right retrocaval ureterdefault in the embryological development of the inferior vena cava.2...
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REFERENCES 1. Yarmohammadi A, Mohamadzadeh Rezaei M, Feizzadeh B, Ahmadnia H. Retrocaval ureter: a study of 13 cases. Urol J. 2006:175-8 2. Minniti S,Visentini S, Procacci C, Minniti S, Visentini S, Procacci C. Congenital anomalies of the venae cavae: embryological origin, imaging features and report of three new variants. Eur Radiol. 2002: 2040-2055. 3. Perimenis P, Gyftopoulos K, Athanasopoulos A, Pastromas V, Barbalias G. Retrocaval ureter and associated abnormalities. Int Urol Nephrol. 2001:19- 22. 4. Cardoza F, Shambhulinga CK, Rajeevan AT. Retrocaval ureter and contra lateral renal agenesis – a case report and review of literature. Int braz j urol. 2016:842-844. A very rare case of right retrocaval ureter with contralateral renal agenesis METHODS A 35 years old male patient of Indian origin with no previous medical history was further investigated due to the diagnosis of acutisation of a symptomatic non previously investigated hypertension (210/160 mmHg). Laboratory tests revealed a severe kidney failure with a plasmatic creatinine at 420 umol/l and BUN at 19.3 mmol/l. Abdominal ultrasound showed a hyperechogenic right kidney with dilated excretory cavities and a distal ureter which could not be detected. Additionally there was an atrophic left kidney. CT scan and MRI-scan confirmed the dilatation of the right renal excretory cavities with a spiroidal ureter crossing posteriorly of the inferior vena cava. Renal scintigraphy (MAG3) demonstrated that the kidney function was exclusively maintained by the right kidney, with a relatively preserved parenchymal tracer uptake on the right but without any signal on the left side. The patient underwent emergency right sided double-J procedure, and later on elective uretherolisis and pyelouretheroanastomosis, with the goal of retardation of progression to a terminal kidney failure. Intraoperative biopsies of the right kidney revealed a microscopic aspect of terminal kidney probably due to a severe hypertensive nephropathy. DISCUSSION Despite a rapid medical work-up, diagnosis and surgery, it was not possible to improve the kidney function in this young patient. Hypertension in young patients may be the only clinical evidence of an underlying kidney dysfunction. After diagnosis of severe hypertension in younger patients a forced imaging work-up is mandatory in order to detect the pathology early and to manage these patients accordingly. Existing guidelines recommend further investigations in younger patients with severe hypertension, especially if there are no family history and no cardiovascular risk factors. BACKGROUND A retrocaval ureter is a rare congenital anomaly affecting 1 in 1000 newborns 1 . It is the result of a default in the embryological development of the inferior vena cava. 2 Additional congenital anomalies are present in up to 21% of such cases. 3 A retrocaval ureter with contralateral renal agenesis is a very rare entity which has been reported only once up to now. 4 We describe and illustrate such a rare case especially what concerns diagnosis, imaging and evolution. RESULTS Despite these therapeutic procedures no improvement of the kidney function was obtained. The patient is still followed by both our nephrology and urology teams and options, such as a preemptive renal transplantation, are considered. Joao Costa dos Santos 1 , Michael Chilcott 1 , Meryll Cassat 2 , Farshid Fateri 4 , Christine Theodoloz 3 , Bernhard Egger 4 1 Department of Surgery, HFR Riaz 2 Clinic of Nephrology HFR Fribourg 3 Department of Radiology HFR Riaz 4 Department of Surgery, HFR Fribourg – Cantonal Hospital Image 1 and 2- CT scan and MRI findings. It is possible to see the compression of the ureter by inferior cava vein (Image 1) and the diminished flow in the right ureter. In both images we can see both dilation of right renal excretory cavities and the absence of left kidney
Transcript of right retrocaval ureterdefault in the embryological development of the inferior vena cava.2...
REFERENCES
1. Yarmohammadi A, Mohamadzadeh Rezaei M, Feizzadeh B, Ahmadnia H.Retrocaval ureter: a study of 13 cases. Urol J. 2006:175-8
2. Minniti S,Visentini S, Procacci C, Minniti S, Visentini S, Procacci C.Congenital anomalies of the venae cavae: embryological origin, imagingfeatures and report of three new variants. Eur Radiol. 2002: 2040-2055.
3. Perimenis P, Gyftopoulos K, Athanasopoulos A, Pastromas V, Barbalias G.Retrocaval ureter and associated abnormalities. Int Urol Nephrol. 2001:19-22.
4. Cardoza F, Shambhulinga CK, Rajeevan AT. Retrocaval ureter and contralateral renal agenesis – a case report and review of literature.Int braz j urol. 2016:842-844.
A very rare case of right retrocaval ureterwith contralateral renal agenesis
METHODS
A 35 years old male patient of Indian origin with noprevious medical history was further investigated due tothe diagnosis of acutisation of a symptomatic nonpreviously investigated hypertension (210/160 mmHg).Laboratory tests revealed a severe kidney failure with aplasmatic creatinine at 420 umol/l and BUN at 19.3mmol/l. Abdominal ultrasound showed ahyperechogenic right kidney with dilated excretorycavities and a distal ureter which could not be detected.Additionally there was an atrophic left kidney. CT scanand MRI-scan confirmed the dilatation of the right renalexcretory cavities with a spiroidal ureter crossingposteriorly of the inferior vena cava. Renal scintigraphy(MAG3) demonstrated that the kidney function wasexclusively maintained by the right kidney, with arelatively preserved parenchymal tracer uptake on theright but without any signal on the left side. The patientunderwent emergency right sided double-J procedure,and later on elective uretherolisis andpyelouretheroanastomosis, with the goal of retardationof progression to a terminal kidney failure.Intraoperative biopsies of the right kidney revealed amicroscopic aspect of terminal kidney probably due to asevere hypertensive nephropathy.
DISCUSSION
Despite a rapid medical work-up, diagnosis andsurgery, it was not possible to improve the kidneyfunction in this young patient. Hypertension in youngpatients may be the only clinical evidence of anunderlying kidney dysfunction. After diagnosis of severehypertension in younger patients a forced imagingwork-up is mandatory in order to detect the pathologyearly and to manage these patients accordingly.Existing guidelines recommend further investigations inyounger patients with severe hypertension, especially ifthere are no family history and no cardiovascular riskfactors.
BACKGROUND
A retrocaval ureter is a rare congenital anomalyaffecting 1 in 1000 newborns1. It is the result of adefault in the embryological development of the inferiorvena cava.2 Additional congenital anomalies arepresent in up to 21% of such cases.3 A retrocaval ureterwith contralateral renal agenesis is a very rare entitywhich has been reported only once up to now.4 Wedescribe and illustrate such a rare case especially whatconcerns diagnosis, imaging and evolution.
RESULTS
Despite these therapeutic procedures no improvementof the kidney function was obtained. The patient is stillfollowed by both our nephrology and urology teams andoptions, such as a preemptive renal transplantation, areconsidered.
Joao Costa dos Santos 1, Michael Chilcott 1, Meryll Cassat 2,
Farshid Fateri 4 , Christine Theodoloz 3, Bernhard Egger 4
1 Department of Surgery, HFR Riaz2 Clinic of Nephrology HFR Fribourg3 Department of Radiology HFR Riaz
4 Department of Surgery, HFR Fribourg – Cantonal Hospital
Image 1 and 2- CT scan and MRI findings. It is possible to see thecompression of the ureter by inferior cava vein (Image 1) and thediminished flow in the right ureter. In both images we can see bothdilation of right renal excretory cavities and the absence of left kidney
![Retrocaval Ureter with Proximal …...anteriorly and laterally to resume its normal course distally.[1] The condition usually becomes symptomatic in the 3rd or 4th decade of life due](https://static.fdocuments.net/doc/165x107/5f423b4b8d684236a37b0660/retrocaval-ureter-with-proximal-anteriorly-and-laterally-to-resume-its-normal.jpg)
