P09.24: Prenatal diagnosis: megalocystis‐microcolon‐intestinal hypoperilstalsis syndrome (MMIHS)
Annotatsiya
MMIHS is a rare non-obstructive cause of megalocystis with a 4 : 1 female predominance. The syndrome involves not only a distended bladder but also functional small bowel obstruction and microcolon. It is important to differentiate this syndrome from the more common posterior urethral valves as reflected in olygohydroamnion while fetal bladder shunting is not indicated. A 22 y.o. woman primagravida presented with a positive pregnancy test for prenatal medical examination. The microbiological, biochemical and cytological test were normal. Ultrasonography at 10 weeks; NT, NB at 14 weeks were normal. There were no complications during pregnancy. Karyotype was normal too. At 22 weeks ultrasonography showed a little ablations of the pelviocaliceal systems of kidneys and cystic formation was found in the pelvis of the female fetus. That cystic formation occupied almost 2/3 parts of fetus' abdomen while hypoplastic lungs, small colon and hypoperilstalsis of intestine were also indicated. Amniotic index coincided to gestational age. Criteria indicating or contra-indicating intrauterine treatment were discussed. In respect that, the fetal survival was defined by complex of malformation and worsening fetus' bradicardia the medical interruption of pregnancy was proposed. Autopsy showed hypoplastic lungs, large abdomen with fulfilled with megalocystis functioning posterior urethral valve, urinary ascites, bladder calcifications, very small atretic intestine and colon. Our results are in concordance with earlier ultrasound examinations demonstrating that intrauterine treatment in this case was ineffective. Supporting information can be found in the online version of this abstract. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.