Acrocephalopolysyndactyly Type II ( @entity50081 ) is determined by autosomal recessive inheritance. Only some 40 cases have been described. Variable clinical signs have been described including prolonged retention of primary teeth and @entity2651 . This paper describes the oral and dental findings in a family containing two affected brothers. The family pedigree is informative, as the mother has had @entity1 by three partners. The two affected individuals are full brothers. The first affected brother has delayed dental development, severe @entity2651 and small tooth crown size. Mesio-distal and bucco-lingual dimensions were measured on the study models and compared with population data. The younger brother also has delayed dental development but only mild @entity2651 . Their half sister has severe @entity2651 but no signs of @entity50081 . This family study demonstrates two affected individuals with typical clinical features and a pedigree compatible with autosomal recessive inheritance. Small tooth crown size has been shown by standardized measurement and evidence advanced that @entity2651 is not part of the syndrome but a coincidental finding which segregates independently. We have also shown that the marked delay in emergence of teeth is associated more with problems of tooth eruption, possibly related to the @entity854 , than to a generalized delay in dental development.

Oral findings in XXXX .

@entity50081 :: ('MESH:C563187', 'Disease') :: ['Carpenter Syndrome'] @entity1 :: ('9606', 'Species') :: ['children'] @entity854 :: ('MESH:D018376', 'Disease') :: ['bony abnormalities'] @entity2651 :: ('MESH:D000848', 'Disease') :: ['hypodontia']
@entity50081 :: (MESH:C563187,Disease) :: ['Carpenter syndrome']
