Peutz-Jeghers综合征的临床及CT表现
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摘要
目的探讨黑斑息肉综合征(PJS)的临床及CT表现。方法收集2014年7月～2016年7月经临床及病理证实的PJS患者资料5例,回顾性分析PJS的临床及CT影像特点。结果 5例均以腹痛为首发症状,伴黑便及多次呕吐入院,CT表现为胃肠道多发息肉,3例合并肠套叠,其中2例为小肠-小肠型肠套叠,1例为结-结型肠套叠; 2例合并肠梗阻,5例均未见明显恶变征象。术后病理结果类型多样,1例增生息肉,1例管状腺瘤息肉,3例错构瘤息肉。结论 PJS患者的主要CT表现为胃肠道多发息肉,可伴肠套叠或肠梗阻,结合病史及临床表现,有助于PJS的确诊。
Objective To investigate the clinical and CT manifestations of Peutz-Jeghers syndrome( PJS). Methods The clinical and CT materials of 5 cases with PJS confirmed by clinical and pathology from July 2014 to July 2016 were collected,andthe CT features were retrospectively analyzed. Results All the five patients presented with abdominal pain,vomit and melena,multiple gastrointestinal polyps and intussusception were shown on CT,3 cases of merger of intussusception,including 2 cases ofjejunojejunal intussusception,1 case of knot-junction intussusception. 5 cases were no signs of malignant transformation. Patholo-gy revealed that there were hyperplastic polyps,and polypoid tubular adenomas for 1 case each,3 cases with hamartomatous pol-yps. Conclusion The main CT features of Peutz Jeghers syndrome are multiple gastrointestinal polyps and intussusception,withor without intussusception or intestinal obstruction. Combining with the history and clinical manifestations is helpfue for PJS confirmation.
Objective To investigate the clinical and CT manifestations of Peutz-Jeghers syndrome( PJS). Methods The clinical and CT materials of 5 cases with PJS confirmed by clinical and pathology from July 2014 to July 2016 were collected,andthe CT features were retrospectively analyzed. Results All the five patients presented with abdominal pain,vomit and melena,multiple gastrointestinal polyps and intussusception were shown on CT,3 cases of merger of intussusception,including 2 cases ofjejunojejunal intussusception,1 case of knot-junction intussusception. 5 cases were no signs of malignant transformation. Patholo-gy revealed that there were hyperplastic polyps,and polypoid tubular adenomas for 1 case each,3 cases with hamartomatous pol-yps. Conclusion The main CT features of Peutz Jeghers syndrome are multiple gastrointestinal polyps and intussusception,withor without intussusception or intestinal obstruction. Combining with the history and clinical manifestations is helpfue for PJS confirmation.
引文
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[9]余捷,吴恩福,陈玲,等.多层螺旋CT在黑斑息肉综合征的诊断价值[J].实用放射学杂志,2014,30(8):1412-1414.
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[11]袁柏思,汪芳裕. Peutz-Jeghers综合征小肠息肉的诊治技术现状和药物治疗进展[J].胃肠病学及肝病学杂志,2013,22(2):185-190.
[12]康素海,程广河,李改兰,等.黑斑-胃肠道息肉综合征息肉恶变途径分析[J].中华临床医师杂志,2015,9(10):3498-3502.
[2] Zheng B,Pan J,Wang Y,et al. Analysis of STK11 gene variantin five Chinese patients with Peutz-Jeghers syndrome[J]. DigDis Sci,2013,58(3):2868-2872.
[3]陈方慧,王弋. Peutz-Jeghers综合征诊治进展[J].中华危重症医学杂志,2014,7(6):53-55.
[4]于妍妍,徐榕苑,白云. Peutz-Jeghers综合征胃肠道息肉及癌变机制的研究进展[J].医学综述,2012,18(23):4016-4018.
[5] Shaco-Levy R,Jasperson KW,Martin K,et al. Morphologiccharacterization of hamartomatous gastrointestinal polyps in Cow-den syndrome,Peutz-Jeghers syndrome,and juvenile polyposissyndrome[J]. Human pathology,2016,49(3):39-48.
[6]袁岸龙,寇继光,夏冰.黑斑息肉综合征患者临床特点调查分析[J].中华消化杂志,2011,31(6):417-419.
[7]葛亮,刘洋,高剑波,等. CT在黑斑息肉综合征中的应用价值[J].放射学实践,2016,31(1):68-71.
[8]冯瑞,宋云龙,王萍,等.多层螺旋CT在黑斑息肉综合征中的应用价值[J].中国医学装备,2016,13(4):67-71.
[9]余捷,吴恩福,陈玲,等.多层螺旋CT在黑斑息肉综合征的诊断价值[J].实用放射学杂志,2014,30(8):1412-1414.
[10]颜禄斌,李斌德,李来元,等.黑斑息肉综合征及其并发症2例报告并文献复习[J].临床小儿外科杂志,2013,12(5):427-429.
[11]袁柏思,汪芳裕. Peutz-Jeghers综合征小肠息肉的诊治技术现状和药物治疗进展[J].胃肠病学及肝病学杂志,2013,22(2):185-190.
[12]康素海,程广河,李改兰,等.黑斑-胃肠道息肉综合征息肉恶变途径分析[J].中华临床医师杂志,2015,9(10):3498-3502.