The main purpose of surgical correction in Poland's syndrome is to improve chest wall symmetry and correct breast hypoplasia. Creation of an anterior axillary fold and smoothing out the infraclavicular defect greatly improves the final result. Cardiorespiratory function may be impaired, but serious conditions requiring early operative correction are rare. When present, unilateral costochondral agenesis involves one to three segments in the mid-anterior chest and sternal depression to that side. Operative planning in such cases includes a multi-layered approach to provide a solid base for soft tissue reconstruction of the more superficial layers.
[Show abstract][Hide abstract] ABSTRACT: Poland's syndrome comprises a spectrum of chest-wall deformities affecting, to various degrees, the rib cage, the chest-wall muscles, neurovascular structures of the ipsilateral arm and the overlying breast. We present a neonate with features of depressed right chest-wall with no respiratory distress. Computed tomography showed hypolasia of the right pectoralis major muscle, with reduced subcutaneous fat on the ipsilateral side of the chest, with abnormalities of the ribs on the same side but absent upper limb deformities. There is negative history of other family members of being affected.
"In 75% of the unilateral cases it is located in the right hemithorax . Reports of bilateral agenesis of the muscle have been infrequently reported in literature [4,5]. Most cases of PS reported are sporadic; however familiar cases have been occasionally described. "
[Show abstract][Hide abstract] 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.
Italian Journal of Pediatrics 02/2010; 36(1):21. DOI:10.1186/1824-7288-36-21 · 1.52 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Poland syndrome (PS) has been described as unilateral pectoral muscle deficiency variably associated with ipsilateral thoracic and upper limb anomalies. Bilateral hypoplasia/aplasia of the pectoralis muscle and upper limb defects in association with variable thoracic muscles, chest wall deformities and lower limb defects have been infrequently reported in the literature. We report on a 3(1/2)-year-old girl with clinical features consisting in bilateral asymmetric pectoral muscle defects (complete agenesis on the left side and agenesis of the sternocostal head on the right side), nipple hypoplasia, left rib defect, and right hand symbrachydactyly. In this study, we reviewed the bilateral features present in our patient and those described in the literature. Hypotheses explaining bilateral features in PS are reviewed.
American Journal of Medical Genetics Part A 07/2009; 149A(7):1597-602. DOI:10.1002/ajmg.a.32922 · 2.16 Impact Factor
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