Poland Syndrome

Mark V Mazziotti, MD, Meghna V Misra, MD, Fizan Abdullah, MD, PhD, David Sigalet, MD, PhD, Jamie C Harris, MD
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Epidemiology

Poland syndrome was first described in the English literature by Poland in 1841. It is a constellation of physical findings including the absence of the pectoralis major and minor muscles, syndactyly, absence of ribs, chest wall depression, athelia or amastia, absence of axillary hair and limited subcutaneous fat. Clarkson was the first to label the syndrome in 1962 [1]. Poland syndrome is rare – it has a sporadic occurrence estimated at 1 in 10,000 to 1 in 100,000 live births [2]. A familial component is present in less than one percent of patients.

Poland syndrome is associated with the Mobius syndrome which involves unilateral or bilateral facial palsy and impaired ocular abduction [3][4][5]. The two may have a common etiology although this has yet to be determined [6].

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Epidemiology

Poland syndrome was first described in the English literature by Poland in 1841. It is a constellation of physical findings including the absence of the pectoralis major and minor muscles, syndactyly, absence of ribs, chest wall depression, athelia or amastia, absence of axillary hair and limited subcutaneous fat. Clarkson was the first to label the syndrome in 1962 [1]. Poland syndrome is rare – it has a sporadic occurrence estimated at 1 in 10,000 to 1 in 100,000 live births [2]. A familial component is present in less than one percent of patients.

Poland syndrome is associated with the Mobius syndrome which involves unilateral or bilateral facial palsy and impaired ocular abduction [3][4][5]. The two may have a common etiology although this has yet to be determined [6].

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Last updated: November 2, 2020