儿童Peutz-Jeghers综合征超声表现

目的总结儿童Peutz-Jeghers综合征(色素沉着-胃肠道多发性息肉综合征)超声声像图特点,探讨超声检查对Peutz-Jeghers综合征的诊断价值.方法 回顾性分析我院经手术病理确诊的19例Peutz-Jegher综合征患儿的超声表现.所有患儿均经消化道内镜或开腹手术行肠息肉摘除术.结果 19例患儿中,1例(5.3%)为单发息肉,其余18例(94.7%)均为多发息肉,其中1例患儿最多发现16枚息肉.本组于术中共发现息肉89枚,超声诊断79枚,诊断符合率88.8%(79/89).息肉最小为0.8 cm×0.5 cm×0.8 cm,位于结肠脾曲;息肉最大为7.6 cm×2.7 cm×3.6 cm,位于直肠上段.息肉超声声像图表现为呈放射状分布的高低相间回声结节,彩色多普勒血流成像示血流信号丰富.11例Peutz-Jeghers综合征患儿合并肠套叠,其中小肠套叠10例,声像图均表现为"同心圆"征象,套叠头端显示为息肉.结论 Peutz-Jeghers综合征患儿超声表现具有一定特征性,术前超声检查诊断准确率高,具有独特的优越性.

The sonographic characteristics of Peutz-Jeghers syndrome in children

Objective To summarize the sonographic characteristics of Peutz-Jeghers syndrome in children and investigate the diagnostic value of ultrasound.Methods Retrospective analysis of sonographic characteristics of 19 cases with pathologically-confirmed Peutz-Jeghers syndrome were conducted.Results Except for one child,other 18 patients all had more than one polyp with the maximum of sixteen.The polyp with the smallest size of 0.8 cm×0.5 cm×0.8 cm was found in left colic flexure,while the largest one was found in superior rectal with a measurement of 7.6 cm×2.7 cm×3.6 cm.The polyps showed radiatiform echotexture.And hypoechoic and hyperechoic components were present alternately.Blood flow was easily identified within these polyps by color Doppler sonography.Intussusceptions were observed in 11/19 patients.Among them,10 cases were located at small intestine.The sonographic finding was a concentric structure with a polyp at the top.Conclusions The sonographic appearence of Peutz-Jeghers syndrome is characteristic.Before surgery,ultrasonographic examination could achieve a accurate diagnosis for Peutz-Jeghers syndrome.