Anesthesia for patients with spondyloepiphyseal dysplasia congenita
Yagi, Y.; Yamazaki, M.; Satone, T.
Masui. Japanese Journal of Anesthesiology 36(5): 793-796
1987
ISSN/ISBN: 0021-4892 PMID: 3656650 Document Number: 296930
Thirty-seven and forty-three year-old brothers with spondyloepiphyseal dysplasia congenita suffering from cervical myelopathy were scheduled to undergo decompression of the cervical canal and the foramen magnum. They showed severe disproportionate short stature. The neck was so short that normally formed head seemed to sit on the shoulders. Respiratory problems were seen resulting from the marked shortening and horizontally lying of the ribs, thoracic kyphosis and pear-shaped vertebrae. Respiratory function showed restrictive pattern. The younger brother showed arterial hypercapnea. Airway examination revealed laryngeal hypoplasia. First, the younger brother's operation was undertaken. Anesthesia was induced with nitrous oxide, oxygen and halothane, and pancuronium bromide was used for muscle relaxation. Laryngoscopy was difficult and intubation was possible with only 4 mm I.D. endotracheal tube. Anesthesia was maintained with nitrous oxide, oxygen and enflurane, and intraoperative course was uneventful. But postoperative respiratory management was troublesome and necessitated tracheostomy. The elder brother received tracheostomy before the operation for the postoperative respiratory management. Intra- and postoperative course was uneventful. For the anesthetic management of this disease the following points should be considered; airway management difficulty, restrictive respiratory insufficiency, atlant-axial instability and the choice of anesthetic method.