Marfan’s Syndrome CombIned WIth SItus Inversus TotalIs

Marfan Syndrome is a connective tissue disorder. Although cardinal manifestations involve skeletal, ocular, and cardiovascular systems, its association with rare conditions have been reported. We present here 4,5 years-old female patient. She has tall stature, increased arm span/height ratio, bilateral lens subluxation, atrial septal defect, aortic valve regurgitation, aortic root dilatation, arachnodactyly, hyperextensible fingers, thumb sign, and situs inversus totalis. Marfan Syndrome combined with situs inversus totalis has been described only once. We have reported the second case as contribution to the literature.