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巨膀胱-小结肠-肠蠕动不良综合征:宫内超声表现及膀胱穿刺术在产前诊断中的作用

Megacystis-microcolon-intestinal hypoperistalsis syndrome: in utero sonographic appearance and the contribution of vesicocentesis in antenatal diagnosis.

作者信息

Hidaka Nobuhiro, Kawamata Kazuya, Chiba Yoshihide

机构信息

Department of Perinatology, National Cardiovascular Center, Osaka, Japan.

出版信息

J Ultrasound Med. 2006 Jun;25(6):765-9. doi: 10.7863/jum.2006.25.6.765.

Abstract

OBJECTIVE

The purpose of this report is to describe the in utero sonographic appearance of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) and examine the role of vesicocentesis.

METHODS

Two cases of the sonographic appearance of MMIHS were reviewed. We performed vesicocentesis in 2 fetuses with MMIHS and performed vesicoamniotic shunting in 1 of them. The sonographic findings after these procedures were compared with those before puncture.

RESULTS

In both cases, ultrasound examination showed a massively enlarged fetal bladder and bilateral hydroureteronephrosis with a normal amount of amniotic fluid. Fetal urinary sodium and chloride concentrations were within normal limits in both cases; this suggested normal fetal renal function. Generally, it is difficult to detect the dilated fetal bowel in MMIHS, probably because of the disturbance caused by a large bladder. In our cases, however, the ultrasound examination clearly showed fetal bowel dilatation after these procedures; this made the antenatal diagnosis of MMIHS more definite.

CONCLUSIONS

The sonographic features after vesicocentesis may be helpful in confirming the antenatal diagnosis of MMIHS.

摘要

目的

本报告旨在描述巨膀胱-小结肠-肠蠕动不良综合征(MMIHS)的宫内超声表现,并探讨膀胱穿刺术的作用。

方法

回顾了2例MMIHS的超声表现。我们对2例MMIHS胎儿进行了膀胱穿刺术,并对其中1例进行了膀胱羊膜分流术。将这些操作后的超声检查结果与穿刺前的结果进行比较。

结果

在这2例病例中,超声检查均显示胎儿膀胱明显增大,双侧肾盂输尿管积水,羊水量正常。2例胎儿尿钠和氯浓度均在正常范围内,提示胎儿肾功能正常。一般来说,MMIHS中扩张的胎儿肠道很难被检测到,可能是由于大膀胱造成的干扰。然而,在我们的病例中,超声检查在这些操作后清楚地显示了胎儿肠道扩张,这使得MMIHS的产前诊断更加明确。

结论

膀胱穿刺术后的超声特征可能有助于确认MMIHS的产前诊断。

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