Physiotherapy Management of Occipito-cervical Fusion in Patients with Morquio Syndrome (Mucopolysaccaridosis Type IVA)

Abstract

<h2>Summary</h2> This paper describes Morquio syndrome as a genetically transmitted, mucopolysaccaride storage disease with dwarfism, orthopaedic and neurological signs. The cervical spine is of particular concern because of instability and spinal cord compression. Myelopathy and instability demonstrable on X-ray are indications for occipito-cervical fusion. At University College Hospital, fusion consisted of extensive grafting and wiring of the occiput to the upper cervical spine. The patient is immobilised with the neck in stable extended position in a halo and plaster jacket. There is extensive physiotherapy involvement, both pre- and post-operatively. Assessment, chest care, handling, early and late rehabilitation are described. It is concluded that a centrally located register to include the names of departments with specific experience of rare conditions should be developed, along with further research into the long-term results following occipitocervical fusion in these patients.