Fact-sheet: Poland syndrome
Updated on 08/13/2020 at 3:40 PM
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Definition
Poland syndrome corresponds to the absence or unilateral hypoplasia of the pectoralis major (most often involving its sternocostal portion) associated with anomalies of the ipsilateral upper limb
This syndrome is probably of vascular origin, due to a defect in subclavian artery irrigation (interruption of its development).
The association of Poland syndrome and neoplastic involvement is often found.
The associated anomalies affect the costal cartilages, the thoracic cutaneous tissues, the hand and the forearms, possibly with humero-radio-ulnar shortening and syndactyly
Clinical features
Variable anomalies:
- of the breasts and nipples (absent nipple, absent pectoralis major)
- variable configuration of the hand and forearm (some patients having a normal hand);
syndactyly, symbrachydactyly, hand agenesis - involvement of shoulder muscles
Radiography
Frontal chest radiograph: asymmetry of the soft tissues due to hypoplasia or agenesis of the pectoral muscle
+/- symbrachydactyly on hand radiograph
