Ehlers-Danlos Syndrome (EDS) Type IV

Karl S. Chiang, MD, Bobby C. Walters, Jr., MD


Appl Radiol. 2003;32(5) 

In This Article

Case Summary

The patient is a 13-year-old boy who was seen in another hospital's emergency department after awakening at night with an episode of sharp "stabbing" chest pain. The pain involved the left shoulder and caused shortness of breath. A chest X-ray revealed mediastinal widening, and a contrast-enhanced CT of the chest (Figure 1A) showed diffuse mediastinal soft-tissue densities, especially around the aorta. He was subsequently transferred to the pediatric intensive care unit (PICU). Admission history and physical examination were notable only for premature birth and pneumonia at 6 months of age, as well as a slightly asymmetrical chest with prominence of the left hemithorax. At this point, Hodgkin's lymphoma was the working diagnosis and fine needle aspiration was performed under CT guidance. The specimen obtained was not diagnostic and a thoracoscopic/open biopsy was scheduled to be performed in 2 days. He was transferred to the general pediatric floor but had to return to the PICU on the following day because of dyspnea and a mild increase in neck swelling. His cardiac examination remained normal.

(A) Chest CT revealing diffuse mediastinal soft-tissue densities, especially around the descending aorta. (B) Repeat chest CT showing an "outpouching" of the aorta.


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