Jennifer Engle, MD, Hazim J. Safi, MD, Omid Abbassi, MD, Dimitrios C

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Presentation transcript:

Mucopolysaccharidosis presenting as pediatric multiple aortic aneurysm: First reported case  Jennifer Engle, MD, Hazim J. Safi, MD, Omid Abbassi, MD, Dimitrios C. Iliopoulos, MD, Douglas Dorsay, MD, Joiner Cartwright, PhD, Donald Weilbaecher, MD  Journal of Vascular Surgery  Volume 26, Issue 4, Pages 704-710 (October 1997) DOI: 10.1016/S0741-5214(97)70074-0 Copyright © 1997 Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter Terms and Conditions

Fig. 1 Preoperative descending thoracic and thoracoabdominal aneurysms. Journal of Vascular Surgery 1997 26, 704-710DOI: (10.1016/S0741-5214(97)70074-0) Copyright © 1997 Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter Terms and Conditions

Fig. 2 Completed graft replacement of descending thoracic and thoracoabdominal aortic aneurysms with bypass graft to right renal artery. Journal of Vascular Surgery 1997 26, 704-710DOI: (10.1016/S0741-5214(97)70074-0) Copyright © 1997 Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter Terms and Conditions

Fig. 3 A, Low-power photomicrograph shows full-thickness aortic wall with cellular intimal proliferation. Severe medial degeneration is present, with marked loss of elastic fibers and increased acid mucopolysaccharides. (VVG stain; original magnification, 50×). B, Electron micrograph of aortic media shows almost total loss of elastic fibers with few fragments remaining (EF). Large cell in center is myofibroblast (MF). Other cells are modified smooth muscle cells (SM) intermixed with collagen (C). Original magnification, 4000×. Journal of Vascular Surgery 1997 26, 704-710DOI: (10.1016/S0741-5214(97)70074-0) Copyright © 1997 Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter Terms and Conditions

Fig. 3 A, Low-power photomicrograph shows full-thickness aortic wall with cellular intimal proliferation. Severe medial degeneration is present, with marked loss of elastic fibers and increased acid mucopolysaccharides. (VVG stain; original magnification, 50×). B, Electron micrograph of aortic media shows almost total loss of elastic fibers with few fragments remaining (EF). Large cell in center is myofibroblast (MF). Other cells are modified smooth muscle cells (SM) intermixed with collagen (C). Original magnification, 4000×. Journal of Vascular Surgery 1997 26, 704-710DOI: (10.1016/S0741-5214(97)70074-0) Copyright © 1997 Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter Terms and Conditions