Poland Syndrome


George Mallat

Case Report

This is a case of a 71-year-old gentleman with Poland syndrome. His clinical findings include absence of his right pectoralis muscles, shortening of his right arm, and previous webbed fingers in the right hand which have been surgically corrected with a skin graft.

Poland Syndrome (PS) is characterized by unilateral, partial or complete absence of the sternocostal head of the major pectoral muscle and ipsilateral shortened, webbed fingers (brachysyndactyly). In most cases, the pectoralis minor muscle is also absent. Patients will usually have a sunken chest wall, due to hypoplasia of the ribs and cartilage, and may display a high-riding scapula (Sprengel deformity). Dextrocardia, lung herniation, renal, vertebral and lower limb malformations have been described in rare cases.   

PS occurs in sporadic fashion, and reported incidence ranges from 1 in 10,000 to 1 in 100,000. The exact cause of PS is unknown; however, the current theory is that it results from an interruption of blood supply to the branches of the subclavian and vertebral arteries, known as “Subclavian Artery Supply Disruption Sequence”, during the sixth week of embryogenesis.