Severe uropathy and normal amniotic fluid volume in a male fetus: sonographic surveillance leading to the diagnosis of megacystis-microcolon-intestinal hypoperistalsis syndrome.
The 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 fetuses 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 pulmonary 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 rapidly 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 likelihood of recurrence in future gestations.
Bornstein
Eran
E
Division of Obstetric and Gynecologic Ultrasound, Department of Obstetrics and Gynecology, New York University Medical Center, New York, New York 10016, USA. eranbor@yahoo.com
Atkins
Kristin
K
Fishman
Shira
S
Monteagudo
Ana
A
Bajor-Dattilo
Ewa B
EB
Arif
Farzana
F
Monda
Susan
S
Greco
Alba M
AM
Timor-Tritsch
Ilan E
IE
D000015:Abnormalities, Multiple / Q000175:diagnosis; D000328:Adult; D000653:Amniotic Fluid; D003106:Colon / Q000002:abnormalities* / Q000000981:diagnostic imaging; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis*; D006801:Humans; D007422:Intestines / Q000002:abnormalities* / Q000000981:diagnostic imaging; D008297:Male; D010528:Peristalsis; D011247:Pregnancy; D012720:Severity of Illness Index; D013577:Syndrome; D016216:Ultrasonography, Prenatal / Q000379:methods*; D014517:Ureteral Obstruction / Q000175:diagnosis*; D001743:Urinary Bladder / Q000002:abnormalities* / Q000000981:diagnostic imaging