154 DEFORMITIES IN CHILDREN WITH NEUROMUSCULAR DISEASES

Abstract

Since the commencement of the Neuromuscular-Unit in the Children’s-Hospital “Agia Sofia”, from December 2002 until December 2008, 306 patients were examined suffering from different neuromuscular diseases (ND). In the present study we examined

1. the frequency of spine deformities,

2. the management in correlation with the poor general health of these patients, analyzing the most frequent presenting disease, that is Duchenne’s-muscular-dystrophy,

3. cases of surgical management of our Unit are presented.

From the analysis of our material we found that 152 patients were suffering from Duchenne’s-Becker muscular-dystrophy, 59 patients from spinal-muscular-atrophy I-III, 13 patients from fascioscapulohumeral muscular-dystrophy, 15 patients from hereditary motorsensory-neuropathies, 5 patients from Friedreich’s Ataxia and 62 patients from different types of dystrophinopathies–myopathies. The ages of the patients varied between 8 months and 37 years.

From the total, 89% of the patients above 10 years presented with spine deformities. Most of them were managed with wheelchairseating modifications and 33 patients were fitted with braces in an attempt to slow curve progression. Surgically were managed 24 patients with spine arthrodesis, 5 of them abroad (USA-Eng-land-France). The older patients (> 17 years wheelchair-bound) were frequently, because of impaired general health, not suitable candidates for surgery.

Pulmonary function was examined in 84 patients suffering from Duchenne’s muscular dystrophy. The crucial age, were pulmonary function has fallen dramatically (FVC< 40%), was between ages 12–15 years.

In conclusion spine deformities are very common in patients suffering from neuromuscular diseases. Because of the rapid deterioration of the general health of these patients spine arthrodesis should be performed early, in the patients severely affected, between ages 12–15 years