LETTERS TO EDITOR Year : 2022  |  Volume : 23  |  Issue : 2  |  Page : 152-153  

Anesthetic management of a rare case of an adolescent with goldenhar syndrome

Shalendra Singh1, Voddepalli Rajesh1, Ankit Singh2
1 Department of Anaesthesiology and Critical Care, Armed Forces Medical College, Pune, Maharashtra, India
2 Department of Anaesthesiology and Critical Care, Command Hospital (SC), Pune, Maharashtra, India

Date of Submission05-Jun-2022Date of Decision30-Jul-2022Date of Acceptance31-Jul-2022Date of Web Publication29-Oct-2022

Correspondence Address:
Dr. Shalendra Singh
Department of Anaesthesiology and Critical Care, Armed Forces Medical College, Pune - 411 040, Maharashtra
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/TheIAForum.TheIAForum_63_22

How to cite this article:
Singh S, Rajesh V, Singh A. Anesthetic management of a rare case of an adolescent with goldenhar syndrome. Indian Anaesth Forum 2022;23:152-3

Sir,

Goldenhar syndrome (GS) is a variant of the oculo-auriculo-vertebral spectrum consisting of hemifacial microsomia (HFM), epibulbar dermoids, and vertebral anomalies. Major manifestations of HFM are orbital distortion, mandibular hypoplasia, ear anomalies, nerve involvement, and soft tissue deficiency.[1] Other issues include heart, kidney, lung, and limb anomalies. The incidence is estimated from 1 in 3000 to 1 in 5000 live births.[1] During general anesthesia, the most common causes of morbidity are airway- and respiratory-related complications.

A 15-year-old 49-kg girl, a case of GS, presented with HFM with American Society of Anesthesiologists (ASA) Physical Status II for maxillomandibular distraction osteogenesis of the right jaw. She had various features of GS such as facial asymmetry, hypoplastic right external ear, preauricular skin tags, a deformed scapula, and scoliosis [Figure 1]a, [Figure 1]b, [Figure 1]c. Airway examination revealed a fissured tongue, high arched palate, submucous cleft palate, and Mallampati class II with limited movements of head and neck. No history was suggestive of any cardiac and central nervous system manifestations. All laboratory investigations were within normal limits. She had no vertebral anomalies or cardiac diseases.

Figure 1: (a) Goldenhar syndrome patient showing facial asymmetry, hypoplastic right external ear and preauricular skin tags, (b) computed tomography image of head and neck showing Maxillo-Mandibular Distraction Osteogenesis of the right jaw, (c) chest X ray image showing scoliosis

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The child was planned for nasal fiber-optic awake intubation. All preparations were made anticipating a difficult airway, having a fiber-optic bronchoscope (FOB), difficult airway trolley with endotracheal tubes, supraglottic airway devices, Guedel airway, and facemasks of different sizes. The patient was nebulized with 4% lignocaine; injection glycopyrrolate 0.2 mg and nasal decongestant drops were administered. Standard ASA monitoring was established. Dexmedetomidine was given as 1 μg/kg over 10 min followed by an infusion of 0.5 μg/kg intravenous. Awake fiber-optic intubation was performed through the left nostril with a size 6.5-cuffed, flexometallic tube; then, sevoflurane and injection atracurium 30 mg were given. The intraoperative course was uneventful. She was shifted to the recovery room in a fully awake state after surgery.

The airway and anesthetic management of the case presented was challenging as the preoperative assessment of her airway revealed the presence of mandibular hypoplasia, as well as limited head and neck movements with Mallampati class II. Obstructive sleep apnea (OSA) is common in patients with major craniofacial anomalies.[2] Mandibular abnormalities have 100% sensitivity and 96% specificity for predicting difficult laryngoscopy. Difficult mask ventilation, laryngoscopy, and intubation should be anticipated in advance; hence, the difficult airway cart kept ready and difficult airway algorithm should be discussed with the team members. Short-acting opioids such as remifentanil are preferred. Patients are more prone to develop airway-related complications such as laryngospasm and bronchospasm. In the absence of FOB, spontaneous ventilation is a key to avoid hypoxia and muscle relaxant should be given once the airway is secured. Difficult mask ventilation may result in insufflation of the stomach with air, increasing the chances of aspiration. Suction with a Ryle's tube is recommended before extubation. Expertise to secure the airway surgically should be in place. Before tracheal extubation, complete recovery of airway reflexes should be ensured. Prolonged postoperative monitoring in the intensive care unit is recommended, and the patient is to be observed for OSA.

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

Nil.

Conflicts of interest

There are no conflicts of interest.

 

  References 
1.Ashokan CS, Sreenivasan A, Saraswathy GK. Goldenhar syndrome-Review with case series. J Clin Diagn Res 2014;8:D17-9.  
    2.Abraham C, Virbalas J, DelRosso LM. Severe obstructive sleep apnea in a child with Goldenhar syndrome and nasal obstruction. J Clin Sleep Med 2017;13:825-7.  
    
  [Figure 1]