Poland syndrome is usually sporadic, although rare familial cases have been described 1. It is rare with an estimated incidence of 1 : 36000 - 50000 1.There does not appear to be a side or gender predilection.
The abnormality is usually evident at birth due to asymmetry of the chest and frequent and ipsilateral abnormalities, most frequently syndactyly 2. Spectrum of abnormalities includes:
- aplasia or hypoplasia of
- pectoralis major (most common)
- pectoralis minor
- ipsilateral 2nd to 5th ribs
- ipsilateral breast or nipple
- upper limb abnormalities
No definite cause has as yet been identified, although it is thought to result from an isolated vascular insult in early gestation (involving embyonic subclavian and vertebral arteries).
May be seen as hypoplasia of the breast or of the pectoralis major muscle (thus absent on MLO view).
On CXR it is one of the classic causes for a unilateral hyperlucent hemithorax, and should be considered when asymmetry of chest is not explained by other causes.
CT / MRI
Although clinical examination usually suffices to make the diagnosis, cross-sectional imaging will elegantly outline abnormalities.
Treatment and prognosis
The condition is benign and depending on the degree of involvement of the upper extremity may be asymptomatic. Cosmetic re-constructive surgery may be considered (e.g. breast implant).
It was first described by Sir Alfred Poland : English surgeon (1822 - 1872) in 1841, after he dissected the body of a convict named George Elt at Guy's Hospital 1,4
For hypoplastic breast / absent tissue on one side on mammography, consider
- amazia : absence of breast parenchyma and
amastia : absence of breast tissue
- this can occur from a an excision of a pre-puberal breast bud or from chest wall irradiation during childhood
For a unilateral hyperlucent hemithorax on a chest radiograph see >>
- 1. Samuels TH, Haider MA, Kirkbride P. Poland's syndrome: a mammographic presentation. AJR Am J Roentgenol. 1996;166 (2): 347-8. AJR Am J Roentgenol (citation) - Pubmed citation
- 2. Jeung MY, Gangi A, Gasser B et-al. Imaging of chest wall disorders. Radiographics. 19 (3): 617-37. Radiographics (full text) - Pubmed citation
- 3. Parker DL, Mitchell PR, Holmes GL. Poland-Möbius syndrome. J. Med. Genet. 1981;18 (4): 317-20. J. Med. Genet. (link) - Free text at pubmed - Pubmed citation
- 4. Alfred Poland from whonamedit.com, the dictionary of medical eponyms. Alfred Poland
- 5. Pisteljić DT, Vranjesević D, Apostolski S et-al. Poland syndrome associated with 'morning glory' syndrome (coloboma of the optic disc). J. Med. Genet. 1986;23 (4): 364-6. J. Med. Genet. (link) - Free text at pubmed - Pubmed citation
- 6. Friedman T, Reed M, Elliott AM. The carpal bones in Poland syndrome. Skeletal Radiol. 2009;38 (6): 585-91. doi:10.1007/s00256-008-0638-x - Pubmed citation
- 7. Paredes ES. Atlas of mammography. Lippincott Williams & Wilkins. (2007) ISBN:0781764335. Read it at Google Books - Find it at Amazon
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Pectoral aplasia-syndactyly syndrome||✓|