoracic scoliosis. Electrocardiogram was suggestive of the right bundle branch block. Two-dimensional echocardiography showed atrial septal defect (ASD) and ejection fraction of 60%. Pulmonary function tests revealed moderate restriction with small airway obstruction. Arterial blood gases were normal.
Figure 1: Dysmorphic features

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Figure 2: Short neck and limited neck extension

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Figure 3: MPC IV

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Awake retrograde intubation was preferred because of anticipated difficult airway. The child was counseled preoperatively. High-risk consent was obtained. Difficult airway cart including surgical tracheostomy was kept ready.

ASA standard monitors were attached. Xylometazoline drops were instilled nasally. Glycopyrrolate was given as an antisialogogue. Sedation was provided with midazolam, fentanyl, and dexmedetomidine. The airway was anesthetized with lignocaine 4% nebulization, Transtracheal block, and superior laryngeal nerve block. An 18G epidural catheter was passed through cricothyroid membrane through 18G intravenous cannula and retrieved orally. A 10 F size nasogastric (NG) tube was passed nasally and retrieved orally. The epidural catheter was threaded into NG tube and taken out nasally. A 5.5 cuffed polyvinyl chloride endotracheal tube (ETT) was advanced into trachea after railroading itover the catheter [Figure 4]. Tracheal intubation was confirmed with end-tidal CO2, bilateral breath sounds, and bag movements. Anesthesia was managed with propofol, atracurium, O2; N2O, and isoflurane. Systemic vascular resistance was maintained to prevent shunt reversal. Meticulous attention was taken to avoid air into circulation. Residual neuromuscular block was reversed, and the patient was extubated when fully awake. The postoperative course was uneventful.
Figure 4: Railroading of the endotracheal tube

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  Discussion Top

Goldenhar syndrome is a rare congenital disorder associated with ocular, auricular, facial, cranial, vertebral, cardiac, and visceral anomalies. The craniofacial malformation poses a difficult airway challenge. About 42% of children with B/L microtia, which was present in our patient, have a difficult laryngeal view.[3]

Successful airway management using various techniques has been reported in patients with Goldenhar syndrome. Kedareshwara et al.[4] described fiberoptic bronchoscope (FOB) intubation under regional nerve block and sedation, in a 12-year-old girl for ophthalmic surgery. Ozlu et al.[5] reported successful intubation at first attempt with FOB after airway anesthesia and sedation, in a 6-year-old child for posterior fossa decompression. As pediatric FOB was not available with us, we preferred awake nasal retrograde intubation technique. The child's cooperation was sought with preoperative counseling, supplemented with airway anesthesia, and sedation with midazolam, fentanyl, and dexmedetomidine.

Videolaryngoscopes such as Airtraq, Mcgrath videolaryngoscope, and intubating video stylet were also kept ready. Milne et al.[6] failed to intubate a 9-year-old girl for ear debridement and tooth extraction under GA with direct laryngoscopy but was able to intubate her with GlideScope after a few attempts. Sugino et al.[7] reported successful intubation using videolaryngoscope in a 5-year-old child. Shukry et al.[8] reported the use of LMA followed by successful Shinkani optical stylet-guided tracheal intubation. Videolaryngoscopy requires adequate mouth opening and optimal patient cooperation, which were lacking in our patient. Hence, videolaryngoscopy was not the technique of choice. However, they were kept ready to secure the airway under GA, after anesthetizing the patient, as were used in the above-mentioned studies.

Alternatively, classic LMA number 2 and 2.5 were also kept ready to secure the airway, after anesthetizing the patient. Although the mouth opening was restricted, it was 2 fingers which was sufficient for LMA insertion. LMA was not introduced as a first choice because BAHA surgery required frequent change of head position. Furthermore, in view of small airway obstruction, ASD, and postoperative intensive care unit admission, ETT was preferred for better oxygenation. Roodneshin and Agah.[9] have reported case series in which neonates were anesthetized with sevoflurane and the airway was secured with classic LMA. Sukhupragarn and Rosenblatt[10] in 2008 used flexible LMA to secure the airway in a 10-year-old child posted for eye surgery. Surgical tracheostomy was planned in event of failure to intubate. However, in our patient, awake nasal retrograde intubation was successful in the first attempt which obviated the need for other techniques.

  Conclusion Top

In patients with Goldenhar syndrome, the airway management plan should include FOB, retrograde intubation, videolaryngoscopes, LMA, and surgical tracheostomy. Awake retrograde intubation with airway anesthesia and sedation can be used as an alternative to FOB-guided intubation even in a pediatric patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Engiz O, Balci S, Unsal M, Ozer S, Oguz KK, Aktas D, et al. 31 cases with oculoauriculovertebral dysplasia (Goldenhar syndrome): Clinical, neuroradiologic, audiologic and cytogenetic findings. Genet Couns 2007;18:277-88.  Back to cited text no. 1
Palminso BW, Rusy LM. Anesthesia for Pediatric Surgery. Pediatric Anesthesia. 4th ed. New York, USA: Churchill Livingstone; 2002. p. 707-45.  Back to cited text no. 2
Uezono S, Holzman RS, Goto T, Nakata Y, Nagata S, Morita S, et al. Prediction of difficult airway in school-aged patients with microtia. Paediatr Anaesth 2001;11:409-13.  Back to cited text no. 3
Kedareshwara KS, Sanikop CS, Shirol S, Jain N. Aneshesia and airway management of oculo auricular vertebral dysplasia: A rare case report. J Sci Soc 2012;39:8992.  Back to cited text no. 4
Ozlu O, Simsek S, Hale Alacakir md, Kazim YigitKanli md. Goldenhar syndrome and intubation with fibre optic bronchoscope. Paeditr Anesth 2008;18:7934.  Back to cited text no. 5
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;17:484-7.  Back to cited text no. 6
Sugino S, Kanaya N, Omote K, Namiki A. Anesthetic management in a patient with goldenhar's syndrome using a perilaryngeal airway and a videolaryngoscope. Masui 2005;54:1367-70.  Back to cited text no. 7
Shukry M, Hanson RD, Koveleskie JR, Ramadhyani U. Management of the difficult pediatric airway with shikani optical stylet. Paediatr Anaesth 2005;15:342-5.  Back to cited text no. 8
Roodneshin F, Agah M. Management of anaesthesia in goldenhar syndrome: Case series study. Tanaffos 2009;8:43-50.  Back to cited text no. 9
Sukhupragarn W, Rosenblatt WH. Airway management in a patient with goldenhar syndrome: A case report. J Clin Anesth 2008;20:214-7.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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