AIRWAY MANAGEMENT IN CHILDREN WITH MORQUI O-BRAILSFORD-SYNDROME

Mucopolysaccharidosis IVA (Morquio-Brailsford syndrome) results from a n inborn deficiency of n-acetyl-galactosamine-6-sulphate sulphatase. C linical features include skeletal deformities with hypoplasia or absen ce of the odontoid process of the axis. The resulting atlanto-axial su bluxation compresses the spinal cord, resulting in cervical myelopathy . Without treatment, quadriplegia ensues sooner or later; consequently , surgical decompression and dorsal fusion of the cervical vertebrae i s recommended, either prophylactically or therapeutically. Anaesthesio logical management must focus on protection of the airway without comp romising integrity of the cervical spinal cord; quadriplegia subsequen t to positioning of the head under anaesthesia has been reported. We h ave performed fiberendoscopic nasotracheal intubation in a 23-month-ol d child presenting for neurosurgical treatment of cervical myelopathy resulting from Morquio-Brailsford syndrome. Case report. A 23-month-ol d girl (84 cm, 11 kg) with Morquio-Brailsford syndrome presented for s urgical decompression and dorsal fusion of the cervical spine. Pre-ana esthetic examination revealed enamel defects, chronic bronchitis, and splenomegaly; the neck was immobilised with a collar. Radiological exa minations (X-ray and NMR) revealed narrowing of the atlanto-occipital and atlantoaxial spaces (Fig. 1) and compression of the cervical spina l cord (Figs. 2 and 3). Pre-anaesthetic medication consisted of midazo lam juice (4 mg). After establishing intravenous access, atropine (0.5 mg), midazolam (1 mg), and ketamine (10 mg) were administered. A 22 F r nasopharyngeal airway (Wendl) was lubricated with local anaesthetic gel and introduced into the right nostril; oxygen was administered thr ough a probe to the left nostril. The Wendl-airway was then removed, a nother 5 mg ketamine was administered, and a 3.5-mm flexible fiberendo scope - over which a 20 Fr armored tube was slipped - was introduced t hrough the right nostril. With the child spontaneously breathing, the glottis was visualised and the fiberscope introduced into the trachea (Fig. 4); 1 mg midazolam and 35 mg ketamine was administered and the e ndotracheal tube was advanced through the nose into the trachea, utili zing the fiberscope as a guide. The distance between endotracheal tube and carina was assessed endoscopically, the fiberscope withdrawn, and the tube connected to the breathing system. Pulse oximetric readings were 98% during induction of anaesthesia including endotracheal intuba tion. Anaesthesia was continued with enflurane, alfentanil, midazolam, and atracurium; 315 min after induction the trachea was extubated and the child discharged to the paediatric intensive care unit. The posts urgical course was uneventful, and the child resumed co-ordinated gait . Discussion. Airway mangement in patients with mucopolysaccharidoses may be extremely difficult. Recommended methods such as blind nasal in tubation are not feasible in small children. Anaesthetic management in children younger than 2 years with Morquio-Brailsford syndrome presen ting for cervical spine surgery has not yet been described. Fiberoptic ally guided nasotracheal intubation is a means of airway management th at does not require repositioning of the head and may be performed wit h the stabilising collar left in place (Fig. 4); preservation of cervi cal spinal cord integrity may hence be assumed. Analgosedation with ke tamine and midazolam allows sufficient spontaneous breathing and - to some extent - maintenance of protective laryngeal reflexes. In conclus ion, anaesthetic management of patients with Morquio-Brailsford syndro me is a challenge that is further increased by extending indications f or surgical intervention to include infants. With respect to protectin g the airway, fiberoptic nasotracheal intubation of the spontaneously breathing child is our method of choice.