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Sep
The currently proposed diagnostic algorithm emphasizes the. To identify the complications and imaging findings related to lymphangioleiomyomatosis LAM after lung transplantation.
The aim of this study was to determine whether a CT-based method shows lung lesions grades disease severity and evaluates lung tissue in areas adjacent to or remote from cysts in patients with lymphangioleiomyomatosis LAM a cystic lung disease.
Lam lung disease radiology. Lymphangioleiomyomatosis LAM is a rare disease characterized by pulmonary cysts at computed tomography CT and proliferation of abnormal smooth muscle cells at lung biopsy. Almost all patients are female and all have pulmonary cysts at high-resolution CT. LAM is characterized pathologically by abnormal proliferation of LAM cells in the lungs and in thoracic and retroperitoneal lymphatics.
Thirty-three cases of LAM were reviewed retrospectively for clinical and radiologic findings. Twenty-eight 85 of 33 women aged 2162 years. Mean 375 years were symptomatic.
Tive imaging technique for diagnosing LAM. The presence of thin-walled cysts uniformly spread throughout the lung is a well-charac-terized feature of pulmonary disease 1 2 5. Several radiologic studiesincluding meth-ods for determining a radiologic score 5 the percentage of pixels with an attenuation of less.
Although historically LAM has been considered an interstitial lung disease it is now considered to be a low-grade destructive metastasizing neoplasm. Typical radiographic findings of. Lymphangioleiomyomatosis LAM is a rare destructive lung disease characterized by multiple thin-walled pulmonary cysts.
The currently proposed diagnostic algorithm emphasizes the. High-resolution CT HRCT scan is the most accurate imaging test for diagnosing LAM providing a correct diagnosis more than 80 of the time. It provides a detailed 2D image of the inside of the lungs and chest revealing cysts or abnormal clusters of cells in the lungs and whether cysts have spread a collapsed lung andor enlarged lymph nodes.
Imaging description Lymphangioleiomyomatosis LAM is characterized on CT imaging by diffuse bilateral thin-walled cysts without a zonal predominance Figures 111 and 112 13. LAM can affect just the lungs but it can also be a systemic disease. Findings of pulmonary LAM are also seen associated with tuberous sclerosis.
The HRCT appearance of pulmonary sarcoidosis varies greatly and is known to mimic many other diffuse infiltrative lung diseases. Approximately 60 to 70 of patients with sarcoidosis have characteristic radiologic findings. In 25 to 30 of cases the radiologic findings are atypical.
In 5 to 10 of patients the chest radiograph is normal. LAM is usually suggested when lungs cysts are seen on a CT scan or in women with tuberous sclerosis. As there are other causes of lung cysts you may also have blood tests or a lung biopsy.
If LAM is suspected you should also have a CT scan of the abdomen to look for kidney angiomyolipomas and lymphatic problems. How is LAM treated. Conditional Low Transbronchial lung biopsy for histopathological diagnosis of LAM When a definitive diagnosis is required in patients who have parenchymal cysts on HRCT that are characteristic of LAM but no additional confirmatory features of LAM ie clinical radiologic or serologic we suggest a diagnostic approach that.
The aim of this study was to determine whether a CT-based method shows lung lesions grades disease severity and evaluates lung tissue in areas adjacent to or remote from cysts in patients with lymphangioleiomyomatosis LAM a cystic lung disease. To identify the complications and imaging findings related to lymphangioleiomyomatosis LAM after lung transplantation. 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.