Airway concerns in a neonate with Treacher Collins syndrome posted for tracheoesophageal fistula repair

Airway concerns in a neonate with Treacher Collins syndrome posted for tracheoesophageal fistula repair: Sushama Raghunath Tandale, Yogesh Gavali, Subhadra Sinha, Chandraprabhu Birnale



Archives of Medicine and Health Sciences 2019 7(2):319-320



Sir,

A 3-day-old male, term neonate, weighing 2.9 kg was referred to our institute for tracheoesophageal fistula repair with provisional diagnosis of  Treacher Collins syndrome ^{More Details} (TCS). Examination revealed tachypnea, heart rate 160 bpm, blood pressure 68/48 mmHg, and SpO₂ reading 98% with 2 L oxygen. Facial dysmorphic features included hypoplasia of facial bones (mandible and maxilla), down-sloping palpebral fissures, coloboma of the left upper eyelid, scanty lower eyelash, micrognathia, retrognathia, and malformation of auricle with atresia of the external auditory canals, diagnosed as TCS [Figure 1]. Thoracotomy for ligation of tracheoesophageal fistula with primary repair of esophagus was planned under general anesthesia. Blood investigations and arterial blood gas analysis were unremarkable. Echocardiography revealed atrial septal defect of 4 mm with left-to-right shunt. Inhalational anesthesia with spontaneous breathing was planned to administer anesthesia. Standard monitoring was done. Difficult airway cart was kept ready in view of difficult airway secondary to micrognathia and retrognathia, including face mask, oropharyngeal, nasopharyngeal airway, endotracheal tube, and laryngeal mask airway of assorted sizes, stylet, laryngoscope with straight and curved blades, and tracheostomy tray. Intravenous glycopyrrolate 30 mcg and dexamethasone 0.3 mg was given as premedication. After preoxygenation, anesthetic plane was deepened with gradually increasing concentration of sevoflurane up to eight volume percent with 100% oxygen via Jackson Rees circuit. Effective mask ventilation was achieved with two-hand technique and jaw thrust. Gentle laryngoscopy was performed by experienced anaesthetist to avoid airway trauma. Laryngoscopy was performed with Miller size 0 blade, which showed Cormack Lehane Grade III view and without any improvement with external laryngeal maneuver. Further attempt of laryngoscopy was performed with Miller size 1 blade which improved laryngoscopic view to Cormack Lehane Grade II with external laryngeal maneuver followed by orotracheal intubation with 2.5-sized endotracheal tube with stylet. Anesthesia was maintained with 100% oxygen (as air not available with institute), sevoflurane, and intermittent doses of fentanyl and atracurium. Surgery was uneventful with minimal intraoperative blood loss. Neonate was shifted to intensive care unit with ventilatory support for postoperative care. He succumbed on the 5^th postoperative day due to septicemia.

Figure 1:(a) Neonate having facial dysmorphic features of Treacher Collins syndrome. (b) Neonate after endotracheal intubation Click here to view

TCS, also referred as mandibulofacial dysostosis, is a congenital malformation of the first branchial arch.^[1] It consists of deformities of ear, eyes, maxilla, and mandible. Cleft lip, cleft palate, and congenital palatopharyngeal incompetence are present in 35% of patients. Congenital heart disease is an important association, seen in index case.^[2] They are susceptible to upper airway obstruction, pharyngeal edema, laryngeal edema, and respiratory distress postoperatively.^[2] Airway management is difficult in these patients due to the presence of upper airway obstruction, relative macroglossia, retrognathia, and difficult intubation.^[2] Airway difficulty increases with age, which warrants reevaluation of airway before each anesthetic.^[3]

Successful airway management is reported with direct laryngoscopy, intubation with flexible fiberoptic bronchoscope, blind nasal intubation, laryngeal mask airway, light wand, retrograde intubation, and tracheostomy in older children.^[4] Most of these techniques are difficult to perform in neonate. In our case, the modified conventional approach was found safe and effective. Forward lift of both mandibles with jaw thrust achieved good ventilation in retrognathic neonate and use of large-sized Miller blade with external laryngeal maneuver helped in visualization of glottis. A large-sized Miller blade was preferred as it causes minimal compression and equals distribution of tongue tissue on either side, making a long clear central path for glottis visualization.^[5]

This implies that modified conventional technique of direct laryngoscopy can be tried in airway management of the neonate with TCS where use of other techniques is impractical.

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.	Agrawal S, Asthana V, Sharma JP, Sharma UC, Meher R. Alternative intubation technique in a case of Treacher–Collins syndrome. Internet J Anesthesiol 2006;11:1-8.
2.	Goel L, Bennur SK, Jambhale S. Treacher-collins syndrome-a challenge for anaesthesiologists. Indian J Anaesth 2009;53:496-500.   [PUBMED]  [Full text]
3.	Inagawa G, Miwa T, Hiroki K. The change of difficult intubation with growth in a patient with Treacher Collins syndrome. Anesth Analg 2004;99:1874.
4.	Sculerati N, Gottlieb MD, Zimbler MS, Chibbaro PD, McCarthy JG. Airway management in children with major craniofacial anomalies. Laryngoscope 1998;108:1806-12.
5.	Bajwa SJ, Bajwa SK, Singh A, Khan B, Parmar SS, Singh G, et al. Anesthetic challenges and difficulties in the management of Treacher Collins syndrome. Anesth Essays Res 2011;5:111-3.     [Full text]

Figures

  [Figure 1]