diagnosis and treatment of the circumcaval...

Diagnosis and Treatment of the Circumcaval Ureter

Andrea Salonia *, Carmen Maccagnano, Arianna Lesma, Richard Naspro,Nazareno Suardi, Giorgio Guazzoni, Francesco Montorsi, Patrizio Rigatti

Department of Urology, Universita Vita-Salute San Raffaele, Milan, Italy

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Keywords:AnatomyAnomaliesCircumcavalDiagnosisImagingRetrocavalTreatmentUreter

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Abstract

Objectives: Updated review about topographic anatomy, clinical andradiologic diagnosis, and management of the circumcaval ureter.Methods: We used Ovid and PubMed (updated January 2006) to conduct aliterature electronic search on MEDLINE which included peer-reviewedarticles. Moreover, we analysed previous publications regarding both‘‘retrocaval’’ and ‘‘circumcaval’’ ureter.Results: Circumcaval ureter predominantly involves the right ureter; itsexact prevalence is still unknown. The anomaly occurs 2.8 times morecommonly in males than females clinically as well. Although the lesion iscongenital, symptoms usually present in the third to fourth decade of life;the patients mostly present with right flank pain and discomfort. Intra-venous urogram and retrograde urography were commonly used to diag-nose a circumcaval ureter. A spiral computed tomography scan and, morerecently, Magnetic Resonance Imaging, have been suggested to differen-tially diagnose a circumcaval ureter as methods of choice. The openureteroureterostomy remained the gold standard surgical approach totreat the circumcaval ureter for many years; however, minimally invasivelaparoscopic procedures have almost replaced open surgery.Conclusions: The circumcaval ureter is a rare congenital anomaly usuallyassociated with upper urinary tract hydronephrosis. Spiral computedtomography scan and Magnetic Resonance Imaging have been recentlyconsidered the tool of choice for the diagnosis of inferior vena caval abnor-malities. Comparisons among historical reports regarding open surgeryand laparoscopic transposition and reanastomosis of a circumcaval ureterhave clearly shown the advantages of minimally invasive approaches.

# 2006 Published by Elsevier B.V.

* Corresponding author. U.O. di Urologia, Istituto Scientifico Universitario Ospedale SanRaffaele, Via Olgettina 60, 20132 Milan, Italy. Tel. +39 02 26437286; Fax: +39 02 26437298.E-mail address: [email protected] (A. Salonia).

1. Topographic anatomy

The incidence of circumcaval ureter develops pre-dominantly on the right side, but when it is reported

1569-9056/$ – see front matter # 2006 Published by Elsevier B.V. doi

on the left side, this congenital anomaly is asso-ciated with either partial or complete situs inversus orduplication of the inferior vena cava (IVC) [1–3]. This

:10.1016/j.eursup.2006.02.009


latter anomaly is a not uncommon variation that iscaused by the persistence of all or part of thesupracardinal vessels and presents an incidence of1%–3% [4]. Recently Wang et al. [5] reported the caseof a 21-year-old man who presented with rightflank pain for about one month and had theradiological finding of moderate hydronephrosisand a right upper ureteral obstruction. The ureterwas tapered and bent superiorly at the level ofapproximately L4. Due to the complete absence ofstones or other significant abnormal findings, acircumcaval ureter was strongly suspected. Atsurgery the authors found that the ureter waskinked by an aberrant vessel, which drainedlaterally into the IVC. The inferior venocavographydemonstrated one duplicated vessel about 0.8 cmin diameter and 6 cm long that originated from theventral side of the IVC and subsequently drainedlaterally into the cava. Pierro et al. [6] reported twocases of a left-sided circumcaval ureter secondaryto persistent left subcardinal vein without situsinversus or caval duplication. Bilateral circumcaval

Fig. 1 – Gladstone’s variant of retrocaval ureter (modified

from Gladstone RI. Development of the inferior vena cava

in the light of recent research, with special reference to

certain abnormalities, and current descriptions of the

ascending lumbar and azygos veins. J Anat 1929;64:70).

Fig. 2 – Hochstetter’s variant of retrocaval ureter (modified

from Hochstetter F. Beitrage zu Entwicklungeschichte des

Venensystems der Amnioten. Morphol Jahrb 1933;20:543).

ureters have also been reported [7]. Several variantsin the anatomic and topographic presentation ofthe anomalous circumcaval ureter have beenobserved in humans. Figs. 1–10 show few variantsbetween those described in the literature [8]. Cir-cumcaval ureters have been associated with severaltypes of embryologic anomalies; recently an unusualpresentation was reported in a 20-year-old womanwith the notable findings of a circumcaval rightureter, Ladd’s bands, and incomplete intestinalmalrotation that resulted in a six-month history ofintermittent right lower quadrant pain [9].

Circumcaval ureters have been classified intotwo clinical types across the literature, in accor-dance with the common radiological appearance(Fig. 11) [10–12]. Type 1 (also named ‘‘low loop’’) hasbeen reported as the more common form; it ischaracterized by the so-called ‘‘typical S’’ or ‘‘fish-hook’’ deformity of the ureter to the level of theobstruction, with the point of obstruction placedsome distance from the lateral margin of the IVC atthe level of the third lumbar vertebra [13]. In thetype 2 variant (also called ‘‘high loop’’), the ureterhas a ‘‘sickle-shaped’’ curve, with the point ofobstruction at the lateral margin of the IVC. Thissecond variant is rare, and represents around 10%of the known cases.

e u r o p e a n u r o l o g y s u p p l e m e n t s 5 ( 2 0 0 6 ) 4 4 9 – 4 6 2 451

Fig. 5 – Kengyel’s variant of retrocaval ureter (modified

from Kengyel H. Ueber einer eigenartige Ursache der

Hydronefrose. Ztschr f Urol Clin 1928;25:417).

Fig. 3 – Kolisko’s variant of retrocaval ureter (modified from

Kolisko F. Ein Fall abnormen Verlaufes des rechten

Ureters. Anat Anz 1909;34:520).

Fig. 4 – De Gironcoli’s variant of retrocaval ureter (modified

from De Gironcoli F. Retrocaval ureter. Acta Chiru Hung

1962;3:137).

Type 1 is usually associated with moderate tosevere hydronephrosis in 50% of the patients andtype 2 with mild or no hydronephrosis [10–12].Indeed, in the latter form, the upper ureter is non-kinked but passes behind the IVC at a higher level;the renal pelvis and upper ureter lies almosthorizontal before it encircles the IVC with a smoothcurve. The ureter is compressed against the peri-vertebral tissue.

Horseshoe kidney is the most common fusionanomaly of the kidney. It occurs in one in 353 casesto one in 1800 cases [14,15]. A great variety ofassociated congenital anomalies have occurred withhorseshoe kidney, but horseshoe kidney accompa-nied by a totally circumcaval ureter has rarely beenreported in the same patient (Fig. 12) [16–22].Moreover, both features are well recognized asuncommon anomalies of the genitourinary systemand rarely is the diagnosis of both anomalies madebefore surgical intervention [18].

2. Clinical diagnosis

As previously introduced, patients who suffer fromboth types of circumcaval ureter usually present in


Fig. 6 – Skamnakis’ variant of retrocaval ureter (modified

from Skamnakis SN. Eine Anomalie der Vena Cava inferior

und abnormale Verlauf des rechten Ureters. Anat Anz

1931;73:50).

Fig. 7 – Wicke’s variant of retrocaval ureter (modified from

Wicke A. Ueber ein Fall Inselbildung im Bereiche der

hinteren Hohlvene und Durchtritt des rechten Harnleiters

beim Erwachsenen. Ztschr f Anat u Entwicklungsgesch

1927;84:524).

their third to fourth decade of life with right flankpain and discomfort caused by ureteral obstructionand resulting hydronephrosis [8,12,23–29]. Painassociated with the hydronephrosis can be inter-mittent, dull, and aching. Patients may also com-plain of urinary tract infections, gross haematuria,or fever [8,12,23–26,29]. Haematuria in varyingdegrees is often present. Calculi occasionally co-exist with a circumcaval ureter. Fillo et al. [29]reported a case of gross haematuria in a patient witha carcinoma of the retrocaval ureter.

Kenawi and Williams [12] reported that in theircohort of patients, 52.5% complained of flank pain,17.9% had significant kidney colic, 23.5% sufferedfrom urinary tract infections, and 21.6% hadhaematuria.

The hydronephrosis in patients with a retro-caval ureter is mainly described as a consequenceof the compression by the psoas muscle, spinalcolumn, and IVC on the anomalous ureter [24,30].In patients who suffer from retrocaval ureter, therenal pelvis and upper ureter are typically elon-gated and dilated in a ‘‘J’’ or fish-hook shape beforethey pass behind the IVC. However, ureteral

obstruction is a frequent but not an inevitablecomplication [31].

3. Radiologic diagnosis

Intravenous urogram (IVU) and retrograde urogra-phy were commonly used to diagnose a circumca-val ureter [8,12] (Figs. 13 and 14). The IVU in theearly stage of ureteral stenosis may only show adilatation of the renal pelvis, calyces, and upperureter above the site of obstruction. Typically, IVUcan fail to visualize the portion of ureter thatextends behind the IVC; a retrograde ureteropye-logram, however, may help demonstrate thetypical fish-hook curve of the upper ureter towardsthe midline, with the retrocaval segment at thelevel of L3 or L4 [8,12,24,32,33].

A spiral computed tomography (CT) scan hasbeen recently considered the tool of choice for thediagnosis of IVC abnormalities and circumcavalureter [2,13,34–36]. In 1999, Pienkny et al. suggestedthat a three-dimensional volume-rendered CT scanin the excretory phase, combined with diuretic


Fig. 8 – Gierke’s variant of retrocaval ureter (modified from

Gierke E. Abnormer Verlauf des rechten Ureters bei

Entwicklunganomalie der unteren Holvene. Ztschr f Urol

Chir u Gynak 1928;25:279).

Fig. 9 – Rotter’s variant N. 1 of retrocaval ureter (modified

from Rotter H. Dorsaler Ureterverlauf bei Abnormitaten der

unteren Hohlvene. Ztschr f Anat u Entwicklungsgesch

1935;104:456).

renography, could be the radiologic evaluation ofchoice for patients with a suspected circumcavalureter, as it is non-invasive and accurately deter-mines the anatomic relationship of the IVC andureter [37]. CT can also represent the procedure ofchoice to confirm an ultrasound diagnosis and avoidretrograde ureteropyelography [38–41]. Spiral CT,which can simultaneously outline the ureter andIVC free of respiratory mis-registration and motionartefacts, may produce overlapping images, andmay represent the diagnostic modality of choice,because it enables the surgeon to avoid the possiblecomplications and expenses of other invasiveprocedures [13].

More recently, however, Uthappa et al. [42]reported data that relate to the potential use ofmagnetic resonance imaging (MRI) to differentiallydiagnose a circumcaval ureter with equal effective-

ness of a spiral CT scan but without radiation risk(see Chapter 3 in this supplement).

In terms of differential diagnosis, circumcavalureter must be differentiated from retroperitonealmass and retroperitoneal fibrosis. Typically, in thefirst case the IVU often reveals lateral deviation ofthe ureter from the compression of the mass. Whenretroperitoneal fibrosis occurs, the ureter is involvedon both sides and the whole segment of the ureter isstiff in the IVU feature [24].

4. Personal case report

A 14-year-old Moroccan boy, normal 46, XY kar-iotype, was referred to the Department of Paedia-trics for failure to thrive (height: 140.6 cm = �3 DS;weight = 32.3 kg), which was documented also by atotal skeleton X-ray that showed a dorsal kyphosisand growth delay. He was the second-oldest of foursiblings born from Moroccan parents; it has not beenpossible to establish a kinship with certainty.

During the hospitalisation he was submitted tovarious instrumental evaluations, among which an


Fig. 10 – Rotter’s variant N. 2 of retrocaval ureter (modified

from Rotter H. Dorsaler Ureterverlauf bei Abnormitaten der

unteren Hohlvene. Ztschr f Anat u Entwicklungsgesch

1935;104:456).

echocardiogram and a cardiac MR, which documen-ted the presence of a right aortic arc, with a right-positioned descending aorta that goes down pro-gressively, anterior to the dorsal vertebral columnbringing itself leftwards in correspondence of thediaphragmatic passage. The cardiac MR also showeda pervious oval foramen, with a diameter of 8 mm,and small ectasia of both atria.

Paediatric examination: hypertelorism, synophriswith dark and thick eyebrows, lengthened filter,small mouth with thinned superior lip, micrognazia,hypoplasia of the dental enamel. Slightly flattenedshort nasal septum, with rounded off tip, ogivalpalate, thickened ear tents were also documented.The boy also suffered from ligamentous laxity,clynodattilia in the left hand, significant bilateralhallux valgus, and gait with intrarotation of thelimb, coupled with valgism of the hind foot. Theophthalmic visit found a bilateral iris coloboma.

The urological history underlined an interventionof bilateral orchipexy at the age of three; enuresiswas still persistent at the time. The physicalexamination showed hypotrophy of the right testiscompared with the contra lateral (testis ultrasound:right testicle = 23 � 15 � 11 mm [2.1 cc]; left testi-cle = of 26 � 15 � 12 mms [2.7 cc]; US presence ofsome small [1–2 mm] hyperechogenic area, possiblyfibrotic, in the left testis).

Abdomen ultrasound was positive for a smalldegree of right dilatation of the pelvis with rightlumbar ureter of a diameter equal to 16 mm. Thecystogram resulted positive for sphincter dyssiner-gia, but negative for vesico-ureteral reflux. The boythus underwent IVU. Although the boy was com-pletely asymptomatic, at 14 years and five months aright circumcaval ureter was diagnosed during anIVU (Figs. 15–17).

The paediatricians diagnosed a recessive auto-somal syndrome called spondylocarpotarsal synos-tosis syndrome and planned a substitutive therapywith growth hormone.

Moreover, the boy had an intervention of rightureteroplasty with a right flank surgical access alongthe prolongation of the XI rib. After the right kidneywas found, a lysis of adherences between the rightcircumcaval ureter and the IVC was performed.Subsequently, a dismembered pyeloplasty wasdone, after having uncrossed the ureter from theIVC. A pyeloureteral anastomosis was then com-pleted using a PDS 6/0 running suture, thus, a D-Jureteral stent was placed. The boy had a regularpostoperative hospital stay, with the removal of theindwelling catheter after a 24-hour period and thehospital discharge during the second postoperativeday. The D-J ureteral stent was then removed at the30-day postoperative visit. A three-month follow-upultrasound evaluation showed mild hypotonia ofthe right pelvis without any sign of hydronephrosis.At 20-month follow-up, a renal scintigraphy docu-mented a bilateral normal renal function, coupledwith a minimal right-sided pyelectasis.

5. Treatment

Various techniques for the management of circum-caval ureter have been reported.

In patients with minimal calicectasis and nosubjective symptoms, surgical correction is notmandatory, but observation should be maintained.Therefore, circumcaval ureter has been defined arare congenital anomaly that requires surgicalcorrection in the symptomatic patients [33]. Con-servative treatment and periodical examination


Fig. 11 – Diagrammatic representation illustrating the radiological features of Type I (left side) and Type II (right side)

circumcaval ureter (modified from Bateson and Atkinson. Circumcaval ureter: a new classification. Clin Radiol 1969;20:173–7).

Fig. 13 – Diagrammatic representation of the IVU feature of

right retrocaval ureter (personal case).

Fig. 12 – Diagrammatic representation of a right retrocaval

ureter and horseshoe kidney (modified from Heffernan

et al. Horseshoe kidney with retrocaval ureter: second

reported case. J Urol 1978;120:358–60).

should be given to those patients without hydrone-phrosis, infection, and stone formation. A periodicexamination has been also suggested for patientswith mild hydronephrosis.


Fig. 14 – Diagrammatic representation of the IVU feature of

right retrocaval ureter (modified from Uthappa et al.

Retrocaval ureter: MR appearances. Br J Radiol

2002;75:177–79).

Fig. 15 – 30-min prone excretory urography showing right-

sided hydronephrosis and dilatation of the proximal

ureter up to the level of the transverse process of L3 in a

14-year-old asymptomatic boy (personal case).

Fig. 16 – Particular of the prone excretory urography in the

14-year-old boy with a type I circumcaval ureter (personal

case).

When either obstructive symptoms or kidneyfunction caused by circumcaval ureter worsen,surgical correction is indicated to preserve renalfunctioning and to provide long-term symptomaticrelief [5,43,44].

5.1. Surgical treatment

Open surgery is the standard treatment and isusually successful. Division of the dilated renalpelvis with transposition and reanastomosis wasinitially described by Harril [45] and has been themost popular form of treatment in patients withsymptomatic hydronephrosis. Both flank and trans-abdominal approaches have been used for thisprocedure.

The choice of open surgery approach dependedprimarily on the severity of the hydronephrosis,impairment of kidney function, and the type ofanomaly [8,24].

Most of the authors that suggested an uretero-pelvis anastomosis also advocated Harril’s method[45] by which a section is made at the level of thepelvis just above the ureteropelvic junction. Char-


Fig. 17 – Post-voidal i.v. urogram feature showing a

persisting right-sided hydronephrosis and dilatation of

the proximal ureter in the 14-year-old boy with a type I

circumcaval ureter (personal case).

Fig. 19 – Puigvert’s technique. After transposition of the

ureter, a ureteropyeloplasty was performed (modified

from Bissi and Rigatti. L’uretere circumcavale. Anatomia

chirurgica – Diagnostica –Terapia. Eds Casa Editrice

Ambrosiana. pp 1–149, 1977).

Fig. 18 – Resection of the hydronephrotic pelvis throughout

the ureteropyeloplasty according to Puigvert et al.

(modified from Bissi and Rigatti. L’uretere circumcavale.

Anatomia chirurgica – Diagnostica –Terapia. Eds Casa

Editrice Ambrosiana. pp 1–149, 1977).

acteristic of this approach is that the abundantvascular supplies of the pelvis and proximal uretermay be kept, and postoperative stricture at theanastomotic site is less likely. Xiaodong et al. [24]underlined that when an 8F catheter could not passthrough the circumcaval segment easily, the steno-tic section of the ureter must be excised, and adismembered pyeloplasty should be done. Puigvertet al. [46], for instance, supported the resection ofthe pelvis just above the ureteropelvic junction, witha subsequent ureteropyeloplasty, just leavinguntouched the segment of the ureter previouslyallocated in a retrocaval fashion (Figs. 18–20).

A number of open surgical approaches havesuggested the transection of the ureter with theuretero-ureteric anastomosis and repositioningin a normal anatomic relationship with the IVC[8,24]. These techniques suggested either theureterotomy or the resection of the stenotic ureterat the site of the segment of ureter compressedby the IVC [47], distally and close to the bladder with


Fig. 21 – Goodwin’s technique. Transposition of the

circumcaval ureter from the inferior vena cava (modified

from Bissi and Rigatti. L’uretere circumcavale. Anatomia



Fig. 20 – Puigvert’s technique. A running suture was also

put to close the upper part of the pelvis (modified from

Bissi and Rigatti. L’uretere circumcavale. Anatomia



Fig. 22 – Goodwin’s technique. Surgical division of the

inferior vena cava (modified from Bissi and Rigatti.

L’uretere circumcavale. Anatomia chirurgica – Diagnostica

–Terapia. Eds Casa Editrice Ambrosiana. pp 1–149, 1977).

Fig. 23 – Goodwin’s technique. Reanastomosis of the

inferior vena cava (modified from Bissi and Rigatti.

L’uretere circumcavale. Anatomia chirurgica – Diagnostica

–Terapia. Eds Casa Editrice Ambrosiana. pp 1–149, 1977).

a concomitant ureterocystostomy [48], or proxi-mally to the ureteropelvic junction [49].

Other surgical options include nephrectomy forthe non-functioning kidney, because of severehydronephrosis and infection [8,24].

In 1957 Goodwin et al. [50] proposed a surgicalapproach aimed to divide and re-anastomose theIVC successfully after relocating the ureter ante-riorly (Figs. 21–23). This technique had greatoperative risks and potentially severe complica-tions. Supporters underlined that it was a goodchoice of treatment, particularly when there wasabsence or disease and malfunction of the contral-ateral kidney.

Xiaodong et al. [24] also suggested to use a so-called ‘‘vena cava supporter’’ (Fig. 24) mainly forcases with lumen stenosis caused only by torsionand compression by the IVC. Adhesive tissue aroundthe circumcaval ureter was well dissected, and thetorsion of the ureter was corrected. A chemical ormetal tool, as well as surrounding soft tissue such


Fig. 24 – Inferior vena cava supporter (modified from

Xiaodong Z et al. Diagnosis and treatment of retrocaval

ureter. Eur Urol 1990;18:207–10).

as the greater psoas muscle, were then placedbetween the IVC and the ureter.

Major criticisms of any kind of open surgery arethat it may require a large skin incision and causesignificant postoperative pain; moreover, the con-valescence might be complicated by wound painand infection.

5.2. Laparoscopic repair of the circumcaval ureter

The open ureteroureterostomy remained the goldstandard surgical approach to treat the circumcavalureter for many years [51]. In the past 10 years,however, technological breakthroughs coupled withthe intensive growth of minimally invasive laparo-scopic procedures have almost replaced opensurgery [52,53].

After that a laparoscopic dismembered pyelo-plasty for ureteropelvic junction stenosis wasreported by Schuessler et al. in 1993 [54], laparo-scopic transposition and reanastomosis of a cir-cumcaval ureter were also performed [55–64]. Thelaparoscopic dismembered pyeloplasty rapidlydemonstrated the obvious advantage of minimaldisfigurement and morbidity to the patients (seeChapter 4 in this supplement).

Baba et al. [55] reported a dismembered pyelo-plasty under transperitoneal laparoscopy. Theseauthors decided to mobilise the entire ascendingcolon by incising the right paracolic gutter aroundthe inferior margin of the cecum, which providedthe best exposure of the retroperitoneal area to theright of the left renal artery above and the inferiormesenteric artery below. The IVC was easilydissected by retracting the ascending colon towardthe midline of the abdomen with the operating

table tilted to keep the patient in the half-recum-bent position. With blunt dissection and gentletraction, the postcaval segment of the right ureterwas safely dissected with instruments insertedthrough two 11-mm trocars placed in the midcla-vicular line. A 5-mm port placed in the middleaxillary line was used for dissection and to lift theextrarenal pelvis and the upper third of the ureter.The difficulty of reapproximating the ureter andthe renal pelvis accounted for almost one-third ofthe operative time. The renogram parametersobtained at two-month follow-up showed markedresolution of the obstruction [55].

A transperitoneal laparoscopic approach was alsoused by others [56,57,59]. Matsuda et al. [56], forinstance, reported that in their 38-year-old patient apart of the ureter, which was about 3 cm long andlocated behind the IVC, had to be removed because itlooked narrow and dysplastic. An end-to-endreanastomosis of the upper and lower ends of theureter was then performed laparoscopically. At theend of the procedure the retroperitoneum wasincised laterally to the ascending colon was com-pletely closed. Bleeding during the procedure wasless than 30 ml; the postoperative period wasuneventful and the patient complained of minimalpost-surgical pain and received no analgesics.

More recently, Ameda et al. [62] reported data thatrelated to the case of a 20-year-old woman whosuffered from a circumcaval ureter that wasdiscovered as she was being treated for urolithiasis.After a spontaneous stone discharge, right hydro-nephrosis remained and a diuretic renography with99mTc-DTPA showed no definite obstruction. Thiswoman was transabdominally laparoscopicallyapproached. Under general anaesthesia the patientwas placed in a modified flank position with theright leg slightly elevated, which eased intraopera-tive manipulation of the ureteral catheter. Opentrocar placement was done at the umbilicus(12 mm), then the second port (10 mm) at themidclavicular line below the costal margin, thethird port (10 mm) midway between the anterosu-perior iliac spine and the umbilicus, and the fourthport (5 mm) was placed on the anterior axillary lineat the same level of the umbilicus (Fig. 25). Theascending colon was reflected medially to exposethe retroperitoneum. After its identification, theright ureter was divided just distal to the crossing ofthe IVC. Therefore, the ureter was repositioned to lieanterior to the IVC itself and then the stenoticportion of about 2 cm was transacted. Tension-freeanastomosis with interrupted 4-0 absorbablesutures was performed with an intracorporealsuturing technique. Total surgical time was 7.5 h


Fig. 25 – Positions of the four trocars placed by Ameda et al.

for the transperitoneal laparoscopic approach in a 20-year-

old woman. Namely, square dot: 5-mm port; small round

dots: 10-mm ports; large round dot: 12-mm port (modified

from Ameda K et al. Laparoscopic ureteroureterostomy for

retrocaval ureter. Int J Urol 2001;8:71–74).

with estimated blood loss less than 20 ml. Thepost-operative course was uneventful without anycomplications.

Salomon et al. [60] in 1999 reported the first caseof purely retroperitoneal laparoscopic repair of acircumcaval ureter, which suggested that in theirpatient the retroperitoneal laparoscopy representedthe more direct approach to the urinary tract. Theoperative time was significantly shorter than withthe previously reported transabdominal laparo-scopic approach [56,57]. Salomon hypothesized thatthe shorter time was obtained because dissection ofthe retroperitoneal space was not hindered by intra-abdominal organs [60]. Mugiya et al. [61] confirmedthat the retroperitoneoscopic treatment could besuperior to the conventional transabdominalapproach to perform the laparoscopic transpositionand reanastomosis of a circumcaval ureter. Theyalso reported the use of an automatic suture device[65] to retroperitoneoscopically correct a circumca-val ureter [61].

The main limiting factor for both the transab-dominal and the retroperitoneal laparoscopic repairof the circumcaval ureter was the intracorporealanastomosis of the ureter that significantlyincreased the surgical time [55,56,60]. Mugiya et al.

[61] first used an automatic suture device to reducethe surgical time. More recently, Tobias-Machadoet al. [66] reported a case of retroperitoneoscopicsurgery coupled with extracorporeal uretero-uret-eral anastomosis for treating circumcaval ureter ina 24-year-old woman who complained of recurringlumbar colic pain associated with repeated epi-sodes of acute pyelonephritis on the same side.After having observed the ureteral point with theleast tension for exteriorization, the ureter wassectioned at that site. The ureteral stumps werethus exteriorized through the incision of the 12-mm port, after having enlarged the skin incision to20 mm. The proximal and distal ureteral marginswere resected and a double running spatulatesuture (namely, posteriorly first and anteriorlysubsequently) was performed. Total surgical timewas 130 minutes; the end-to-end anastomosis took40 minutes.

6. Conclusions

The circumcaval ureter is a rare congenital anomalycaused by an error in the embryogenic developmentof the IVC. It is usually associated with upper urinarytract hydronephrosis, and patients present in theirthird to fourth decade of life with right flank painand discomfort. Patients may also complain ofurinary tract infections, haematuria, or fever.

Intravenous urogram and retrograde urographyhave been commonly used to diagnose a circumca-val ureter. However, a spiral CT scan has beenrecently considered the tool of choice for thediagnosis of IVC abnormalities and circumcavalureter. Even more recently, the potential use of theMRI to differentially diagnose a circumcaval ureterwas suggested to have equal effectiveness of a spiralCT scan but without radiation risk.

Comparisons between historical reports aboutopen surgery and laparoscopic transposition andreanastomosis of a circumcaval ureter have clearlyshown the advantages of minimally invasiveapproaches, less intraoperative bleeding, specifi-cally a shorter post-operative hospital stay, reducedpostoperative pain, earlier return to daily activities,and a significant superior aesthetics effect, whilepreserving therapeutic efficacy.

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