Tracheal stenosis due to cervicothoracic hyperlordosis in patients with cerebral palsy treated with posterior spinal fusion: a report of the first two cases

BACKGROUND: Spinal deformity is frequently identified in patients with cerebral palsy (CP). As it progresses, tracheal stenosis often develops due to compression between the innominate artery and anteriorly deviated vertebrae at the apex of the cervicothoracic hyperlordosis. However, the treatment strategy for tracheal stenosis complicated by spinal deformity in patients with CP remains unknown. CASE PRESENTATION: This study reports two cases: a 19-year-old girl (case 1) and a 17-year-old girl (case 2), both with CP at Gross Motor Function Classification System V. Both patients experienced acute oxygen desaturation twice within the past year of their first visit to our department. X-ray and computed tomography revealed severe scoliosis and cervicothoracic hyperlordosis causing tracheal stenosis at T2 in case 1 and at T3-T4 in case 2, suggesting that their acute oxygen desaturation had been caused by impaired airway clearance due to tracheal stenosis. After preoperative halo traction for three weeks, both patients underwent posterior spinal fusion from C7 to L5 with Ponte osteotomy and sublaminar taping at the proximal thoracic region to correct cervicothoracic hyperlordosis and thoracolumbar scoliosis simultaneously. Postoperative X-ray and computed tomography revealed that the tracheal stenosis improved in parallel with the correction of cervicothoracic hyperlordosis. Case 1 did not develop respiratory failure 1.5 years after surgery. Case 2 required gastrostomy postoperatively due to severe aspiration pneumonia. However, she developed no respiratory failure related to impaired airway clearance at one-year follow-up. CONCLUSIONS: We present the first two cases of CP that developed tracheal stenosis caused by cervicothoracic hyperlordosis concomitant with progressive scoliosis and were successfully treated by posterior spinal fusion from C7 to L5. This enabled us to relieve tracheal stenosis and correct the spinal deformity at the same time. Surgeons must be aware of the possibility of coexisting tracheal stenosis in treating spinal deformity in patients with neurological impairment because the surgical strategy can vary in the presence of tracheal stenosis. This study demonstrated that some patients with CP with acquired tracheal stenosis can be treated with spinal surgery.