No abstract available.
Marfan Syndrome*

We report a case of a 12-year-old Filipino female with crumpled ears, arachnodactyly, camptodactyly, and congenital joint contractures, consistent with Beals syndrome. Marfan syndrome is a common differential diagnosis, since both are caused by mutations in two homologous genes, namely FBN1 and FBN2. Both syndromes share similar characteristics hence, it is essential to differentiate the two, since Marfan syndrome may develop fatal complications, not encountered in Beals Syndrome. Management of Beals syndrome is mainly supportive including physiotherapy and ophthalmologic and cardiovascular evaluation.
Beals syndrome ; Marfan Syndrome

Sliding annuloplasty has been used for mitral valve repair in conjunction with posterior leaflet quadrangular resection to avoid systolic anterior motion of the anterior leaflet of the mitral valve. Herein, we report on a case of successful mitral valve repair with using the annular plication technique to facilitate sliding annuloplasty and extensive quadrangular resection was also done for treating a Marfan's syndrome patient who had an extremely redundant leaflet and a severely dilated annulus.
Humans ; Marfan Syndrome* ; Mitral Valve

The Marfan's syndrome is a collagenous hereditary disorder affected by the long bone, heart and eyes. Two cases of Marfan's syndrome are presented. Both two cases showed upward dislocation of lenses. We performed extraction of lenses on 4 eyes among the two cases by means of Cryo-extractor. The results of surgical treatment are described.
Collagen ; Dislocations ; Heart ; Marfan Syndrome*

No abstract available.
Aortic Aneurysm, Abdominal ; Marfan Syndrome

Marfan syndrome is an uncommon congenital disease originally described by Marfan in 1896. It is inherited as an autosomal dominant trait and can affect the tissues of mesodermal origin, mainly skeletal, ocular, and cardio-vascular systems. We report 4 cases of Marfan syndrome occurring in one family, one of which was found to have an acute attack of glaucoma and underwent extract operation of a right dislocated lens. A brief summary of the literature is submitted.
Glaucoma ; Humans ; Marfan Syndrome ; Mesoderm

From march, 1983 to May, 1987, we experienced 17 patients of Marfan syndrome. The clinical profile and course of 17 patients of Marfan syndrome were reviewed. 1) Their ages ranged from 13 to 50 years with a mean age of 30 years and 16 patients were male and 1 patient was female. 2) musculoskeletal features were present in 14(88%) of the 16 examined. Ocular features were found in 5(42%) of 12 examined. Familial features were present in 7(47%) of 15 examined. 3) Among cardiovascular manifestations dissecting aneurysm was present in 10(59%) patients, aortic annuloectasia in 15(94%), MR in 4(24%) and MVP in 1(6%). 4) Modified Bentall operation was performed to 8 patients. Two of them died. The other 8 patients took a conservative medical therapy. Four of them died.
Aneurysm, Dissecting ; Female ; Humans ; Male ; Marfan Syndrome*

No abstract available.
Anesthesia, Spinal* ; Dilatation, Pathologic* ; Marfan Syndrome*

Marfan's syndrome is a widespread disturbance of mesodermal tissue throughout the body. The main disturbances are characteristic skeletal anomaly, cardiopathy and eye disturbance such as lens dislocation, glaucoma, etc. Marfan originally described it under the name of dolichostenomelie in 1896. Since then, many cases have been reported in the literature. Authors experienced 3 cases of Marfan's syndrome and 2 cases were operated. The result of surgical treatment. other clinical findings, and brief review of literature are reported as following.
Glaucoma ; Lens Subluxation ; Marfan Syndrome* ; Mesoderm

The authors experienced a case of Marfan's syndrome in whom the right sided lens was dislocated into the anterior chamber and the left side inferio-temporally into vitreous. In addition, the lens of the right eye was complicated by cataract, and a favorable outcome was obtained by intracapsular lens extraction.
Anterior Chamber* ; Cataract ; Lens Subluxation* ; Marfan Syndrome*