目的 探讨Poland综合征的多层螺旋CT（multislice spiral computer tomography，MSCT）特征及鉴别诊断。 方法 回顾2014年10月至2022年1月9例Poland综合征患者（男8例、女1例）临床及影像资料，分析其CT特征。患者年龄18~83岁，中位年龄37（27，56）岁，平均（42±20）岁；1例因明显胸廓异常就诊，余8例为胸部CT检查时偶然发现。 结果 9例均为单侧发病（右侧5例、左侧4例），均有患侧胸小肌缺如，胸壁软组织变薄、塌陷，均无同侧短指或/和并指畸形；胸大肌发育不良7例，完全缺如2例。MSCT表现为原胸肌区域未见正常的肌肉组织密度灶，仅有轻度下陷的均匀低密度脂肪组织，胸壁皮肤连续。重组图像表现为患侧胸部稍下陷，两侧乳头位置不对称，对侧胸壁形态正常。其中1例复杂胸廓畸形CT表现为胸壁多重畸形，包括左侧胸大肌发育不良、胸小肌与前锯肌缺如、骨性胸廓塌陷以及第3、4肋与乳头发育不良，胸壁皮下软组织变薄，心右偏。 结论 Poland综合征的CT表现具有特征性，可通过MSCT横轴位图像结合重组图像逐层观察胸壁肌肉及骨的发育解剖，明确诊断该病并作出鉴别。
Abstract
Objective To investigate the characteristics and differential diagnosis of Poland syndrome by Multi-slice Spiral Computed Tomography (MSCT). Methods The clinical and imaging data of 9 patients (8 males and 1 female) with Poland syndrome from October 2014 to January 2022 were collected, and their CT features were retrospectively analyzed. The ages of patients ranged from 18 to 83 years old, with an average age of (42±20) years old. . One patient was treated for obvious thoracic abnormality, and the other 8 patients were found accidentally during chest CT examination. Results All 9 cases were unilaterally involved, with 5 cases on the right side and 4 cases on the left side. Pectoralis major dysplasia was observed in 7 cases and complete absence in 2 cases. All patients’ pectoralis minor were absent, and the soft tissue of the chest walls were thin and collapsed. The MSCT images showed no normal muscle tissue density in the original pectoral muscle area, and the low-density of subcutaneous adipose tissue was uniform, while the skin of chest wall was continuous. Reconstructed images showed loss of the pectoralis and subcutaneous soft tissue in the affected side, the position of the two papillae was asymmetrical, while the structure of contralateral pectoral muscle was normal. Among them, multiple malformations of the chest wall was shown on the one patient’s CT, which included pectoralis major dysplasia, pectoralis minor absence, serratus anterior absence, skeletal thoracic collapse, the 3rd and 4th rib dysplasia, nipple dysplasia, thinning of the subcutaneous soft tissue of chest wall, and dextrocardia. Conclusions The CT manifestations of Poland syndrome are typical. By combination of axial MSCT images and multiplanar reconstruction, the anatomical structure of chest wall muscles and bones can be clearly shown, which helps to make definite diagnosis and differentiation.
关键词
Poland综合征 /
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先天畸形 /
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胸壁异常 /
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胸大肌发育不良 /
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MSCT
Key words
Poland syndrome /
Congenital malformation /
Abnormal thoracic wall /
Pectoralis major dysplasia /
Multislice spiral computer tomography (MSCT)
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参考文献
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