Chyluria

CHYLURIA DAVID B. CRANE, WILLIAM

M.D.

E. WHEELER,

M. J. VERNON

SMITH,

M.D. M.D.

From the Division of Urology and Department Radiology, Medical College of Virginia, Richmond, Virginia

of

ABSTRACT -A twenty-four-year-old black female presented through the outpatient department complaining of “white urine. ” Pertinent history, physical examination, and diagnostic studies are reviewed with special emphasis on lymphangiography. The literature is reviewed particularly with regard to lateralixation of the fistulas, thoracic duct obstruction, and methods of therapy.

Chyluria, the passage of chyle (a turbid, white fluid containing suspended fat globules) in the urine, may be secondary to anything capable of causing lymphangiostasis. The very rare nonparasitic form, also known as nontropical or European, may be secondary to pregnancy, diabetes mellitus, pernicious anemia, congenital malformations, thoracic duct or abdominal lymphatic obstruction (trauma, tuberculosis, abscess, carcinoma). l4 The parasitic form, more common in the Far East, is rare in North America. Recently a de novo parasitic case appeared in Richmond, Virginia. Only 10 other cases of parasitic chyluria have been reported in the continental United States in the last fifteen years. 1*5-14Two cases of nonparasitic chyluria have been reported in the United States.4s15

at age nineteen. She had returned to British Guiana in 1966 and 1968. During her visit in 1966, she required medical treatment for severe mosquito bites. The ectomorphic patient weighed 98 pounds. Other than bilateral fibrotic breast masses and dry skin, her physical examination was normal. Her chest x-ray film was also normal. Her urine was creamy white, with a specific gravity of 1.030, pH 6, and 3 plus protein (Fig. 1). Microscopic examination showed many white blood cells, rare red blood cells, and amorphous

Case Report A twenty-four-year-old gravida I, para I, black female was seen in the urology clinic complaining of “white urine” for the past two weeks. She denied any dysuria, frequency, nocturia, chills, fever, back pain, trauma, previous urinary tract infections, or vaginitis. She recalled initially passing a white gelatinous clot. Subsequently she noted some diurnal variation in the cloudiness of her urine and some relation to exercise. She also related a gradual weight loss over the past year. She was born in British Guiana but moved to England at age ten and then the United States

UROLOGY

/ APRIL 1977 / VOLUME

IX

NUMBER4

FIGURE

1.

appearance

Left: initial appearance after shaken with ether-.

of urine; right:

FIGURE 2. (A) lntravenous pyelogram showing mild pyelocalyceal irregularities of right lower pole. (B) Pedal lymphangiogram showing collections of contrast around papillocalyceal lymphatic channels. (C) Chest film shows patent thoracic duct.

material. The urine cleared almost completely when shaken with ether. Her complete blood count showed no anemia or eosinophilia. Electrolytes, blood urea nitrogen, glucose, and SMA-12 were normal, Urine cultures showed no growth. Multiple morning urines for tuberculosis were negative on smear and culture; purified protein derivative was negative. No microfilaria were noted on late night thick blood smears. An intravenous urogram was normal except for mild pyelocalyceal irregularities of the right lower pole (Fig. 2A). Cystoscopy revealed chyle effluxing from the right ureteral orifice. Right retrograde pyelogram showed no pyelolymphatic backflow. Dorsal lymphatic vessels of both feet were cannulated by the technique of Kinmouth.16 Eight ml. of ethiodized oil were injected into each cannulated lymphatic over one hour. Films taken at the end of the injection revealed ectatic and tortuous lymphatic channels in the pelvis, especially on the left side. Varicose lymphatic vessels around the right renal hilus were evident. Circular and semicircular collections of contrast outlined the papillocalyceal lymphatic channels (Fig. 2B). Contrast was seen in the renal pelvis, and radiographs of the patient’s urine demonstrated contrast in the urine. No obstruction to lymph flow was evident in the abdominal para-aortic nodes or cysterna chyli. Chest films revealed a normal thoracic duct without evidence of obstruction (Fig. 2C). Twenty-four-hour films revealed a small amount of residual contrast in the pelvic and renal lymph channels. The para-aortic nodes were unremarkable, and the main abdominal lymphatic channels had emptied. Biopsy of the left breast mass showed chronic cystic mastitis.

430

She continued to demonstrate significant chyle in her urine at bed rest and on a low-fat diet. Under general anesthesia her right renal pelvis was irrigated with 2 per cent silver nitrate solution. Postoperatively mild flank tenderness and a low-grade fever developed. By the third postoperative day her urine had cleared and her symptoms abated. She was tried on a regular diet and then a fatty meal; her urine remained clear. Diethylcarbamazine (Hetrazan) 100 mg. orally three times a day was given for ten days as a precaution against any residual active disease. She has remained asymptomatic for one year. Comment Parasitic chyluria is far more common than nonparasitic, and it is inconclusive that the disease is not parasitic simply because the organism is not found. The parasitic form may follow an infection with filariasis, Echinococcus, Cysticercus, Ascaris, or malaria. Most patients never have filaria demonstrated in their blood, urine, or tissue, but previous exposure is sufficient to make the diagnosis. In the endemic areas of northern India, southern China, southern Japan, Australia, British Guiana, Puerto Rico, and Africa as many as 10 per cent of the population may be infected.” In only about 2 per cent of the infested population does chyluria develop. Filariasis resulting from Wuchereria bancrofti is most common in human subjects. Invasion of microfilaria occurs after the bite of the mosquito vector (Anopheles, Culex, or Aedes). Man is the sole host. The worms migrate to the lymphatics where both alive and dead they stimulate fibrin disposition on the endothelial surface. Eosinophilia, edema, and finally granulation result. As the parasites are slowly destroyed, the

UROLOGY

/ APRIL1977 / VOLUMEIX

NUMBER4

granulation tissue accumulates and may calcify. The length of infection is estimated at five years. The period of time between last exposure and onset of chyluria ranges from four months to forty-five years with an average between five and ten years. “,18 Males and females are equally infected usually between twenty and forty years of age, but with a range of seventeen months and seventy years. In reviewing 226 cases, lateralization was noted by either cystoscopy or lymphangiographic filling of the renal pelvis in 193, the fistula appeared in the right in 61, the left in 63, and bilaterally in 69. While the renal fornix is the most common site of rupture, fistulas to the bladder, ureter, urethra, and seminal vesicles have also been reported (Fig. 3).2-4~‘o,‘5,18 The most remarkable symptom is the milky urine, the color being in proportion to the amount of fat. There are also many red and white blood cells, albumin, globulin, and fibrinogen. The onset may be related to cough (asthma, upper respiratory infection), position (orthostatic pressure),‘,lg exercise (usually of the lower extremity),‘s3 a fatty meal, pregnancy,” or trauma.3 On standing, the urine will form three layers: fat, albumin, and fibrin, and red and white cells mixed with debris. Mixture with ether 1: 1 will dissolve the fat. Weight loss leads to cachexia and lethargy. The skin is dark and dry. A dull back ache may be present; vesical or ureteral obstruction may occur secondary to gelatinous clots; lymphangitis of the lower extremities and/or genitalia may occur. Complete blood count may show anemia with long-standing disease or eosinophilia with acute infection. The addition of Sudan III (100 mg. to 10 Gm. of butter) will color the urine; similarly injection of I 131 triotin or ingestion of C-14 cholesterol will appear in the urine. An intravenous urogram is usually normal. Cystoscopy during acute chyluria will lateralize the side, and a retrograde pyelogram may show pyelolymphatic backflow. l3 Pedal lymphangiogram, the diagnostic method of choice, may show the fistulas and the level of obstruction. Thoracic duct obstruction with resultant engorgement of the retroperitoneal lymphatics has been proposed as the etiology for chylous reflux.‘O Increased hydrostatic pressure ruptures the lymphatic vessels at their weakest point the renal fomices - creating the fistula. A review of 60 cases reporting lymphangiography showed 32 patent ducts, 10 obstructed ducts,

UROLOGY

/

APRIL 1977

/

VOLUME

IX

NUMBER4

FIGURE

3.

Lateralization

and

localization

of

jistulas.

and 18 failed to report the status. Thoracic duct obstruction does not appear to be the primary cause of chylous reflux.21-23 Alteration of the para-aortic lymphatic valves and nodes is the more likely etiology.‘7,22 Differential diagnosis should include severe pyuria, lipiduria secondary to fat emboli (urine will show fat globules but no fibrin), pseudochylous urine (specific gravity less than 1.012 with less than 2 per cent solid),3 caseous material from cavitating renal tuberculosis and amorphous urates. The natural history of the disease is unpredictable with varying periods of remission. In the severe form, there is a progressive wasting with anemia, debility, and exhaustion. Medical management should be tried initially. For a small amount of chyluria without symptoms, reassurance and time may suffice. Diethylcarbamazine 0.5 to 2 mg. per kilogram three times a day for one to three weeks is effective only in the active disease.18 Once chyluria is established, diethylcarbamazine is of limited value. A fat diet of only medium-chain triglycerides (less than 12 carbons) which enter the circulation through the portal system rather than the thoracic duct may be of value.‘O Similarly a strict low-fat diet may decrease the loss of fat.’ Bed rest is also helpful, Use of a tight corset to increase intra-abdominal pressure has been reported successful in 2 cases6 but is not generally accepted as a primary method of treatment. The lymphangiogram has been noted to be

431

therapeutic in several cases with subsequent resolution of the chyluria. 8~12~22 For severe or continued chylous urine irrigation of the renal pelvis with normal saline, 1 to 2 per cent silver nitrate, 10 to 25 per cent sodium iodide or sodium bromide, or 15 per cent potassium iodide has been utilized to sclerose the fistula. Several attempts may be necessary, and only transient improvement may result. Follow-up is mandatory. Surgical intervention should be limited to unresponsive progressive cases with persistent colic, urinary retention, or persistent weight 10~s.‘~~~~Ligation of the renal lymphatics is the treament of choice and has a high incidence of success. 20*21,24-26 Renal decapsulation has been tried with less success as has a combination of the two procedures. 11,27Cockett and Goodwin’ attempted a lymphatic-venous anastomosis, but because of the small size of the vessels it was a technical failure. Long-term follow-up on any of the methods of treatment are few. Nephrectomy has occasionally been advocated primarily2s or required after technical failure of other methods.24s25 Nephrectomy is inadvised secondary to the bilaterality of the disease; the removal of one kidney may result in shunting of lymphatic flow to the opposite kidney, 5 and the disease is rarely severe enough to warrant removal of a vital organ. With the increased mobility of the world’s population, parasitic chyluria will be seen more frequently in nonendemic areas as a result of earlier infection. The prolonged intermittent course requires close follow-up. Washington University School of Medicine 4960 Audubon Avenue St. Louis, Missouri 63110 (DR.

Chyluria and pregnancy, Am. J. Obstet. 83: 521 (1962). HERTZ, A. F.: Observations on a case of chyluria occurring in England, Lancet 172: 733 (1967). LAZARUS, J. A., and MARKS, M. S.: Non-parasitic chyluria with special reference to traumatic chyluria, J. Ural. 56: 246 (1946). EHRLICH, R. M., HECHT, H. L., and VEENEMA, R. J .: Chyluria following aorto-iliac bypass graft: a unique method of radiologic diagnosis and review of the literature, ibid. 107: 362 (1972). JOHNSTON, D. W.: Chyluria: a case report and review of literature, Ann. Intern. Med. 42: 931 (1955). BLOMSTRAND, R., THORN, N. A., and AHRENS, E. H., JR.: The absorption of fats, studied in a patient with SALL,

3.

4.

5. 6.

432

9.

10.

11.

12.

13.

14.

15.

16.

17.

18.

19. 20. 21.

22.

S.:

Gynecol.

2.

8.

CRANE)

References 1.

7.

23. 24 25.

26.

27. 28.

chyluria. I. Clinical investigation, Am. J. Med. 24: 958 (1958). and GOODWIN, W. COCKETT, A. T. K., E.: Chyluria: attempted surgical treatment by lymphatic-venous anastomosis, J. Urol. 88: 566 (1962). SWANSON, G. E.: Lymphangiography in chyluria, Radiology 81: 473 (1963). of lymphaKI~REDCE, R. D., et al. : Demonstration tic abnormalities in a patient with chyluria, Am. J. Roentgenol. 90: 159 (1963). HASHIM, S. A., ROHOLT, H. B., BABAYAN, V. K., and VAN ITALLIE, T. B.: Treatment of chyluria and chylothorax with medium-chain triglyceride, N. Engl. J. Med. 270: 756 (1964). CALLAHAN, D. H., GRAF, E. C., GERSACK, J., and TURBOW, A. M.: Lymphangiography and simultaneous excretory urography as a diagnostic aid in chyluria, J. Urol. 93: 417 (1965). LANG, E. K., REDETZKI, J. E., and BROWN, R. demonstration of lymL. : Lymphangiographic phaticocaliceal fistulas causing chyluria (filariasis), ibid. 108: 321 (1972). STAMS, U. K., GURSEL, E. O., and VEENEMA, R. J.: An unusual radiologic finding in a patient with chyluria, ibid. 112: 128 (1974). MORGAN, R. R., and LAROTUNDA-FORMATO, M. . Chyluria: report of a case and review of the liter&-e ibid. 87: 200 (1962) CHOI: J. K., and WIEDE~ER, H. S.: Chyluria: lymphangiographic study and review of literature, ibid. 92: 723 (1964). in man. A KINMOUTH, J. B.: Lymphangiography method outlining lymphatic trunks at operation, Clin. Sci. 11: 13 (1952). YAMAUCHI, S. : Chyluria: clinical, laboratory and statistical study of 45 personal cases observed in Hawaii, J. Ural. 54: 318 (1945). SANJURJO, L. A.: Parasitic diseases of the genitourinary system, in Campbell, M. F., and Harrison, J. H., Eds.: Urology, 3rd ed., Philadelphia, W. B. Saunders Company, 1970, vol. 1, p. 480. WIGGELINKHUIZEN, J., LANDMAN, C., and GREENBERG, E.: Chyluria, Am. J. Dis. Child. 124: 99 (1972). SERVELLE, M., et al. : Chyluria in abnormalities of the thoracic duct, Surgery 54: 536 (1963). LLOYD-DAVIES, R. W., EDWARDS, J. M., and KINMOUTH, J. B.: Chyluria. A report of five cases with particular reference to lymphography and direct surgery, Br. J. Ural. 39: 560 (1967). AKISADA, M., and TANI, S.: Filarial chyluria in Japan. Lymphography, etiology and treatment in 30 cases, Radiology 90: 311 (1968). CHEN, K.: Lymphatic abnormalities in patients with chyluria, J. Ural. 106: 111 (1971). CHANG, C., LUE, Y. B., and LAPIDES, J.: Surgical treatment for chyluria, ibid. 109: 299 (1973). OKAMOTO, K., ASECHI, S., and NACATA, K.: Distribution of chyluria and its treatment in Japan, Urol. Int. 17: 241 (1964). KISHIMOTO, T., HICUCHI, T., ENDO, M., and KAI, . Lymphography in a patient with unilateral ~h$uria, J. Ural. 92: 574 (1964). TORRES, L. F., and ESTRADA, J.: Experiences in treatment of chyluria, ibid. 87: 73 (1962). SANGMIT, S. : Non-parasitic chyluria: report of a case, J. Int. Coll. Surg. 42: 273 (1964).

UROLOGY

/ APRIL 1977 / VOLUME

IX NUMBER4