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.

Left-sided Poland's syndrome in a girl with rare associations like spina bifida and diaphragmatic hernia

“…Dextroposition of the heart in left-sided Poland's syndrome is not a coincidence but secondary to reduced volume of the left hemithorax 4. Lacorte et al 3 also supports the same view. Our patient also had left-sided diaphragmatic hernia with herniation of part of left lobe of the liver and fundus of the stomach into the left hemithorax which is also a rare but documented association of Poland's syndrome 5.…”

Mild form of Poland syndrome: A case report and review of the literature

“…10 ise %16 sıklıkta bulmuştur. Dekstrokardi bildirilen vakaların hemen hemen tamamında göze çarpan sendromun bu hastalarda sol taraf tutulumu göstermesi ve sağda eşlik eden kosta anomalilerinin olmasıdır 11 . Hastamız sağ taraf tutulumluydu ve beklendiği gibi dekstrokardisi yoktu.…”

Hybrid procedure for Poland syndrome associated with a Gerbode‐type defect

“…It may also be associated with disorders such as vertebral defects, Moebius syndrome, and renal aplasia or hypoplasia (Fokin and Robicsek 2002). Although dextrocardia in Poland syndrome had been reported before (Zhang et al 2011) (Lacorte et al 2010), associated malformations in the cardiovascular system are still very rare. Gerbode-type defect is a LV to RA shunt, which was first introduced in 1958 (Gerbode et al 1958).…”