| Key
Facts |
- Women of child-bearing
age
- Chest radiography:
paradoxical reticular interstitial disease with increased lung volumes
- CT: numerous thin-walled
cysts with intervening normal lung
- Often present with
spontaneous pneumothorax
- Most eventually
develop pleural or pericardial chylous effusion
- Associated findings
include mediastinal and retroperitoneal adenopathy
- Renal angiomyolipoma
(15%)
|
| Imaging
Findings |
Best
Imaging Clue
- Coarse interstitial
thickening with hyperinflated lungs
Chest radiography
- Reticular interstitial
thickening (coarse honeycomging)
- Normal or enlarged
lung volume
- Paradoxical
observation as interstitial lung disease (ILD) is restrictive and
decreases lung volumes
- Small to moderate
pleural effusions
- Spontaneous
pneumothorax (40%)
CT/HRCT
- Reticular thickening
on chest radiography represents superimposed cysts
- Thin-walled cysts
uniform in size, increase in size and number as disease progresses
- cysts will eventually
completely replace lung
- Diffuse distribution,
no predilection for any region of the lung
- Intervening lung
normal
- CT may demonstrate
cysts when chest radiograph and PFT’s normal
- Pleural or pericardial
effusion (chylous, 60%)
- Mediastinal and
retroperitoneal adenopathy
- Renal angiomyolipoma
(15%)
- Scattered ground-glass
opacities (may represent hemorrhage)
|
| Differential
Diagnosis |
- Langerhans cell
histiocytosis
- Emphysema
- Neurofibromatosis
|
| Pathological
Features |
- Hamartomatous proliferation
of smooth muscle around lymphatics, airways, and blood vessels
- Identical pathologic
findings found in 1-2% of patients with tuberous sclerosis
- Predilection for
premenopausal women suggests estrogen plays a role in pathogenesis
- Cysts uniformly
distributed throughout the lung
|
| Clinical
Presentation |
- Present with dyspnea
and pneumothorax
- hemoptysis 30%
- Treatment
- discourage
air travel (increased risk pneumothorax)
- pregnancy may
exacerbate disease
- progesterone
and oophorectomy (variable success)
- lung transplant
(may recur in transplanted lung)
- pleurodesis
for effusions or pneumothorax may worsen pulmonary function
- Prognosis:
50% five year survival after diagnosis
|
| References |
Sullivan
EJ. Lymphangioleiomyomatosis: A review. Chest 114: 1689-703, 1998.
|
