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Thoracic Imaging |
1 Departments of Radiology (J.C.)
2 Cardiothoracic Surgery (R.B.L.), University of Wisconsin Hospital and Clinics, E3/311 CSC, 600 Highland Ave, Madison, WI 53792-3252
3 Department of Radiology, University of British Columbia Hospital and Health Sciences Centre, Vancouver, Canada (N.L.M.)
4 Department of Radiology, University of Michigan Medical Center, Ann Arbor (E.A.K.)
5 Department of Radiology, Duke University Medical Center, Durham, NC (H.P.M.)
6 Department of Radiology, Stanford University Medical Center, Stanford, Calif (A.N.L.).
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Abstract |
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MATERIALS AND METHODS: The authors retrospectively reviewed the clinical histories and imaging studies of 13 patients from five major medical centers who underwent unilateral (n = 8) or bilateral (n = 5) lung transplantation for LAM between 1991 and 1997. Complications related to LAM, both before and after transplantation, were recorded.
RESULTS: The following LAM-related complications were found during and after transplantation: excessive pleural adhesions (n = 4), native lung pneumothorax (n = 3), chylous effusion (n = 1), chylous ascites (n = 3), complications from renal angiomyolipomas (n = 4), and recurrent LAM (n = 1). Diagnosis could be made or suggested with computed tomography (CT) in all cases. Four patients (31%) died; one patient died of complications of LAM.
CONCLUSION: Patients who have undergone lung transplantation for LAM have increased morbidity and mortality due to complications related to their underlying disease. These LAM-related complications can be diagnosed or suggested with CT.
Index terms: Lung, CT, 60.12115, 60.12118 • Lung, transplantation, 60.458 • Lymphangiomyomatosis, 94.829 • Lymphatic system, 993.39
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Introduction |
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LAM is believed to be hormonally mediated, and therapies have been directed toward reducing circulating estrogen levels. Even with treatment, the survival rates in patients with LAM from two reported series were 78% and 38% at 8.5 years (3,4). Lung transplantation has recently become an option for patients with end-stage LAM, with 73 patients recorded in the St Louis International Registry as of 1996 (5). In addition to infection, rejection, airway anastomotic complications, and lymphoproliferative disease, patients undergoing lung transplantation for LAM are also susceptible to the development of complications related to their underlying disease. We performed a retrospective review of the clinical course and imaging studies of all patients who underwent lung transplantation for LAM at five major lung transplantation medical centers to determine the type and frequency of complications related specifically to LAM and the imaging features of these complications.
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MATERIALS AND METHODS |
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Review of Clinical Course
The patients' medical records were reviewed to determine the pretransplantation frequency of complications related to LAM (eg, hemoptysis, chylous ascites, spontaneous pneumothorax, and chylous pleural effusion) and all medical or surgical interventions performed to treat LAM.
The frequency of complications related to lung transplantation in general, including acute and chronic rejection, infection, airway anastomotic complications, and lymphoproliferative disease, was recorded. In addition, the frequency of posttransplantation complications related to LAM, including excessive pleural adhesions and whether these adhesions resulted in excessive bleeding at surgery, pneumothorax of the native lung in single lung transplant recipients, chylous effusion, chylous ascites, hemorrhage or other complications related to renal AML, and recurrent LAM, was noted. The immunosuppressive protocol for each patient was recorded.
Pulmonary Function Tests
Pulmonary function tests were performed in all patients. Spirometry was performed to measure FEV1 (forced expiratory volume in 1 second), FVC (forced vital capacity), FEV1/FVC (ratio of FEV1 to FVC), and TLC (total lung capacity) with equipment and procedures meeting the standards recommended by the American Thoracic Society (8). The single-breath diffusing capacity of the lungs for carbon monoxide (DLCO) was measured with the single-breath technique of Ogilvie et al (9) as modified by Jones and Meade (10). To correct for the influence of age, sex, and height, all values are expressed as absolute and percentages of predicted values. Pulmonary function tests were performed (a) just prior to transplantation, (b) after transplantation at peak functioning, and (c) most recently after transplantation.
Image Analysis
All computed tomographic (CT) scans of the chest were reviewed for the presence of parenchymal disease, pleural effusion, pneumothorax, adenopathy, and bronchial anastomotic complications. CT was performed with a variety of scanners by using a variety of protocols, including thin-section CT at 1.0- or 1.5-mm collimation, helical CT at 8.0- or 10.0-mm collimation, and with or without the use of intravenous contrast material. To view pulmonary parenchyma, window width was varied from 1,300 to 2,000 HU and level from -550 to -700 HU. To view mediastinal structures, window width was varied from 350 to 450 HU and level from 34 to 40 HU. Chest radiographs obtained at the same time as the CT scans were evaluated for parenchymal disease, effusions, and pneumothorax.
CT scans of the abdomen and pelvis were reviewed for the presence of renal AML and associated hemorrhage, enlarged abdominal and retroperitoneal lymphatic vessels, and ascites.
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RESULTS |
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Review of Clinical Course and Image Analysis
Pretransplantation complications related to LAM included hemoptysis (n = 3), chylous ascites (n = 1), spontaneous pneumothorax (n = 10) (Fig 1), and chylous effusion (n = 3). One patient underwent shunt placement for the treatment of chylous ascites. Two patients required multiple thoracenteses and one required pleurodesis to treat chylous effusions. Twenty-five pneumothoraces occurred in 10 patients and involved the right lung in 12 cases and the left lung in 11. The side of the pneumothorax was not recorded in two patients. Seven of the 10 patients required an interventional procedure for the treatment of pneumothoraces; thoracotomy was performed in five patients and pleurodesis in seven.
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years, cerebral vascular accident at transplantation, paralyzed diaphragm, renal failure with uremic pericarditis at 14 months, cyclosporine renal toxicity, migraine headaches, spontaneous colon perforation related to steroids, and chronic ventilator dependency at 4 months after transplantation.
At the time of our review, nine patients were alive and four had died. The cause of death in one patient was related to LAM. This patient, who had multiple bilateral renal AMLs and renal colic, died of renal insufficiency 4 years 7 months after transplantation. The other three deaths were a result of multiple organ failure 4 years after transplantation in one patient, aspiration of pills (time after transplantation was not recorded) in one, and exsanguination in one. The patient with exsanguination had chronic necrotizing pulmonary Aspergillus infection and developed a 0.6-cm fistula between the superior left upper lobe pulmonary artery and the left upper lobe bronchus 5 months after transplantation. This led to hemorrhage and exsanguination as a cause of death.
Pulmonary Function Tests
The results of the pulmonary function tests are listed in the Table. All measured indexes improved after transplantation and subsequently decreased; however, the indexes in all but six patients remained improved compared with pretransplantation values. All but two patients had an obstructive pattern, which is defined as TLC of at least 80% of the predicted value and FEV1/FVC of less than 75%, on pulmonary function tests performed before transplantation (12). Recorded pretransplantation TLC values were 66%–168% of the predicted values. Pretransplantation FEV1 values ranged from 0.21 (8% of the predicted value) to 1.80 (60% of the predicted value), and FEV1/FVC values ranged from 0.20 to 0.83 (predicted percentages for patients with these values were not recorded). Recorded FEV1/FVC values were 28%–80% of the predicted values. This compares with the percentage of predicted values from another study (12), in which the percentage predicted values for 26 of 32 patients with LAM before transplantation were 24% ± 12% (range, 13%–66%) for FEV1 and 38% ± 12% (range, 20%–61%) for FEV1/FVC.
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DISCUSSION |
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TOPAbstractIntroductionMATERIALS AND METHODSRESULTSDISCUSSIONReferences |
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Reported complications related to LAM after lung transplantation include excessive bleeding from pleural adhesions, pneumothorax of the native lung, chylothorax, chylous ascites, hemorrhage from renal AML, and recurrence of the primary disease (17). All of these complications occurred in at least one of our patients.
Of four patients who were found to have excessive pleural adhesions at surgery, excessive bleeding required postoperative emergent exploratory surgery in one patient. Extensive pleural adhesions are said to represent the main intraoperative problem in patients with LAM (12). In a study of 34 patients with LAM who underwent lung transplantation (12), 18 patients had substantial adhesions. These lesions were moderate in eight patients and severe in 10. The adhesions were due to LAM in 13 patients and to LAM and prior pleurectomy after spontaneous pneumothorax in five. Moderate to severe hemorrhage occurred in four patients, leading to intraoperative death in one patient and repeat thoracotomy in two patients.
The most important posttransplantation complication in patients with LAM is reported to be pneumothorax in the native lung after single-lung transplantation; this occurred in five of 34 patients (15%) in one study (12). Most patients with LAM have received instrumentation of the thoracic cavity or pleurodesis, and have had, and remain at high risk for, pneumothorax (2). This increases both the intraoperative and postoperative risk. Hemodynamic instability due to dynamic hyperinflation and intraoperative pneumothorax have been reported, and, in the latter case, institution of cardiopulmonary bypass was required (18). Three of our 13 patients (23%) developed four episodes of persistent native lung pneumothorax, which is a higher rate than that seen in 70 lung transplant recipients with a variety of lung diseases (4%) (19). One of our patients had a tension pneumothorax, which has been reported to be a cause of death after lung transplantation (20).
Ipsilateral pleural fluid occurs in all lung transplant recipients, beginning immediately after transplantation and continuing for up to 9 days (21). An increase in pleural fluid output or failure of resolution of the effusion by day 9 has been suggested as a sign of pulmonary reimplantation response, infection, or acute rejection (21). When persistent pleural effusions are seen in patients after lung transplantation for LAM, chylothorax related to LAM should be considered. One of our 13 patients (8%) developed chylous effusions after transplantation, compared with three of 34 patients with LAM (9%) in a previous study (12). Other patients, and our one patient, required thoracic duct ligation and tube thoracostomy as treatment (12). An important point regarding the complication of chylothorax is that bilateral lung transplantation will not prevent patients from developing chylothorax because the mediastinal involvement, if present, cannot be cured irrespective of the surgical procedure adopted (22).
Three of our 13 patients (23%) developed chylous ascites, which, to our knowledge, has not been reported as a complication in other studies of patients undergoing transplantation for LAM. Chylous ascites has been reported in one of 32 patients (3%) with LAM who did not undergo lung transplantation (17). In one of our patients, recurrent and large-volume chylous ascites required placement of two peritoneal-subclavian vein shunts, multiple paracenteses, and radiation therapy of lymphatic vessels. The ascitic fluid became infected with Torulopsis species, a fungal agent, which necessitated removal of all indwelling medical devices.
Five of our 13 patients (38%) had renal AMLs and four (31%) had complications related to renal AML, including retroperitoneal hemorrhage, renal colic, and renal failure. Two patients required nephrectomy. Renal AMLs, hamartomatous lesions composed of smooth muscle, adipose tissue, and blood vessels, are radiographically present in almost 50% of patients with LAM (2). Edelman and Kotloff (17) reported one episode of retroperitoneal hemorrhage from a renal AML in a patient who underwent transplantation for LAM, which was precipitated by the need to administer an anticoagulant for a thrombus at the left atrial suture line. In one of our patients with multiple AMLs and renal colic, renal insufficiency led to the patient's death.
As with sarcoidosis (23,24), giant cell pneumonia (25), and diffuse panbronchiolitis (26), LAM can recur after transplantation, a complication that has been reported in two patients (27,28). Both patients developed parenchymal LAM after unilateral transplantation from male donors. The recurrence of this disease raises interesting questions regarding the etiology of LAM. Because LAM occurs almost exclusively in premenopausal female patients, the use of male donors should preclude the development of recurrence from the donor itself. LAM, however, has been reported in women as old as 72 years, in a man, and a male bottlenose dolphin (2). Hypotheses regarding the etiology of the recurrence of LAM include (a) metastatic spread of benign smooth muscle proliferation of pulmonary vasculature (29), (b) direct spread through mediastinal lymph nodes from the nontransplanted lung, and (c) a circulating or secretory stimulating substance produced within the host (recipient) (27). It has been suggested that bilateral transplantation for LAM should be considered to eliminate the possibility of recurrence (27); however, one of our patients who underwent bilateral transplantation developed recurrence in a retroperitoneal lymph node site. Enlarged lymph nodes in patients after lung transplantation are suggestive of neoplasm (including lymphoproliferative disease) or infection. In patients who have undergone transplantation for LAM, enlarged lymph nodes should suggest the possibility of recurrent LAM. Mediastinal or hilar nodal enlargement may also represent recurrent sarcoidosis in the appropriate patient population. Despite its questionable beneficial effect, hormonal therapy has been recommended after transplantation for LAM in an attempt to retard or prevent disease recurrence in the allograft (28). An important consideration regarding the recurrence of LAM is the potential problem of differentiating deteriorating pulmonary function resulting from bronchiolitis obliterans (considered to represent chronic rejection) from that resulting from LAM. Both produce an obstructive pattern on pulmonary function tests.
Our study has several limitations, including the small number of patients, incomplete medical records, and differences among the five medical centers in pre- and posttransplantation protocols regarding pulmonary function tests, drug therapy, and diagnostic imaging. Chest CT was performed with a variety of protocols, and, although this was unlikely to result in an inability to diagnose chylous effusion or pneumothorax, parenchymal cysts indicating recurrent LAM may not be visualized without high-resolution CT and adenopathy may not be appreciated on unenhanced scans. In general, patients did not undergo CT unless they were symptomatic. For this reason, some abnormalities may have gone undiagnosed. Even massive pneumothorax can occur and not be clinically recognized, as occurred with one of our patients.
In summary, patients with LAM have increased morbidity owing to the unique complications related to their underlying disease. These complications include intraoperative bleeding due to pleural adhesions, native lung pneumothorax, chylous effusion, ascites, and the recurrence of LAM both within the transplanted lung parenchyma and at nodal sites. Chest CT is superior to chest radiography in the detection of pneumothorax, small effusions, parenchymal changes of LAM, and lymph node enlargement. It remains to be seen whether preoperative CT of the chest will be useful when screening for extensive pleural adhesions and whether this information will aid the surgeon and result in decreased morbidity from excessive intraoperative bleeding. Increased mortality is seen in patients who have undergone transplantation for LAM as a result of massive hemorrhage from pleural adhesions and renal failure due to renal AMLs.
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Acknowledgments |
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Footnotes |
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Abbreviations: ALM = angiomyolipoma DLCO = diffusing capacity of lung for carbon monoxide FEV1 = forced expiratory volume in 1 second FVC = forced vital capacity LAM = lymphangioleiomyomatosis TLC = total lung capacity
Author contributions: Guarantor of integrity of entire study, J.C.; study concepts, J.C., R.B.L.; definition of intellectual content, J.C., N.M.L.; literature research, J.C., E.A.K.; data acquisition, J.C., N.L.M., E.A.K., H.P.M., A.N.L., R.B.L.; data analysis, J.C.; manuscript preparation, J.C.; manuscript editing, J.C., N.L.M., E.A.K., H.P.M., A.N.L.; manuscript review, J.C., N.L.M., E.A.K., H.P.M., A.N.L., R.B.L.
Received April 21, 1998;
revision requested July 2, 1998; revision received July 16, 1998;
accepted September 28, 1998.
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TOPAbstractIntroductionMATERIALS AND METHODSRESULTSDISCUSSIONReferences |
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