Skeletal  System (80% cases) [7,10]

·             Hands and feet involved in 60-70% *

·             May be asymmetrical and variable

·             Lobster claw hand deformity - almost characteristic *

·             Polydactyly, syndactyly*

·             Claw hand, split hand or foot 

·             Hypoplasia,  aplasia of digits*

·             Microcephaly*

·             Scoliosis

·             Osteopathia striata- commonly present*

Ocular  System (40% cases) [10]

·             Asymmetry of eyes

·              Microphthalmos*

·             Strabismus*

·             Colobomas of iris, choroid or retina*

·             Epiphora

·             Visual acquity usually normal

Dental  System (nearly 50% cases) [10]

·             Enamel defects

·             Recurrent caries

·             Anodontia, Delayed eruption*

·             Malocclusion

·             Notched incisors