Abstract
Poland's syndrome is a rare congenital anomaly characterized by agenesis or hypoplasia of the pectoralis major and minor muscles, agenesis of costal cartilages and absence of anterior parts of ribs, abnormalities of breast, hypoplasia of subcutaneous tissue and unilaterally brachysyndactyly. In some cases other associated ipsilateral anomalies have been described. One of the most important associated anomalies is dextrocardia. We report five cases of left-sided Poland syndrome combined with dextrocardia to discuss the correlations between Poland syndrome and dextrocardia.
The diagnosis of left sided Poland‘s syndrome with dexrocardia was identified in 5 patients between July 2006 and November 2011. All of the 5 patients were male and their mean age was 20.6 (19-24). There were left pectoral muscle agenesis and hypoplasia of subcutaneous tissue in all of the patients. There were amastia and athelia in one patient, hypomastia and hypothelia in 4 patients, agenesis of costal cartilages and absence of anterior parts of ribs in 2 patients, brachydactyly in one patient, right sided pectus carinatum in one patient, pectoral and axillary alopesia in 3 patients, sternal defect in one patient, agenesis of serratus anterior inferior in one patients. All cases were sporadic.
Dextrocardia has been reported in approximately 20 patients with Poland syndrome. In all these patients, Poland syndrome was left-sided, although Poland syndrome usually occurs on the right.
As a result, our cases support the view that dextrocardia may be a part of the left-sided. Poland syndrome. Further studies would be helpful to confirm this combination.
- © 2013 ERS