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Kozo Ishino
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Ann Thorac Surg 2005;80:729-731
© 2005 The Society of Thoracic Surgeons

Case report

Coarctation of the Thoraco-Abdominal Aorta Associated With Mucopolysaccharidosis VII in a Child

Osami Honjo, MD a , Kozo Ishino, MD a , * , Masaaki Kawada, MD a , Shin-Ichi Ohtsuki, MD b , Shunji Sano, MD a

a Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine and Dentistry, Okayama City, Japan
b Department of Pediatrics, Okayama University Graduate School of Medicine and Dentistry, Okayama City, Japan

Accepted for publication February 3, 2004.

* Address reprint requests to Dr Ishino, Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine and Dentistry, 2-5-1 Shikata-cho, Okayama City 700-8558, Japan (Email: ishino{at}tb3.so-net.ne.jp).

Herein we describe a case of atypical coarctation of the thoraco-abdominal aorta associated with mucopolysaccharidosis VII in a 4-year-old girl. Aortography showed diffuse narrowing of the descending aorta. An extra-anatomic bypass was constructed using an 8-mm Dacron graft (Meadox Medicals Inc, Oakland, NJ) between the proximal and distal portion of the descending aorta. Balloon angioplasty was necessary to treat stenoses in the infrarenal abdominal aorta. Two years postoperatively at age 6, aortography revealed no stenosis at the anastomotic sites or in the prosthesis, but the hypoplastic segment of the descending aorta between the anstomoses was completely occluded.






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