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Robotic-assisted Left Pneumonectomy For Vanishing Lung Syndrome
Published on: January 23, 2026
314
[A rare form of obstructive pulmonary disease]
C J van Loenhout1, M A den Bakker, M S van Wijsenbeek
1Erasmus MC, Rotterdam.
Nederlands Tijdschrift Voor Geneeskunde
|January 12, 2017
Summary
Lymphangioleiomyomatosis (LAM) is a rare lung disease causing shortness of breath and lung destruction. High-resolution CT scans showing diffuse cysts can suggest LAM, especially in women, prompting further investigation.
Area of Science:
- Pulmonology
- Radiology
- Pathology
Background:
- Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease.
- LAM presents with dyspnea, spontaneous pneumothorax, and cystic lung destruction.
- Clinical and radiological features of LAM can mimic emphysema.
Observation:
- A 44-year-old woman with a 6-year history of dyspnea and severe obstructive pulmonary function disorder was evaluated for lung transplantation.
- Her presentation, including young age and non-smoking status, raised doubts about a Chronic Obstructive Pulmonary Disease (COPD) diagnosis.
- Pulmonary cysts identified on high-resolution computed tomography (HRCT) suggested LAM, later confirmed by reviewing a prior lung biopsy.
Findings:
- Severe obstructive pulmonary disease and decreased diffusion capacity in a non-smoker warrants further investigation beyond COPD.
- Diffuse, homogenous pulmonary cysts on HRCT are highly suggestive of LAM, particularly in women.
- Re-evaluation of previous lung biopsies can aid in confirming the LAM diagnosis.
Implications:
- High-resolution CT (HRCT) is a crucial diagnostic tool for identifying LAM in patients with unexplained severe obstructive pulmonary disease.
- Early and accurate diagnosis of LAM is essential for appropriate management and potential lung transplantation consideration.
- This case highlights the importance of considering LAM in the differential diagnosis of obstructive lung diseases, especially in women presenting with characteristic imaging findings.
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