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Case 9

Lymphangioleiomyomatosis

A young female with chronic breathlessness

This 34 year old female had a long history of worsening breathlessness. Her chest X-ray demonstrated severe reticular shadowing, but with relative preservation of lung volumes. This high resolution CT scan was obtained prior to consideration for a lung transplant.

Describe the abnormalities. What is the diagnosis?

Findings

There are multiple well defined cystic spaces, 2-20mm, throughout both lungs. There is a pneumothorax on the right with a chest drain in situ (arrow). No pleural effusion was seen.

Diagnosis

Lymphangioleiomyomatosis
Pneumothorax

Differential

Tuberous sclerosis
Histiocytosis X (see below)
Neurofibromatosis
Alpha-1 antitrypsin deficiency

Discussion

This is a rare disorder confined to women of child bearing age. It resembles closely the lung changes in tuberous sclerosis, and may be a form fruste of that condition.

Presentation is with breathlessness, and spontaneous pneumothorax is particularly common.

In early disease it resembles cryptogenic fibrosing alveolitis, but with preservation of lung volume (due to air trapping). There is generalised, or predominantly basal, coarse reticulonodular shadowing. The late appearance is of honeycombing. CT shows well defined thin walled cysts, 2mm-50mm in size. Mediastinal lymphadenopathy may be present. Pleural effusions are common, large, recurrent and chylous in origin. Lung function tests give an obstructive picture.

Pathology

Macroscopically the cut lung shows cysts with thickened interstitial tissue. Microscopically the hallmark is smooth muscle proliferation in the interstitium, which it is postulated causes bronchiolitis, lymphatic and venous obstruction, and hence many of the typical findings.

Histiocytosis X

Compare with this example of Histiocytosis X, albeit less severe. Note that Histiocytosis X tends to spare the lung bases, the cysts have thicker walls, and there is a nodularity to the lung parenchyma due to granulomas, especially at the apices.

References

Fraser, Paré, Paré, Fraser, Genereux. Diagnosis of diseases of the chest, 3rd Ed. 1991. Vol IV, p.2672-7.

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