Poland Syndrome

Discussion:

The differential diagnosis of hyperlucent hemithorax includes:

Poor patient positioning

Scoliosis

Pneumothorax

Contralateral pleural effusion

Mastectomy

Swyer James Syndrome

Pulmonary Agenesis

Mucous Plug-ball valve mechanism

Pulmonary Embolism Westermark sign of unilateral pulmonary oligemia

Emphysema-asymmetric

Congenital Lobar Overinflation

Unilateral bullous Disease

Contralateral Chest wall mass

 

Poland Syndrome, the Poland “Sequence”, or “Subclavian Artery Supply Disruption Sequence” is a pattern of abnormalities believed to arise from an interruption of the blood supply via the subclavian artery in the 40-50^th days in utero.

The abnormalities are typically on the right.

The etiology is unknown, and is usually sporadic, however there are rare familial cases.

The syndrome affects males three time more frequently than females.

The syndrome is characterized by unilateral underdevelopment or absence of the sternal portion of the pectoralis major and/or the entire pectoralis minor muscle.

Associated ipsilateral defects include syndactyly (webbing), brachydactyly (short finger), oligodactyly (missing fingers), absent or abnormal humerus or ulna, additional absent or underdeveloped chest wall muscles, i.e. latissimus dorsi or serratus anterior, absence of subcutaneous fat, short ribs, Sprengel deformity, hypoplastic or absent nipple, abnormally located axillary hair, and renal agenesis.

In females there may be absence or underdevelopment of the ipsilateral breast.

 

References:

1.      RSNA, http://pubs.rsna.org/doi/abs/10.1148/101.3.619

2.      National Organization of Rare Disorders. https://rarediseases.org/rare-diseases/poland-syndrome/

3.      Wikipedia, https://en.wikipedia.org/wiki/Poland_syndrome

Radiopedia, https://radiopaedia.org/articles/unilateral-hypertranslucent-hemithorax