Severe Uropathy and Normal Amniotic Fluid Volume in a Male Fetus Sonographic Surveillance Leading to the Diagnosis of Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome Eran Bornstein, MD, Kristin Atkins, MD, Shira Fishman, MD, Ana Monteagudo, MD, RDMS, Ewa B. Bajor-Dattilo, MD, Farzana Arif, MD, Susan Monda, RDMS, Alba M. Greco, MD, Ilan E. Timor-Tritsch, MD, RDMS he widespread use of sonography as a screening tool for fetal anomalies has facilitated prenatal detection of several fetal conditions characterized by urinary tract dilatation. These conditions are more common in male fetus- es and are generally a result of an anatomic defect causing obstruction along the urinary tract system. Although the prognosis of these conditions largely depends on the specific anomaly, several poor prognostic factors have been described. These factors include detection at an early gestational age, bilateral marked dilatation, a persistently obstructed bladder, oligohydramnios causing pul- monary hypoplasia, and the presence of associated fetal or chromosomal anomalies. We report a case in which a male fetus at 14 weeks’ gestation had a diagnosis of rapid- ly progressing bilateral hydronephrosis, massive bladder dilatation, hydroureter, and a surprisingly normal amniotic fluid volume. Serial sonographic surveillance assisted us in obtaining the correct diagnosis, which was important for adequately consulting the patient regarding the fetal prognosis in the affected index pregnancy as well as the like- lihood of recurrence in future gestations. Received January 29, 2008, from the Division of Obstetric and Gynecologic Ultrasound, Department of Obstetrics and Gynecology (E.B., K.A., S.F., A.M., S.M., I.E.T.-T.) and Department of Pathology (E.B.B.-D., F.A., A.M.G.), New York University Medical Center, New York, New York USA. Revision requested February 4, 2008. Revised manuscript accepted for publication February 7, 2008. Address correspondence to Eran Bornstein, MD, Department of Obstetrics and Gynecology, New York University Medical Center, 462 First Ave, 9E, New York, NY 10016 USA. E-mail: eranbor@yahoo.com Abbreviations MMIHS, megacystis-microcolon-intestinal hypoperistalsis syndrome Case Report A 41-year-old woman, gravida 3, para 1011, underwent a routine early anatomic survey at 14 weeks’ gestation. She was anxious because of a previous first-trimester preg- nancy loss. Her medical history was unremarkable, and her obstetric history was noteworthy for a previous uncomplicated pregnancy resulting in a healthy term neonate and the above-mentioned spontaneous abor- tion. Her partner’s medical history was unremarkable, and there was no history of consanguinity. The current gestation was the result of an uncomplicated in vitro fer- tilization cycle. © 2008 by the American Institute of Ultrasound in Medicine • J Ultrasound Med 2008; 27:1099–1104 • 0278-4297/08/$3.50 T Case Report