Poland Syndrome is a topic covered in the Pediatric Surgery NaT.

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Epidemiology

Poland syndrome is a congenital condition characterized by variable defects Including agenesis of the pectoralis major, absence or hypoplasia of the pectoralis minor, a varying number of rib defects, anomalies of the breast and nipple, a lack of subcutaneous tissue, an absence of pectoral and axillary hair, and hand anomalies [1]. Of these, the absence of the pectoralis major is the most common component of Poland syndrome[1]In 1841, Sir Alfred Poland, a British surgeon, was the first to describe the association of the absence of the sternocostal head of the pectoralis major muscle with an ipsilateral upper extremity deficiency[2]. He described the syndrome when he was a student. However, it wasn’t until 1962 that the nomenclature of Poland syndrome was first used by Dr. Patrick Clarkson[3]. Other non-eponymous terms used to describe the condition include "pectoral aplasia-dysdactylia syndrome" or "hand and ipsilateral thorax syndrome" [4].

Poland syndrome is rare, with a sporadic occurrence estimated at 1 in 10,000 to 1 in 100,000 live births [5]. A familial component is present in less than one percent of patients. It has also been associated with Moebius syndrome which involves unilateral or bilateral facial palsy and impaired ocular abduction [6][7][8]. The two may have a common etiology, although this has yet to be determined [9].

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Epidemiology

Poland syndrome is a congenital condition characterized by variable defects Including agenesis of the pectoralis major, absence or hypoplasia of the pectoralis minor, a varying number of rib defects, anomalies of the breast and nipple, a lack of subcutaneous tissue, an absence of pectoral and axillary hair, and hand anomalies [1]. Of these, the absence of the pectoralis major is the most common component of Poland syndrome[1]In 1841, Sir Alfred Poland, a British surgeon, was the first to describe the association of the absence of the sternocostal head of the pectoralis major muscle with an ipsilateral upper extremity deficiency[2]. He described the syndrome when he was a student. However, it wasn’t until 1962 that the nomenclature of Poland syndrome was first used by Dr. Patrick Clarkson[3]. Other non-eponymous terms used to describe the condition include "pectoral aplasia-dysdactylia syndrome" or "hand and ipsilateral thorax syndrome" [4].

Poland syndrome is rare, with a sporadic occurrence estimated at 1 in 10,000 to 1 in 100,000 live births [5]. A familial component is present in less than one percent of patients. It has also been associated with Moebius syndrome which involves unilateral or bilateral facial palsy and impaired ocular abduction [6][7][8]. The two may have a common etiology, although this has yet to be determined [9].

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Last updated: November 23, 2021