Table 2. Proposed diagnostic criteria for BHD.
Definite pulmonary BHD |
|
Probable pulmonary BHD | |
Possible pulmonary BHD | Compatible or characteristic HRCT |
Characteristic lung HRCT findings: Multiple thin-walled round, elliptical or lentiform well-defined air-filled cysts, without internal structure, in a basilar, medial and subpleural predominant distribution, with preserved or increased lung volume, and no other significant pulmonary involvement (specifically no interstitial lung disease)
Compatible HRCT findings: Thin walled cysts without the more typical elliptical shape or subpleural distribution
Failure to meet modified Gomez criteria for TSC. Other features that may exclude TSC and LAM include absence of positive serum VEGF-D, surgical lung biopsy negative for LAM, negative genetic testing for TSC, CT or MRI of the head negative for tubers, subependymal nodules or subependymal giant cell astrocytomas
Radiographic diagnosis based on fat content by MRI or CT acceptable