Case 14
History:
25 year old female with history of recurrent pneumothorax
Findings:
Multiple well-defined cystic spaces throughout both lungs. No pleural effusion was seen.
Diagnosis:
Lymphangioleiomyomatosis (LAM)
Discussion:
Lymphangioleiomyomatosis (LAM) is a rare disorder resulting from proliferation of smooth muscle cells along lymphatics in lung, thorax and abdomen. The etiology of the disease is unknown. The disorder affects young patients with female predominance. It presents with spontaneous pneumothorax, chylothorax, hemoptysis, and slowly progressive dyspnea. Similar lesions may be seen in tuberous sclerosis (Some suggest LAM is a forme frust of tuberous sclerosis). Extrapulmonary LAM is rare.
Radiographically presents with numerous thin walled cysts, 90 percent with size of cysts < 5-10 mm. Recurrent pneumothotax, 70 percent. Chylous pleural effusions, 25 percent.
References:
- Alberle DR, Hansell DM, Brown K, Tashkin DP: Lymphangioleiomyomatosis: CT, chest radiography, and functional correlations. Radiology 176:381-387, 1990.
