Case descriptions
Four patients underwent surgical procedures for TALS.
All the patients had history of hematological malignancies diagnosed during adolescence. All the patients received allogenic HSCT; two of them received a second allogenic HSCT for relapsed disease.
All the patients developed pulmonary graft-versus-host disease (pGvHD) as a late complication of allogenic HSCT, and presented with exertional dyspnea and dry cough, radiologic evidence of air trapping, bilateral ground glass lesions and bronchiectasis (Fig 1) and restrictive or mixed restrictive/obstructive pattern at pulmonary function tests.
Pulmonary function tests showed progressive reduction of forced vital capacity (FVC), forced expiratory volume (FEV1) and forced expiratory flow (FEF 25-75%).
All these patients had associated comorbidities, including extra-pulmonary GvHD, malnutrition, defined as age- and sex-adjusted body mass index below 17.0 10, and cardiac dysfunction.
Clinical characteristics and pulmonary function tests of these patients are summarized in table 1 and table 2 in the Supporting Information, respectively.
Surgery was indicated as an emergency in case of respiratory distress with radiological evidence of tension pneumothorax, or as an elective procedure in case of failure of initial treatment (Fig 2).
Patient One had two episodes of TALS that were managed conservatively and underwent emergency right tube thoracostomy at the third episode for acute respiratory distress and evidence of tension pneumothorax; this patient rapidly worsened towards respiratory failure, was admitted to Intensive Care Unit and passed away 25 days after emergency tube thoracostomy.
Patient Two underwent emergency left tube thoracostomy for respiratory distress and tension pneumothorax at the second episode of TALS. This patient had persistent pneumothorax after 24 days of negative pressure chest drain and underwent left thoracotomy and wedge resection. This patient had contralateral tension pneumothorax that required emergency chest drain insertion and, 30 days later, thoracoscopy and pleural scarification. Pulmonary function progressively worsened with the development of chronic respiratory failure. This patient was referred for pulmonary transplant but was judged non-eligible due to history of recent hematologic malignancy, previous thoracic surgery, ventricular systolic dysfunction and malnutrition. This patient had right tension hydropneumothorax five months later that required emergency chest drain; general conditions progressively deteriorated and the patient eventually passed away for respiratory failure.
Patient Three underwent elective right thoracoscopy and chemical pleurodesis at the second episode of TALS after failure of conservative management. This patient underwent contralateral thoracoscopic bullectomy and chemical pleurodesis one and half months after initial surgery, followed by thoracotomy and wedge resection for persistent left pneumothorax after 10 days. This patient had left tension pneumothorax 40 days after thoracotomy that required emergency chest drain; respiratory function rapidly deteriorated and the patient died 12 days after the last episode of TALS.
Patient Four underwent elective right thoracoscopy and chemical pleurodesis at the third episode of TALS after failure of conservative management. This patient had contralateral pneumothorax 14 months after surgery and two more episodes of TALS, all managed conservatively. Respiratory failure slowly developed; the patient was referred for pulmonary transplant but was judged non-eligible due to history of recent hematologic malignancy, previous thoracic surgery, ventricular systolic dysfunction and malnutrition. The patient ultimately died for respiratory failure two years after surgery.
Surgical procedures and outcomes are summarized in table3 and table 4 in the Supporting Information, respectively.