Entry - %174600 - POLYDACTYLY, PREAXIAL III; PPD3 - OMIM
% 174600

POLYDACTYLY, PREAXIAL III; PPD3


Alternative titles; symbols

INDEX FINGER POLYDACTYLY


Clinical Synopsis
 
Phenotypic Series
 

Limbs
- Preaxial polydactyly
- Thumb replaced by one or two triphalangeal digits
- Occasionl polydactyly of 1st or 2nd toes
Radiology
- Distal epiphyses for metacarpals of accessory digits
Inheritance
- Autosomal dominant

TEXT

Clinical Features

Manoiloff (1931) reported a 5-generation Russian family (Scipion family), descended from Italian and Polish ancestors, in which hexadactyly involving the index finger had been transmitted for approximately 700 years. Examination of the proband showed that all fingers were well developed with normal nails. His left foot showed 'double toes' at the second toe, with cutaneous syndactyly but 2 distinct nails; the same double toe was not quite as developed on the right foot, appearing as a broad toe with single nail in published photos. Muscles of the extremities appeared to be normal. Hand x-rays showed a normal array of carpal bones, but the presence of an extra metacarpal articulating with the trapezium; similarly, foot x-rays showed that the medial os cuneiformis was connected with 2 metatarsal bones instead of one. The author stated that both thumbs should be considered 'supplementary fingers;' the published x-rays appear to show triphalangeal thumbs bilaterally. James and Lamb (1963) noted that in some cases the feet may show preaxial polydactyly of the first toes.


See Also:

REFERENCES

  1. Atasu, M. Hereditary index finger polydactyly: phenotypic, radiological, dermatoglyphic, and genetic findings in a large family. J. Med. Genet. 13: 469-476, 1976. [PubMed: 1018306, related citations] [Full Text]

  2. James, J. I. R., Lamb, D. W. Congenital abnormalities of the limbs. Practitioner 191: 159-172, 1963. [PubMed: 14048524, related citations]

  3. Manoiloff, E. O. A rare case of hereditary hexadactylism. Am. J. Phys. Anthrop. 15: 503-508, 1931.


Contributors:
Marla J. F. O'Neill - updated : 10/11/2019
Creation Date:
Victor A. McKusick : 6/2/1986
carol : 10/11/2019
carol : 05/03/2019
joanna : 03/18/2004
mimadm : 2/25/1995
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/27/1989
marie : 3/25/1988
reenie : 6/2/1986

% 174600

POLYDACTYLY, PREAXIAL III; PPD3


Alternative titles; symbols

INDEX FINGER POLYDACTYLY


SNOMEDCT: 723446006;   ORPHA: 93337;  



TEXT

Clinical Features

Manoiloff (1931) reported a 5-generation Russian family (Scipion family), descended from Italian and Polish ancestors, in which hexadactyly involving the index finger had been transmitted for approximately 700 years. Examination of the proband showed that all fingers were well developed with normal nails. His left foot showed 'double toes' at the second toe, with cutaneous syndactyly but 2 distinct nails; the same double toe was not quite as developed on the right foot, appearing as a broad toe with single nail in published photos. Muscles of the extremities appeared to be normal. Hand x-rays showed a normal array of carpal bones, but the presence of an extra metacarpal articulating with the trapezium; similarly, foot x-rays showed that the medial os cuneiformis was connected with 2 metatarsal bones instead of one. The author stated that both thumbs should be considered 'supplementary fingers;' the published x-rays appear to show triphalangeal thumbs bilaterally. James and Lamb (1963) noted that in some cases the feet may show preaxial polydactyly of the first toes.


See Also:

Atasu (1976)

REFERENCES

  1. Atasu, M. Hereditary index finger polydactyly: phenotypic, radiological, dermatoglyphic, and genetic findings in a large family. J. Med. Genet. 13: 469-476, 1976. [PubMed: 1018306] [Full Text: https://doi.org/10.1136/jmg.13.6.469]

  2. James, J. I. R., Lamb, D. W. Congenital abnormalities of the limbs. Practitioner 191: 159-172, 1963. [PubMed: 14048524]

  3. Manoiloff, E. O. A rare case of hereditary hexadactylism. Am. J. Phys. Anthrop. 15: 503-508, 1931.


Contributors:
Marla J. F. O'Neill - updated : 10/11/2019

Creation Date:
Victor A. McKusick : 6/2/1986

Edit History:
carol : 10/11/2019
carol : 05/03/2019
joanna : 03/18/2004
mimadm : 2/25/1995
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/27/1989
marie : 3/25/1988
reenie : 6/2/1986