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A case of Poland Syndrome associated with dextroposition

A case of Poland Syndrome associated with dextroposition,10.1186/1824-7288-36-21,Italian Journal of Pediatrics,Doriana Lacorte,Maria Marsella,Pietro G

A case of Poland Syndrome associated with dextroposition   (Citations: 1)
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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.
Journal: Italian Journal of Pediatrics - Ital J Pediatr , vol. 36, no. 1, pp. 21-3, 2010
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    • ...These include absence of the minor pectoralis or total absence of both pectoralis muscles, hypoplasia of the 2nd/3rd/4th or 3rd/4th/5th ribs, hypoplasia of the scapula and its muscles and infrequent manifestations such as dextrocardia [18, 28], pneumothorax at the affected side [5], shoulder dislocation [30], abnormal hemostasis [10] and thrombocytopenia [20], growth hormone deficiency [19] or facial nerve palsy [15]...
    • ...Clinicians firstly observe the abnormal hemithorax and then pay attention to other possible features of the syndrome [18, 23, 28]...
    • ...Poland’s syndrome has been referred to cause transposition of the heart in many cases [18, 28]...
    • ...When Poland’s syndrome occurs on the left side, the heart is moved to the right and it seems in some cases to cause total dextroposition of the heart [18, 28]...
    • ...12 Lacorte D. et al. [18] 1 – Left Hypoplasia of both pectoralis muscles and reduction of the intercostal spaces, 3rd–5th rib...

    Kapetanakis Stylianoset al. Muscle abnormalities of the chest in Poland’s syndrome: variations and...

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