dentinogenesis imperfecta (di) is a hereditary dentin defect caused by an autosomal dominant mutation in dentin sialophosphoprotein gene. defective dentin development results in discolored teeth that are prone to wear and fracture. early diagnosis and proper treatment are necessary to achieve better functional and esthetic results and minimize nutritional deficiencies and psychosocial distress....

crouzon syndrome is a rare genetic disorder inherited in autosomal dominant pattern with complete penetration and variable expressivity. its most notable characteristic feature is premature synostosis of cranial sutures the case presented is of a 4 yr old boy with box like head with microcephaly, protuberant eyes, hydrocephalus, low visual acquity diagnosed as a case of crouzon syndrome after c...