Clinical Analysis of 113 Patients With Poland Syndrome
Clinical Analysis of 113 Patients With Poland Syndrome
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DOI：
10.1016/j.athoracsur.2014.10.036
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发表时间：
2015-03-01
影响因子：
4.6
通讯作者：
Oksuz, Sinan
中科院分区：
文献类型：
--
作者：
Yiyit, Nurettin;Isitmangil, Turgut;Oksuz, Sinan
背景。波兰综合征是一种罕见的先天性异常，其特征是胸肌部分或完全缺失、不同程度的胸部畸形和手部异常。迄今为止，已经报道了这种综合征的许多变体及其伴随的异常。方法。 1990年至2014年间，我们诊所有113名患者被诊断患有波兰综合征。其中6名患者接受了背阔肌移植术。结果。 113 例患者中，63 例（55.7%）诊断为右侧综合征，42 例（37.1%）诊断为左侧综合征，8 例（7%）诊断为双侧综合征。所有患者均检测到胸大肌部分或完全缺失。尽管 81 名（71.6%）患者完全缺乏胸大肌，但 32 名（28.3%）患者仅缺乏胸大肌。在分析的病例中，还发现波兰综合症伴有特定的异常现象。这些患者中最常见的伴随波兰综合征的异常是 Sprengel 畸形，在 18 名患者中出现。 6例患者通过背阔肌移植术实现了胸壁的对称性和稳定性。结论。波兰综合征是一种罕见的先天性异常，有多种变异和伴随异常。波兰综合征患者除胸肌外还可见其他几块肌肉的缺失。 Sprengel 畸形是最常见的伴随异常。据报道，有几种针对该综合征的外科手术方法。例如，就稳定胸壁和提供最佳对称身体外观而言，转位背阔肌是一种有效的手术。 (C) 2015 年，胸外科医师协会
Background. Poland syndrome is a rare congenital anomaly characterized by the partial or complete absence of pectoral muscles, varying thoracic deformities, and hand anomalies. To date, many variants of this syndrome and its accompanying anomalies have been reported.Methods. In our clinic, 113 patients were diagnosed with Poland syndrome between 1990 and 2014. A latissimus dorsi muscle transfer was performed on 6 of these patients.Results. Out of 113 patients, 63 (55.7%) were diagnosed with the syndrome on the right side, 42 (37.1%) were diagnosed on the left side, and 8 (7%) had a bilateral diagnosis. The partial or complete absence of the pectoralis major muscle was detected in all patients. Although 81 (71.6%) patients had a complete absence of the pectoralis major muscle, 32 (28.3%) were lacking only the sternocostal head of the muscle. In the analyzed cases, Poland syndrome was also found to be accompanied by specific anomalies. The most common anomaly accompanying Poland syndrome in these patients was Sprengel deformity, seen in 18 patients. Symmetry and stabilization of the chest wall were performed in 6 patients through transfer of the latissimus dorsi muscle.Conclusions. Poland syndrome is a rare congenital anomaly, which has several variants and accompanying anomalies. The absence of several muscles in addition to the pectoral muscle can be seen in patients with Poland syndrome. Sprengel deformity is the most common accompanying anomaly. Several surgical procedures have been reported for the syndrome; for example, transposing the latissimus dorsi muscle is an effective procedure in terms of stabilizing the chest wall and providing optimum symmetric body appearance. (C) 2015 by The Society of Thoracic Surgeons