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Case Reports
. 2017 Jan-Mar;9(1):73-77.
doi: 10.4103/jpbs.JPBS_326_16.

Marfan Syndrome

T Sivasankari  1 , Philips Mathew  2 , Ravi David Austin  3 , Sakthi Devi  4
Affiliations
Case Reports

Marfan Syndrome

T Sivasankari et al. J Pharm Bioallied Sci. 2017 Jan-Mar.

Abstract

Marfan syndrome (MFS) is the autosomal dominant-inherited multisystem connective-tissue disorder, with a reported incidence of 1 in 10,000 individuals and equal distribution in both genders. The main clinical manifestation of this disorder consists of an exaggerated length of the upper and lower limbs, hyperlaxity, scoliosis, alterations in the cardiovascular and pulmonary systems, and atypical bone overgrowth. Orofacial manifestations such as high-arched palate, hypodontia, long narrow teeth, bifid uvula, mandibular prognathism, and temporomandibular disorders are also common. Early diagnosis of MFS is essential to prevent the cardiovascular complications and treatment of orofacial manifestations, thus to increase the quality of life of the patient.

Keywords: Autosomal dominant; Marfan syndrome; oral manifestation.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Flat foot
Figure 2
Figure 2
Upper limbs were long and slender (arachnodactyly)
Figure 3
Figure 3
Wrist (walker's sign)
Figure 4
Figure 4
Thumb or Steinberg sign
Figure 5
Figure 5
Long and narrow face with a convex profile
Figure 6
Figure 6
High arch palate
See this image and copyright information in PMC

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