Poland’s Syndrome: A Concise Review of the Clinical Features Highlighting Associated Dermatologic Manifestations

Abstract

Poland's syndrome is a rare congenital condition characterized by absence of the pectoralis major muscle and a wide spectrum of associated ipsilateral chest wall and upper extremity anomalies. Associated dermatologic anomalies classically include pectoral and axillary alopecia, anhidrosis due to the absence of sweat glands, and deficiency in subcutaneous fat. Functional limitations are minimal, and thus surgical correction is primarily indicated for aesthetic purposes. Chest wall reconstruction typically involves transposition of a latissimus dorsi myocutaneous flap with or without a temporary subcutaneous tissue expander prior to surgical reconstruction. Using the PubMed database, a literature review was done on Poland's syndrome. We summarize the key features of Poland's syndrome, including the epidemiology, clinical presentation, pathogenesis, and management, and highlight the dermatologic associations reported in the literature.

Poland Syndrome: A Case of a Left Handed Infant

“…4 Other theories for development of PS include disruption of the lateral embryonal plate mesoderm after 16-28 days post fertilization, atrophy of the cord anterior motor neurons, intrauterine trauma, viral infections and teratogens. 2,5 In this case, the patient was noted to have a hand preference strong enough for a parent to identify a preference by the ninth month. The development of a functioning reach in an infant requires Straightness, which is kinematially defined as the ratio of the path traveled by the hand over a distance to the target, and Smoothness, which is a lack of vacillations between accelerations and decelerations along that path.…”

“…5 The primary theory behind PS development is Subclavian Artery Supply Disruption Sequence (SASDS), in which a vascular disruption occurs in the sixth week of development causing hypoplasia at some point along the Subclavian artery or its branches.…”

“…Furthermore, digits begin to form as digital rays. 2,5 How much and in which locations the artery and branches are disrupted account for the widely varied presentation. 4 Other theories for development of PS include disruption of the lateral embryonal plate mesoderm after 16-28 days post fertilization, atrophy of the cord anterior motor neurons, intrauterine trauma, viral infections and teratogens.…”

“…1,2,3,4,5 Eighty-one years later, Patrick Clarkson credited Poland with the discovery of this anatomic anomaly after examining a series of three similar cases. 2,3,4 Poland Syndrome (PS) is thought to occur sporadically, with an unclear genetic component, due to a vascular insult occurring in the 6th week of fetal development.…”

Poland's Syndrome: When there is no breast

“…The most commonly cited theory is a vascular injury due an interruption of blood supply to the branches of the subclavian and vertebral arteries during the sixth week of embryogenesis when the pectoralis muscle is developing from the pectoral mass and the digital rays are developing into fingers . Other possible etiologies include teratogens, intrauterine trauma, infections, and malformation of the lateral embryonic plate mesoderm (which gives rise to the pectoralis muscle) . While the majority of Poland's Syndrome cases are sporadic, there are documented cases of heritability which display an autosomal dominant inheritance pattern with incomplete penetrance …”