| 张琳， 刘俊刚， 李欣.部分型Poland综合征的CT诊断[J].放射学实践,2014,(07):834-836 |
| 部分型Poland综合征的CT诊断 |
| CT diagnosis of partial Poland syndrome |
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| DOI：10.13609/j.cnki.1000 0313.2014.07.025 |
| 中文关键词: Poland综合征 胸壁畸形 体层摄影术，X线计算机 儿童 |
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| 【摘要】目的：探讨部分型Poland综合征的CT表现特征，提高对本病的认识。方法：回顾性分析9例Poland综合征患儿的CT原始图像及VR重组图像，分别观察胸肌、肋骨、肋软骨、乳房、肺部、心脏等情况。结果：9例患儿中，累及右侧5例（55.56%），左侧4例（44.44%）。所有病例胸大肌胸肋部缺如，其中1例累及全部胸大肌。全部患儿胸小肌缺如，7例肋间肌缺如（77.78%），2例肋间肌菲薄（22.22%），4例前锯肌变薄（44.44%），2例背阔肌变薄（22.22%），2例斜方肌变薄（22.22%）。全部患儿患侧胸廓均小于对侧，胸骨向患侧倾斜。3例患儿患侧乳芽缺如（33.33%），6例发育不良并低位（66.67%）。肋骨缺如或发育不良6例（66.67%），肋软骨缺如或发育不良8例（88.89%），高位肩胛2例（22.22%）。4例可见心脏向健侧移位或旋转（44.44%），全部患儿未见肺疝。结论：Poland综合征是一类少见的先天畸形，特征为单侧胸壁发育不良、同侧手部畸形并伴随多种其他畸形。部分型Poland综合征由于不合并手部畸形的表现，临床易漏诊。MSCT可对患儿胸壁肌肉、乳房、骨骼、肺部、心脏及合并畸形进行一站式检查，早期诊断及评价对手术治疗具有重要帮助。 |
| 【Abstract】Objective：To investigate the CT characteristics of partial Poland syndrome and to improve the understanding of that disease.Methods:The CT original images and reconstructed VR images of 9 pediatric cases with Poland syndrome were retrospectively analyzed.The musculi pectoralis,ribs,costal cartilage,breast,lung,heart were investigated respectively.Results:Of the 9 cases,involvement of right side was found in 5 cases (55.56%) and left side (4 cases,44.44%).All cases showed absence of sternocostal part of musculi pectoralis major (100%),including one case having absence of the whole musculi pectoralis major.All cases had musculi pectoralis minor absence (100%),moreover,7 cases had musculi intercostalis absence (77.78%).Thinning of intercostal muscle was found in 4 cases (22.22%),involvement of musculi serratus anterior (4 cases,44.44%),involvement of musculi latissimus dorsi (2 cases,22.22%) and involvement of musculi trapezius (2 cases,22.22%) were observed.The affected side of thorax was smaller than the contralateral side (100%) and the sternum tilted to the affected side (100%).3 patients (33.33%) had absence of ipsilateral preliminary breast,6 patients (66.67%) had mamma dysplasia and erratica. Involvement of rib was revealed in 6 patients (66.67%),of costal cartilage in 8 patients (88.89%),2 patients (22.22%) had elevated scapula.Displacement or rotation of heart toward the non affected side could be seen in 4 cases (44.44%).No lung hernia was observed in all patients.Conclusion:Poland syndrome is a rare congenital malformation,with the characteristics as unilateral chest wall hypoplasia and ipsilateral abnormalities of hand and associated with many other malformations.Partial Poland syndrome could be easily missed in clinical practice due to lack of hand abnormalities.MSCT can be a one stop examination in children with abnormalities of chest wall muscles,bones,breast,lung,heart and other accompanied malformations.Early diagnosis is helpful for treatment planning. |
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