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Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome

In Utero Sonographic Appearance and the Contribution of Vesicocentesis in Antenatal Diagnosis

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

Address correspondence to Nobuhiro Hidaka, MD, Department of Obstetrics and Gynecology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashiku 812-8582, Fukuoka, Japan. E-mail: n-hidaka{at}kpa.biglobe.ne.jp

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.

Key Words: fetal urinalysis • megacystis-microcolon-intestinal hypoperistalsis syndrome • sonographic appearance • vesicocentesis

Abbreviations: MMIHS, megacystis-microcolon-intestinal hypoperistal-sis syndrome

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