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Anaesthesia and orphan disease

Difficult ventilation following intubation in Goldenhar syndrome

Hasham, Faiz; van Helmond, Noud; Sidlow, Richard

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European Journal of Anaesthesiology: March 2017 - Volume 34 - Issue 3 - p 181-183
doi: 10.1097/EJA.0000000000000556
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Airway management difficulties have been reported in children with Goldenhar syndrome.1 The Goldenhar syndrome (oculo-vertebro-auricular syndrome) is characterised by macrostomia, facial asymmetry and hypoplasia, micrognathia, hypoplastic zygomatic arch, external ear malformations, hearing loss and ocular dermoids or lipodermoids. Other oral cavity anomalies may include a high-arched or cleft palate, and abnormalities of the tongue.1 We report ventilation problems in a 2-year-old girl with Goldenhar syndrome who presented for surgical repair of macrostomia.

On a recent medical mission with Healing the Children (Healing the Children Inc, Florida, USA) to Cusco, Peru, a 2-year-old girl presented at preoperative screening for the correction of macrostomia. Apart from features characteristic of Goldenhar syndrome, routine examination of her respiratory and cardiovascular systems was normal and the child appeared to be in good health. Assessment of the airway was difficult because of the unilateral deformity of the mandible, but on direct visualisation with a tongue depressor, the tip of the uvula was visible. We decided to proceed with the surgery the next day, and she was admitted to the hospital that evening to ensure that the mother complied with our NPO (nil per os) instruction.

On the morning of surgery, the child was examined again to rule out any possible respiratory infection that she could have acquired during the night in the hospital. The child was apyrexial, her chest was clear and her NPO status had been maintained.

Her preanaesthetic vital signs were normal and her oxygen saturation on room air was 96% (Nihon Kohden monitor, Tokyo, Japan). Cusco is at an altitude of 11 152 feet (3340 m) and we attributed the 96% oxygen saturation to this high altitude. Anaesthesia was induced with sevoflurane in oxygen, after which intravascular access was secured. Vital signs remained normal during induction. Laryngoscopy was then performed and the epiglottis and vocal cords were clearly visible. A Mallinckrodt (Covidien-Nellcor, Boulder, Colorado, USA) uncuffed orotracheal tube of 4.5 mm internal diameter was easily passed. We anticipated no further difficulty. However, when we connected the breathing circuit to the tracheal tube and attempted manual ventilation, we encountered a high airway pressure. On auscultation, we could not hear any breath sounds on either side of the chest. The child's oxygen saturation and end-tidal carbon dioxide concentration started to decrease. Sevoflurane anaesthesia was discontinued and 100% oxygen was administered. However, the child desaturated further, and as the saturation was now in the 70 to 80% range, we extubated the trachea and manually ventilated the lungs with a facemask with 100% oxygen. Surprisingly, the saturation came back to normal and auscultation revealed good bilateral air entry, with no expiratory rhonchi or other added sounds. After a quick equipment check, we felt that it could have been an oesophageal intubation, even though the vocal cords had been clearly visible. After deepening anaesthesia again, we performed laryngoscopy and an orotracheal tube was easily passed. Passage through the vocal cords was confirmed by a second anaesthesiologist. However, after we had maintained anaesthesia for a few minutes using sevoflurane, the same situation occurred: the child began to desaturate. We extubated the trachea, decided to cancel the procedure and allowed her to awaken. We transported her to the recovery room, and after she was stable and completely awake, a chest radiograph was ordered.

The chest radiograph revealed a right tracheal bronchus ending blindly (a so-called ‘tracheal diverticulum’), originating just below the vocal cords, with the trachea bifurcating at the normal level to supply the right and left lungs. We concluded that the desaturations had been a result of intubating the right tracheal diverticulum, thus occluding the airway. We felt that the delays in desaturation occurring were probably a result of preoxygenation prior to induction.

A tracheal bronchus is an anatomical deformity of the bronchial system that arises from the trachea above the carina. The incidence is reported to be 0.1 to 5% of the population.2 Although the existence of a tracheal bronchus was described as early as 1957, it is not a common differential diagnosis for intraoperative desaturation. The abnormal bronchus usually supplies the apical segment of the right upper lobe (with the normal right upper lobe bronchus supplying the rest of the lobe), an accessory segment within the upper lobe, an accessory segment completely separate from the upper lobe or the whole right upper lobe (in which case the normal right upper lobe bronchus is absent).2

Our patient presented with a blind-ending tracheal bronchus, a rare anomaly that can be considered a lesser form of an accessory tracheal bronchus.2 This ‘diverticulum-variant’ tracheal bronchus is probably a tracheal bud that has not developed fully or that has been resorbed partially. A tracheal diverticulum is usually asymptomatic, but may give rise to symptoms when it becomes infected. A tracheal diverticulum may be visualised on conventional radiograph or computed tomography (CT)-scan, with definitive confirmation on fibreoptic examination.2 Unfortunately, we did not have access to a fibreoptic laryngoscope given our geographic location at the time and we cannot present a copy of the radiograph from the hospital in Peru, as the hospital was unwilling to release the image. The chest radiograph showed bifurcation of the trachea just below the vocal cords with the right branch ending blindly and the left branch bifurcating again into right and left lungs at the normal level of the carina.

Goldenhar syndrome was first described in 1952 by Maurice Goldenhar. The incidence is reported as occurring in one in every 3000 to 5000 live births.1 It is a complex malformation of varying severity involving the structures arising from first and second branchial arches, first pharyngeal pouch, first branchial cleft and primordia of the temporal bone. There may be associated anomalies in other major organ systems, including cardiac structural defects, renal agenesis, pulmonary agenesis and vascular anomalies. There have been a few reports of Goldenhar syndrome with associated pulmonary anomalies in the literature.3–8 Usually, there is involvement of the lung on the same side as that of facial anomalies,3,5,6 but contralateral pulmonary involvement has also been reported.7,8 Our patient presented with a tracheal diverticulum on the same side as that of the facial anomalies, without any other pulmonary involvement. The relationship between Goldenhar syndrome and pulmonary anomalies should be considered in the preoperative evaluation of patients with this condition. Pulmonary imaging may be useful to assess if there is any lung involvement to avoid intraoperative airway management difficulties.

Acknowledgements relating to this article

Assistance with the case report: the authors extend their gratitude to the parents of the patient described for granting their permission to publish this instructive case report.

Financial support and sponsorship: none.

Conflicts of interest: none.


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© 2017 European Society of Anaesthesiology