|Year : 2004 | Volume
| Issue : 2 | Page : 79-85
Chyluria - a clinical and diagnostic stepladder algorithm with review of literature
Igbal Singh, P Dargan, N Sharma
Department of Surgery, University College of Medical Sciences (University of Delhi) and GTB Hospital, Delhi, India
F-14 South Extension Part-2, New Delhi - 110 049
Source of Support: None, Conflict of Interest: None
| 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
| Introduction|| |
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.  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|| |
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|| |
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. , 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. 
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.  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.  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.  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).  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.  Routine radio imaging may not be necessary in filarial chyluria.  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%. 
| Management of Chyluria|| |
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. 
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  has been found to be a safe, effective and minimally invasive procedure with an initial success rate of about 70-80% , 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.  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  and life-threatening hemorrhage with massive hematuria  and renal papillary necrosis  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.  Recently localized chyluria (post-radical nephrectomy) has been cured using cyanoacrylate adhesives.  Other agents such as (0.2%) povidone iodine's and radiographic contrast media (Urografin TM )  have also been used successfully for EST of chyluria. Subcutaneous octreotide has also been used to treat post-traumatic chyluria. , Thus the immediate results with EST are good but the long term follow up and recurrence rates tend to be high. 
(B) Surgical lymphatic disconnection: Traditional open surgical ligation , and excision of renal pedicle lymphatics to cure persistent chyluria has now been largely replaced by the minimally invasive approach of retroperitoneoscopy. ,,, Nephrolympholysis, ureterolympholysis, hilar vessel stripping, fasciectomy and nephropexy has been also been accomplished retroperitonoscopically. ,, 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  and procedures such as subcutaneous emphysema, port site infections and inadvertent clipping of other structures at the renal hilum.  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  as well retroperitoneally.  Retroperitoneoscopic access avoids the potential problems of transperitoneal breach such as prolonged ileus, peritonitis and bowel injury. , 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).  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. 
(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. , 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. , 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|| |
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.
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[Figure - 1]
[Table - 1], [Table - 2], [Table - 3]