January Case of the Month
Authors: Andrea L. Magee, MD, PGY2, radiology resident, and Steven M. Montner, MD, assistant professor of radiology, Thoracic Imaging, Department of Radiology, University of Chicago, Chicago, Ill.
Why did you select this case for submission?
Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary arterial hypertension (PAH), and many radiologists often don’t encounter it. Dr. Montner and I chose to submit the PCH case to provide a refresher of the clinical and imaging findings associated with this diagnosis.
What should readers learn from this case?
Patients with PCH may present with nonspecific findings such as progressive dyspnea, fatigue, and chronic cough. Pulmonary function tests may be completely unremarkable. Critically, the management of PCH differs from the management of other causes of PAH, and the routine use of vasodilators can be fatal in patients with PCH. Radiologists are well-positioned to recognize the imaging findings and suggest PCH in the correct clinical setting.
What did you learn from working on the case?
Although imaging findings may suggest PCH, it is a microscopic diagnosis. Patients are often unable to tolerate lung biopsy, and so most cases are confirmed only at autopsy, if at all.
How did guidance from senior staff at your institution impact your learning and case development?
Working with Dr. Montner was essential in understanding the pathophysiology of PCH and how this manifests in the imaging findings. He walked me through the findings in other PCH cases and provided additional cases with common differential considerations for PCH.
Why did you choose Case in Point for submission of your case?
We chose to submit this case in order to provide a digestible, but still informative, summary of PCH for radiologists who might not have seen a case recently. CIP allowed us to share our case with a large number of radiologists in varying levels of training and practice.
Are you a regular reader of Case in Point? What are your favorite types of cases?
Yes, I receive the daily CIP emails and make it a point to complete each case. I especially appreciate cases showing unusual etiologies or abnormal presentations of common etiologies.
What else should we know about the case that you'd like to share?
We feel that we provided high-yield facts in our case and would like to emphasize to others the importance of considering PCH in patients with PAH and appropriate imaging findings. We're glad our case was well-received!