A case of Poland Syndrome associated with dextroposition

Abstract

Classical Poland Syndrome (PS) is characterized by unilateral, partial or complete absence of the sternocostal head of the major pectoral muscle and brachysyndactyly of fingers on the same side. We report the case of a newborn infant with dextrocardia and PS located on the left side. This association is very rare: to date only 19 cases have been described in scientific literature. In all reported cases, as in the present, the Poland defect involved the left side and was associated to rib defects, whereas most cases of PS are on the right side and few have rib defects. This case supports the view that dextrocardia follows the loss of volume of the left hemithorax caused by Poland sequence and that the combination of PS and dextrocardia is not coincidental.

Figure 1

Depression of the left anterior chest wall (1a); brachysyndactyly of the second, third and fourth finger of the left hand (1b).

Figure 2

Chest X-ray: asymmetric chest with reduction of the third to fifth left intercostal spaces and dextrocardia (2a). Left hand X-ray: hypoplasia of the main phalanx of the I finger, absence of the intermediate phalanx of the II and III fingers, and substitution of the intermediate phalanx of the IV and V fingers with a small ossification nucleous (2b).