Retrocaval Ureter: Report of 12 Cases and Literature Review

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1 Case Analyses Retrocaval Ureter: Report of 12 Cases and Literature Review Kuo-How Huang, Shiou-Chun Chang 1, Shih-Chieh Chueh, Chao-Yuan Huang, Yeong-Shiau Pu, Jun Chen Department of Urology, National Taiwan University Hospital, Taipei, Taiwan R.O.C, 1 Far-Eastern Memorial Hospital, Taipei, Taiwan, R.O.C. OBJEIVE: Retrocaval ureter is a rare congenital anomaly. For clarification of clinical presentations, radiological features, diagnosis, and treatment outcomes, we retrospectively reviewed 12 cases in our hospital. MATERIALS AND METHODS: From January 1989 to December 2004, 12 cases with retrocaval ureter were treated in our hospital. Their medical records were analyzed, and the relevant literature was reviewed. RESULTS: Among the 12 cases, there were 11 males and 1 female aged 15~71 (mean, 32.3) years. The main clinical features included flank pain in 9 cases (75%) and hematuria in 3 cases (25%). Retrocaval ureter was diagnosed preoperatively by imaging studies in 11 cases (91.7%). All cases received an operation including 1 laparoscopic ureteroureterostomy. Clinical symptoms and hydronephrosis had improved in all patients by 6 months postoperatively. CONCLUSIONS: An accurate preoperative diagnosis in most cases with retrocaval ureter can be achieved by imaging studies. Surgical treatment can achieve successful results. A laparosopic ureteroureterostomy is minimally invasive with good convalescence and outcome.(jtua 16:163-8, 2005) Key words: retrocaval ureter, ureteroureteral anastamosis, laparosopic ureteroureterostomy. INTRODUION Retrocaval ureter is a rare congenital anomaly which leads to ureteral external compression by the inferior vena cava (IVC) [1]. Only sporadic cases were reported in the literature. We reviewed 12 cases with retrocaval ureter in our hospital. The relevant literature was also reviewed. MATERIALS AND METHODS received operation in our hospital. A retrospective review of their medical records was undertaken, including clinical presentations, associated anomalies, imaging features, treatments and postoperative outcomes (Table 1). The outcomes of treatment were evaluated by clinical symptoms and imaging studies (ultrasonography and intravenous urography) about 6 to 12 months postoperatively. The successful treatment was defined as improvement of hydronephrosis in imaging studies and clinical symptoms about 6-12 months postoperatively. Between January 1989 and December 2004, 12 cases were diagnosed as having retrocaval ureter and Received: Feb. 23, 2005 Revised: May 30, 2005 Accepted: Oct. 27, 2005 Address reprint requests and correspondence to: Shyh-Chyan Chen, MD Department of Urology, National Taiwan University Hospital, No. 7 Chung Shan S. Road, Taipei, Taiwan 100, R.O.C. 台灣泌尿醫誌第十六卷第四期 (94 年 12 月 ) 163

2 Retrocaval Ureter: Report of 12 Cases and Literature Review Table 1. Clinical and radiological features of 12 retrocaval ureter cases Case Gen- Age no. der (yr) Symptoms Combined Compli- Results Imaging study Treatment anomalies cations #1 M 16 Flank pain Schizophrenia IVP, RP (type 1), Ureteroureterostomy Urine Successful leakage, urosepsis #2 M 71 Hematuria, UTI (-) IVP (type 1) Pyelolithotomy, pyeloureteral anastamo- #3 M 37 Flank pain, hematuria (-) IVP, RP (type 1), sis Pyelolithotomy, pyeloureteral anastamosis #4 M 15 Flank pain (-) IVP, RP (type 1) Ureteroureterostomy #5 F 23 Asymptomatic hydronephrosis Imperforate anus, agensis of left kidney, uterus, and vagina IVP, RP,(type 1), Ureteroureterostomy (-) Successful #6 M 26 Flank pain (-) IVP, AP, RP (type 1), Ureteroureterostomy #7 M 20 Flank pain (-) IVP (type 1), Ureteroureterostomy #8 M 46 Flank pain (-) IVP (type 1), Ureteroureterostomy #9 M 19 Flank pain (-) IVP (type 1), Ureteroureterostomy #10 M 46 Flank pain (-) IVP (type 1), Ureteroureterostomy #11 M 26 Hematuria Hypospadias IVP (type 1), Tc 99m DTPA scintigraphy Ureteroureterostomy #12 M 43 Flank pain (-) IVP (type 1), Laparoscopic ureterourterostomy Type 1, Bateson and Atkinson classifcation type 1; RP, retrograde pyelography; AP, antegrade pyelography; Successful result, improved hydronephronephrosis and symptoms 6-12 months postoperatively. RESULTS Among the 12 patients, there were 11 males and 1 female aged 15~71 (32.3 ±17.1) years. Three cases had combined anomalies. One had latent schizophrenia, another had glandular hypospadias, the other was a female with an imperforated anus and agenesis of left kidney, uterus, and vagina. Concomitant stone formation was noted in 4 patients (33.3%). Diagnosis was made by ultrasonography, intravenous urography (IVU), retrograde pyelography (RP), antegrade pyelography (AP), computed tomography (), or Tc-99m DTPA renal scan (Fig.1, 2). The main clinical features included flank pain in 9 cases (75%), hematuria in 3 cases (25%), asymptomatic hydronephrosis in 1 case (8.3%), and urinary tract infection in 1 case (8.3%). In all cases, preoperative imaging study showed a type I retorcaval ureter according to the Bateson and Atkinson classification. Retrocaval ureter was diagnosed pre- operatively in all cases except one (case no.2), because the upper ureter was not visualized clearly in IVP. The patient underwent surgery under the diagnosis of a right staghorn stone with hydronephrosis. A pyelolithotomy and pyeloureteric anastamosis were performed for reconstruction. In another 10 cases, the standard open operations were performed (resection of the stenotic segment of the ureter in all 10 cases; ureteroureteral anastamosis in 9 cases and pyeloureteral anastamosis in 1 case). Among them, 9 cases underwent intraoperative ureteral stent (double J stent) insertion. All of them had uneventful postoperative courses. The other patient (case no.1) who received ureteroureteral anastamosis without ureteral stent suffered from the complication of urine leakage and urosepsis. One patient (Case no. 12) received laparoscopic resection of the retrocaval stenotic segment and urteroureterostomy. The total operative time is JTUA Vol.16 No.4, Dec. 2005

KH Huang, SC Chang, SC Chueh, et al Fig. 2 Enhanced scan showing the ureter posterior to the IVC. Fig. 1 Antegrade pyelography showing a type 1 retorcaval ureter according to the Bateson and Atkinson classification.

The period of hospital stay is shorter in the laparoscopic case than that in open surgery gruop (average 9.5 days). The patient felt minimal post- operative pain and received no analgesics.

3 KH Huang, SC Chang, SC Chueh, et al Fig. 2 Enhanced scan showing the ureter posterior to the IVC. Fig. 1 Antegrade pyelography showing a type 1 retorcaval ureter according to the Bateson and Atkinson classification. minutes which was longer than open surgery group (average: 228 minutes). Blood loss was less than 10 ml. He could walk on day 1 and was discharged on day 7. The period of hospital stay is shorter in the laparoscopic case than that in open surgery gruop (average 9.5 days). The patient felt minimal post- operative pain and received no analgesics. The ureteral stents were removed about 4-8 weeks later (the average duration of ureteral stent placement: 36.5 days). Postoperative symptoms and hydronephrosis were improved in all cases. Operative findings showed that the retrocaval segments were stenotic and lacked elasticity. The pathology of the resected stenotic segments all showed smooth muscle hypertrophy with infiltration of inflammatory cells. DISCUSSION Retrocaval ureter, also known as circumcaval ureter was first reported by Hochstetter in 1893 [2]. Over 200 cases have been reported to the present. Heslin et al. showed the incidence of retrocaval ureter at autopsy to be about 1 in 1500 [3]. Totally speaking, the incidence of retrocaval ureter is reported to be 0.06%~0.17% of autopsy materials. The incidence is greater in males than in females with a ratio of 2.8:1 [1-4]. In our series, the ratio of males to females was 11:1. In embryology, the inferior vena cava normally develops from a plexus of fetal veins. The posterior cardinal and supracardinal veins lie dorsally, and the subcardinal veins lie ventrally. The left supracardinal veins and the lumbar portion of the right posterior cardinal vein atrophy. The subcardinal veins become the internal spermatic veins. A definitive right-side inferior vena cava forms from the right supracardinal vein. If the subcardinal vein in the lumbar portion fails to atrophy and becomes the primary right-sided vein, the ureter is trapped dorsally to it [1]. Retrocaval ureter almost invariably involves the right side [5][6]. Other anomalies associated with retrocaval ureter in past reports include IVC congenital variations, brachial arch syndrome, horseshoe kidney, myelomeningocele, hypospadias, Turner's syndrome, esophageal atresia, an abnormal left kidney (agenetic, ectopic, or malrotated), and cardiovascular anomalies such as situs inversus. [7-10]. Common presentations are right flank pain, recurrent urinary tract infections, and microscopic or gross haematuria as the presentations in our cases. There is a high incidence of calculi due to stasis. Flank pain is the predominant symptom. Hydronephrosis may be entirely silent without any symptoms. The patients usually are in their third or fourth decades because of gradual development of hydronephrosis. In our series, the age of onset is from (32.3±17.1) years. Imaging studies are usually accurate and sufficient for diagnosis of the disorder. Ultrasonography is a noninvasive method to demonstrate the anatomy of the retrocaval ureter and follow-up patients for hydrone- phro- 台灣泌尿醫誌第十六卷第四期 (94 年 12 月 ) 165

4 Retrocaval Ureter: Report of 12 Cases and Literature Review sis, parenchymal atrophy, and nephrolithiasis [11]. IVU in the early stage of ureteral stenosis only shows dilatation of the renal pelvis, calyces, and upper ureter above the site of obstruction. AP and RP is helpful in revealing the characteristic "S" shape and midline deviation of the ureter. Retrograde pyelography combined with inferior venocavography can clearly confirm the diagnosis but is invasive [12]. Enhanced scan with ureteral catheterization can demonstrate the opacified catheter posterior to IVC [13]. New imaging studies such as spiral scan and magetic resonance imaging (MRI) are of great help in delineating the anatomy noninvasively. An isotope renal scan can reveal the degree of obstruction, and differential renal function to help decide the therapeutic modalities [14,15]. In our series, retrocaval ureter can be correctly diagnosed preoperatively by imaging studies in 11 cases (91.7%). Bateson and Atkinson distinguished 2 types of retrocaval ureter according to the radiological appearance and site of ureteral narrowing. In type 1 (low loop), the ureter crosses behind the inferior vena cava at the level of the third lumbar vertebra and has a fish hook-shaped (S-shaped) deformity of the ureter. Marked hydronephrosis is seen in over 50% of patients. In type 2 (high loop), the renal pelvis and upper ureter lie horizontally, and the retrocaval segment of the ureter is at the same level as the renal pelvis. The retrograde pyelogram shows a "sickle shape" of the involved ureter, generally with mild hydronephrosis. Type 2 is less common at around 10% of all cases [16]. In our series, all 12 cases are type 1 according to the classification. The main causes of hydronephrosis are lumen stenosis, torsion, and adhesion of the retrocaval segment. This segment is compressed by the psoas muscle, spinal column, and vena cava, which leads to inflammation and fibrosis. All of these above-mentioned features can be identified pathologically in our series. Treatment depends primarily on the clinical presentations, severity of the hydronephrosis, and impairment of renal function. Conservative treatment and periodic examinations is necessary to those patients who have mild hydronephrosis without obvious symptoms, infection, worsening renal function, or stone formation. In 1935, Kimbrough performed the first successful surgical correction [17,18]. Various operative methods have been performed for retrocaval ureter including nephrectomy, ureteroureteral reanastamosis with or without resection of the retrocaval segment, and ligation or transection of the IVC with or without reanastomosis. Now, ureteroureteral reanastamosis anterior to the vena cava with resection of the retrocaval segment is the widely favored standard treatment [18,19]. If there is severe hydronephrosis, Anderson Hynes pyeloplasty with precaval transposition of the ureter has been advocated. Occasionally nephrectomy may be required in the presence of nonfunctional kidney.uueteral stent may be helpful for stenting and drainage. All 11 cases with ureteral stent were uneventful postoperatively, and the only 1 case without ureteral stent was complicated with urine leakage from anastamotic site. The period of 4-8 weeks may be sufficient for stent placement to achieve good result. Laparoscopic or retroperitoneoscopic ureterolysis and reconstruction for retrocaval ureter become popular in recent years with satisfactory success rate, minimal pain, and short convalescence time. However, laparoscopic ureteral reanastamosis is still time-consuming and technically demanding as is seen in the case no. 12 [20-23]. CONCLUSIONS Imaging studies are sufficient for an accurate diagnosis of retrocaval ureter. With advances in imaging studies and surgical equipment, the diagnosis and treatment of retrocaval ureter also have made great progress at present. Minimally invasive surgery may be the major trend in the future. REFERENCES 1. Walsh PC, Retik AB, Vaughan ED Jr. Cambell s Urology. 8 th ed. Philadelphia: W.B. Saunders 2003: Ouyang CC, Chueh SC, Hsu TC, Chen J, Tsai TC, Chiu TY. Retrocaval ureter- three case reports and review of the literature J Urol ROC 1994;5: Heslin JE, Mamonas C. Retrocaval ureter: report of four cases and review of the literature. J Urol 1951; 65: Carrion H, Gatewood J, Politano V, Morillo G, Lynne C. Retrocaval ureter: report of 8 cases and the surgical management. J Urol 1979;121: Kokubo T, Okada Y, Yashiro N. diagnosis of retrocaval ureter associated with double inferior vena cava: report of a case. Rad Med 1990;8: Sasai K, Sano A, Imanaka K. Right periureteric venous ring detected by computed tomography. J Comput Assist Tomogr 1986;10: Ishitoya S, Arai Y, Okubo K. Left retrocaval ureter associated with the Goldenhair Syndrome (brachial arch syndrome). J Urol 1997;158(2): Watanabe M, Kawamura S, Nakada T. Left pre- ureteral vena cava associated with partial situs inversus J Urol 1991;145: JTUA Vol.16 No.4, Dec. 2005

5 KH Huang, SC Chang, SC Chueh, et al 9. Heffernan JC, Lightwood RC, Snell ME. Horse-shoe kidney with retrocaval ureter, second reported case. J Urol 1978;120: Piercy SL, Gregory JG, Young PH. Ventriculoperitoneal shunt pseudocyst causing ureteropelvic junction obstruction in a child with my- elomenigocele and retrocaval ureter. J Urol 1984; 132: Uson AC, Braham SB, Abrams CA, Berdon WE. Retrocaval ureter in a child with Turner's syndrome. Am J Dis Child 1970;119: Thomas ES, Moore CA. Retrocaval ureter: 4 cases. J Urol 1971;105: Schaffer RM, Sunshine AG, Becker JA, Macchia RJ, Shih YH. Retrocaval ureter: sonographic appearance. J Ultras Med 1985;4: Zhang X, Hou S, Zhu J, Wang X, Meng G, Qu X. Diagnosis and treatment of retrocaval ureter. Eur Urol 1990;18: Singh DD, Sanjeev P, Sharma RK. Spiral evaluation of circumcaval ureter. Ind J Radiol Imag 2001:11: Bateson EM, Atkinson D. Circumcaval ureter: a new classification. Clin Radiol 1969;20: Kimbrough JC. Surgical treatment of hydronephrosis. J Urol 1935;33: Harrill HC. Retrocaval ureter: report of a case with operative correction of the defect. J Urol 1940;44: Shown TE, Moore CA. Retrocaval ureter: 4 cases. J Urol 1971;105: Matsuda T, Yasumoto R, Tsujino T. Laparoscopic treatment of a retrocaval ureter Eur Urol 1996;29: Ramalingam M, Selvarajan K. Laparoscopic transperitoneal repair of retrocaval ureter:report of two cases. J Endourol 2003;17: Gupta NP, Hemal AK, Singh I, Khaitan A. Retroperitoneoscopic ureterolysis and reconstruction of retrocaval ureter. J Endourol 2001;15: Polascik TJ, Chen RN. Laparoscopic ureteroureterostomy for retrocaval ureter. J Urol 1998;160: 台灣泌尿醫誌第十六卷第四期 (94 年 12 月 ) 167

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40 Case report Retrocaval ureter: a report on two neonates Ibrahim E. Bassiouny, Tariq O. Abbas and Ayman Mosleh Retrocaval ureter is a relatively rare anomaly that usually manifests in the third or the