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REVIEW ARTICLE
Year : 2004  |  Volume : 20  |  Issue : 2  |  Page : 79-85
 

Chyluria - a clinical and diagnostic stepladder algorithm with review of literature


Department of Surgery, University College of Medical Sciences (University of Delhi) and GTB Hospital, Delhi, India

Correspondence Address:
Igbal Singh
F-14 South Extension Part-2, New Delhi - 110 049
India
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   Abstract 

Objectives: Chyluria is an infrequently discussed urological problem and a rare urological manifestation of filariasis. Apart from few isolated case reports the lit­erature regarding the etiology, diagnostic approach and management of chyluria is grossly inadequate. We un­dertook the present study to review chyluria in its entirety so as to have a broader insight in to its etiopathogenesis and to suggest the clinician with a proposed stepladder protocol approach (algorithm) towards its management.
Methods: We made a detailed systematic data search for the period covering the last 37 years on the "Pubmed" for published English literature using the key words 'chy­luria', `milky urine' and 'hematochyluria'. The signifi­cant findings and recent advances on chyluria were reviewed.
Results: About 250 articles were found; these were analyzed, tabulated and reviewed for their clinical ap­proach and management of chyluria.
Conclusions: Though generally a harmless condition in a majority, chyluria should not be ignored, instead all cases must be aggressively investigated to arrive at a cause. These should then be managed on the lines similar to as proposed in our 10-stepladder protocol.


Keywords: Chyluria, milky urine, hematochyluria.


How to cite this article:
Singh I, Dargan P, Sharma N. Chyluria - a clinical and diagnostic stepladder algorithm with review of literature. Indian J Urol 2004;20:79-85

How to cite this URL:
Singh I, Dargan P, Sharma N. Chyluria - a clinical and diagnostic stepladder algorithm with review of literature. Indian J Urol [serial online] 2004 [cited 2017 May 30];20:79-85. Available from: http://www.indianjurol.com/text.asp?2004/20/2/79/20727



   Introduction Top


Chyluria is a state of chronic lymphourinary reflux via fistulous communications secondary to lymphatic stasis caused by obstruction of the lymphatic flow. It is more commonly encountered in the tropics and subtropics (filarial belt) and is rather uncommon in the western world. Though classified in to parasitic / non-parasitic causes the former predominate mainly due to filariasis, especially in the filarial belt. While in the endemic areas up to 10% may be afflicted by filariasis, chyluria actually occurs in only 2% of them. [1] The natural history of this chronic con­dition is still unclear and the treating surgeon or urologist must be well versed with its etiopathogenesis, diagnosis and management to prevent some of the later sequels.


   Methods Top


We reviewed over 250 published articles on chyluria and its manifestations in the English literature. The arti­cles were analyzed in detail under the headings; etiopatho­genesis, clinical features, diagnosis, management and current advances. On the basis of these studies we have proposed a 10-step algorithm to assist the treating sur­geon in their work-up and management.


   Results Top


Etiopathogenesis: Lymphatic drainage of the kidney occurs in a trilaminar fashion. The first lamina lies within the renal parenchyma, the second lies at a sub-capsular level and the third lies within the perinephric fat. The lymphatics in the second and third lamina freely inter­communicate. The intrarenal lymphatics emerge as 4-7 trunks, which emerge at the renal hilum to join the 2 nd and 3 rd level lymphatics. These then eventually converge along the renal vessels to the lateral aortic nodes. Chyluria oc­curs after rupture of lymphatic varies into renal tubules. The lymphatic varices are the result of high intralymphatic pressure, usually due to an obstruction or stenosis of the major lymphatic ducts. Various parasitic agents includ­ing Wuchereria bancrofti, Echinococcus, Cysticercus cellulose, Ascaris lumbricoides, Tinea nana, Cercorrenas hominis and malaria can induce this obstruction. The nonparasitic causes can be congenital lymphatic malfor­mation, injury to kidney with lymphourinary fistulas and obstruction of the lymphatics caused by trauma, abscess, neoplasms, diabetes, pernicious anemia, pregnancy and tuberculosis. [1],[3] It is vital to distinguish whether chyluria is of a parasitic or nonparasitic origin [Table - 1] since the prognosis of nonparasitic chyloria is usually very good.

The most common etiological factor for chyluria is filariasis and chyluria should be considered filarial unless proven otherwise particularly in the filarial belt. Block­age of the major retroperitoneal lymphatics and thoracic duct by the mature parasite and retrograde flow of lymph from gut and pelvis to lumen of genitourinary tract, lym­phangiectasis (as a result of inflammatory reaction to the parasitic components) and heralds the subsequent devel­opment of urinary fistulae. Ultimately, chyluria is the end result of impairment of the retroperitoneal lymphatics due to a vicious cycle of infection, sclerosis, obstructive retro­grade dilatation, stasis, backflow and spontaneous rupture with fistulization in to the urinary tract. These abnormal lymphaticourinary communications most commonly es­tablish at the renal level but may occur anywhere along the ureter, bladder, prostate or urethra. In the kidney, these fistulae have been most commonly demonstrated by lym­phography at the lymphaticoforniceal level in the renal pelvis and calyces. [1]

Clinical features: Chyluria though often asymptomatic (monosymptomatic) may be also polysymptomatic at times due to the existence of associated conditions such as dysu­ria, hematuria, renal colics, backache, urinary tract infec­tions (UTI), pedal lymphangitis, edema, hydroceles, hypoproteinemia, cachexia, weight loss and malnutrition. Chyluria is a urological manifestation of lymphatic sys­tem disease which when prolonged may lead to nutritional deficiency, recurrent clot colic, urinary retention, UTI, hematuria (hematochyluria) and a state of compensated immunosuppression. Prolonged massive chyluria may lead to serious immunological and homeostasis deficits due to increasing IgG, and particularly IgA deficiency. [4] This may lead to a state of depressed Immoral and cellular immunity (lymphocytopenia) with opportunistic fungal in­fections and promotion of malignant tumors (due to sup­pression of cellular immunity).

Diagnosis: The diagnosis of chyluria can be confirmed by evaluating a sample of a postprandial urine for chylomicrons and triglycerides. The intermittent passage of milky cloudy urine should be differentiated from phos­phaturia (clears on adding 10% acetic acid), amorphous urates, severe pyuria, lipiduria secondary to fat embolism, pseudochylous urine and caseousuria due to renal tuber­culosis. [5] The typical properties of chylous urine have been shown in [Table - 2]. All patients should also be screened for filariasis with: hemoglobin, total and differential counts (eosinophilia), serum proteins, blood urea and serum cre­atinine, urine for acid-fast bacilli and UTI. Intravenous urography, retrograde pyelography and lymphangiography have been traditionally used to demonstrate abnor­mal lymphaticourinary fistulas, this in some cases may be even therapeutic, due to the contrast induced chemical pyelitis causing an obliterative sclerosis of the lymphatics leading to cessation of chyluria. [6] Intravenous urography is generally within normal limits but it may demonstrate dilated paracalyceal lymphatics. Retrograde pyelography may demonstrate the fistulous connection and dilated lymphatics in about half the cases but this must be done gently because the reflux of contrast in to lymph / veins may produce false positive results if the contrast is in­jected forcefully at high pressure. Lymphangiography (ultrafluid lipiodol injection via bipedal lymphangiogram) has a sensitivity of 90% (lymphatopelvic fistulization) but being invasive, time-consuming, technically cumbersome and prone to complications it has now become largely obsolete with the emergence of lymphoscintigraphy (al­bumin tagged radioisotopes). [7] Lymphographic evidence of numerous perihilar lymphatics is the most pathogno­monic sign of chyluria. Te-99m diethylenetriamine penta­acetic acid radionuclide lymphoscintigraphy has now replaced traditional lymphography to precisely reveal the location of chyluria in a noninvasive manner. [8] Routine radio imaging may not be necessary in filarial chyluria. [9] CT scan can demonstrate enlarged para-aortic lymphad­enopathy. Detection of filarial antigens in serum and urine can be done routinely with ELISA sandwich assay but for rapid accurate diagnosis of Wuchereria bancrofti infec­tion immunochromatographic test (ICT) is best because of its high sensitivity of 96.7%. [10]


   Management of Chyluria Top


Medical management: Chyluria should be initially managed conservatively. If the initial general condition is good, chyluria is mild and stable and there is absence of microfilaria no therapy may be necessary. The natural history of chyluria is still unknown and it must be appre­ciated that up to 50% of cases may undergo spontaneous remission. The conservative measures include dietary ma­nipulations with omission of long chain triglycerides (use fat as medium chain triglycerides only), use of coconut oil, drug therapy with diethyl carbamazine, bed rest and use of abdominal binders (to decrease the lymphourinary reflex through higher intra-abdominal pressure). Medium chain triglycerides (<12 carbon atoms) have been advo­cated since these are directly absorbed via the portal vein bypassing the lacteals and lymphatic channels, unlike the long chain fatty acids. Filarial chyluria is also known to undergo spontaneous exacerbations and remissions related to causative factors, physical activity, posture (orthostatic chyluria) and fatty meal ingestion. Chyluria associated with other coexistent conditions [Table - 1] and may often be controlled by treatment of these coexistent conditions, which should be the initial therapeutic approach for pa­tients suffering from these conditions. [11]

Surgical management: Surgical management of chyluria is indicated in patients with refractory severe chylu­ria associated with recurrent colics, urinary retention, pro­gressive weight loss, ill health due to immunosuppression and failed medical therapy. The severity may be judged by (1) persistence of the symptoms, (ii) history of frequent chylous clots, clot colics, urinary retention; and (iii) sig­nificant weight loss with ill health. [Table - 3] shows the major worldwide reports and their experience on the sur­gical options that have been used to manage chyluria till date.

(A) Endoscopic sclerotherapy (EST): Instillation of silver nitrate (AgNO 3 ) in the renal pelvis [12] has been found to be a safe, effective and minimally invasive procedure with an initial success rate of about 70-80% [10],[12] and the long-term recurrence rate is 50%. The success rate with EST is known to fall with subsequent reinstallations. The chylous efflux is identified after a fatty meal by (post­prandial cystoscopy) and a ureteric catheter is passed up to the renal pelvis on affected side. Graded higher con­centrations of AgNO, (10 ml) 0.1-0.5-1% are instilled every half hour to two hours under strict asepsis and che­moprophylaxis. AgNO 3 induces an intense aseptic scleros­ing obliterative inflammatory reaction in the lymphatic channels leading to immediate relief. The subsequent heal­ing by fibrosis leads to permanent remission. Flank pain, nausea, vomiting and hematuria can occur after instilla­tion but usually subsides by 24-28 hours. Higher concen­trations of AgNO 3 (3%), which may lead to dangerous massive obstructive pelvicalyceal casts and life threaten­ing anuria especially following bilateral EST, should be avoided. [13] It is safer to manage bilateral chyluria one side at time, and doing EST on the severe side first. EST with AgNO 3 should not be taken lightly as occasional fatal epi­sodes [14] and life-threatening hemorrhage with massive hematuria [15] and renal papillary necrosis [16] have also been reported to occur. Timely recognition and adequate backup emergency angiographic facilities should be available in centers opting to manage such cases by EST. [15] Recently localized chyluria (post-radical nephrectomy) has been cured using cyanoacrylate adhesives. [17] Other agents such as (0.2%) povidone iodine's and radiographic contrast media (Urografin TM ) [19] have also been used successfully for EST of chyluria. Subcutaneous octreotide has also been used to treat post-traumatic chyluria. [20],[21] Thus the imme­diate results with EST are good but the long term follow up and recurrence rates tend to be high. [22]

(B) Surgical lymphatic disconnection: Traditional open surgical ligation [23],[24] and excision of renal pedicle lym­phatics to cure persistent chyluria has now been largely replaced by the minimally invasive approach of retroperi­toneoscopy. [25],[26],[27],[28] Nephrolympholysis, ureterolympholysis, hilar vessel stripping, fasciectomy and nephropexy has been also been accomplished retroperitonoscopically. [25],[26],[27] The retroperitoneoscopic approach to the management of chyluria though well suited to the identification of the frag­ile lymphatics may occasionally lead to complications re­lated to retroperitoneal access [26] and procedures such as subcutaneous emphysema, port site infections and inad­vertent clipping of other structures at the renal hilum. [17] Retroperitoneal or transinguinal access is the most physi­ological method though it is technically cumbersome since the lymphatics are hard to identify and are fragile. Laparo­scopic renal pedicle stripping and ligation has been per­formed successfully both transperitoneally [28] as well retroperitoneally. [29] Retroperitoneoscopic access avoids the potential problems of transperitoneal breach such as pro­longed ileus, peritonitis and bowel injury. [30],[31] Retroperi­toneoscopic lymphatic disconnection is a safe, effective and minimally invasive procedure for intractable chylu­ria; ideally it should encompass nephrolympholysis (dis­section of peri-renal fascia all around); hilar stripping (skeletonising renal vessels with perihilar lymphatic clip­ping); ureterolysis (skeletonising the ureter till the iliac vessels); fasciectomy (Gerota's fascia removal) and ne­phropexy (three polar fixation of renal capsule to the psoas fascia). [31] Failure of improvement after renal stripping may be due to incomplete stripping or fistulas in the lower uri­nary tract. In such cases renal auto-transplantation has been advocated. [32]

(C) Microsurgery: Recurrent intractable chyluria that has failed EST and surgical management may be cured by transinguinal spermatic lymphangiovenous anastomosis or inguinal lymph node-saphenous vein anastomosis. [33],[34] The lymphonodovenous anastomosis may be superior since it can avoid damage to the afferent, efferent lymphatics and provides a larger anastomotic stoma for free drainage of lymph in to the vein. [35],[36] Due to the spon­taneous emergence of collateral lymphatic channels even these lymphatic shunts tend to fail over 6 months.

[Figure - 1] shows the stepwise ten stepladder protocol to diagnose and manage chyluria suggested by us based on the current review of literature.

Follow-up: Involves subjective and objective evalua­tion of the patient and postoperative evaluation of urine for chyle, lipids and cholesterol and thereafter to be checked 6-monthly.


   Conclusions Top


Non-parasitic chyluria is rare in the western world. After investigations in to the parasitic/nonparasitic etiology, one must determine whether it is mono or poly-symptomatic chyluria since the latter often has associated coexistent conditions, the treatment of which leads to remission of chyluria. Routine etiological demonstration of lymphatic urinary reflux is unnecessary, it should be advocated only for intractable/persistent chyluria that persists despite the initiation of conservative measures. The next step should be to initiate 1-2 trials of endoscopic sclerotherapy, fail­ing which one of the minimally invasive (lap retroperi­toneoscopic) procedures must be considered, the latter has a high success rate. For recurrent or incurable chyluria microsurgical procedures should be considered. We sug­gest a 10-step protocol towards the diagnosis and man­agement of chyluria.

 
   References Top

1.Diamond E, Schapira HE. Chyluria - A review of the literature. Urology 1985; 26(5): 427-31.  Back to cited text no. 1    
2.Wadsworth DE, Glazer HS. McClennan BL. Chyluria. Urol Radiol 1983: 5: 113-9.  Back to cited text no. 2    
3.Onyeije CI, Sherer DM. Trambert J. Nonfilarial chyluria during pregnancy. Obstet Gynecol 1997; 90: 699-700.  Back to cited text no. 3    
4.Kuzniar J, Uzar J. Kopec W. Herbec R, Modrakowska A. Certain aspects of clinically mild, non-tropical chyluria. Pol Tyg Lek 1991; 46(4-5): 81-3.  Back to cited text no. 4    
5.Esquirol Mallol R, Servelle M, Turpyn L, Verminck JP, Esquirol Caussa JR. Lymphatic malformation and chyluria: presentation of a treated case without relapse. Med Clin (Barc) 1991; 97(15): 576-8.  Back to cited text no. 5    
6.Nunez Mora C, Carcamo Valor P, de Cabo Ripoll M, Kabani MH, Martinez-Pineiro Carames JA. Recurrent nonparasitic chyluria. Arch Esp Urol 1998: 51(9): 932-4.  Back to cited text no. 6    
7.Nishiyama Y, Yamamoto Y, Mori Y, Satoh K. Usefulness of Tc­99m human serum albumin. Clin Nucl Med 1998: 23(7): 429-31.  Back to cited text no. 7    
8.Thet L. Takeda T, Kuramochi M, Sato M, Wu J, Myo-Min, Itai Y. Tc-99m diethylenetriamine penta-acetic acid (DTPA)-human se­rum albumin (HAS) radionuclide lymphography for detecting the location of chyluria. Ann Nucl Med 1998: 12(4): 205-7.  Back to cited text no. 8    
9.Dalela D, Kumar A, Ahlawat R, Goel TC, Mishra VK, Chandra H. Routine radio-imaging in filarial chyluria - is it necessary in devel­oping countries? Br J Urol 1992; 69(3): 291-3.  Back to cited text no. 9    
10.Zheng H, Tay Z, Fang R, Chang B, Zhang Y. Turner P. Application of immuno-chromatographic test for diagnosis and surveillance of bancroftian filariasis. Zhongguo Ji Sheng Chong Xue Yu Ji Sheng Chong Bing Za Zhi 1998; 16(3): 168-71.  Back to cited text no. 10    
11.Koga S, Arakaki Y. Matsuoka M, Ohyama C. Remission of filarial chyluria after treatment of coexistent conditions. Lymphology 1990: 23(3): 164-6.  Back to cited text no. 11    
12.Sabnis RB, Punekar SV. Desai RM, Bradoo AM. Bapat SD. Instil­lation of silver nitrate in the treatment of chyluria. Br J Urol 1992; 70(6): 660-2.  Back to cited text no. 12    
13.Gulati MS, Sharma R, Kapoor A, Berry M. Pelvi-calyceal cast for­mation following silver nitrate treatment for chyluria. Australas Radiol 1999; 43(1): 102-3.  Back to cited text no. 13    
14.Mandhani A. Kapoor R. Gupta RK, Rao HS. Can silver nitrate in­stillation for the treatment of chyluria be fatal? Br J Urol 1998: 82(6): 926-7.  Back to cited text no. 14    
15.Srivastava DN, Yadav S. Hemal AK, Berry M. Arterial haemor­rhage following instillation of silver nitrate in chyluria: treatment by coil embolization. Australas Radiol 1998: 42(3): 234-5.  Back to cited text no. 15    
16.Dash SC, Bhargav Y, Saxena S, Agarwal SK, Tiwari SC, Dinda A. Acute renal failure and renal papillary necrosis following instilla­tion tion of silver nitrate for treatment of chyluria. Nephrol Dial Trans­plant 1996; 11(9): 1841-2.  Back to cited text no. 16    
17.Kumar M. Kumar R. Hemal AK, Gupta NP. Complications of retroperitoneoscopic surgery at one center. BJU Int 2001; 87(7): 607-12.  Back to cited text no. 17    
18.Shanmugam TV, Prakash JV, Sivashankar G. Povidone iodine used as a sclerosing agent in the treatment of chyluria. Br J Urol 1998: 82(4): 587.  Back to cited text no. 18    
19.Hsieh JT, Chang HC. Law HS, Shun CT. Cyst-like chylous coagu­lum in the urinary bladder of a patient with recurrent chyluria. J Formos Med Assoc 1999; 98(8): 586-8.  Back to cited text no. 19    
20.Giordano M. Crillo D. Baron I, Sorrentino F. Tomasino G. Cicchella T, De Bellis L, Giordano C. Treatment of post-traumatic chyluria with subcutaneous octreotide administration. Nephrol Dial Trans­plant 1996; 11(2): 365-7.  Back to cited text no. 20    
21.Campieri C, Raimondi C. Dalmastri V. Sestigiani E, Neri L. Giudicissi A, Zompatori M, Stefoni S, Bonomini V. Post-traumatic chyluria due to lymphorenal fistula regressed after somatostatin theraphy. Nephron 1996: 72(4): 705-7.  Back to cited text no. 21    
22.Okamoto K, Ohi Y. Recent distribution and treatment of filarial chyluria in Japan. J Urol 1983; 129(l): 64-7.  Back to cited text no. 22    
23.Punekar SV, Kelkar AR. Prem AR, Deshmukh HL, Gravande PM. Surgical disconnection of lymphorenal communication for chylu­ria: a 15-year experience. Br J Urol 1997; 80(6): 858-63.  Back to cited text no. 23    
24.Tan LB, Chiang CP. Huang CH, Chou YH. Wang CJ. Experiences in the treatment of chyluria in Taiwan. J Urol 1990; 144(3): 710-3.  Back to cited text no. 24    
25.Hemal AK, Gupta NP. Retroperitoneoscopic lymphatic manage­ment of intractable chyluria. J Urol 2002; 167(6): 2473-6.  Back to cited text no. 25    
26.Hemal AK, Kumar M. Pawar RS, Gupta NP. The laparoscopic man­agement of chyluria by retroperitoneal access. BJU Int 2000; 86(3): 402.  Back to cited text no. 26    
27.Hernal AK, Kumar M, Wadhwa SN. Retroperitoneoscopic nephrolympholysis and ureterolysis for management of intractable filarial chyluria. J Endourol 1999; 13(7): 507-11.  Back to cited text no. 27    
28.Chui AW, Chen MT, Chang LS. Laparoscopic nephrolysis for chy­luria - case report of long-term success. J Endourol 1995; 9(4): 319-22.  Back to cited text no. 28    
29.Gomella LG. Shenot P, Abdel-Meguid TA. Extraperitoneal laparoscopic nephrolysis for the treatment of chyluria. BJU 1998; 81: 320-1.  Back to cited text no. 29    
30.Singh I, Hemal AK. Reconstructive procedures, retroperitoneal and transperitoneal ureteral surgery, In: Hemal AK. ed. Laparoscopic Urologic Surgery - Retroperitoneal and Transperitoneal, Section IV, Ch.27, B.IChurchill Livingstone, New Delhi. 2000; Pp 231­-236.  Back to cited text no. 30    
31.Ansari MS, Pawan Kumar. Reconstructive procedures, laparoscopic management of chyluria. In: Hemal AK, ed. Laparoscopic Urologic Surgery - Retroperitoneal and Transperitoneal, Section IIb, Ch.27, B.I.Churchill Livingstone, New Delhi, 2000; Pp 197-202.  Back to cited text no. 31    
32.Brunkwall J, Simonsen 0, Bergquist D, Jonsson K, Berentz S. Chy­luria treated with renal auto-transplantation: a case report. J Urol 1990;143:793.  Back to cited text no. 32    
33.Xu YM, Ji RJ, Chen ZD, Qiao Y, Jin NT. Microsurgical treatment of chyluria: a preliminary report. J Urol 1991; 145(6): 1184-5.  Back to cited text no. 33    
34.Ji YZ, Zheng JH. Chen JN. Zu ZD. Microsurgery in the treatment of chyluria and scrotal lymphangial fistula. Br J Urol 1993; 72(6): 952-4.  Back to cited text no. 34    
35.Hou LQ, Liu QY. Kong QY, Luo CZ. Kong QA. Li LX, Li GJ. Lymphonodovenous anastomosis in the treatment of chyluria. Chin Med J (Engl) 1991: 104(5): 392-4.  Back to cited text no. 35    
36.Zhao WP, Hou LQ, Shen JL. Summary and prospects of fourteen years' experience with treatment of chyluria by microsurgery. Eur Urol 1988; 15(3-4): 219-22.  Back to cited text no. 36    


    Figures

  [Figure - 1]
 
 
    Tables

  [Table - 1], [Table - 2], [Table - 3]



 

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