urethral agenesis
Newborn with
oligohydramnios on prenatal US and bilateral large abdominal masses and Potter’s facies on physical exam. Postmortem CXR and AXR shows an extremely distended abdomen due to bilateral abdominal masses resulting in a small thorax. An umbilical catheter fragment is also present.The diagnosis was urethral atresia causing bladder outlet obstruction resulting in bilateral hydroureteronephrosis and subsequent cystic renal dysplasia.
Urethral agenesis (or urethral atresia) refers to a situation where there is a congenital absence of the urethra. It can be a cause of fetal obstructive uropathy.
Pathology
Associations
- prune belly syndrome
- bladder agenesis
Radiographic features
Antenatal ultrasound
May show a dilated fetal bladder + / - dilated upper tracts.
Ancillary sonographic features include
- presence of oligohydramnios
Prognosis
It is incompatible with life unless an alternative communication between the bladder and amniotic sac exists. In selected situations intra uterine vesico-amniotic shunts can be considered.
Differential diagnosis
For a dilated bladder and upper tract in utero consider
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Assoziationen und Differentialdiagnosen zu urethral atresia: