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Anaesthetic approach in a case of Goldenhar's syndrome

Kaymak, C.; Gulhan, Y.; Ozcan, A. O.; Baltaci, B.; Unal, N.; Safak, M. A.; Oguz, H.

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European Journal of Anaesthesiology (EJA): November 2002 - Volume 19 - Issue 11 - p 836-838
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A spectrum of oculoauriculo-vertebral anomalies also called hemifacial microsomia or Goldenhar's syndrome is rarely seen. The frequency of occurrence is estimated to be 1 in 3000-5000, with male predominance. In this syndrome, variable combinations are seen of facial, ear, oral and vertebral anomalies, tending to be asymmetric and 70% unilateral [1].

Facial abnormalities are congenital hemicranio-facial dysmorphologies, hypoplasia of the malar, maxillary-mandibular region, especially the temporomandibular joint, and lateral cleft-like extension of the corner of the mouth. Ear abnormalities are microtia, accessory preauricular tags or pits, auricular appendices - most commonly in a line from tragus to the corner of the mouth - middle ear anomaly with variable degrees of deafness. Oral abnormalities are diminished to absent parotid secretions, anomalies in function or structure of the tongue and malfunction of the soft palate. Occasionally vertebral, ocular, cardiac, genitourinary and other (mental, intracranial, brachial, laryngeal) abnormalities are seen [1].

Upper airway dysmorphology is important in the anaesthetic management of a child with Goldenhar's syndrome. Airway obstruction and difficulty in tracheal intubation may result from a combination of micrognathia, unilateral mandibular hypoplasia and vertebral anomalies including vertebral fusion and odontoid elongation. Cooper and Murray-Wilson [2] reported that difficult or impossible tracheal intubation should be anticipated and that retrograde intubation in a 5-month-old male with Goldenhar's syndrome.

An 8-yr-old, 20 kg, female previously diagnosed with Goldenhar's syndrome was scheduled for ear canal reconstruction surgery. The features of Goldenhar's syndrome included hemifacial dysostosis (Fig. 1), left-sided mandibular hypoplasia, preauricular skin pit (Fig. 2), external auditory canal atresia (Fig. 3a,b) and a high-arched palate. Congenital heart disease or vertebral abnormalities were not present. In the physical examination, there were no pathological signs or symptoms. She had no difficulty in feeding, and had no airway obstruction or snoring or sleep apnoea problems. The patient had a general anaesthetic 2 yr previously because of a vesicoureteral reflux operation, and her family had been told about difficulty in intubating her. The arterial oxygen saturation rate was 98% during air breathing. No abnormality of the airway was demonstrated by either upper airway radiographs or chest radiography. Preoperatively, laboratory findings were normal, except cranial computed tomography showed external auditory canal atresia (Fig. 3a,b). The intubation score was Class III according to the Mallampati classification. Mouth opening was assessed as Grade 2 (<35°), one-third decreased according to the angle between the upper teeth occlusial face and the horizontal plane. Atlanto-occipital joint movements were evaluated using the angle of the line between the corner of the mouth, the tragus and the horizontal plane. It was <80° in our patient and difficult intubation was expected. The thyrohyoid distance was within the normal range. After 6 h fasting, atropine 0.02 mg kg−1 and midazolam 0.1 mg kg−1 were given intramuscularly for premedication. Non-invasive blood pressure, electrocardiogram (ECG) and SPO2 were monitored and venous access secured. After induction with thiopental 4 mg kg−1, anaesthesia was maintained with sevoflurane 2% in oxygen. Lung ventilation was achieved easily via a facemask. Laryngoscopy was performed using a Macintosh 2 blade, under deep inhalational anaesthesia, without the aid of a muscle relaxant, and both the epiglottis and glottis could be seen. Following pressure on the larynx, tracheal intubation was performed using a 5.5 mm internal-diameter endotracheal tube. Sevoflurane 2% in 50% O2/N2O was used for maintenance of anaesthesia. Atracurium 0.2 mg kg−1 was given when needed. At the end of the surgery, the patient was extubated after antagonism of residual neuromuscular block. The patient was closely observed in the recovery room and no anaesthetic complications were seen.

Figure 1
Figure 1:
Our patient with Goldenhar's syndrome demonstrating a hemifacial dysostosis.
Figure 2
Figure 2:
Our patient with Goldenhar's syndrome demonstrating a preauricular skin pit.
Figure 3
Figure 3:
Coronal tomographic section demonstrating external earway atresia.

Anaesthetists should be aware of congenital heart disease, vertebral anomalies and difficult tracheal intubation when dealing with the anaesthetic management of a patient with Goldenhar's syndrome [1]. Craniofacial anomalies often cause mechanical airway obstruction, especially during sleep, and cause obstructive sleep apnoea. Therefore, a patient with Goldenhar's syndrome should be monitored before anaesthesia during sleep with respiratory inductance plethysmography and pulse oximetry to assess respiratory patterns and arterial oxygen saturation [3]. There was no airway problem in our patient.

Prediction of difficult intubation is possible by a detailed clinical assessment. The classical features, Mallampati score, mouth opening and thyromental distance, etc., should be sought during an anaesthetic consultation [4]. In addition to these, Janssens and Hartstein [5] established an intubation score based on the various criteria. This score, the ADS (airway difficulty score), represents the sum of the points for five criteria of difficult intubation: thyromental distance, Mallampati class, mouth opening, neck mobility and upper incisors. The ADS also allows the clinician to distinguish difficulty in maintaining the patency of the upper airway.

A difficult intubation is 'an intubation during which the insertion of the endotracheal tube takes more than 10 min, and/or requires more than three attempts by an experienced anaesthesiologist' [5]. When tracheal intubation is difficult in both adults and children, the possible aids include: laryngeal mask airway®, Fastrach®, Combitube®, retrograde intubation, fibreoptic intubation and percutaneous cricothyroidotomy [4]. Whenever a difficult intubation is suspected, spontaneous ventilation of the lungs must be maintained until the airway has been secured. In most cases, an awake intubation is chosen and blind nasotracheal intubation has been the technique of choice for many years. Awake fibreoptic intubation has become the 'gold standard' in cases of anticipated difficult intubation [5].

Sculerati and colleagues [6] assessed airway management in children with major craniofacial anomalies in a 5 yr retrospective study. They reported that while patients with craniofacial synostosis had the highest rate of tracheostomy (48%), patients with mandibulofacial dysostoses had the next highest rate (41%); those with oculoauriculo-vertebral anomalies were less likely to require tracheotomy (22%). Madan and colleagues [7] reported that the laryngeal mask airway®, and also awake fibreoptic intubation via the laryngeal mask, had been used as alternative methods for difficult intubation. In their study, use of a laryngeal mask established a clear airway in an awake infant. In our case, because of hemifacial dysostosis and mandibular hypoplasia, difficult intubation was expected and laryngeal masks were ready for any possibility of difficult intubation. Use of laryngeal masks provides a safe airway in these cases.

Fibreoptic laryngeal intubation is limited to tracheal tubes of at least 4.5-5.0 mm internal diameter and requires considerable experience. In addition, tracheostomy carries many risks and requires considerable skill. Retrograde intubation may be successful in cases of prolonged intubation difficulty. However, retrograde intubation may cause complications. It is reported that after childhood as the child grows, conventional tracheal intubation becomes easier [2].

In our case, we also made preparations for retrograde intubation and tracheostomy. Our patient was not an infant: she was older than most of the other cases in the literature. In our opinion, this was the major reason why we did not have difficulty in intubation. We conclude that probable risks must always be anticipated in cases of Goldenhar's syndrome and according to the age group of the patient the anaesthetist must be ready for every possibility and be able to deal with a difficult intubation.

C. Kaymak

Y. Gulhan

A. O. Ozcan

B. Baltaci

N. Unal

Department of Anaesthesiology and Reanimation; The Ministry of Health; Ankara Research and Training Hospital; Ankara, Turkey

M. A. Safak

H. Oguz

Department of Otorhinolaryngology; The Ministry of Health; Ankara Research and Training Hospital; Ankara, Turkey


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© 2002 European Academy of Anaesthesiology