186200 SYNDACTYLY, TYPE IV
Alternative
titles; symbols
HAAS TYPE SYNDACTYLY
POLYSYNDACTYLY, HAAS TYPE
table OF
CONTENTS
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TEXT
Haas (1940) reported
this type of syndactyly in a mother and her 2
children. The syndactyly was complete, affecting
the fingers of both hands, and there was
polydactyly, with 6 metacarpals and 6 digits.
Flexion of the fingers gave the hands a cup-shaped
form. In contradistinction to the type of
syndactyly in Apert syndrome, there was no bone
fusion. There was no mention of the condition of
the feet and there were no associated
malformations. Gillessen-Kaesbach
and Majewski (1991) described an affected
2-month-old girl with complete cutaneous syndactyly
and hexadactyly of both hands. Partial cutaneous
syndactyly of toes 2 and 3, involving the proximal
segment, was present on both feet. Although neither
parent had syndactyly, 2 brothers of the mother and
the maternal grandmother showed complete bilateral
cutaneous syndactyly of the second and third toes.
Development was otherwise normal. Each hand had 1
supernumerary ray, which may have been pre- or
postaxial. Rambaud-Cousson et
al. (1991) described type IV syndactyly with
bilateral hexadactyly of the hands and feet in 6
members of 3 generations with 1 instance of
male-to-male transmission. The proband also had
unilateral absence of the tibia. The possibility
was raised that syndactyly type IV with hexadactyly
of the feet is in fact a complex entity which can
include a variety of lower limb malformations.
REFERENCES
- 1.
Gillessen-Kaesbach, G.; Majewski, F. :
- Bilateral complete polysyndactyly
(type IV Haas). Am. J. Med.
Genet. 38: 29-31, 1991.
PubMed ID : 1849351
- 2. Haas, S. L.
:
- Bilateral complete syndactylism of
all fingers. Am. J. Surg. 50:
363-366, 1940.
- 3. Rambaud-Cousson,
A.; Dudin, A. A.; Zuaiter, A. S.; Thalji, A.
:
- Syndactyly type IV/hexadactyly of
feet associated with unilateral absence of the
tibia. Am. J. Med. Genet. 40:
144-145, 1991.
PubMed ID : 1654744
CLINICAL
SYNOPSIS
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Clinical Synopsis Entry
CREATION DATE
Victor A. McKusick : 6/2/1986
EDIT HISTORY
mimadm : 5/10/1995
carol : 4/17/1992
supermim : 3/16/1992
carol : 10/3/1991
carol : 3/12/1991
carol : 3/11/1991
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