A Challenging Case of PTLD-Related Broncho-Esophageal Fistula After Lung Transplantation

Abstract

<h3>Introduction</h3> Post-transplant lymphoproliferative disease (PTLD) is a complication often encountered after lung transplantation (LTx). We present a case where necrotic subcarinal lymph nodes (LNs) affected by PTLD caused a large broncho-esophageal fistula, for which all treatment options were exhausted. <h3>Case Report</h3> A 64-year-old LTxrecipient presented with a 1.5-month history of fever and general weakness, 11 years after bilateral LTx for IPF and 4 years after redo LTx for BOS. PET/CT revealed hypermetabolic subcarinal, paratracheal, supraclavicular and infra-diaphragmatic LNs (image A). EBV-associated diffuse large B-cell lymphoma (DLBCL)-PTLD was confirmed on supraclavicular LN biopsy. The patient had to be intubated for respiratory failure due to pneumonia, prior to initiating PTLD-specific treatment. An underlying bilateralbroncho-esophageal fistula was diagnosed by chest computed tomography (CT) (image B) and confirmed with esophagoscopy and bronchoscopy. Treatment of the DLBCL consisted of reduction of tacrolimus, discontinuation of mycophenolate mofetil, steroids, rituximab, and cyclophosphamide. Esophagostomyand gastrostomy were performed to prevent airway aspiration. Despite broad-spectrum antibiotics and antifungals, the patient developed neutropenic sepsis due to respiratory infection as well as probable invasive pulmonary aspergillosis. Twenty days after surgical intervention, chest CT (image C) and bronchoscopy (image D) showed that the posterior side of the airway below the carina was completely eroded. No therapeutic options remained, and the patient passed away. <h3>Summary</h3> Management of a broncho-esophageal fistula resulting from PTLD following LTx is challenging. Due to erosion of necrotic subcarinal LNs, surgical intervention was performed to prevent ongoing infection precipitated by PTLD-specific treatment.