Poland anomaly with dextrocardia: a case report.
Tabish Hazir, Malik Ms
Abstract
Tabish Hazir, Malik Ms
Abstract
Poland anomaly was first reported in Guys Hospital report, 1841. Since then many cases have been described. Among the upper limb developmental defects, Poiand anomaly has a prevalence of 1 in 20,000. According to anotherreport 10% of all cases of upper limb syndactyly have Poland anomaly also. Poland anomaly is a sporadic developmental disorder involving the upper limb. Primary defect seems to be in the development of the proximal subclavian artery, with early deficit of blood flow to the distal limb and the pectoral region, yielding partial loss of tissue in those regions There is a male preponderance with 70% involving the right side. The clinical spectrum is variable depending upon the extent of expression. Upperlimb defect is distal agenesis of varying severity with syndactyly being the commonest presentation. Thoracic deformities include hypoplasia and absence of subcutaneous tissue of anterior chest on the affected side; absence orhypoplasia, with displacement of nipple and breast; absence of sternocostal part of pectoralis major muscle; absence of pectoralis minor and costal cartilage abnormalities.
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Poland anomaly was first reported in Guys Hospital report, 1841. Since then many cases have been described. Among the upper limb developmental defects, Poiand anomaly has a prevalence of 1 in 20,000. According to anotherreport 10% of all cases of upper limb syndactyly have Poland anomaly also. Poland anomaly is a sporadic developmental disorder involving the upper limb. Primary defect seems to be in the development of the proximal subclavian artery, with early deficit of blood flow to the distal limb and the pectoral region, yielding partial loss of tissue in those regions There is a male preponderance with 70% involving the right side. The clinical spectrum is variable depending upon the extent of expression. Upperlimb defect is distal agenesis of varying severity with syndactyly being the commonest presentation. Thoracic deformities include hypoplasia and absence of subcutaneous tissue of anterior chest on the affected side; absence orhypoplasia, with displacement of nipple and breast; absence of sternocostal part of pectoralis major muscle; absence of pectoralis minor and costal cartilage abnormalities.
Key concepts: Syndactyly, Medicine, Poland syndrome, Pectoralis major muscle, Hypoplasia, Dextrocardia, Anatomy, Upper limb