|Year : 2017 | Volume
| Issue : 2 | Page : 126-129
Variable presentation of retrocaval ureter: Report of four cases and review of literature
Muhammed Ahmed, Abdullahi Alhassan, Muhammad Abubakar Sadiq, Ahmad Tijjani Lawal, Ahmad Bello, Hussaini Yusuf Maitama
Department of Surgery, Division of Urology, Ahmadu Bello University, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria
|Date of Web Publication||24-Jul-2017|
Department of Surgery, Division of Urology, Ahmadu Bello University, Ahmadu Bello University Teaching Hospital, Zaria
Source of Support: None, Conflict of Interest: None
Retrocaval ureter is a rare anomaly in which the ureter passes behind the inferior vena cava due to abnormal embryogenesis. Very few cases have been reported from Africa. Although the anomaly is congenital, patients become symptomatic in the third or fourth decade of life. We reviewed the records of four patients with the diagnosis of retrocaval ureter and managed in our centre between January 2010 and December 2016. Three patients presented with recurrent colicky right flank pain while one was asymptomatic. Two patients each had Type I and Type II retrocaval ureters, respectively. Surgical repairs were achieved in the three symptomatic cases and recovery was uneventful. Retrocaval ureter, though congenital, manifests in young adults and it may be symptomatic. Pre-operative diagnosis may be difficult when the lesion is high and mimics pelviureteric junction obstruction. Thus, a high index of suspicion is required for pre-operative diagnosis. Under-reporting and asymptomatic cases may account for the low incidence.
Keywords: Management, presentation, retrocaval ureter
|How to cite this article:|
Ahmed M, Alhassan A, Sadiq MA, Lawal AT, Bello A, Maitama HY. Variable presentation of retrocaval ureter: Report of four cases and review of literature. Niger Postgrad Med J 2017;24:126-9
|How to cite this URL:|
Ahmed M, Alhassan A, Sadiq MA, Lawal AT, Bello A, Maitama HY. Variable presentation of retrocaval ureter: Report of four cases and review of literature. Niger Postgrad Med J [serial online] 2017 [cited 2020 Feb 19];24:126-9. Available from: http://www.npmj.org/text.asp?2017/24/2/126/211461
| Introduction|| |
Retrocaval ureter, also called circumcaval ureter or pre-ureteral vena cava, is a rare congenital anomaly in which the ureter passes posterior to the inferior vena cava (IVC). The proximal ureter deviates medially passing behind the IVC and winding around it before crossing anteriorly and laterally to resume its normal course distally. It occurs in 1 in 1500 live births at autopsy,,, with an overall incidence of about 0.006%–0.17%., It was first reported by Hochstotter in 1893. It is more common in males, with a male-to-female ratio between 3 and 4:1. In clinical practice, reported cases have also shown male preponderance.,,,
The anomaly is thought to occur because of the abnormal formation of infrarenal IVC from anteriorly located subcardinal instead of supracardinal veins which are located posteriorly. In normal circumstances, the infrarenal IVC originates from dorsally located supracardinal vein, but when it develops from ventrally located subcardinal vein, the ureter is trapped posteriorly leading to pre-ureteral vena cava. The subcardinal vein in the lumbar region on the right atrophies under normal circumstances.
It becomes symptomatic usually in the third or fourth decade of life due to hydronephrosis from kinking of the ureter, compression or aperistaltic segment (retrocaval segment).,,, However, symptoms can occur earlier., It usually occurs on the right side but can be on the left side in patients with the very rare situs inversus, duplication of IVC or persistent left subcardinal vein., Most patients present with right flank pain, recurrent urinary tract infection (UTI), renal stones and hydronephrosis. It is also one of the causes of pelviureteric junction (PUJ) obstruction.
Retrocaval ureter may be asymptomatic; discovered only during imaging or surgery for unrelated pathology or at autopsy. It may be associated with other anomalies mainly in the urogenital and cardiovascular systems. Some of the associated anomalies include duplication of IVC, situs inversus, imperforate anus, oesophageal atresia, myelomeningocele, renal agenesis, horse shoe kidney, ureteral duplication, congenital absence of vas deference, hypospadias, syndactyly, intestinal malrotation, VECTERL and Turner's branchial arch or Goldenhar syndromes. Repair usually involves resection of the redundant retrocaval ureteral segment and reanastomosis.
| Case Report|| |
A retrospective review of the clinical records of all patients with the diagnosis of retrocaval ureter and managed in the division of Urology of our University Teaching Hospital was conducted between January 2010 and December 2017. The data extracted are summarised in [Table 1].
|Table 1: Summary of the four cases showing the clinical characteristics of patients, diagnosis and type of retrocavalureter retrocaval ureter|
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A 9-year-old boy presented with 5-month history of right flank colicky pain with occasional fever. There was an episode of passage of stones in urine. Physical examination revealed only mild renal angle tenderness. Abdominal ultrasound showed right hydronephrosis and intravenous urography (IVU) [Figure 1]a showed right hydroureteronephrosis with an 'S' shaped or 'fish hook' deformity of the proximal ureter, which terminated abruptly. A diagnosis of retrocaval ureter was made and the findings at operation were that of right retrocaval ureter, proximal dilated ureteral segment and a normal distal segment lying between the aorta and IVC. The redundant retrocaval segment was mobilised and excised, and end-to-end anastomosis was achieved over a stent. The patient's symptoms resolved at follow-up.
|Figure 1: (a and b) Intravenous urograms of cases 1 (a) and 2 (b) showing the typical Type I retrocaval ureter; moderate hydronephrosis, dilatation of the proximal ureter with a sharp upward curving (‘S' shape or ‘fish hook' deformity) of the proximal ureter and abrupt termination of contrast in the ureter at the level of L3 with non-visualisation of the distal ureteral segment|
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A 32-year-old male presented with recurrent right-sided colicky abdominal pain for over 10 years. The pain was occasionally associated with nausea and vomiting and he had been admitted on two occasions for severe exacerbation of the pain. There was no haematuria or passage of stone in urine. Ultrasound showed right-sided hydronephrosis, which was confirmed on IVU with additional features of a dilated proximal ureter with a gentle medial and upward turn [Figure 1]b. A diagnosis of retrocaval ureter was made. The diagnosis was confirmed at operation through a flank subcostal approach. The retrocaval segment was excised and ureteral continuity was restored over a double J stent with an excellent outcome.
A 38-year-old woman presented to our outpatient clinic with recurrent right flank pain of 4-year duration, the pain was dull aching but occasionally colicky, it was often exacerbated by pregnancy and was relieved by analgesics. On examination, there was no renal angle tenderness and the kidneys were not ballotable. Abdominal ultrasound showed right-sided moderate hydronephrosis. The IVU [Figure 2] confirmed the hydronephrosis with obstruction noted at the PUJ. She had open surgery through a subcostal flank incision with the intent to effect a pyeloplasty, however at operation, marked hydronephrosis was observed with a retrocaval ureter. The redundant retrocaval segment was dissected and excised. Upper tract continuity was restored by Anderson–Hyne's pyeloplasty over a double J stent. Post-operative period was uneventful.
|Figure 2: Intravenous urography of the third patient (case 3) similar to that of pelviureteric junction obstruction|
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A 48-year-old woman referred to our outpatient clinic for evaluation based on an incidental finding on magnetic resonance imaging (MRI) of a right retrocaval ureter while she was being evaluated for peptic ulcer disease. She had no symptoms referable to the right kidney and there was no hydronephrosis. She was managed by observation.
| Discussion|| |
Over 200 cases have been reported worldwide since the first report by Hochstotter in 1893. We could only find a few reported cases in Africa. It is important to bear in mind that under-reporting is common, especially in Africa, and the fact that we managed four cases within a period of 8 years (2010–2017) suggests that it may not be as rare as previously assumed. Although it is congenital in origin, most of the reported cases presented in the third or fourth decade because of the gradual nature of the hydronephrosis  as was the case in our second and third patients. However, presentation can occasionally be early, as in our first case in which symptoms manifested at 9 years of age.
Patients usually complain of right-sided flank pain and features of recurrent UTI, which typify the mode of presentation of our cases. In some cases, haematuria and renal stone may also be present. Our first case passed a stone in urine at some point while the second had haematuria. However, it should be noted that some cases remain asymptomatic and are only incidentally discovered during evaluation for unrelated disease conditions, as was the case in our fourth patient. They may also be discovered at operations for other conditions. While our first and second cases were diagnosed preoperatively, diagnosis was only made at operation in the third case. This was because the IVU of the third patient [Figure 2] displayed the characteristic features of PUJ obstruction with non-visualisation of the subsequent course of the ureter. Thus, pre-operative diagnosis of the patient was that of a PUJ obstruction and he was scheduled to have pyeloplasty until retrocaval ureter was found at operation.
Bateson and Atkinson  classified retrocaval ureter radiologically into two types in 1969. Type I has the typical S-shaped, 'fish hook' or 'Shepherd crook' deformity and associated with extreme medial deviation in 50% of the cases at the level of third lumbar vertebral segment and moderate-to-severe hydronephrosis. This type accounts for most of the symptomatic cases. Type II is associated with a more gentle curve appearing as J-shaped or 'sickle'-shaped deformity with mild medial deviation at the level of renal pelvis; with mild or no hydronephrosis in 10% of the cases and most are asymptomatic. Another classification scheme uses the level of obstruction in which Type I is at the level of third lumbar vertebra and Type II crosses at the level of ureteropelvic junction. In this series, patients 1 and 2 had Type I [Figure 1]a and [Figure 1]b while patients 3 and 4 had the Type II [Figure 2]. However, patients 1, 2 and 3 were symptomatic and had moderate hydronephrosis.
The diagnosis of retrocaval ureter can be confirmed preoperatively with antegrade or retrograde pyelography. Abdominal ultrasound can at best demonstrate the presence of hydronephrosis since it poorly delineates the ureter, while IVU readily demonstrates hydroureteronephrosis with upward curving and abrupt termination of the ureter and non-visualisation of the middle and distal thirds of the ureter as seen in Type I [Figure 1]. Spiral computerised tomography (CT) is considered the investigation of choice compared to IVU because it can delineate both the ureter and IVC. MRI may be better than the CT as it can delineate the course of the entire ureter and is not associated with exposure to radiation as compared to IVU or CT.,, The asymptomatic (4th) case in this series was discovered incidentally on MRI for unrelated condition. Diuretic renography with diethylenetriaminepentaacetic acid or mercaptoacetyltriglycine can assess the level of obstruction and determine the best therapeutic modality.
Asymptomatic patients and those with mild hydronephrosis and infection can be managed non-operatively and followed up subsequently while surgical treatment is required in symptomatic patients or those with severe hydronephrosis. Treatment can be by open or laparoscopic approach. The first successful surgical correction was by Kimbrough in 1935. It involved division of the ureter with or without excision of the narrowed or aperistaltic segment, anteriorisation, uretero-ureterostomy or dismembered pyeloplasty over a stent. These two methods of treatment were used in our cases, but we excised the redundant retrocaval segments in all. Laparoscopic approach through transperitoneal or retroperitoneal route  is less invasive and associated with less morbidity, less post-operative pain, early recovery, short hospital stay and cosmetically acceptable scar. Patients who are treated generally have an uneventful course and an excellent prognosis, as observed in our cases.
| Conclusion|| |
Retrocaval or pre-ureteral vena cava is a rare anomaly with over 200 cases reported worldwide. The cases are under-reported in Africa due to inefficient health information management systems and low or non-visibility of publications. The low clinical incidence may also be attributed to a number of asymptomatic cases that go undetected in the patient's lifetime. Imaging studies are often sufficient to establish the diagnosis preoperatively, some of which are not readily available or may not be affordable to most patients or health facilities in sub-Saharan Africa. Surgical treatment of the symptomatic cases or those with severe hydronephrosis leads to complete resolution of symptoms and restoration of the normal anatomy and function.
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[Figure 1], [Figure 2]