Case Report
Urologia
Received: March 17, 2015 Accepted: March 28, 2015 Published online: June 5, 2015
Urol Int 2015;95:243–245 DOI: 10.1159/000382132
Internationalis
Right-Crossed, Fused Renal Ectopia L-Shaped Kidney Type with Urinary Chyluria Ding-yi Liu a Hao-fei Wang b Wei-mu Xia a Hong-chao He b Zhou-jun Shen b
Departments of a Urology, Punan Hospital, and b Urology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China
Key Words Crossed fused renal ectopia · Chyluria
Abstract Crossed fused renal ectopia combined with chyluria is extremely rare. Here we report the case of a patient who was admitted to our institution since milky urine and was finally found to have an L-shaped fused kidney and renal pelvis fistula. The patient was cured by renal pelvic instillation sclerotherapy. © 2015 S. Karger AG, Basel
Case Presentation A 60-year-old female was admitted to our institution complaining of recurrent milky urine. The patient presented with an 8-month history of intermittent abdominal pain in the right flank region and chyluria, which had become worse in the past 4 months (presented as constant chyluria) with feebleness. Moderate pitting edema was observed in bilateral lower limbs. The urinary chyluria test was positive and 24-hour urine protein was 1.2 g. Night blood filarial examination and filarial serology were negative. The results of the routine blood test and liver and renal function were all within normal limits. A plain film of kidney, ureter and bladder (KUB), intravenous urography showed an enlarged right kidney shadow with indepen-
© 2015 S. Karger AG, Basel 0042–1138/15/0952–0243$39.50/0 E-Mail [email protected] www.karger.com/uin
dent renal pelvis and normal distal ureter. The right pyelocaliceal system was arranged vertically with an anterior-medial orientation, while the left pyelocaliceal system was oriented horizontally. The right ureter descended vertically with a slight curve to the right, while the left ureter crossed the midline at the level of promontorium. Both of them entered the bladder wall in the normal anatomic location (fig. 1). Computed tomography (CT) was performed for further diagnostic imaging. We identified an L-shaped, right-crossed fused renal ectopia with arteriovenous malformation using the CT angiography technique (fig. 1). Two kidneys were found in the right renal fossa joined by parenchymal tissue with two renal arteries and two renal veins. The maximum length, width and thickness of the fused kidney were 15, 9.5 and 4.5 cm, respectively. Cystoscopy examination revealed the presence of lots of white lumps. Each side of the ureteric orifice was observed at least five times but no observable excretion of chyle was demonstrated. Unilateral pedal lymphography was performed for localization diagnosis. When local anesthesia was achieved using 0.5% lidocaine, methylene blue was injected subcutaneously between the first and second toe. After 2 min of gentle massage, the blue-dyed lymphangion could be seen when the skin incision had been made. CT and KUB were performed 30 min after the injection of contrast agent (Lipiodol Ultra-Fluide 10 ml with a 0.1–0.2 ml/min injection speed) [1]. Wire-like, half-round shadow could be seen at the kidney area with some contrast agent flowing into the bladder. Renal pelvis fistula was detected. The fistula was derived from the upper
H.W. is listed as co-first author. This work was supported by the fundation for health system key disciplines construction of Shanghai Pudong District (PWZx 2014-19).
Dr. Hong-chao He Department of Urology, Ruijin Hospital School of Medicine, Shanghai Jiaotong University No. 197, Ruijin Er Road, Shanghai 200025 (China) E-Mail dr_john_he @ 163.com
Downloaded by: Siriraj Medical Library, Mahidol University 198.143.39.97 - 1/28/2016 2:39:20 AM
a
b
c
Fig. 1. Right-crossed fused renal ectopia (L-shaped kidney type) had independent renal pelvis and normal distal ureter.
a
renal parenchyma of the fused kidney and communicated to the collecting system (fig. 2). Low-fat and high-protein diet with nutrition therapy was given in vain. Renal pelvic instillation was then performed; 20 ml 50% glucose solution was administrated through ureteral catheter and repeated twice within 30 min. The patient was finally cured and no recurrence was observed during the 2-year follow-up.
Discussion
Crossed fused renal ectopia is the second most common fusion abnormality of the kidney [2] and is divided into six types (in decreasing order of frequency): (1) inferior crossed fused ectopia, (2) sigmoid or S-shaped kidney, (3) unilateral lump kidney, (4) unilateral disc kidney, (5) L-shaped kidney, and (6) superior crossed fused ectopia [3]. It occurs more frequent in men than in women, and the crossover of the left kidney to the right side is the most common form. In the present case, the patient was 244
Urol Int 2015;95:243–245 DOI: 10.1159/000382132
b
a female with a crossover from left to right. Most cases are diagnosed incidentally since they are often asymptomatic. Flank or abdominal pain, abdominal mass, hematuria, dysuria, urinary tract infections, renal failure, fever and hypertension are the most common clinical manifestations as previously reported [4]. In our case, however, the female patient had chyluria. Chyluria is a rare condition defined as the presence of chyle in the urine. Although most reported cases are attributed to parasitic infections (especially filarial infections), they can also be secondary to non-parasitic causes, such as trauma, tuberculosis, and tumors [5]. Localization diagnosis has become the most important thing once chyluria was confirmed by the chyluria test. Cystoscopy plus ureteral urine collection for chyluria test and unilateral pedal lymphography are commonly used for localization. In our case, night blood filarial examination and filarial serology proved negative, and this indicated a nonparasitic etiology. In crossed fused renal ectopia, the Liu/Wang/Xia/He/Shen
Downloaded by: Siriraj Medical Library, Mahidol University 198.143.39.97 - 1/28/2016 2:39:20 AM
Fig. 2. Renal pelvis fistula derived from the upper renal parenchyma of the fused kidney.
vascular and urinary tract structures are reestablished, which might make the fistula easier to form between the collecting system and the lymphatic system. Conservative treatments consist of diet control/nutrition therapy and renal pelvic instillation sclerotherapy. Most patients with mild-to-moderate chyluria have good response to conservative treatment. Surgical intervention to disconnect the fistulous communication is mandatory for patients
with a severe or refractory disease [5]. In our case, the patient was cured by renal pelvic instillation sclerotherapy with no recurrence during the 2-year follow-up.
Acknowledgment There are no financial or commercial interests.
References Liu DY, He HC, Zhou WL, Chao ZF, Wang J, Wang MW, He XZ, Xia WM, Zhang CY: The advantages of unilateral pedal lymphography in the diagnosis of chyluria. Urol Int 2015;94: 215–219.
Crossed Fused Renal Ectopia with Chyluria
2
3
Kaur N, Saha S, Mriglani R, Saini P, Gupta A: Crossed fused renal ectopia with a single ureter: a rare anomaly. Saudi J Kidney Dis Transpl 2013;24:773–776. Türkvatan A, Olçer T, Cumhur T: Multidetector CT urography of renal fusion anomalies. Diagn Interv Radiol 2009;15:127–134.
Urol Int 2015;95:243–245 DOI: 10.1159/000382132
4 5
Boyan N, Kubat H, Uzum A: Crossed renal ectopia with fusion: report of two patients. Clin Anat 2007;20:699–702. Sharma S, Hemal AK: Chyluria – an overview. Int J Nephrol Urol 2009;1:14–26.
245
Downloaded by: Siriraj Medical Library, Mahidol University 198.143.39.97 - 1/28/2016 2:39:20 AM
1
Urologia
Received: March 17, 2015 Accepted: March 28, 2015 Published online: June 5, 2015
Urol Int 2015;95:243–245 DOI: 10.1159/000382132
Internationalis
Right-Crossed, Fused Renal Ectopia L-Shaped Kidney Type with Urinary Chyluria Ding-yi Liu a Hao-fei Wang b Wei-mu Xia a Hong-chao He b Zhou-jun Shen b
Departments of a Urology, Punan Hospital, and b Urology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China
Key Words Crossed fused renal ectopia · Chyluria
Abstract Crossed fused renal ectopia combined with chyluria is extremely rare. Here we report the case of a patient who was admitted to our institution since milky urine and was finally found to have an L-shaped fused kidney and renal pelvis fistula. The patient was cured by renal pelvic instillation sclerotherapy. © 2015 S. Karger AG, Basel
Case Presentation A 60-year-old female was admitted to our institution complaining of recurrent milky urine. The patient presented with an 8-month history of intermittent abdominal pain in the right flank region and chyluria, which had become worse in the past 4 months (presented as constant chyluria) with feebleness. Moderate pitting edema was observed in bilateral lower limbs. The urinary chyluria test was positive and 24-hour urine protein was 1.2 g. Night blood filarial examination and filarial serology were negative. The results of the routine blood test and liver and renal function were all within normal limits. A plain film of kidney, ureter and bladder (KUB), intravenous urography showed an enlarged right kidney shadow with indepen-
© 2015 S. Karger AG, Basel 0042–1138/15/0952–0243$39.50/0 E-Mail [email protected] www.karger.com/uin
dent renal pelvis and normal distal ureter. The right pyelocaliceal system was arranged vertically with an anterior-medial orientation, while the left pyelocaliceal system was oriented horizontally. The right ureter descended vertically with a slight curve to the right, while the left ureter crossed the midline at the level of promontorium. Both of them entered the bladder wall in the normal anatomic location (fig. 1). Computed tomography (CT) was performed for further diagnostic imaging. We identified an L-shaped, right-crossed fused renal ectopia with arteriovenous malformation using the CT angiography technique (fig. 1). Two kidneys were found in the right renal fossa joined by parenchymal tissue with two renal arteries and two renal veins. The maximum length, width and thickness of the fused kidney were 15, 9.5 and 4.5 cm, respectively. Cystoscopy examination revealed the presence of lots of white lumps. Each side of the ureteric orifice was observed at least five times but no observable excretion of chyle was demonstrated. Unilateral pedal lymphography was performed for localization diagnosis. When local anesthesia was achieved using 0.5% lidocaine, methylene blue was injected subcutaneously between the first and second toe. After 2 min of gentle massage, the blue-dyed lymphangion could be seen when the skin incision had been made. CT and KUB were performed 30 min after the injection of contrast agent (Lipiodol Ultra-Fluide 10 ml with a 0.1–0.2 ml/min injection speed) [1]. Wire-like, half-round shadow could be seen at the kidney area with some contrast agent flowing into the bladder. Renal pelvis fistula was detected. The fistula was derived from the upper
H.W. is listed as co-first author. This work was supported by the fundation for health system key disciplines construction of Shanghai Pudong District (PWZx 2014-19).
Dr. Hong-chao He Department of Urology, Ruijin Hospital School of Medicine, Shanghai Jiaotong University No. 197, Ruijin Er Road, Shanghai 200025 (China) E-Mail dr_john_he @ 163.com
Downloaded by: Siriraj Medical Library, Mahidol University 198.143.39.97 - 1/28/2016 2:39:20 AM
a
b
c
Fig. 1. Right-crossed fused renal ectopia (L-shaped kidney type) had independent renal pelvis and normal distal ureter.
a
renal parenchyma of the fused kidney and communicated to the collecting system (fig. 2). Low-fat and high-protein diet with nutrition therapy was given in vain. Renal pelvic instillation was then performed; 20 ml 50% glucose solution was administrated through ureteral catheter and repeated twice within 30 min. The patient was finally cured and no recurrence was observed during the 2-year follow-up.
Discussion
Crossed fused renal ectopia is the second most common fusion abnormality of the kidney [2] and is divided into six types (in decreasing order of frequency): (1) inferior crossed fused ectopia, (2) sigmoid or S-shaped kidney, (3) unilateral lump kidney, (4) unilateral disc kidney, (5) L-shaped kidney, and (6) superior crossed fused ectopia [3]. It occurs more frequent in men than in women, and the crossover of the left kidney to the right side is the most common form. In the present case, the patient was 244
Urol Int 2015;95:243–245 DOI: 10.1159/000382132
b
a female with a crossover from left to right. Most cases are diagnosed incidentally since they are often asymptomatic. Flank or abdominal pain, abdominal mass, hematuria, dysuria, urinary tract infections, renal failure, fever and hypertension are the most common clinical manifestations as previously reported [4]. In our case, however, the female patient had chyluria. Chyluria is a rare condition defined as the presence of chyle in the urine. Although most reported cases are attributed to parasitic infections (especially filarial infections), they can also be secondary to non-parasitic causes, such as trauma, tuberculosis, and tumors [5]. Localization diagnosis has become the most important thing once chyluria was confirmed by the chyluria test. Cystoscopy plus ureteral urine collection for chyluria test and unilateral pedal lymphography are commonly used for localization. In our case, night blood filarial examination and filarial serology proved negative, and this indicated a nonparasitic etiology. In crossed fused renal ectopia, the Liu/Wang/Xia/He/Shen
Downloaded by: Siriraj Medical Library, Mahidol University 198.143.39.97 - 1/28/2016 2:39:20 AM
Fig. 2. Renal pelvis fistula derived from the upper renal parenchyma of the fused kidney.
vascular and urinary tract structures are reestablished, which might make the fistula easier to form between the collecting system and the lymphatic system. Conservative treatments consist of diet control/nutrition therapy and renal pelvic instillation sclerotherapy. Most patients with mild-to-moderate chyluria have good response to conservative treatment. Surgical intervention to disconnect the fistulous communication is mandatory for patients
with a severe or refractory disease [5]. In our case, the patient was cured by renal pelvic instillation sclerotherapy with no recurrence during the 2-year follow-up.
Acknowledgment There are no financial or commercial interests.
References Liu DY, He HC, Zhou WL, Chao ZF, Wang J, Wang MW, He XZ, Xia WM, Zhang CY: The advantages of unilateral pedal lymphography in the diagnosis of chyluria. Urol Int 2015;94: 215–219.
Crossed Fused Renal Ectopia with Chyluria
2
3
Kaur N, Saha S, Mriglani R, Saini P, Gupta A: Crossed fused renal ectopia with a single ureter: a rare anomaly. Saudi J Kidney Dis Transpl 2013;24:773–776. Türkvatan A, Olçer T, Cumhur T: Multidetector CT urography of renal fusion anomalies. Diagn Interv Radiol 2009;15:127–134.
Urol Int 2015;95:243–245 DOI: 10.1159/000382132
4 5
Boyan N, Kubat H, Uzum A: Crossed renal ectopia with fusion: report of two patients. Clin Anat 2007;20:699–702. Sharma S, Hemal AK: Chyluria – an overview. Int J Nephrol Urol 2009;1:14–26.
245
Downloaded by: Siriraj Medical Library, Mahidol University 198.143.39.97 - 1/28/2016 2:39:20 AM
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