Airway management in an infant with tessier N. 4 anomaly.

Children with craniofacial abnormalities provide a challenge to an anesthesiologist being one the commonest cause of expected difficult airway. Difficult airway management should be predicted and planned in advance to avoid critical problems. It is important to understand the development and characteristics of the more common anomalies and their peculiar anesthetic challenges in order to construct a safe anesthetic plan. We describe the successful airway management of a Tessier N. 4 anomalous child with left orofacial cleft, cleft lip and cleft palate.

Introduction

Orofacial clefts are among the rarest malformations, with an incidence of 1.43-4.85 per 100,000 births, and of 1% among children born with cleft lip and/or palate.[12] Surgical treatment of clefts during the infancy is not only a challenge for the surgeon but also for the anesthesiologist. We describe the successful anesthetic management of a Tessier N. 4 anomalous child with left orofacial cleft, cleft lip and cleft palate.

Case Report

A 5-month-old infant, weighing 5 kg, in functional class ASA I was scheduled for reconstructive surgery of facial cleft and cleft lip. The child had been diagnosed as Tessier N. 4 anomaly but had no other associated congenital anomalies. On examination, he had left corneal opacity, absent left lower lid with the left orbit communicating with ipsilateral oral cavity. Maxilla on the left side was hypoplastic and eventrated. The cleft extended medially from left medial canthus to the left side of upper lip bordering nose laterally [Figure 1]. He also had cleft palate with cleft extending from alveolus to uvula. Systemic examination and blood investigations were normal.

Figure 1

Tessier N. 4 anomalous child with orofacial cleft on left, hypoplastic and eventrated maxilla, cleft palate extending from alveolus to uvula

The child was fasted as per standard guidelines. Anesthesia was induced with air, oxygen and sevoflurane. A 24-G intravenous cannula was placed. Adequate seal of face mask was initially attempted with size 1 mask but failed, snug fit was achieved with adult sized 3 face mask. However, mask ventilation was difficult and improved with packing the orofacial cleft with saline-soaked sterile gauze with the adhesive on top, and use of an appropriate sized Guedel's oral airway [Figure 2]. There was no interruption to breathing and normal levels of peripheral oxygen saturation were maintained (SpO2 = 99%). Fibreoptic bronchoscopy and equipments for tracheostomy was kept available in case of difficult intubation. Adequate depth of anesthesia was ensured. Laryngoscopy revealed a good view of vocal cords (Cormack-Lehane I) and orotracheal intubation with uncuffed south-pole tracheal tube size 4 was accomplished easily on spontaneous ventilation. Intraoperative monitoring included electrocardiogram, pulse oximetry, noninvasive blood pressure, temperature and capnography. Anesthesia was maintained with air, oxygen, isoflurane, IV morphine 0.5 mg, IV fentanyl 15 mcg, IV atracurium and paracetamol suppository 170 mg. The total duration of surgery was 3 hours. He had an uneventful intraoperative course. Trachea was extubated when the patient was awake. Patient was monitored in pediatric high dependence unit in the postoperative period. The postoperative period was uneventful and patient was discharged on the 2nd postoperative day. The child was advised cleft palate repair at 10 months of age.

Figure 2

Orofacial cleft with saline-soaked sterile gauze piece with adhesive on top and oral airway in situ

Discussion

The etiology of craniofacial cleft is multifactorial and includes maternal infections such as toxoplasmosis, alterations in phenylalanine and intake of thalidomide drugs. Tessier in 1990 classified craniofacial defects according to the anatomical defects as facial, craniofacial and laterofacial. Tessier N. 4 is described as a rare 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] The complete N. 4 oro-oculofacial cleft includes bone and soft tissue defects to a variable degree. It extends vertically from the lacrymal portion of the inferior eyelid medially to the infraorbital foramen on the orbital floor, continuing down through the maxillary sinus and cheek to penetrate the maxillary arch in the classic location of the labiomaxillary cleft, that is, between the lateral incisor and canine. Extrophy of the maxillary sinus is frequently associated and there is usually a marked decrease in the oculo-oral distance, as was observed in our case.

Initial priorities in such cases include airway patency, feeding, and protection of the cornea.[2] Airway problems in children with cleft lip and palate are well recognized. 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.[3] Age (<6 months), bilateral harelip, and retrognathism are considered predictive factors for difficult laryngoscopy and intubation.[4]

Airway management in these patients is often difficult when using conventional facemasks because of the large asymmetrical cheek and due to the need of avoiding excessive pressure on the opposite eyeball.[5] Our patient had a large perioral cleft involving cheek with a hypoplastic maxilla with eventration, which could compromise the ability to ventilate with face mask, if tracheal intubation proved difficult. To combat these problems, we used saline-soaked sterile gauze to fill the defect, appropriate size Guedel's airway and used adult-sized face mask (size 3) after induction of anesthesia. The laryngeal mask airway (LMA) insertion after sedation with transmucosal midazolam and lignocaine spray in pediatric age group may be an acceptable alternative for the similar group of patients.[3] We kept the equipments available to deal with difficult intubation and emergency tracheostomy as there are reports of failure to ventilate children with LMA in abnormal airway anatomy.[6-8]

Conclusion

Although the airway appears difficult in an infant with Tessier N. 4 anomaly, it can be managed with easily available technique by filling the defect by saline-soaked sterile gauze piece, appropriate sized Guedel's oral airway and using oversized face mask.