Florid Hypercapnic Respiratory Failure Due to Idiopathic Fibrothorax With Excellent Response to Decortication

Abstract

INTRODUCTION: Idiopathic fibrothorax may result in severe debility and is a treatment challenge. Decortication is not frequently considered as a routine component of management of fibrothorax (1). CASE PRESENTATION: The patient is a 45 year old male treated at an outside institution for nonspecific interstitial pneumonitis with azathioprine and prednisone for 8 months prior to presenting to our institution for a second opinion. His co-morbidities included morbid obesity (BMI 52), obstructive sleep apnea requiring nocturnal CPAP and chronic back pain. He never smoked. He worked retail in a car repair shop for many years. At the time of presentation PFTs revealed a severe restrictive lung pattern and reduced diffusing capacity with hypercapnic respiratory failure requiring 5 liters of oxygen with activity. Chest CT scanning showed diffuse patchy ground glass and bilateral pleural scattered calcifications. He underwent BAL initially and infection was ruled out and then VATS assisted biopsy of the lung with pathology showing only mild pleural fibrosis and normal lung structure. He became more hypercapnic and required tracheostomy with nocturnal ventilator support with oxygen continuously. His hypercapnia stabilized in the mid 70s. He was assessed for surgical intervention by transplant surgery and they deemed him not a candidate based on his weight. After 120 lbs weight loss assisted with gastric sleeve surgery, no significant improvement in his respiratory failure was evident. Repeat imaging of the chest at this point clearly revealed diffusion pleural thickening. Serologic work up was negative but his back disease was deemed seronegative spondyloarthopathy by Rheumatology. He then underwent sequential bilateral decortication procedures. Three months after the procedures he had considerable improvement in functional status, pulmonary functions, arterial blood gases and almost complete resolution of his sleep disordered breathing, and he was liberated from oxygen. After more than a year of follow-up he remains off oxygen, pulmonary function tests and exercise capacity steadily improve with each visit and his hypercapnia has resolved. He retains mild OSA which requires CPAP at night. DISCUSSIONS: We believe that this represents a case of idiopathic fibrothorax in a morbidly obese patient where treatment with pleural decortication resulted in a significant improvement in lung function and the overall health status, which was not fully appreciated until his profound weight loss alone did not correct the hypercapnia. There are only rare reports of treatment with decortication for idiopathic fibrosis (2). CONCLUSION: Our experience shows that pleural decortication is a viable option in treating idiopathic fibrothorax.