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Click Prenatal US images of a 27 weeks old female fetus with the diagnosis of Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) using a Philips scanner with a 3.5 MHz curved transducer. FINDINGS: There is a single fetus in a breech presentation with anteriorly located placenta and increased amniotic fluid volume. The abdominal circumference is abnormally enlarged. There is marked distension of the urinary bladder with no evidence of bladder wall thickening or filling defect. The kidneys appear normal in echogenicity and no cystic changes are identified within the parenchyma. There is bilateral hydroureteronephrosis, right greater than left. There is no evidence of perinephric collection or fetal ascites. The constellation of findings is in keeping with megacystis microcolon hypoperistalsis syndrome or lower urinary tract obstruction. The increased amniotic fluid volume could be secondary to the diminished peristalsis in gastrointestinal tract.

Click MR images of a female fetus with a diagnosis of MMIHS at 27 weeks of gestational age in planes. (Protocol: 1.5T MR scanner, T2 Half Fourier Acquisition Single Shot Turbo Spin Echo (HASTE) sequence, TR=1100, TE=84, no contrast). FINDINGS: There is a single fetus in breech presentation with anterior placenta and a slightly increased amount of amniotic fluid volume. There is marked bilateral pelvicaliectasis with renal parenchymal thinning and tortuous, dilated bilateral ureters. The urinary bladder is massively enlarged without obvious wall thickening. The urethra is not distended. The entire colon is diminutive in size. The stomach is normal in appearance. There is no ascites. The constellation of findings is in keeping with megacystis microcolon hypoperistalsis syndrome.

Click MR images of a female fetus with a diagnosis of MMIHS at 27 weeks of gestational age in coronal plane: (Protocol: 1.5T MR scanner, T2 Half Fourier Acquisition Single Shot Turbo Spin Echo (HASTE) sequence, TR=1100, TE=84, no contrast). FINDINGS: There is a single fetus in breech presentation with anterior placenta and a slightly increased amount of amniotic fluid volume. There is marked bilateral pelvicaliectasis with renal parenchymal thinning and tortuous, dilated bilateral ureters. The urinary bladder is massively enlarged without obvious wall thickening. The urethra is not distended. The entire colon is diminutive in size. The stomach is normal in appearance. There is no ascites. The constellation of findings is in keeping with megacystis microcolon hypoperistalsis syndrome.

Click MR images of a female fetus with a diagnosis of MMIHS at 27 weeks of gestational age in sagital plane: (Protocol: 1.5T MR scanner, T2 Half Fourier Acquisition Single Shot Turbo Spin Echo (HASTE) sequence, TR=1100, TE=84, no contrast). FINDINGS: There is a single fetus in breech presentation with anterior placenta and a slightly increased amount of amniotic fluid volume. There is marked bilateral pelvicaliectasis with renal parenchymal thinning and tortuous, dilated bilateral ureters. The urinary bladder is massively enlarged without obvious wall thickening. The urethra is not distended. The entire colon is diminutive in size. The stomach is normal in appearance. There is no ascites. The constellation of findings is in keeping with megacystis microcolon hypoperistalsis syndrome.

Click A contrast enhanced CT scan of the abdomen was performed with pediatric dose reduction protocols at the age of 2 months in this female infant with megacystis microcolon hypoperistalsis syndrome (MMIHS) (protocol: 36mAs, 80kV, 3mm slice thickness, with 8ml Iohexol (Omnipaque 300, GE health care, Princeton, NJ) injection). FINDINGS: The esophagus is distended with gas and fluid. There is an enteric tube within the esophagus which terminates in the stomach. In the abdomen, there is hepatomegaly. There is bilateral ureteropelvocaliectasis with mild renal renal parenchymal thinning. The small bowel loops are markedly dilated and fluid filled. The colon is diminutive and not well visualized. There is no free intraperitoneal gas. No focal extraluminal fluid collections are visualized in the abdomen or pelvis. The urinary bladder in the pelvis is distended there is no ascites.