Definition of Pectoralis muscle absence with syndactyly
Pectoralis muscle absence with syndactyly definition - medical term
A
unique pattern of one-sided
malformations characterized by a defect of the chest
(pectoralis) muscle on
one side of the body and webbing of the fingers (cutaneous
syndactyly) of
the ipsilateral hand (the hand on the same side). Often
called Poland
syndrome. It is right-sided three times more often than it
is left-sided.
It is not common, affecting 1 child in about 20,000. For
reasons that are
unfathomable, Poland syndrome is 3 times more frequent in
boys than girls. The cause of Poland syndrome is uncertain. The
disorder is currently
considered "a nonspecific developmental field defect"
occurring at about the
sixth week of fetal development. Diminished blood flow
through the
subclavian artery that goes to the arm has been blamed, but
final proof for
this idea is lacking. In Poland syndrome there is aplasia
of the sternal
head of the pectoralis major. In other words, the end of
the main chest
muscle that normally attaches to the breastbone is missing.
On that side of
the body, nearby chest muscles (the serratus anterior and
latissimus dorsi
muscles) may also be absent as may be the armpit (axillary)
hair. In girls,
the breast on that side is also usually absent. The fingers
show webbing and
shortening (symbrachydactyly) on the hand on the same side.
The child with
Poland syndrome usually is entirely normal except for the
problems already
noted. Upon rare occasions, the Poland syndrome is
associated with more
severe finger and arm involvement or vertebral or kidney
problems.
Intelligence is not impaired by Poland syndrome.
The syndrome occurs sporadically ("out of the blue") and is
not familial.
The risk of recurrence of Poland syndrome in the family is
minute, except in
the very small fraction of cases where there is a parent or
other relative
known with Poland syndrome. Reconstructive surgery has in
the past been the
main recourse. Now bioengineered cartilage may be implanted
to help give the
chest a more normal look. Other names for Poland syndrome
include: Poland sequence, Poland anomaly, and Poland syndactyly.
The syndrome is named for
Alfred Poland. Born in 1822 in London, he became
demonstrator in anatomy in
1839 at Guy's Hospital. There he dissected the body of a
deceased convict
named George Elt whom he reported had "Deficiency of the
pectoral muscles"
(Guy's Hosp. Rep. 6:191, 1841). Poland became a celebrated
surgeon and
ophthalmologist but had to retire in 1867 due to a chronic
cough. He died in
1872 at the age of 51 of "consumption of the lungs"
(pulmonary
tuberculosis). Although Poland could never have discovered
this syndrome
without George Elt, Mr. Elt has not been credited nor has
his name ever been
associated with the syndrome.
Common Misspellings: pectoralis muscel absence with syndactyly
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