PubMed1993
Mucopolysaccharidosis IVA (Morquio-Brailsford syndrome) results from an inborn deficiency of n-acetyl-galactosamine-6-sulphate sulphatase. Clinical features include skeletal deformities with hypoplasia or absence of the odontoid process of the axis. The resulting atlanto-axial subluxation 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 is recommended, either prophylactically or therapeutically. Anaesthesiological management must focus on protection of the airway without compromising integrity of the cervical spinal cord; quadriplegia subsequent to positioning of the head under anaesthesia has been reported. We have performed fiberendoscopic nasotracheal intubation in a 23-month-old child presenting for neurosurgical treatment of cervical myelopathy resulting from Morquio-Brailsford syndrome. CASE REPORT. A 23-month-old girl (84 cm, 11 kg) with Morquio-Brailsford syndrome presented for surgical decompression and dorsal fusion of the cervical spine. Pre-anaesthetic examination revealed enamel defects, chronic bronchitis, and splenomegaly; the neck was immobilised with a collar. Radiological examinations (X-ray and NMR) revealed narrowing of the atlanto-occipital and atlanto-axial spaces (Fig. 1) and compression of the cervical spinal cord (Figs. 2 and 3). Pre-anaesthetic medication consisted of midazolam juice (4 mg). After establishing intravenous access, atropine (0.5 mg), midazolam (1 mg), and ketamine (10 mg) were administered. A 22 Fr nasopharyngeal airway (Wendl) was lubricated with local anaesthetic gel and introduced into the right nostril; oxygen was administered through a probe to the left nostril. The Wendl-airway was then removed, another 5 mg ketamine was administered, and a 3.5-mm flexible fiberendoscope--over which a 20 Fr armored tube was slipped--was introduced through 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 endotracheal tube was advanced through the nose into the trachea, utilizing 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 The distance between endotracheal tube and carina was assessed endoscopically, the fiberscope withdrawn, and the tube connected to the breathing system.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 oxymetric readings were 98% during induction of anaesthesia including endotracheal intubation. 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 postsurgical course was uneventful, and the child resumed co-ordinated gait. DISCUSSION. Airway management in patients with mucopolysaccharidoses may be extremely difficult. Recommended methods such as blind nasal intubation are not feasible in small children. Anaesthetic management in children younger than 2 years with Morquio-Brailsford syndrome presenting for cervical spine surgery has not yet been described. Fiberoptically guided nasotracheal intubation is a means of airway management that does not require repositioning of the head and may be performed with the stabilising collar left in place (Fig. 4); preservation of cervical spinal cord integrity may hence be assumed. Analgosedation with ketamine and midazolam allows sufficient spontaneous breathing and--to some extent--maintenance of protective laryngeal reflexes. In conclusion, anaesthetic management of patients with Morquio-Brailsford syndrome is a challenge that is further increased by extending indications for surgical intervention to include infants. With respect to protecting the airway, fiberoptic nasotracheal intubation of the spontaneously breathing child is our method of choice.