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Pediatric radiology · Jan 1994
Case ReportsCavitary pulmonary lesions in type IV Ehlers-Danlos syndrome.
- T E Herman and W H McAlister.
- Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis Children's Hospital Department of Radiology, MO 63110.
- Pediatr Radiol. 1994 Jan 1; 24 (4): 263-5.
AbstractType IV Ehlers-Danlos syndrome (EDS) is characterized by large vessel bleeding, visceral rupture and early death. Pulmonary complications are uncommon. We report an 18-year-old patient with EDS type IV who developed recurrent large, thick-walled lung cavitary lesions, probably a manifestation of focal lung rupture. Such hemorrhagic cavities are probably the basis of the bullous disease and recurrent pneumothoraces occasionally observed in type IV EDS.
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