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Recurrent lymphangioleiomyomatosis

Imaging findings

Serial chest CT scans post-double lung transplant show the development of small, thin-walled pulmonary cysts in the right allograft representing recurrent lymphangioleiomyomatosis, along with progressive upper-lobe-predominant consolidative scarring, peri-bronchovascular nodularity, and air trapping in the left lung consistent with restrictive allograft syndrome.

Key takeaways

Lymphangioleiomyomatosis can recur in the transplanted lungs, typically presenting as slowly developing thin-walled cysts. Chronic allograft dysfunction can also manifest as restrictive allograft syndrome (characterized by upper-lobe fibrosis and elastosis) and bronchiolitis obliterans.

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