Anesthetic management for a patient with spondyloepiphyseal dysplasia congenita

An 11-year-old girl with spondyloepiphyseal dysplasia congenita in the spine was scheduled for virectomy under general anesthesia. She had slight scoliosis in the thoracic and lumbar spine, moderate funnel chest and slight thoracic kyphosis. Preoperative laboratory data were within normal range and her intelligence was normal. Her Mallanpathi's score, however, was Grade 3 and effective mandibular length/posterior depth of mandible ratio was 3.48 on the lateral view of head X-ray. From these data, difficult intubation was expected. Although anesthesia was induced uneventfully using thiamylal and vecuronium, her vocal cord was not visualized under laryngoscope. Using cricoid pressure procedure, slightly left-shifted vocal cord was exposed. However a 26 Fr. size endotracheal tube without cuff was too large and finally a 22 Fr. tube was inserted. During the operation patient's general condition was stable and the operation was finished without any episodes. She did not have any complications in the postoperative period. In a case of SDC, pathological changes in laryngotracheal resion should be examined and evaluated preoperatively and difficult intubation should also be always taken into account.