Coarctation of the thoraco-abdominal aorta associated with mucopolysaccharidosis VII in a child

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.