Breath Stacked Odds: Phrenic Neuropathy in a Single Lung Transplant Recipient with Scoliosis, A Case Report

Case Diagnosis: Phrenic neuropathy (PhN) and diaphragm dysfunction following lung transplant (LTx) is a known complication (6-41%) associated with prolonged intensive level care and ventilator weaning failure with unclear impact on LTx rehabilitation.

Case Description or Program Description: 70-year-old male with chronic hypercapnic respiratory failure from thoracic restriction related to scoliosis and end-stage hypoxemic respiratory failure from emphysema underwent single LTx. Postoperatively, he developed diaphragmatic hypomobility from PhN leading to diminished transplanted lung function, native emphysematous lung hyperinflation, and exacerbation of baseline thoracic restriction contributing to tracheostomy placement and tenuous respiratory status. Deconditioned from his 3-month hospitalization, the patient initially required maximum assistance (maxA) with self-care and transfers. In addition to managing various problems (e.g., pain, immunosuppression, renal dysfunction), the medical team notably optimized sleep and anxiety, which improved nocturnal noninvasive positive pressure ventilation (NPPV) adherence, and, in turn, enhanced overall endurance and respiratory muscle strength. Close coordination with transplant medicine, pulmonology, and neuromuscular medicine was critical in advancing the patient's respiratory function.

Setting: Inpatient rehabilitation (IPR)

Assessment/Results: Diaphragmatic recovery was evaluated via diaphragmatic thickness ratio on ultrasound. Despite no objective change, the patient demonstrated significant clinical improvement: he was decannulated, had improved NPPV adherence, and demonstrated self-care improvement from maxA to minimal assistance and mobility improvement from maxA to supervision. Ultimately, he met goals for discharge to home with family support.

Discussion (relevance): Given the increasing number of LTx annually, physiatrists will undoubtedly encounter the common complications of these complex patients. This is a case of significant functional improvement in a single LTx patient with severe restrictive and obstructive lung disease further complicated by PhN.

Conclusions: In addition to standard transplant risks, LTx recipients are at a further risk of postoperative complications (including PhN), debility, critical illness neuromyopathy, and ongoing pathologies in their non-transplanted lung. Physiatrists must be equipped to effectively manage and coordinate the interdisciplinary care for this growing population.