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.