British Journal of Urology (1975), 41, 419-423 0

Non-parasitic Chyluria (presenting with Urinary Retention) T. V. TAYLOR, A. W. B. STRACHAN, 1. 1SHERWOOD

and

THOMAS MOORE

Manchester Royal Infirmary and Barrow-in-Furness Hospitals

Chyluria was recognised by Hippocrates, Galen and Pheophile. Pecquet in 1653 attempted to give a detailed description of the lymphatic system. It was not until 1741 that Morgagni first associated chyluria with abnormalities of the lymphatic circulation. It was not until the advent of retrograde pyelography and later percutaneous lymphangiography that abnormal connections between the lymphatic and urinary systems were demonstrated in the living subject. Pyelolymphatic reflux was demonstrated by Wood during retrograde pyelography in 1929 though it was as recently as 1963 that Kittredge er al. outlined these communications by lymphangiography. Chyluria is broadly classified into two main aetiological groups: (1) parasitic or tropical and (2) non-parasitic. Non-parasitic chyluria is rare. Clinically it presents as a metabolic disturbance due to loss of fat (Mogg and Jones, 1971), or more rarely with obstructive symptoms. Two cases of this latter group are described in adult males presenting with recurrent attacks of retention of urine. Case I

In May 1964, a 25-year-old furniture remover presented at the Manchester Royal Infirmary complaining of the passage of white milky urine. He first noticed this in 1959, and again in 1960 on isolated occasions. At the time of presentation it had been present continually for several weeks, most marked on rising in the morning. On several occasions he had experienced difficulty in micturating and complained of a continuous dull aching pain in the right loin. There was no past medical history of note, nor any family history of similar complaints. The only time he had left his home town in Derbyshire was for a period of a few months whilst stationed on Army duty in the Mediterranean during 1953. The patient was healthy in appearance and there were no abnormalities on physical examination. His urine was obviously “milky” and contained 740 mg of fatty acid per 100 ml, 8.9 g in a 24-hour specimen. Haemoglobin, blood urea, liver function tests, serum albumin and serum electrolytes were all normal. The urine was normal apart from the presence of chyle, with the exception of one sample which contained 50 red blood cells per high power field. Radiology of the chest and abdomen together with excretion pyelography were all within normal limits. Cystoscopy revealed milky urine coming through the right uretero-vesical orifice. Bilateral retrograde pyelography demonstrated incompletefilling of the right upper and middle pole calyces, but no other abnormality. Bilateral percutaneous lymphangiography outlined an abnormal connection between the para-aortic lymph channels and the right renal lymphatics (Fig. I). A number of widely dilated lymph vessels in the hilum of the right kidney were demonstrated. The thoracic duct was well outlined, indicating that no obstruction existed to the normal cranial flow of chyle (Fig. 2). The appearances represented a right lymphatico-renal fistula. Symptoms resolved spontaneously but in September 1965 the patient was re-admitted after some months of continuous chyluria. The right kidney was explored and large lymphatic vessels exposed, particularly around the right renal vein anteriorly. These vessels were mobilised and divided. Postoperatively the patient was asymptomatic until January 1969, when he had slight recurrence of chyluria which disappeared spontaneously following hospital admission. The patient is now on a low fat diet and has remained free of symptoms to date. Case 11

A 34-year-old English male presented at the Manchester Royal Infirmary in January 1966 complaining of the passage of “milky” urine accompanied by suprapubic discomfort, an aching pain in the right renal angle and some difficulty in passing urine. His symptoms began whilst he was a naval rating in Malta in 1953. Investigation in 419

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Fig. I . Case I. Lymphangiography. Abnormal connection between para-aortic lymph channels and right renal lymphatics. Fig. 2.

Case 1. Lymphangiography. Normal thoracic duct.

Malta at that time showed no abnormality apart from the occasional demonstration of fat in the urine. His symptoms recurred 2 years later when it was noticed that his urine was heavily loaded with fat and albumin and contained some red blood cells, but no pus cells were found. The chyluria cleared spontaneously after a few days. Later that year he developed a urinary tract infection, the organism being Staph. pyogenes. Excretion and retrograde pyelography were normal and the infection responded to antibiotic therapy. He was again investigated in 1956, following several attacks of incomplete urinary retention culminating in acute retention which required catheterisation. The pain which accompanied the attacks on these occasions was in both loins and both iliac fossae. The possibility that the chyluria was associated with filariasis was considered at this time, but microbiological investigation of nocturnal specimens failed to reveal any abnormality. The patient had never visited an area where this condition is endemic. He was at this stage placed on a low fat diet, whereupon the chyluria again disappeared. The patient felt that the exacerbations of colic and retention seemed less frequent and severe, and also that the quantity of lymph in his urine during the exacerbations was reduced whilst adhering to the diet. Retrograde pyelography, performed in 1959, demonstrated connections between the right renal pelvis and the abdominal lymphatics (Fig. 3). In 1964 lymphangiography was performed. In addition to abnormal lymphatic communications to both kidneys, more marked on the right side, an extensive lymphangiomatous malformation was demonstrated in the pelvis with lymphatic connections extending over the posterior abdominal wall. Contrast was demonstrated in the bladder at an early stage of the examination (Fig. 4). The thoracic duct was well outlined and showed no evidence of obstruction (Fig. 5). Manv examinations were made on the urine. When it was cloudy. fat could always be demonstrated. Sudan 111 dye was taken with a fatty meal and the pink colour of the next specimen of urine was obvious. Estimation of the quantity of fat in the urine gave various readings; those on 2 consecutive days in 1963 were 120 mg per 100 ml in 1,950 ml of urine, i.e. 2.34 g per day, and 207 mg per 100 ml in 1,800 ml of urine, 4.7 g per day. Protein was always present along with fat, and on standing at room temperature clots formed within the urine. Red blood corpuscles and polymorphonucleocytes were often demonstrated and on a few occasions organisms were found, mainly E. coli and Staph. pyogenes. Blood urea, full blood count, erythrocyte sedimentation rate, serum proteins, and liver function tests were checked on many occasions and always were within normal limits. The eosinophil count was never greater than 300/cu.m. Numerous nocturnal smears for microfilaria were negative.

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Fig. 5 Fig. 3. Case 11. Right retrograde pyelography. Abnormal connections between para-aortic lymph channels and right renal lymphatics. Fig. 4. Case 11. Lymphangiography. Abnormal lymphatic communications to both kidneys. Lymphangiomatous malformation in the pelvis. Contrast medium in the bladder. Fig. 5. Case 11. Lymphangiography. Normal thoracic duct.

The continued attacks of colic and retention inconvenienced the patient greatly and he was therefore admitted with a view to surgical exploration of both kidneys. On clinical examination at this time the only abnormality was some slight tenderness in the right renal angle and right hypochondrium. At operation there were numerous large lymphatic channels surrounding the hilum of the right kidney and a mass of lymphatic tissue was seen at the junction of the right renal vein and inferior vena cava. On the left a leash of lymphatics was present, extending from the kidney across to the cysterna chyli. All the dilated lymphatic trunks wereligated and divided. Ths patient’s postoperative condition was good. He has now remained free from symptoms for almost 10 years.

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Discussion Prout (1840) proposed the first theory to explain the aetiology of chyluria postulating that fat was secreted from the blood stream into the urine. This theory substantiated the findings that increased intake of fat increased the chyluria but failed to explain the effect of posture and exercise on chyluria and the rarity of the condition. Golding Bird (1843) finally discounted the secretory theory by showing that the fat in chylous urine was in the form of molecular particles rather than globules. In 1862 Carter suggested that chyluria might be caused by rupture of a lymphatic varix into the urinary tract. Ackermann in the following year explained the development of chyluria on the basis of a mechanical obstruction of the lymphatics between the bowel and the thoracic duct. The obstruction would produce an increase in intralymphatic pressure, lymphangiectasia, valvular incompetence and retrograde flow of chyle resulting in a lymphatico-renal shunt. Rupture of lymphatic vessels into the kidney has been explained on the basis of a relative lack of supporting tissue elements in the renal parenchyma associated with its inadequate supply of collateral blood vessels (Ackermann, 1963). Fistulous openings and chylous reflux have been reported in the trigone by Pope (1909) and by Lazarus and Marks (1946). In the embryo, lymphatic channels develop from primitive lymph sacs, 2 of which lie in the peri-renal area. The diffuse and dilated peri-renal lymphatics described in these 2 cases and demonstrated by lymphangiography may represent an abnormal or incomplete differentiation of these peri-renal lymph sacs. In favour of such a congenital abnormality is the absence of any evidence of obstruction to these lymphatics or to the thoracic duct. This suggests abnormal development in one or more of the primitive lymph sacs, the posterior lymph sacs in the region of the sacro-iliac joints are abnormal as well as the peri-renal sacs in Case 11. It would appear not to be a generalised abnormality but perhaps a localised lymphatic malformation such as lymphangioma involving one or more of the primitive lymph sacs. Clinicalfeatures

The disorder is usually unilateral, the left kidney being more frequently affected and symptoms are characteristically intermittent. Chyluria has been reported in an infant of 17 months by Brandenberg (1909), but most cases present in the second or third decade. Pain is a common symptom, presumably due to obstruction along the urinary tract and acute retention of urine is known to occur (Craig and Woodroffe, 1969). These features are presumably referrable to the viscosity of the urine from the chyle which formed “clots” in our 2 cases. More commonly there is genera1 impairment of health with weight loss, debility or anaemia. Superimposed urinary tract infection is common (Yamauchi, 1945). Treatment A fat-free diet has a palliative effect on the exacerbations of symptoms and during an attack the effect may well be dramatic (Ray and Rao, 1939). Treatment based upon injection of sclerosing agents into the lymphatics or local diathermy of the fistula tends to be unsatisfactory (Mogg and Jones, 1971). Visualisation of the lymphatics by percutaneous lymphangiography and the use of dyes such as Sudan I11 and methylene blue have enabled elaborate by-pass procedures to be carried out such as jugulo-thoracic duct anastomosis and anastomosis of a varicose lymphatic and the testicular or ovarian vein (Cockett and Goodwin, 1962). Surgical stripping of the renal pedicle lyniphatics is the treatment of choice in most cases where surgery is necessary though in infective cases abnormal communications may recur.

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Summary Two cases of non-parasitic chyluria are presented each presenting with retention of urine. Abnormal communications in both cases have been demonstrated lymphangiographically. The thoracic ducts were patent and there was no evidence of filariasis. Surgical removal of the anomalous dilated lymphatics was carried out, the patients being symptom-free at 7 and 10 years. The two cases seem to have lymphatic malformations which may be the basis of the disorder.

References ACKERMANN, T. (1863). Ein Fall von Galacturie (Urina chylosa). Deutsche Klinik, 15, 233. BIRD,GOLDING (1843). Remarks on fatty urine. London Medical Gazette, 33, 110-112. BRANDENBERG, F. (1909). Ein Fall von Chylurie mit Glykosurie. Deutsche medizinische Wochenschri/i, 35,934-935. CARTER,H. V. (1862). On the connection between a local affection of the lymphatic system and chylous wine. Medico-chirurgical Transactions, 45, 189-208. COCKETT, A. T. K. and GOODWIN, W. E. (1962). Chyluria: attempted surgical treatment by lymphatic venous anastomosis. Journal of Urology, 88, 566-568. F. J. (1969). Chyluria and lymphatic renal fistula. Clinical Radiology, 20, 465-472. CRAIG,0. and WOODROFFE, HIPPOCRATES (1665). The eight sections of Hippocrates’ aphorisms reviewed and rendered into English according to the translation of Anutius Foesius, 7, 35. London: Crofts. KIITREDGE,R. D., HASHIM,S., ROHOLT,H . B., VAN ITALLIE,T. B. and FINBY, N. (1963). Demonstration of lymphatic abnormalities in a patient with chyluria. American Journal of Roentgenology, 90, 159-165, LAZARUS, J. A. and MARKS,M. S. (1946). Non-parasitic chyluria with special reference to traumatic chyluria. Journal of Urology, 56, 246-258. MOOG,R. A. and JONES,J. H. (1971). Non-parasitic chyluria. British Journal of Urology, 43, 28-34. MORGAGNI, G. B. (1741). De Sedibus et Causis Morborum, Liber 42, vol. 2., p. 44. PECQUET, J. (1653). New anatomical experiments by which the hitherto unknown receptacle of the chyle, and the transmission from thence to the subclavian veins by the new discovered lacteal channels of the thorax is plainly made appear in brutes. London. pp. 141-148. POPE,S. (1909). Chyluria with a vesical sinus. California State Journal of Medicine, 7 , 285-288. PROUT,W. (1840). On the Nature and Treatment of Stomach and Urinary Diseases, 3rd ed., London: Churchill. p. 120. RAY,P. N. and RAO,S. S. (1939). Chyluria of filarial origin. British Journal of Urology, 11, 48-64. SCHIELD,P. N., Cox, L. and MAHONY, D. T. (1966). Anatomic demonstration of mechanism of chyluria by lymphangiography with successful surgical treatment. New England Journal of Medicine, 274, 1495-1497. WOOD,A. H. (1929). Unilateral renal chyluria. Journalof Urology, 21, 109-117. YAMAUCHI, S. (1945). Chyluria: clinical laboratory and statistical study of forty-five personal cases observed in Hawaii. Journal of Urology, 54, 318-347.

The Authors T. V. Taylor, FRCS, Surgical Registrar, Manchester Royal Infirmary. A. W. B. Strachan, FRCS, Consultant Surgeon, Barrow-in-Furness Hospitals. I. Isherwood, FFR, Consultant Radiologist, Manchester Royal Infirmary. Thomas Moore, MS, FRCS, Consultant Urological Surgeon, Manchester Royal Infirmary. Requests for reprints to T. V. Taylor, FRCS. University Department of Surgery, The Royal Infirmary, Manchester M13 9WL.

Non-parasitic chyluria (presenting with urinary retention).

Two cases of non-parasitic chyluria are presented each presenting with retention of urine. Abnormal communications in both cases have been demonstrate...
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