PURPOSE: Hand surgeons are often the first specialists to see @entity1 with @entity16138 ( @entity16138 ), when @entity1 with ulnar-sided syndactyly are referred. Major associated problems include neurologic, ophthalmologic, dental, and other @entity853 . The purposes of this study were to investigate the incidence of the reported associated conditions in the families of our @entity1 with @entity16138 , correlate them with the severity of syndactyly, and provide better counseling with more accurate information for these @entity1 and families. METHODS: We reviewed medical records from Texas Scottish Rite Hospital for @entity1 from 1980 to 2009 to identify @entity1 with @entity16138 . These @entity1 and families were invited to return for a detailed medical and family history and physical examination documenting hand, foot, eye, dental, and facial findings. RESULTS: A total of 73 pediatric @entity1 from 47 families were diagnosed with @entity16138 , and 38 individuals in 31 families agreed to participate in the study. We observed bilateral syndactyly in 32 @entity1 , with symmetric involvement in 31 of these. Abnormalities of the shape and size of the middle phalanx of the small finger were common. Ophthalmological findings were present in 31 and dental abnormalities identified in 33 @entity1 . Neurologic findings likely related to @entity16138 were found in 11 @entity1 . There appeared to be an association between the severity of syndactyly and the severity of dental and urologic findings, but not ophthalmologic or neurologic findings such as paraparesis or @entity73 . CONCLUSIONS: We found a 29% incidence of neurologic manifestations in @entity1 with @entity16138 . In addition, associated ophthalmologic, dental, and developmental conditions are frequent and a heightened awareness will allow appropriate referrals for @entity1 with ulnar-sided syndactyly in @entity16138 . TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic IV.

XXXX : ulnar-sided syndactyly and its associated disorders.

@entity1 :: ('9606', 'Species') :: ['patients', 'Children', 'infants'] @entity73 :: ('MESH:D003072', 'Disease') :: ['cognitive deficits'] @entity853 :: ('MESH:D009139', 'Disease') :: ['skeletal abnormalities'] @entity16138 :: ('MESH:C563160', 'Disease') :: ['ODDD', 'oculodentodigital dysplasia']
@entity16138 :: (MESH:C563160,Disease) :: ['Oculodentodigital dysplasia']
