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CASE REPORT
Year : 2012  |  Volume : 39  |  Issue : 2  |  Page : 89-92

Anaesthesia and airway management of occulo auricular vertebral dysplasia: A rare case report


1 Department of Anaesthesiology, Jawaharlal Nehru Medical College, KLE University, Belgaum, Karnataka, India
2 Department of Plastic Surgery, Jawaharlal Nehru Medical College, KLE University, Belgaum, Karnataka, India

Date of Web Publication1-Oct-2012

Correspondence Address:
K S Kedareshvara
Department of Anaesthesia, Jawaharlal Nehru Medical College, Belgaum - 590 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-5009.101854

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  Abstract 

A 12 year old girl child from the department of Ophthalmology posted for right eye limbal dermoid excision. Opthlamic examination and history revealed bilateral restricted eye movements, limbal dermoids, and decreased vision since birth. Fundus examination showed pathological myopia. There were multiple preauricular appendages in front of both ears. The lateral X-ray of cervical spine showed fused second and third cervical vertebra with loss of intervertebral disc space. Chest X-ray showed scoliosis of Cervico-Thorasic vertebra with convexity to right. Clinical examination of the airway revealed restricted head and neck movements with Mallampatti class II which indicates difficult intubation. The history, clinical examination and radiological findings confirmed her to be a case of Goldenhar syndrome. Difficult airway continues to be a major cause of anaesthesia related complications. Therefore fibre optic intubation under regional nerve block and sedation was planned for the surgery. Lack of associated cardiac and neurological anomalies encouraged us to present the advantage of fibre optic intubation. The larynx was adequately relaxed because of regional nerve block which helped us by decreasing the number of attempt of scopy and vocal cords injury. The intraoperative and postoperative period was uneventful.

Keywords: Fibre optic intubation, goldenhar syndrome, occulo-auricular-vertebral dysplasia


How to cite this article:
Kedareshvara K S, Sanikop C S, Shirol S, Jain N. Anaesthesia and airway management of occulo auricular vertebral dysplasia: A rare case report. J Sci Soc 2012;39:89-92

How to cite this URL:
Kedareshvara K S, Sanikop C S, Shirol S, Jain N. Anaesthesia and airway management of occulo auricular vertebral dysplasia: A rare case report. J Sci Soc [serial online] 2012 [cited 2019 Jun 17];39:89-92. Available from: http://www.jscisociety.com/text.asp?2012/39/2/89/101854


  Introduction Top


Oculo-Auricular-Vertebral dysplasia is a rare congenital disorder also known as Goldenhar syndrome. This syndrome with malformations of face, eyes and ears was first recorded by the nineteenth-century German physician Carl Ferdinand von Arlt (1812-1887) but was first described by Dr. Maurice Goldenhar in 1952. [1]

The syndrome occurs randomly, with no apparent cause. Most cases of Goldenhar syndrome are not inherited, however few families show unclear inheritance pattern. The male to female ratio is 2:1 and its occurrence is estimated from one in every 3,000 to one in every 5,000 live births. Usually children with Goldenhar syndrome have normal life span. [2],[3],[4],[5]

The presence of mandibular abnormalities has more sensitivity and specificity for predicting difficult laryngoscopy. As the number of associated craniofacial anomalies of Goldenhar syndrome increases, the risk of difficult intubation also increases. The airway and anaesthesia management of the case presented was challenging as the preoperative assessment of her airway revealed the presence of maxillary hypoplasia, fused cervical vertebra and limited head and neck movements with Mallampatti class II.

Genetic profile
Goldenhar syndrome is caused by disruption of normal facial development which is formed between 8 and 12 weeks of intrauterine developmental life. Its aetiology may be environmental or due to certain medication taken during pregnancy. Some cases of positive family history suggested autosomal dominant or recessive inheritance. There may be interaction of many genes possibly in combination with environmental factors (multifactorial inheritance). [6]


  Case Report Top


A 12 year old female child presented to the ophthalmic outpatient department with history of decreased vision, restricted movements of eyes and painless swelling in both eyes since birth.

On ophthalmic examination, visual acuity in the right eye was counting fingers at three meters and the left eye was counting fingers at two meters distance which improved with refraction. In the right eye lateral limbal dermoid measures about 18x14 mm size extending up to medial cantus with 30 to 35 degree divergent squint with hair particles [Figure 1]. In the left eye dermal lipoma in the lateral canthus measuring 8x10 mm. Fundus examination showed pathological myopia and normal intraocular pressure in bilateral eyes. The physical examination showed facial asymmetry due to under development of left maxilla and normally appearing mandible. Airway evaluation showed Mallampatti class II with restricted movements in the neck, which suggested possibility of difficult laryngoscopy and intubation.
Figure 1: Fused 2nd and 3rd cervical vertebra

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On ear examination, the child had multiple preauricular skin tags in front of both the ears, lying in line joining tragus and angle of mouth [Figure 2]. The external ears were normal in shape, size and function. Cardiovascular and central nervous system examinations were normal with no other associated congenital anomaly.
Figure 2: Cervicothoracic vertebral scoliosis

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Lateral view of the cervical spine X-ray showed fused second and third cervical vertebral bodies with loss of intervertebral disc space [Figure 3]. X-ray chest showed scoliosis of Cervico-thoracic spine with convexity towards right, [Figure 4] and computed tomography of head was normal.
Figure 3: Bilateral limbal dermoid

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Figure 4: Preauricular appendages

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Haemogram, bleeding time, and clotting time were within normal limits. Fenigold and Baun criteria (5) were used to diagnose her as a case of Goldenhar syndrome as she had bilateral pre-auricular appendages lateral limbal dermoid on both eyes and vertebral anomalies.

Airway and anaesthesia management
Preanesthetic evaluation of airway showed restricted mouth opening with Mallampatti classification II, normal thyromental distance, and restricted neck movements. Nasal patency was checked for both nostrils. The fibre optic intubation technique under nerve block and sedation was planned for airway management during surgery. Only sedation is not sufficient to keep vocal cords relaxed and it causes more number of attempts in the insertion of scope with endotracheal tube through the glottic aperture. This will lead to vocal cords injury may leads to laryngospasm, bronchospasm and stridor and during extubation.

The child was counselled for regional nerve block in the neck and procedure of fibreoptic intubation. A written informed parental consent was taken after explaining the risks involved in the management of difficult airway. Local anesthetic, Lignocaine sensitivity test was done. The 'difficult airway cart' with percutaneous tracheostomy set, was kept ready in the operation theatre.

A 20 gauge IV cannula secured in the left dorsum of hand, fluid ringer lactate was started at a rate 6 to 8 ml kg -1 .Child was taken inside the operation room and standard monitors ECG, NIBP and SpO 2 were attached. The Xylometazoline 0.1% nasal drops, instilled in both nostrils 15 minutes prior to procedure and topical anesthesia was achieved by nasal packing with 4% Lignocaine. The pharynx was sprayed with 4 to 6 puffs of Lignocaine10% aerosol. She was premedicated with intravenous Glycopyrrolate (.01mg kg -1), fentanyl (1μg kg -1) and Midazolam (0.05mg kg -1). The Superior laryngeal nerve and recurrent laryngeal nerve in the neck were blocked with 2%Llignocaine. After pre oxygenation with 100% oxygen for three minutes, fibre optic intubation (Karl strorz 3.7 x 65 intubating fibrescope model no 11302 BD2 made in Germany) was performed through nasal route with 6 mm portex cuffed endotracheal tube.

During the procedure sedation was supplemented with fentanyl. The correct positioning of tracheal tube was confirmed with capnograph and was firmly secured after confirming the equal bilateral air entry on auscultation. Afterwards induced with intravenous Thiopentone sodium dose of 2.5 mg kg -1 sufficient to abolish the eyelash reflex. She was maintained with Atracurium 0.5 mg kg -1 , Sevoflorane 0.5 to 1% nitrous oxide and oxygen on controlled ventilation via closed circuit.

The surgical procedure was smooth, uneventful performed only in the right eye. The duration of surgery about 60 minutes. Residual neuromuscular blockade was reversed with Neostigmine 0.05 mg kg -1 and Glycopyrrolate 0.01 mg kg -1. She was extubated, when fully awake and breathing spontaneously with adequate tidal volume. The postoperative period was also uneventful.


  Discussion Top


The Occulo Auricular Vertebral dysplasia or Goldenhar syndrome of our patient associated with under development of maxilla, fused 2-3rd cervical vertebra and scoliosis of cervico-thorasic vertebra with convexity to right and neck movements are restricted. All these features predicted difficult airway. Management of difficult air way in 12 year old girl is a debatable issue. Flexible fibre optic intubation under nerve block with sedation is seems to be better choice in the management of anticipated difficult airway. It is generally regarded as a gold standard method for endotracheal intubation in patients with cervical spine instability or immobility. [7],[8] A study [9] found that intravenous induction was preferable to the inhalational one. Other options are lighted stylet, retrograde intubation and percutaneous tracheostomy.

We did fibre optic intubation under both regional nerve block and sedation. Giving regional nerve block in 12 year old girl child is not an easy task, we convinced her to get co-operation for the procedure. This prevented us more number of attempts in doing fibre optic intubation in relaxed glottis and reduced cord injury, there by complications like airway oedema, stridor, laryngospasm and bronchospasm.

Finally we conclude Occulo Auricular Vertebral dysplasia also called Goldenhar syndrome is a rare congenital disorder of unknown aetiology, characterized by spectrum of physical features that vary in range and severity. [10] No single airway test can provide high index of sensitivity or specificity for predicting difficult airway. The fibre optic intubation under both regional nerve block and sedation is a better approach in the management of airway in this age group (10 to 12 years), instead of sedation alone.


  Acknowledgement Top


The authors would like to thank Principal, Jawaharlal Nehru Medical College, Belgaum for the permission to publish this case report. Also we would like to thank staff of Anaesthesiology, Ophthalmology and Plastic Surgery Department and patient for their cooperation.

 
  References Top

1.Goldenhar M. Association malformations of the eye and especially I'oreill epibulbar dermoid syndrome - Appendices-fistula auris congenita Auriculares-and its relationship with mandibulofacial dysostosis. J Genet Hum 1952;1:243-82.  Back to cited text no. 1
    
2.Kulkarni VV, Shah MD, Parikh AA. Goldenhar syndrome (a case report). J Postgrad Med 1985;31:177.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.Kumar R, Balani B, Patwari AK, Anand VK, Ahuja B. Goldenhar syndrome with rare association. Indian J Pediatr 2003;67:231-3.  Back to cited text no. 3
    
4.Gorlin RJ, Jue KL, Jacobson NP, Goldschmidt E. Oculo-auriculovertebral dysplasia. J Pediatrics 1963;63:991-9.  Back to cited text no. 4
    
5.Feingold M, Baum J. Goldenhar's syndrome. Am J Dis Child 1978;132: 136-8.  Back to cited text no. 5
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6.Tsui FJ, Tsai CH. Autosomal dominant inherited ocolu-auriculo-vertebral spectrum report of one family. Acta Paediatr Sin 1993;34:27-31.  Back to cited text no. 6
    
7.Uezono S, Holzman RS, Goto T, Nakata Y, Nagata S, Moriya S. Prediction of difficult airway in school-aged patients with microtia. Pediatric Anaesth 2001;11:409-13.   Back to cited text no. 7
    
8.Grewal A, Bhat D, Sood D, Garg S, Singh A, Bharti R, et al. Goldenhar syndrome: Anaesthetic and airway management. J Anaesth Clin Pharmacol 2005;3:313-6.  Back to cited text no. 8
    
9.Chen PP, Cheng CK, Abdullah V, Chu CP. Tracheal intubation using suspension laryngoscopy in an infant with Goldenhar's syndrome. Anaesth Intensive Care 2001;29:548-51.  Back to cited text no. 9
[PUBMED]    
10.Madan R, Trikha A, Venkataraman RK, Batra R, Katia P. Goldenhar syndrome: An analysis of anaesthetic management - A retrospective study of seventeen cases. Anaesthesia 1990;45:49-52.  Back to cited text no. 10
    


    Figures

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



 

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