Airway management in an infant with congenital centrofacial dysgenesia

B. Carenzi*,1, R. M. Corso2, V. Stellino1, G. D. Carlino1, C. Tonini2, L. Rossini2 and G. Gentili1

1Department of Neurosurgery, Section of Anaesthesia and Intensive Care, A.S.L. Città di Bologna, Ospedale ‘Bellaria-C.A. Pizzardi’, via Altura 3, Bologna, Italy. 2Institute of Anaesthesiology and Intensive Care, University of Bologna, Ospedale Policlinico S. Orsola Malpighi, Italy*Corresponding author: via Renata di Francia 44, I-44100 Ferrara, Italy

{dagger}LMA® is the property of Intavent Limited.

Accepted for publication: December 12, 2001


    Abstract
 Top
 Abstract
 Introduction
 Case report
 Discussion
 Conclusions
 References
 
The use of a laryngeal mask airway (LMA{dagger}) on two occasions, in a 53-day-old and 270-day-old male infant with Tessier N.3 and N.4 facial defects, using sedation and topical anaesthesia is described. The LMA was used to manage the airway and facilitate inhalation induction of anaesthesia as the facial deformities were thought to be too extensive for the safe use of a facemask. The LMA is an alternative to a facemask and secures the airway and facilitates the inhalation induction of anaesthesia in paediatric patients with severe facial deformities.

Br J Anaesth 2002; 88: 726–8

Keywords: complications, dysgenesia; airway, complications


    Introduction
 Top
 Abstract
 Introduction
 Case report
 Discussion
 Conclusions
 References
 
Congenital cleft cheeks are rare and disfiguring malformations with an incidence of 1.43–4.85/100 000. The aetiology is multifactorial and includes maternal infections such as toxoplasmosis, alterations in phenylalanine, and intake of anticomitial and thalidomide drugs. Tessier in 1990 classified them according to the anatomical defects as facial, craniofacial and laterofacial.1 Tessier N.3 includes harelip extending to a wing of the nose and connected to the medial palpebral angle;2 Tessier N.4 is described as an anomaly of the bony tissue with a wide harelip extending to the cheek, orbital dystopia, anomalies of the eyeball and reduced ocular-oral distance.3 Surgical treatment of these malformations involves several corrective operations over the years and include: (1) medial canthopexy to safeguard the integrity of the eyeball; (2) reconstruction of the cheek defect; (3) bone graft to the orbital region; and (4) attempts to expand the orbital cavity in patients presenting with michrophthalmia and anophthalmia.2 Airway management in these patients is often problematic when using conventional facemasks.4

The laryngeal mask airway (LMA{dagger}) has become an accepted aid in cases of difficult tracheal intubation in both adults and children.49 In addition the LMA has been used successfully as a conduit for fibrescope-assisted tracheal intubation in patients with congenital syndromes such as Pierre-Robin, Treacher-Collins, and Goldenhar, known to present with difficult airways.1014 We describe the use of an LMA as a part of the anaesthetic strategy in an infant with congenital centrofacial dysgenesia.


    Case report
 Top
 Abstract
 Introduction
 Case report
 Discussion
 Conclusions
 References
 
A 53-day-old male infant, weighing 4.4 kg, with Tessier’s facial anomaly N.4 on the left side and N.3 on the right side and with right ocular agenesis, presented for urgent surgery consequent to the detection of a corneal ulceration in the residual eye, as a result of blepharal dysgenesis. The infant was normally delivered at term (3.750 kg) with no other abnormalities.

The presenting problem with airway management was the inability to use a correctly sized facemask because of the large asymmetrical cleft cheek as well as the need not to exert pressure on the other eyeball (Fig. 1).



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Fig. 1. Frontal photograph of a 53-day-old male infant with Tessier’s facial anomaly N.4 on the left side and N.3 on the right side, and with right ocular agenesis.

 
Monitoring in the operating theatre, before the induction of anaesthesia, included continuous ECG, non-invasive arterial pressure (NIAP), pulse oximetry (SpO2), and rectal temperature. Preoxygenation of the patient’s lungs was carried out for 5 min and required the use of an adult sized facemask (size 4) in order to cover the entire face including the anatomical defect without exerting pressure on the residual eyeball. After sedation with transmucosal midazolam (0.25 mg kg–1), i.v. access was obtained, the pharynx was sprayed with lignocaine 4 mg in two divided doses, and a size 1.5 LMA was inserted successfully using the recommended insertion technique. The neck was flexed and the head extended and the deflated LMA cuff was pressed against the hard palate and then pushed backwards along the anterior and posterior pharyngeal wall into its final position. The insertion of the LMA caused the arterial pressure to rise to 85/50 mm Hg from 75/40). There was no interruption to breathing and normal levels of peripheral oxygen saturation were maintained (SpO2=99%). The LMA provided a secure airway both for oxygen administration and provided a good seal for manual ventilation of the lungs. Inhalation induction of anaesthesia with 2.5% sevoflurane and 50% nitrous oxide was commenced and larger venous access secured. After induction vecuronium 0.6 mg and fentanyl 10 µg i.v. were given and the LMA removed. Laryngoscopy revealed a good view of the vocal cords (Cormack-Lehane I) and orotracheal intubation with a flexible uncuffed tracheal tube size 3.5 (Rusch®) was accomplished easily. An arterial line was then inserted for additional perioperative monitoring. The surgical procedure of partial correction of cleft palate, harelip, and blepharoplasty lasted 6 h and was uneventful as were extubation and recovery.

A second corrective surgical procedure was carried out on this infant with the same technique at the age of 5.5 months (weight 6 kg). On this occasion the patient was pre-medicated with oral diazepam 0.3 mg kg–1 and atropine 0.01 mg kg–1 given 30 min before surgery. After 5 min of preoxygenation, i.v. access was obtained. After administration of oropharyngeal topical anaesthesia (lignocaine 1 mg kg–1) a size 2 LMA was inserted using the standard insertion technique. Once again the airway was secured easily and the ability to apply positive pressure ventilation was confirmed. Anaesthesia was induced with 2.5% sevoflurane and nitrous oxide (FIO2 50%) and further venous access established. Propofol 10 mg, fentanyl 20 µg, and vecuronium 0.2 mg kg–1 were administered and an additional minute of manual ventilation with 100% oxygen was carried out. Laryngoscopy revealed a good view of the vocal cords (Cormack-Lehane I) and the trachea was intubated with a flexible, uncuffed size 4 tracheal tube (Rusch®). Pulse oximetry and NIAP were unchanged, and an arterial line was placed for perioperative monitoring during the 4.5 h surgical procedure. There were no perioperative problems and the infant was observed for 1 h in the postoperative surgical unit. He was discharged to the ward 1 h after extubation of the trachea and discharged home after 4 days.


    Discussion
 Top
 Abstract
 Introduction
 Case report
 Discussion
 Conclusions
 References
 
Airway problems in children with cleft lip and palate were recognized by Magill more than 70 yr ago15. A careful preoperative examination is, therefore, essential in order to recognize potential difficulties and select the most appropriate technique to secure adequate oxygenation and ventilation. Recent studies in paediatric patients undergoing corrective procedures for cleft palate have identified several variables associated with difficult laryngoscopy and intubation. Age (<6 months), bilateral harelip, and retrognathism are considered predictive factors for difficult laryngoscopy and intubation.16 In addition to these factors this patient presented with severe facial deformity because of the large perioral and right cheek defect, both of which could compromise the ability to use a facemask for the administration of oxygen, inhalation agents for induction of general anaesthesia, or to assist ventilation if tracheal intubation proved difficult.

The use of an LMA has been advocated in children with potentially difficult anatomy in order to avoid excessive airway instrumentation, thereby minimizing the risk of trauma and airway obstruction. Insertion of an LMA while our patient was still awake secured a patent airway before the induction of general anaesthesia. Once the infant was anaesthetized, we were able to proceed calmly with the task of tracheal intubation.

We were prepared for laryngospasm, coughing, or breath holding. If laryngospasm is encountered during the insertion of the LMA, we would recommend the use of succinylcholine and subsequent ventilation through the LMA, striving for an adequate depth of anaesthesia.

As LMA failure to ventilate children with abnormal airway anatomy has also been reported, equipment to deal with difficult intubation and emergency tracheostomy should be available.1719


    Conclusions
 Top
 Abstract
 Introduction
 Case report
 Discussion
 Conclusions
 References
 
The use of sedation and topical anaesthesia of the pharynx and larynx allowed placement of the LMA to secure safely the airway in a paediatric patient with severe facial abnormalities on two separate occasions. The LMA was used as a conduit to facilitate inhalation induction of general anaesthesia in a patient in whom the use of facemask was not desirable.


    References
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 Abstract
 Introduction
 Case report
 Discussion
 Conclusions
 References
 
1 Tessier P. Anatomical classification of facial, craniofacial and laterofacial clefts. In: McCarthy JG ed. Plastic Surgery Vol. 4. Philadelphia: W. B. Saunders, 1999; 2922–73

2 Darzi MA, Chowdri NA. Oblique facial clefts: a report of Tessier numbers 3, 4, 5 and 9 clefts. Cleft Palate Craniofacial J 1993; 30: 414–5

3 Longaker MT, Lipshuts GS, Kawamoto HK jr. Reconstruction of Tessier no. 4 clefts revisited. Plast Reconstr Surg 1997; 99: 1501–7[ISI][Medline]

4 Hatch DJ. Airway management in cleft lip and palate surgery. Br J Anaesth 1996; 76: 755–6[Free Full Text]

5 Berry AM, Brimacombe JR, Verghese C. The laryngeal mask airway in emergency medicine, neonatal resuscitation, and intensive care medicine. Int Anesthesiol Clin 1998; 36: 91–109[ISI][Medline]

6 Boehringer LA, Bennie RE. Laringeal mask airway and the paediatric patient. Int Anesthesiol Clin 1998; 36: 45–60

7 Lopez-Gil M, Brimacombe J, Alvarez M. Safety and efficacy of the laryngeal mask airway. A prospective survey of 1400 children. Anaesthesia 1996; 51: 969–72[ISI][Medline]

8 Brain AIJ, McGhee TD, McAteer EJ, Thomas A, Abu-Saad MAW, Bushman JA. The laryngeal mask airway. Development and preliminary trial of a new type of airway. Anaesthesia 1985; 40: 356–61[ISI][Medline]

9 Johnston DF, Wrighley SR, Robb PJ, Jones HE. The laryngeal mask airway in paediatric anaesthesia. Anaesthesia 1990; 45: 924–7[ISI][Medline]

10 White AP, Billingham IM. Laryngeal mask guided intubation in paediatric anaesthesia. Paediat Anaesth 1992; 2: 265

11 Goldie AS, Hudson I. Fibreoptic tracheal intubation through a modified laryngeal mask. Paediat Anaesth 1992; 2: 343–4

12 Walker RWM. The laryngeal mask airway in the difficult paediatric airway: an assessment of positioning and use in fibreoptic intubation. Paediat Anaesth 2000; 10: 53–8[ISI]

13 Hansen TG, Joensen H, Henneberg SW, Hole P. Laryngeal mask airway guided tracheal intubation in a neonate with the Pierre Robin Syndrome. Acta Anaesthesiol Scand 1995; 39: 129–31[Medline]

14 Wheatley RS, Stainthorp SF. Intubation of a one-day-old baby with the Pierre Robin Syndrome via a laryngeal mask. Anaesthesia 1994; 49: 733

15 Hatch DJ. Magill’s endotracheal catheter device for use during repair of cleft lip and palate. Paediatr Anaesth 1995; 5: 199–201[ISI][Medline]

16 Gunawardana RH. Difficult laryngoscopy in cleft lip and palate surgery. Br J Anaesth 1996; 76: 757–9[Abstract/Free Full Text]

17 Brimacombe JR, Berry AM, White PF. The laryngeal mask airway: limitations and controversies. Int Anesthesiol Clin 1998; 36: 155–82

18 ElHammar F, Dubreuil M, Benoit I, et al. A difficult intubation of an infant with McKusick-Kaufman syndrome. Failure of the laryngeal mask-fibroscope sequence. Ann Fr Anesth Reanim 1998; 17: 240–2[ISI][Medline]

19 Busoni P, Fognani G. Failure of the laryngeal mask to secure the airway in a patient with Hunter’s syndrome (mucopolysaccharidosis type II). Paediat Anaesth 1999; 9: 153–5[ISI]





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