Medical Central Resource
Lymphangiomyomatosis
Nusser, Christopher
11/30/96
Presentation
35 year old female with shortness of breath.
Imaging Technique
Plain X-ray
CT
Imaging Findings
Diffuse mild interstial prominence, normal to mild hyperaeration, normal heart size and pulmonary vascularity.
Diagnosis
Lymphangiomyomatosis
Discussion
Lymphangioliomyomatosis:
- Clinical:
Lymphangioleiomyomatosis is characterized by a progressive proliferation of
spindle cells which resemble immature smooth muscle in the lung parenchyma
and along lymphatic channels which dilate. Proliferation of spindle cells along
the bronchioles leads to air trapping and the formation of thin walled cysts.
The disorder progresses to end-stage lung disease secondary to
post-obstructive emphysema. The disorder occurs only in females of child
bearing age (17-50y) and is characterized by progressive interstitial lung
disease, chylous effusion (chylous ascites), and spontaneous pneumothorax.
On CXR the disorder is characterized by bibasilar coarse reticular interstitial
lung disease with normal to hyperinflated lungs. A chylous effusion is noted in
50-75% of cases and pneumothorax in 40%. The end-stage of the disorder
produces a honeycomb lung.
On HRCT there are characteristically numerous thin walled cysts (2 mm to 5
cm) which are surrounded by relatively normal lung parenchyma. The cysts
tend to enlarge as the disease progresses, are distributed diffusely throughout
the lungs, and irregular shapes are uncommon (a distinction from eosinophilic
granuloma). A slight increase in linear interstitial markings, interlobular septal
thickening, or patchy areas of ground-glass opacification can also be seen, but
findings of significant fibrosis are usually absent.
Submitted by: Christopher Nusser,Capt,USAF,MC,Wilford Hall Medical Center
Reviewed by:
