Chyluria: introduction, etiology, diagnosis and treatment

Chylous urine

Introduction:

•  Chyluria  -  Milky urine

•         Defined as leakage of lymphatic fluid in urine.

•         An infrequently discussed problem which is not uncommon in our area.

•         Described by CHARAK in 300B.C. as ‘ SHUKLAMEHA’

•         Prevalent in African countries and Indian Subcontinent.

•         Seen in rural and economically weaker population

Etiopathogenesis:

Chyluria is a state of chronic lymphourinary reflux caused by obstruction to the lymphatic flow.

•         Parasitic

•         Non – Parasitic

            Parasitic infestation

                         /

            Obliterative lymphangitis

                         \

            Lymphatic hypertension

                          /

            Varicosity and collateral formation

                           \

            Failure of valvular system

                           /

            Back flow

                        \

            Rupture of varicosities into renal calyces and pelvis.

 

PARASITIC (Primary, Tropical)

•         Wuchereria  bancrofti (most important and most common)

•         Eustrongilus gigas

•         Taenia echinococcus

•         Taenia nana

•         Malarial parasites

•         Cereonomas nominitis

NON – PARASITIC (Secondary, Non – Tropical)

•         Congenital

•         Lymphangiomas of urinary tract

•         Megalymphatics with ureteral or vesical fistulae

•         Stenosis of thoracic duct

•         Retroperitoneal lymphangiectasia.

•         Traumatic lymphangio urinary fistulae

•         Obstruction of lymphatics due to

–        thoracic duct obstruction by tumor

–        granuloma glands, aortic aneurysm

Other causes

•         Pregnancy

•         Diabetes

•         Pernicious  Anemia

Clinical features:

1. Monosyptomatic
2.  Polysymptomatic

         nutritional deficiencies

         recurrent clot colic

         urinary retention

          UTI

          Hematuria

          Immunosuppression (loss of Ig A and Ig G cause lymphopenia leading to                                                               promotion of opportunistic fungal infections, malignant tumors.)

Diagnosis:

1 .Confirm chyluria

2. Ruleout other causes of milky urine(pyuria, phosphaturia, caseousuria)

3. Confirm the cause of chyluria (99% is failarial)

4. Exclude secondary causes like tuberculosis, tumour by ultrasound abdomen or CT scan.

Urine sample:

Naked eye examination: Urine settles down into 3 layers-fat  on the top,clots in the middle and debris in the bottom

Ether test: Milky urine becomes transperant on adding Ether

Biochemical examination: For Triglycerides

Microscopy: Chylomicrons,  RBC’s,Lymphocytes

TREATMENT

•         Disease of unknown natural history

•         It is a self limiting disorder with intermittent remissions and exacerbations.

•        
Man of them require:
Reassurance
Antifilarial treatment
Dietary modifications
Correction of anemia
 Bed rest                                           Abdominal Binders          

DIETARY  MODIFICATIONS:

•         Minimal oil in diet

•         Use nonstick pans to minimize the amount of oil

•         Avoid ghee

•         Use coconut oil as cooking medium

•         Skimmed milk is better

•         Cow milk is better than buffalo `

         (Boil, cool, refrigerate for 12hrs and strain top cream layer          before use)

•         Avoid fried foods like poori, cream biscuits, parantha, chat, pastry

•         Avoid dry fruits

•          Restrict sweets

•          Avoid mutton

•          Fish and chicken are better ;roast or boil instead of   fry

•          Boiled egg is better than having it as fried or omlette  (Not more than 1-2 whole eggs/ week)

•          Routine diet should include roti, broken wheat,rice, corn flakes,dals,fruits and vegetables               

Indications for Intervention  : 

•         Weight Loss

•         Hypoproteinemia

•         Recurrent clots

•         Anaemia due to haematochyluria

•         Refractory chyluria

•         Psychological disturbance

Interventional therapy

•          Cystoscopy &sclerosant instillation(RPIS)

•          Surgical –open / laparoscopic

           Nephrolympholysis

•          Microsurgery  

RPIS  (Retrograde pelvic instillation of sclerosants)

      Agents:

           Silver Nitrate 1%

           Povidone Iodine 0.2%

           Dextrose 50%

           Hypertonic saline 3%

           Urograffin 

Mechanism:

Installation of agent – reaches lymphatics through fistulae – chemical lymphangitis – blockade of lymphatics due to edema – immediate relief.

Healing by fibrosis – permanent relief.

Procedure :

•              High fat diet evening before sclerotherapy

•              NBM for 5hrs

•                      Anesthesia – local, caudal

•              IV – antibiotics + Lasix (steroids – sos)

•              Cystoscopy to identify the side (85% unilateral, 15% Bilateral)

•              RGP – 6Fr ureteric catheter

•              Size of pelvis estimated injecting water till pain develops ( 7 – 10ml)

•              Only one side at a time

•              Contralateral side planned after 6 weeks.

Preparation and schedule

•         AgNO3 – 1gr in 100ml

•         Povidone – 1:50 dilation of 10% W/v in water

•         Povidone Iodine  + 50% dextrose

              8th hrly instillation for 3 days

              12th hrly instillation for 2 days

              ½  hrly  instillation  for  2hrs.

Response

•         70% - permanent remission with single course

•         30% - recurrence

•         2nd course of RPIS -   50% remission

                                                       50% need surgery

•         Late failure is due to recanalization of lymphatics – better response with 2nd RPIS.

SURGERY:

Indications:

         Failure of instillation therapy 

    Techniques:

      Lympho-urinary disconnection (open / retroperitoneoscopy)

                       

•         High fat diet 24 – 36hrs prior surgery

•         Kidney is freed all around

•     NEPHROLYMPHOLYSIS HILAR STRIPPING -    Renal hilar lymphatics are cleared

  URETEROLYMPHOLYSIS -   Downward mobilization of ureter up to pelvic brim                                                                                    

•         Nephropexy , fasciectomy – optional

     Lymphangio – venous anastomosis

    Men – Inguinal region

•         Women – dorsum of foot, leg, thigh

  Renal autotransplantation

To  summarise :

Ø      Chyluria – an alarming symptom with benign course

Ø      Can be diagnosed by simple urinary tests

Ø      Rare secondary causes should be ruled out

Ø      Most of them do not need any intervention except for reassurance

Ø      70-80% can be cured with RPIS

Dietary modifications may help preventing recurrence.