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产前超声诊断胎儿膈下隔离肺1例

2018-10-29 吕白雪 姜镔 王学梅 中国医学影像技术

孕妇33岁,孕1产0,因“孕24+2周产前超声检查发现胎儿腹部包块”就诊,无家族遗传病史。超声检查显示胎儿左肾上方肾上腺区见大小约2.1 cm×1.7 cm×1.5 cm高回声包块,类圆形,内部回声均匀,边界清晰,与肾上腺分界清(图1A)。CDFI示高回声包块内部供血动脉起源于腹主动脉(图1B)。超声诊断为膈下隔离肺。

孕妇33岁,孕1产0,因“孕24+2周产前超声检查发现胎儿腹部包块”就诊,无家族遗传病史。超声检查显示胎儿左肾上方肾上腺区见大小约2.1 cm×1.7 cm×1.5 cm高回声包块,类圆形,内部回声均匀,边界清晰,与肾上腺分界清(图1A)。CDFI示高回声包块内部供血动脉起源于腹主动脉(图1B)。超声诊断为膈下隔离肺。
 
图1 胎儿膈下隔离肺 A.超声示胎儿左肾上腺区高回声包块(箭);B.CDFI示供血动脉起源于腹主动脉;
 
孕妇孕26周超声复查,发现病变无明显改变,孕27周经穿颅术引产一男性胎儿,尸体解剖发现肿物位于胎儿左肾上腺右上方、胰腺后方,重约7g,包膜完整,表面光滑(图1C),病理检查见发育异常、结构紊乱的肺组织,包括囊性扩张的支气管及畸形血管,病理诊断为隔离肺(图1D)。
 
图1 C.尸体解剖示肿块位于左肾上腺右上方、胰腺后方(箭);D.病理检查示肿块内存在紊乱的肺组织(HE,×400)
 
讨论 
 
隔离肺是少见的先天性肺发育畸形,发病率约为0.15%~1.70%。隔离肺的病因是在肺发育过程中,连接原始主动脉与原始肺的血管未退化,高压血流压迫肺组织,使其发生囊性变和纤维性变,形成无功能的肺组织团,异常血供大多来自胸主动脉和腹主动脉。未合并其他畸形的隔离肺胎儿预后较好,可能于产前逐渐缩小或消失,目前治疗原则为手术切除。90%以上的隔离肺位于胸腔,左肺与膈肌之间多见,少数位于胸腔其他部位、心包和膈下(左侧多见),本例为膈下型。
 
典型隔离肺超声表现为边界清晰的高回声团,圆形、三角形或楔形,大多内部回声均匀,少数内部可观察到囊肿,为扩张的支气管或并存肺囊腺瘤,随访观察生长缓慢或缩小消失。CDFI发现其血供来自体循环为特征性表现。膈下隔离肺需与肾上腺神经母细胞瘤相鉴别,膈下隔离肺与肾上腺分界清晰,可见患侧完整的肾上腺;肾上腺神经母细胞瘤与肾上腺分界不清,常为分叶状或不规则形,肿瘤内部回声杂乱,可不同程度包绕下腔静脉和腹主动脉,肿瘤生长速度较快。
 
原始出处:

吕白雪,姜镔,王学梅.产前超声诊断胎儿膈下隔离肺1例[J].中国医学影像技术,2018(02):274.

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    2018-10-31 changjiu

    谢谢分享,学习一下。

    0

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    2018-10-31 qilu_qi
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    2018-10-30 医者仁心5538

    学习了

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