POLAND SYNDROME
Victor A. McKusick, OMIM, 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 SYNDROME
Victor A. McKusick, OMIM, 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, OMIM, 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 ...
Sindrome de Poland: un caso con dextrocardia
Tori Tori, Carlos et al., Rev Med Hered v.10 n.2 Lima abr., June 1999
[Spanish]
Reportamos un caso de Anomalía de Poland (ausencia del músculo pectoral mayor y braquisindactilia en la mano ipsilateral ) con dextrocardia. La ausencia del músculo pectoral mayor sin anomalías del miembro superior puede ser una variante de esta anomalía (1). En la mayoría de casos este sindrome ocurre en el hemicuerpo derecho (2), usualmente sin defectos costales ...
Poland Syndrome
I.B.I.S. Birth Defects, February 5, 2002
[Ukrainian]
Factsheet in Ukrainian
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Last Updated: 2008/3/3
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