Anaesthetic management of a child with a rare congenital malformation: Bilateral macrostomia as an isolated asyndromic entity

Sir,

Macrostomia is one of the rarest facial anomalies classified among facial clefts with an incidence of 1 in 2,25,000 live births and constitutes about 1:100-1:300[1] of all facial clefts. Bilateral macrostomia as an isolated asyndromic entity is extremely uncommon with the present one being probably the 22^nd reported in the literature so far.

A 2-year-old child with bilateral macrostomia without any other associated congenital anomaly was posted for correction of facial deformity. Clinical examination revealed bilateral facial clefts across the cheeks extending up to the anterior border of masseter [Figure 1]. The risk of the difficult airway was explained, and consent for tracheostomy was taken. A Gamgee pad (obtained from the central sterile services department at our hospital) with an oval opening corresponding to normal oral aperture was kept ready. Difficult airway cart including laryngeal mask airway (LMA), different sized face masks, Magill's forceps, bougie, stylet, McCoy's laryngoscope blade and a tracheostomy kit were kept ready. Premedication with intranasal midazolam 0.2 mg/kg allowed easy parental separation. Sevoflurane 8% was insufflated using size 4 transparent face mask which covered the lateral clefts. Once unresponsive, the Gamgee pad was put with few drops of distilled water around the margins of the oval opening for a complete seal and ventilation with size 2 Rendell Baker Soucek (RBS) mask was possible. Venous access was secured with 22G cannula and glycopyrrolate, ondansetron and fentanyl were administered. Paediatric xylometazoline drops were administered in nostrils. As direct laryngoscopic view was Cormack–Lehane grade 1, succinyl choline was administered, and a lubricated 4.5 ID endotracheal tube was passed nasally followed by throat packing. Electrocardiogram, oxygen saturation, end tidal CO2 and temperature were continuously monitored. Surgical repair consisted of straight line closure, creating a neocommisure and repair of orbicularis oris.

Figure 1.

Child with bilateral macrostomia

Macrostomia, classified as Tessier cleft type 7, is a poorly characterized malformation. It may be found as part of cranio facial anamolies. Bilateral forms are more often isolated than unilateral ones.[1,2] The embryonic origin may be related to disruption during fusion of the maxillary and mandibular processes of the first branchial arch in the 7^th embryonic week. The aetiopathogenesis remains unclear involving interactions of several genetic and environmental factors. Various factors like placental infarction, amniotic bands, diabetes, Vitamin E deficiency, rubella infection and radiation have been suggested.[1,2]

Macrostomia is evidenced by an increased diameter of the mouth ranging from a slight widening of the mouth to a severe cleft extending back to the ears causing disfigurement. It is troublesome due to sialorrhoea, speech problems, compromised suckling and mastication.[2,3]

There is no detailed literature available regarding the anaesthetic problems. The anaesthetic challenges primarily involve the airway management.[4] There may be difficult or impossible mask ventilation. Intubation can also become difficult when associated with congenital syndromes.

We opted for intranasal midazolam as macrostomia itself was a limiting factor for oral administration in this child. Intranasal route of administration ensures rapid and complete absorption and provides predictable and effective sedation.[5] In our patient, it helped in creating a calming effect and easy mask acceptance.

Huge macrostomia can lead to a difficult mask fit resulting in difficult inhalational induction and mask ventilation as the lateral extension of cleft would not be enclosed within the appropriate size face mask.[4,6] We preferred inhalational induction over intravenous in view of expected difficult airway and venous access. We opted for a bigger mask just to cover the clefts allowing insufflation with sevoflurane until the child became unresponsive. Gamgee pad with an oval opening corresponding to oral aperture helped ineffective mask ventilation using size 2 RBS mask. Wetting of the margin of the aperture helped in achieving a complete seal.

Evidence from closed claim studies suggests that the commonest cause of airway related morbidity in paediatric patients is due to the inability to ventilate.[7] Safe, effective airway management requires a systematic individualised approach with clear strategies. Preoperative evaluation, anticipation of difficulty, preparation, planning for alternatives, judicious use of airway aids and management of complications constitute the key to success in difficult paediatric airway management.[8] Adequate planning and preparation helped us in achieving airway control and avoiding a major airway catastrophe.

In the literature, various techniques including LMA, fibreoptic intubation and use of micropore adhesive[6] have been described to manage the airway. Gamgee pad is easily available, cheap, soft, atraumatic and acceptable. It can be easily reapplied any time in emergency situations. The surgical procedure necessitates introduction of a nasotracheal tube. It gives way for more critical assessment of new points of commissure and avoids reconstruction of orbicularis oris under tension. This repair is significant to achieve normal sphincter like function necessary for articulation and mastication.[1]

To summarize, difficult paediatric airway is every anaesthesiologist's nightmare. Airway management technique depends on the availability of suitable equipment, experience and expertise of the attending anaesthesiologist. We devised a simple solution to overcome the problem of difficult mask ventilation by using a Gamgee pad with an oval aperture corresponding to the normal mouth aperture.