INTRODUCTION: Severe scoliosis distorts thoracic anatomy and produce marked asymmetry within the chest cavities. These characteristics contribute to concerns for donor-to-recipient size mismatch in lung transplantation. For this reason, some patients with severe scoliosis end stage lung disease are denied lung transplantation (LTx). Two cases of patients with severe scoliosis who underwent successful double LTx are presented.
CASE PRESENTATION: Our first patient was a 53 year old female who developed end stage interstitial lung disease secondary to sarcoidosis. Work-up showed a normal forced expiratory volume in 1 second percent of predicted (FEV1%) 74%, forced vital capacity percent of predicted (FVC%) 79% and diffusion lung capacity for carbon monoxide (DLCO) 19% of predicted. The patient desaturated to 88% using 6 liters of oxygen by nasal cannula (NC). A quantitative ventilation-perfusion (VQ) scan demonstrated a right to left distribution of 70% and 30%, respectively. Pulmonary artery systolic pressure (PASP) was 70 mmHg. She had severe scoliosis with a Cobb angle of 80 degrees (Figure 1A). Due to her pulmonary hypertension, double LTx was recommended. Double LTx was performed with an unremarkable hospital course. She was discharged on postoperative day 10. Nine months after surgery, her chest radiograph was clear (Figure 1B). With an FEV1% of 68%, she required no supplemental oxygen, did not desaturate and reported an excellent quality of life. Our second patient was a 46 year old female who developed end-stage bronchiectasis due to congenital primary ciliary dyskinesia. Work-up showed FEV1% of 29% and FVC% of 32%. The patient was unable to tolerate DLCO measurement. With 6L of oxygen by NC, she desaturated on exertion to 90%. VQ scan demonstrated a right to left distribution of 73% and 27%, respectively. PASP was normal. She also had severe scoliosis with a Cobb angle of 72 degrees (Figure 2A). Due to her recurrent respiratory infections and bilateral bronchiectasis, double LTx was recommended. The postoperative course was complicated by myopathy, reoperation for bleeding and prolonged airleak. The patient was discharged on postoperative day 30. Eight months after surgery, her chest radiograph was clear (figure 2B), FEV1% 36% and no supplemental oxygen is required. Bronchoscopy demonstrated moderate, right mainstem stenosis which has not required any intervention.
DISCUSSIONS: Severe scoliosis is considered a technical contraindication to lung transplantation. Asymmetry of the hemithoraces is thought to prohibit appropriate graft size matching and chest wall distortion may cause poor functional outcome. This, however, was not our experience. No significant technical problems were encountered for either patient during lung extraction or implantation. Interestingly, the second patient developed a complication directly related to her scoliosis: right-sided airway narrowing secondary to dynamic airway compression against her scoliotic vertebral body. This seemed to negatively impact her FEV1% which is less than expected. Fortunately, this has not required intervention and the patient reports a good quality of life.
CONCLUSION: We conclude severe scoliosis alone should not preclude patients from double lung transplantation. Although surgically challenging, double LTx in patients with severe scoliosis is possible with outcomes similar to non-scoliosis patients.
DISCLOSURE: Jang Wen Su, No Financial Disclosure Information; No Product/Research Disclosure Information