June 2025 Medical Image of the Month: Neurofibromatosis-Associated Diffuse Cystic Lung Disease

By: Ali A

Abstract: 

No abstract available. Article truncated after 150 words. A 61-year-old man with a history of neurofibromatosis type 1 (NF1) and NF1-associated cystic lung disease presented for a routine follow-up visit 5 years post-bilateral lung transplantation.  The patient’s physical examination revealed multiple cutaneous neurofibromas, consistent with his diagnosis of NF1 (Figure 1).  Additionally, he had a prior tracheostomy scar; he temporarily required tracheostomy post lung transplant surgery.

Pre-Transplant History:

The patient had progressive chronic hypoxic respiratory failure, requiring home oxygen supplementation at up to 8 L/min. His medical history included a 15-pack-year smoking history, though he quit smoking 16 years prior to his lung transplant.  His diagnostic workup prior to transplant consisted of a high-resolution computed tomography (HRCT) of the chest, which  revealed diffuse cystic lung disease with intervening ground-glass opacities (Figure 2), an echocardiogram, which demonstrated severe pulmonary hypertension, and pulmonary function tests (PFTs), which  showed a combined restrictive and obstructive pattern with severely reduced DLCO at 25%. …

URL: https://www.swjpcc.com/imaging/2025/6/2/june-2025-medical-image-of-the-month-neurofibromatosis-assoc.html