A girl was born at 36 weeks of gestation, with no pathological findings on prenatal screening, with vaginal delivery and cephalic presentation.
On physical examination after birth, vesicular murmur is abolished in the lower 2/3 of the left lung field and there is an abdominal mass dependent on the left renal fossa with posterior peloteus.
Chest X-ray and pulmonary scintigraphy showed left lung hypoplasia.
Abdominal ultrasound did not detect the presence of the left kidney, so a thoracic ultrasound examination was performed, showing left intrathoracic kidney with duplicity pyeloouret.
Parenchymatous stricture of the cortex of the upper pyelone and tortuosus ureter was observed, dilating to the bladder in normal situation.
The lower hemi-rain has mild ectasia with preserved cortical bone.
Normal right kidney.
Intravenous urography, computed tomography angiography and magnetic resonance imaging were performed, revealing a left kidney in an intrathoracic situation, probably due to diaphragmatic hernia, with severe dilation of the ureter in the superior hemi-rhinoma associated with a hernia.
Lower pylon and right kidney were normal.
The left kidney has anomalous vascularization, depending on the superior pyelone of the pulmonary artery and the inferior pyelone of the aorta.
Right renal vascularization was normal.
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Renal scintigraphy shows an abnormally high left kidney with good tracer uptake in its lower pyelone and functional superior annulation.
With the diagnosis of left pyeloureteral duplication with intrathoracic kidney, annulation of the upper pyelone and diaphragmatic hernia, we proceeded to left upper polar nephroureterectomy via retroabdominal urethra, renal mobilization, pepexy
The postoperative course was uneventful.
Follow-up visits at 6 months with ultrasound, UIV and DMSA showed a kidney located in the left renal fossa with normal morphology and functionality.
After 3 years of follow-up in controls and controls with DMSA, both renal morphology and functionality remain stable.
