Respiratory failure in postpneumonectomy syndrome complicated by thoracic lordoscoliosis: treatment with prosthetic implants, partial vertebrectomies, and spinal fusion.

STUDY DESIGN: This study investigated the case of a 17-year-old girl with postpneumonectomy syndrome, complicated by a thoracic lordoscoliosis, who was successfully treated with prosthetic implants, partial vertebrectomies, and anteroposterior spinal fusion. OBJECTIVE: To report a unique case and describe the authors' method of treatment. SUMMARY OF BACKGROUND DATA: Postpneumonectomy syndrome is an uncommon complication of pneumonectomy. Many case reports describe successful treatment with insertion of prosthetic implants into the empty hemithorax to shift the mediastinum to its original position. Thoracic lordoscoliosis reportedly has contributed to pulmonary compromise, but no cases have shown its occurrence in the setting of postpneumonectomy syndrome. METHODS: The patient was observed at the National Children's Hospital in Tokyo, referred to Children's Hospital in Los Angeles, California for surgical correction, and followed in Tokyo for the next year. RESULTS: Two prosthetic implants with an injection port for further expansion were positioned in the right hemithorax to restore the mediastinum to its normal position. Anterior discectomies, partial vertebrectomies, and fusion of T5-T10 was performed concurrently. Then 5 days later, posterior spinal fusion of T1-T12 with instrumentation and bone graft were performed to correct the thoracic lordoscoliosis and increase the chest cavity space. At 1 month after the surgery, the patient was extubated after being ventilator dependent for 5 months. At the time of operation, the girl was ventilator dependent and nonambulatory, but 1 year later could participate in all activities of daily living without any oxygen supplementation. CONCLUSIONS: Postpneumonectomy syndrome can be treated successfully with prosthetic implants to restore the normal position of the mediastinum. Thoracic lordoscoliosis can complicate the syndrome and may be corrected to help restore normal pulmonary function.

Unilateral absence of the clavicle with rapidly progressive scoliosis in an 8-year-old.

We report a possible association of unilateral absence of a clavicle with rapidly progressive scoliosis. Cleidocranial dysplasia (CCD) is an autosomal dominant disorder that is characterized by defective bone formation. The clavicle, pelvis, and skull are the most commonly affected bones. A review of the literature found two cases of CCD and scoliosis. Unilateral absence of the clavicle in association with rapidly progressing scoliosis has not been previously reported. Review of the patient's charts and radiographs from age 8 to 17 years, 5 years after treatment with posterior spinal instrumentation is presented, together with a review of the literature. Our patient initially presented without any spinal deformity until age 9, when she had a 10 degree curve between C-8 and L-T. Eighteen months later, the curve progressed to 52 degrees, Risser 1. Associated anomalies include posterior-element hypoplasia of the thoracic spine and posterior fusion of C4-6. She was treated with posterior spinal instrumentation from C-8 to L-4 without complications. Correction was maintained at 5-year follow-up. There may be an association between unilateral absence of the clavicle and rapid progression of scoliosis in immature children. We hypothesize that the asymmetrical influence of the unilateral absent clavicle may have played a causative role in her rapidly progressive scoliosis.

Mild gait abnormality and leg discomfort in a child secondary to extradural ganglioneuroma.

Ganglioneuromas are benign and slow-growing tumors that most commonly originate from the sympathetic trunk. Ganglioneuromas often decrease in size and rarely require reoperation. Changes in gait or the onset of limb pain without a discernible local cause are indications for investigation of patients for possible intraspinal pathology. We report the case of a 5-year-old boy who presented with seemingly static symptoms, while the slow-growing tumor had enveloped nerve roots and caused bone destruction of the vertebrae.