POLAND SYNDROME
Victor A. McKusick, Johns Hopkins University, February 27, 2006
[for Professionals mainly]
Alternative titles; symbols
POLAND SYNDACTYLY
POLAND ANOMALY
PECTORALIS MUSCLE, ABSENCE OF, INCLUDED
This condition consists of unilateral symbrachydactyly and ipsilateral aplasia of the sternal head of the pectoralis major muscle. Sometimes called Poland sequence, this disorder was first describe by Poland (1841). Trier (1965) found 2 instances of parent and child with Poland syndrome. Fuhrmann et al. (1971) reported a family with father - to - son transmission and referred to other cases ... In British Columbia, McGillivray and Lowry (1977) found an incidence of 1 per 32,000 live births ...
Riccardi (1978) described unilateral gluteal hypoplasia and brachysyndactyly and proposed that it is the lower limb equivalent of the Poland anomaly. In support of Riccardi's view, Parano et al. (1995) reported on an extended family in whom 3 individuals, all women, had unilateral gluteal hypoplasia and the propositus in addition had unilateral hypoplasia of a pectoral muscle ...
Czeizel et al. (1990) reported that in Hungary for the period 1975 to 1984 there was 1 case of full - blown Poland sequence in 87,550 births including 12 cases with Poland sequence - type symbrachydactyly but without pectoralis major defect ...

Parents of Poland Syndrome
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Poland's Syndrome - an Overview
Helen Williams RN, Sydney, NSW, Australia, July 2007.
First described in a cadaver examined by A. Poland at Guy's Hospital in 1841, "this combined anomaly of unilateral webbed fingers and the absence of the sterno - costal portion of ipsilateral pectoralis major muscle is recently known as Poland's Anomaly, Poland's Syndactyly, or Poland's Syndrome (Sugiura, 1976, p. 17) ... classified ... as ... (pectoral dysplasia - dysdactyly)" ...
Most researchers currently find that there is no familial cause, but there are some who link this Syndrome with several others ...
Sugiura (1976) proposes four types based on observation of the hand in his research with 45 cases ...
Brachymesophalangeal triphalangy type ...
Diphalangy type ...
Monophalangy type ...
Ectrodactyly type ...
Implications for children 0 - 14 years.
Birth to Two years : Issues for the child at this age are minimal ...
Ages Three to Five : These years are perhaps some of the busiest for the parent ... Children even at this early age will "feel sorry" for the child with a disability and will offer to help ...
Ages Six to Ten : There is a lot of peer pressure to keep up and the child may withdraw unless they have had a chance to do an activity before ...
Ages Ten to Fourteen : By this stage, activities such a Tennis, Netball, Music (practical or non - practical), and Athletics should be fully implemented ...

POLAND SYNDROME
Victor A. McKusick, Johns Hopkins University, February 27, 2006
CLINICAL SYNOPSIS
... Aplasia of sternal head of pectoralis major
Breast aplasia in females
Serratus anterior, and latissimus dorsi muscle aplasia ...
Unilateral symbrachydactyly ...
Patchy absence of axillary hair ...
Overwhelmingly sporadic
Causally nonspecific developmental field defect ...
Rarely familial

Poland syndrome
Philippe Jeanty, MD, et al., TheFetus.net, May 14, 1999
Definition: Unilateral symbrachydactyly and ipsilateral aplasia of the sternal head of the pectoralis major muscle, Ispilateral aplasia of the breast exists is females. Dextrocardia has been reported in several cases ...
Synonyms: Poland - Moebius syndrome ...
Etiology: Sporadic interruption of the early embryonic blood supply in the subclavian arteries ... Several familial cases have been described ...

BREASTS AND NIPPLES, ABSENCE OF
Victor A. McKusick, Johns Hopkins University, January 13, 2005
Alternative titles; symbols
ATHELIA
Pedigrees consistent with dominant inheritance have been reported. Fraser (1956) found absent breasts in 7 members of 3 generations ... Hypoplasia or aplasia of the breasts and nipples occurs in anhidrotic ectodermal dysplasia ...

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Last Updated: 2008/1/07

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