Left-sided Poland Syndrome Research Articles

Left-sided Poland Syndrome Presenting as Chronic Respiratory Symptoms without Hand Deformity: A Rare Case Report

Left-sided Poland Syndrome Presenting as Chronic Respiratory Symptoms without Hand Deformity: A Rare Case Report

A rare case report of left-sided Poland syndrome in females with hand anomalies and aplastic anemia with pancytopenia

A rare case report of left-sided Poland syndrome in females with hand anomalies and aplastic anemia with pancytopenia

Bilateral Free Deep Inferior Epigastric Artery Perforator Flaps for Reconstruction following Mastectomy in Poland Syndrome Patients.

Poland syndrome is classically described as symbrachydactyly, with hypoplasia of the pectoralis major and other upper thoracic musculoskeletal structures. It is thought to be caused by intrauterine interruption in subclavian arterial flow and often includes breast hypoplasia. Affected vasculature can pose a challenge for reconstruction with free flaps because inflow may not be reliable in this patient population. We present the rare case of a 28-year-old woman with left-sided Poland syndrome, significant family history of breast cancer, and BRCA1+ mutation who underwent bilateral prophylactic nipple-sparing mastectomies with successful immediate bilateral deep inferior epigastric artery perforator free flap reconstruction. The surgical literature in this clinical scenario is also reviewed. Preoperative computed tomography angiography of the chest successfully demonstrated the patency and quantified the caliber of the internal mammary vessels to support free flap breast reconstruction. Free tissue transfer is a viable option for breast reconstruction in patients with Poland syndrome undergoing mastectomy guided by preoperative computed tomography angiography to characterize the internal mammary vasculature.

Poland syndrome

Poland syndrome is a congenital anomaly with unilateral agenesis or hypoplasia of the pectoral muscles, deformity of the anterior chest wall and upper extremity anomalies. In patients with Poland syndrome, varying rates of breast asymmetry, from hypomastia to amastia, accompany. It is observed that the incidence of dextrocardia is increased in patients with left-sided Poland syndrome. Vital lung capacity may be reduced due to anterior chest wall deformity. This syndrome is thought to be due to a temporary impairment of circulation in the arteria subclavia or any of its branches or hypoplasia of these vessels during the development of the upper extremities in the intrauterine period. We aimed to emphasize the characteristics of Poland syndrome in a 26-yearold female case. On physical examination, pectus excavatum deformity was observed besides the absence of left pectoralis major and minor muscles with plica axillaris anterior. In terms of congenital or acquired pathologies that m ay accompany, the cases should be evaluated clinically in detail and followed up.

An unusual case of a neonate with left sided type 3 poland syndrome with dextrocardia

Poland syndrome (PS) is right sided in 75 percent of cases. Only 10 percent are associated with dextrocardia. It is usually detected late for the milder nature of the condition. Only in severe cases it comes to attention during neonatal period. Left sided Poland syndrome with dextrocardia is extremely rare. To the best of our knowledge this is the first case of left sided type 3 Poland syndrome with dextrocardia diagnosed at birth with chest wall getting sucked in and bulging out during inspiration and expiration respectively

Multimodality Imaging of Poland Syndrome With Dextrocardia and Limb Anomalies

Poland syndrome is a rare disorder featuring unilateral anterior thoracic and upper extremity anomalies. These include hypoplasia of the pectoralis major costosternal head, absent pectoralis minor, breast hypoplasia, and absent upper rib cartilage. Pulmonary hypoplasia may occur secondarily. The postulated etiology is underdevelopment of the subclavian artery. Less than 50 cases of Poland syndrome with dextrocardia have been reported. Rib anomalies cause rightward cardiac displacement in fetal life. This case of left-sided Poland syndrome in a young girl is unique not only because of dextrocardia, but also because the condition preferentially afflicts the right side and males.

Dextrocardia in patients with Poland syndrome: Phenotypic characterization provides insight into the pathogenesis

Poland syndrome is a rare congenital anomaly characterized by complete or partial agenesis of the pectoralis major muscle variably associated with other thoracic malformations, upper limb malformations, or both. More than 20 patients with dextrocardia and left-sided Poland syndrome have been previously described. The association between these 2 rare anomalies suggests a causal relationship, but the etiopathogenetic mechanism has not been clarified yet. We studied the clinical correlation between these 2 anomalies, and we tried to elucidate whether dextrocardia or Poland syndrome comes first. This is a multicentric multidisciplinary study conducted over the last 5 years. We identified 122 patients with Poland syndrome, and we investigated heart position through different imaging techniques. Logistic regression statistical analyses were carried out. We observed dextrocardia in 14 (11.5%) patients, which was never associated with situs inversus. All of them presented with left-sided Poland syndrome and partial agenesis of 2 or more ribs. Conversely, all patients with Poland syndrome with partial agenesis of 2 or more ribs presented with dextrocardia, whereas dextrocardia was never associated with partial agenesis of a single rib. Three patients with dextrocardia presented with simple congenital heart defects. These findings suggest that mechanical factors during embryonic life could explain the strong association between left-sided Poland syndrome and dextrocardia. According to this hypothesis, partial agenesis of 2 or more ribs is needed to displace the heart toward the right side. The peculiar features of dextrocardia when associated with Poland syndrome (neither associated with situs inversus nor complex intracardiac anomalies) support our hypothesis.

Dextrocardia Associated With Left-Sided Poland Syndrome

Dextrocardia Associated With Left-Sided Poland Syndrome

Dextrocardia is a component of left-sided Poland syndrome

DOI:€10.1016/j.jtcvs.2005.06.048 J Thorac Cardiovasc Surg 2005;130:1471-1472 Atilla Eroglu, Duran Yildiz and Habil Tunc Dextrocardia is a component of left-sided Poland syndrome http://jtcs.ctsnetjournals.org/cgi/content/full/130/5/1471 located on the World Wide Web at: The online version of this article, along with updated information and services, is

Poland Syndrome and Associated Dextrocardia in Kuwait

During the 15-year period from January 1979 through December 1993, 13 unrelated sporadic cases of Poland syndrome were ascertained in Kuwait. Two of 5 cases (40%) of left-sided Poland syndrome were associated with dextrocardia. This study supports the previous findings of male sex predilection, right-sided predominance and the association of left-sided involvement with dextrocardia. An overview of the disease is presented with particular emphasis on the possible pathogenetic mechanism, worldwide familial cases and the broad spectrum of phenotypic heterogeneity.