| The Results of Surgical Treatment
for Cardiovascular Disorder in Shprintzen-Goldberg Syndrome |
(Department of Surgery, Kurume University School
of Medicine, Kurume, Japan)
| Shogo Yokose |
Shuji Fukunaga |
Toru Takaseya |
| Hideki Sakashita |
Shingo Chihara |
Ryoichi Hiratsuka |
| Seiji Onitsuka |
Shigeaki Aoyagi |
|
|
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.
Jpn. J. Cardiovasc. Surg. 30: 206-209 (2001) |
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