Poland syndrome
Clinical:
Poland syndrome is a rare congenital malformation of the chest wall consisting of a variable degree of hypoplasia or aplasia of the pectoralis major muscle and ajacent cartilagenous, osseous, and soft tissue structures [1]. Scoliosis and upper extremity anomalies such as syndactyly may also be rpesent [2]. The disorder is felt to be the result of ipsilateral subclavian artery disruption and is nearly always unilateral [1]. Males are affected more than females (2-3:1) and the right side is involved in 60-75% of cases [1]. Brachysyndactly is an associated anomaly [1].
X-ray:
The condition produces hypolucency of the affected hemithorax on CXR [1]. At CT, there is ipsilateral partial or complete absence of the chest wall musculature and hypoplasia of the ipsilateral ribs [2].
REFERENCES:
(1) Radiol Clin N Am 2005; Fefferman NR, Pinkney LP. Imaging evaluation of chest wall disorders in children. 43: 255-370
(2) Radiographics 2011; Dillman JR, et al. Expanding upon the unilateral hyperlucent hemithorax in children. 31: 723-741
