ABSTRACT
Shprintzen-Goldberg syndrome (SGS) is a rare disorder with many characteristics of generalized connective tissue dysplasia. SGS is characterized by Marfanoid habitus with craniosynostosis and mental retardation. Patients with SGS have cardiovascular disorders similar to Marfan syndrome (MFS) and those disorders seem to play an important role in the prognosis of SGS. To our knowledge, only 19 patients with SGS have been reported, and 7 of them had cardiovascular disorders. The major cardiovascular disorders of SGS are aortic root dilatation and mitral valve prolapse. We reported the first case of SGS successfully treated surgically for cardiovascular disorders. Since then, we performed another operation in a patient with SGS. In this paper, we report our surgical results in patients with SGS.