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      Off-pump coronary artery bypass in poland syndrome with dextrocardia: case report

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          Abstract

          Poland Syndrome is a congenital disorder characterised by hypoplasia of the pectoral muscles along with upper extremity deformities. We encountered a patient with Poland syndrome associated with dextrocardia and also failed pectus excavatum repairs who presented to us with symptomatic ischaemic heart disease requiring intervention. He underwent successful off-pump coronary artery bypass surgery (OPCABG). As far as we are aware, this is the first case report of OPCABG in a case of Poland syndrome with dextrocardia. We describe here the management of this complex patient and wish to emphasise that the off-pump option is feasible in dextrocardia with some technical modifications.

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          Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil, and Möbius anomalies.

          A hypothesis is presented to explain the pathogenesis of the Poland, Klippel-Feil, and Möbius anomalies, isolated absence of the pectoralis major with breast hypoplasia, isolated terminal transverse limb defects, and the Sprengel anomaly. We propose that these conditions are the result of an interruption of the early embryonic blood supply in the subclavian arteries, the vertebral arteries and/or their branches, and hypothesize that the occlusions occur at specific locations in these vessels during or around the sixth week of embryologic development and produce predictable patterns of defects. The term subclavian artery supply disruption sequence (SASDS) is suggested for the group of birth defects represented by the above conditions. Possible causes for interruption of embryonic blood supply are discussed.
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            Poland's syndrome revisited.

            Poland's syndrome is a rare congenital anomaly characterized by unilateral chest wall hypoplasia and ipsilateral hand abnormalities. Literary data suggest its sporadic nature. The prevailing theory of its cause is hypoplasia of the subclavian artery or its branches, which may lead to a range of developmental changes. The incidence of Poland's syndrome varies between groups (male versus female patients, congenital versus familial cases, and so on) and ranges from 1 in 7,000 to 1 in 100,000 live births. Cases of Poland's syndrome associated with leukemia, carcinoma of the hypoplastic breast, and other conditions, confirm the relationship between developmental defects and tumors, and require oncologic awareness. Various manifestations, age, and gender require different surgical approaches. Our experience, which includes 27 patients (15 male, 12 female), 20 of whom (12 male, 8 female) underwent operation, suggests that the repair should be done in two stages in children and in a single stage in adults. Reconstruction and/or stabilization of the aplastic ribs may be achieved using bone grafts or prosthetic mesh. Muscle flaps and breast implants may be used to correct muscle deficiency and breast hypoplasia and to help achieve a complete cosmetic repair.
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              Should you stand on the left or the right of a patient with dextrocardia who needs coronary surgery?

              A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was which side of the operating table you should stand on when carrying out surgical revascularization on a patient with dextrocardia. Altogether 40 papers were found using the reported search, of which 19 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes, and results of these papers are tabulated. The side on which the operating surgeon stood was mentioned in 20 out of the 24 cases. Surgery was carried out from the conventional right side of the patient in 5 cases, while in 10 cases, it was carried out from the left side. The surgeon needed to switch sides to facilitate surgery in three cases. In addition, the right internal mammary artery (RIMA) was anastomosed to the left anterior descending artery (LAD) in 16 cases. Of these, surgery was carried out from the left side in 11 cases. The left internal mammary artery (LIMA) to LAD anastomosis was carried out in two cases, one of which was a free LIMA graft. In six cases, only vein grafts were used. Fourteen cases were carried out using cardiopulmonary bypass while 10 cases were carried out as off-pump cases with one conversion. The majority of patients were operated on from the left of the table. More cases were performed with the RIMA as the conduit of choice to the LAD.
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                Author and article information

                Journal
                J Cardiothorac Surg
                Journal of Cardiothoracic Surgery
                BioMed Central
                1749-8090
                2011
                18 May 2011
                : 6
                : 75
                Affiliations
                [1 ]Department of Cardiothoracic Surgery, Lancashire Cardiac Centre, Victoria Hospital, Blackpool, FY3 8NR, UK
                [2 ]Department of Cardiology, Lancashire Cardiac Centre, Victoria Hospital, Blackpool, FY3 8NR, UK
                Article
                1749-8090-6-75
                10.1186/1749-8090-6-75
                3120655
                21592399
                44102940-c523-4723-ac83-3986981af3d8
                Copyright ©2011 Srivastava et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 13 March 2011
                : 18 May 2011
                Categories
                Case Report

                Surgery
                opcab,coronary artery bypass graft surgery,off-pump surgery,cabg,pectus excavatum,poland syndrome

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