Chyluria is a distressing condition known for remissions and exacerbations. It is shown that chyluria despite being intermittent does not significantly affect general health and no chyluria related death has been reported. Spontaneous remissions are reported in nearly 50% of cases and this has to be kept in mind when analysing conservative treatments claiming success. Various conservative measures include dietary modification, anti filarial drugs, retrograde intrapelvic instillation of sclerosants have been reported with reasonable success. Problem with any of these treatments is recurrence. Surgery assumes importance in such refractory cases. This article discusses indications of open surgery, various techniques their rationale and outcome with review of literature.
INDICATIONS FOR SURGERY
Various terminologies are used to define the severity of chyluria as an indication for surgery. It is known that 20% of chylurics have unrelenting course requiring surgery. The indications are a weight loss, hypoproteinemia, anasarca, recurrent clot retention, hematochyluria, recurrent urinary tract infection because of Chyluria, refractory chyluria (defined as failure of conservative treatment with adequate dietary modification, medical management and two or more instillations of sclerosants), altered immune status, marked psychological disturbance.
Hemal et al. based on retrograde pyelogram stratified chyluria into three grades: mild - involvement of one calyx, moderate - involvement of two or more calices and severe where most of the calices are involved. They advocated surgery in failed cases of mild to moderate grade and in severe grade of chyluria. Dalela et al. used recurrent 'clinically significant chyluria' (defined as those associated with chylous clots, or hematochyluria or duration of more than 1 year and or failure to respond to conservative medical measures) as their indication for surgery.
ROLE OF IMAGING
In routine practice intravenous urogram (IVU), cystoscopy followed by lymphoscintigraphy or pedal lymphangiography is performed before surgery. Yu et al. considered IVU necessary in all cases, which are reiterated by others. Punekar et al. did not find IVU useful either in pre-op diagnosis or any detrimental effect on renal function following surgery. We feel that, IVU or some sort of functioning study is a must before and after operation as there are reports of kidney becoming non-functional following lymphovenous disconnection.
Similarly, the role of radioimaging in developing countries is debated. The advantages of lymphangiography are localization of reflux, severity and extent of reflux, lateralisation and tackling of recurrence after surgery. Argument against routine lymphangiography is that the radiological lesion may not be associated with chylous leak clinically or endoscopically. If microsurgical procedures are used then there is no need to do either imaging or cystoscopy.
Various procedure described are lympho-venous disconnection, ureterolympholysis (Patna operation), Cockett and Goodwin procedure, microsurgical techniques, renal autotransplanta-tion and nephrectomy.
It is the most commonly performed procedure, first described by Katamine in 1952 and popularised by others with some modifications. Apart from routine preparation for any surgery, patients were put on high fat diet 24-36 h prior to surgery. This is to facilitate intra-op detection of lymphatic channels. Some do routine pedal lymphangiography followed by continuous use of patent blue for perirenal lymph vessels. The disadvantage of latter is that, if there is a tear in lymphatic vessel during surgery, discoloration of tissues will render further dissection difficult. For similar reasons Chang et al. discontinued routine use of methylene blue during surgery.
Pedal lymphangiography was done initially, by canulation of lymphatics on both sides. But, with demonstration of significant cross over flow to the contralateral side, currently one-side injection is been done. Punekar et al. after initial experience with bilateral injections, performed lymphangiography on one side only in rest of their 62% cases with equal results. For similar reasons bilateral surgery at same time is not required as majority will resolve or become so negligible that patient wanting additional surgery is very rare.
Approach is by extraperitoneal standard flank incision. Either turner warwick or 11th rib incision can also be used. Renal fascia containing fat, areolar tissue and the superficial lymph plexus was divided preferably with sharp dissection rising flaps anteriorly and posteriorly upto the renal hilum like, flipping of page in a book. Subsequently mobilizing the flap superiorly securing vessels and lymphatics in between ligatures. Once the kidney is freed all around, the dilated lymphatics in the renal hilum coursing along the renal vein are dissected gently without tearing (which leads to oozing and pooling of lymph in the operative field). If torn it is better to ligate the oozing vessel with absorbable suture rather than coagulating as the lymphatics have lower protein content and fewer cellular moieties than blood vessels making elctro cautery ineffective. The lymphatics are divided between the ligature to bare at least one cm of the artery and vein along the entire circumference. 100 ml of 12.5% I.V Mannitol is administered before dissecting around the renal hilum as it prevents arterial spasm and transient renal ischemia.
After lympho-venous disconnection at the renal hilum, which is an essential and crucial step, the lymphatics around the upper ureter are similarly disconnected. The extent of ureteral disconnection varies from 3 to 4 cm to the entire length including around the bladder. Prasad et al. introduced ' ureter in a lymphatic tunnel concept ' (formed by pampiniform plexus in front and pre and para aortic plexus behind). They did lymphatic disconnection of the abdominal ureter only (so called Patna operation) without renal hilar disconnection. In their series of 15 cases followed for 6 months to 14 years, eight had surgery on one side (right). Of the seven who had bilateral surgery, one had synchronous procedure and the rest had contralateral procedure after 2 months. The reason for doing contralateral operation after an interval of 2 months has not been explained. They claimed an overall success of 93% with one recurrence. Results of lympho-venous disconnection are given in Table 1.
Routine stripping of entire ureter up to bladder is not recommended as majority of the leak occurs around the renal hilum. In case of recurrence or demonstration of obvious leak at the bladder level either on radioimaging or on pedal lymphangiography dissection up to the bladder is mandatory.
Additional procedures included diversion of a single hilar lymphatic to spermatic or gonadal vein in an end to side fashion. Theoretically this would obviate the need of complete stripping and the complications thereof. It also provides a safety valve mechanism for renal lymphatic hypertension, which is the basis for chyluria. Of the four cases, one never regained renal function because of renal vascular damage. The advantage claimed was decreased lympho cutaneous fistula. The disadvantages were increased operative time, need for operative loop or microscope and it is technically demanding. Some people routinely fix the kidney in the retroperitoneum to avoid torsion of the renal vessels and wound drain to prevent lymph collection but these are not necessary.
Includes lymphangio-venous anstamosis and lymphnode-saphenous vein anastamosis. These procedures are more physiological as they rapidly decrease the intralymphatic pressure by increasing drainage of lymph fluid into the venous system, bring about decrease in lymphangiectasis and facilitates healing of the lymphatic fistula in the renal papilla. The advantage is that the procedure is relatively superficial which makes it simpler and easier and is less traumatic to the surrounding tissues. Other advantage is that it obviates the use of lymphangiography and cystoscopy since there is no relation between site of operation and side of lymphorenal fistula. But some do routine cystoscopy before the operation.
The disadvantage of the operation is that it is technically demanding and some times it takes up to 6 months before it starts showing the results. Of the two microsurgical procedures, lymphangio-venous anastamosis is much more technically challenging more so in females as the caliber of the vessels is very thin. In one series, it was observed that, 90% of lymph vessels had diameter of < 1 mm and the vessel wall consisted of one layer of endothelium making magnification very essential for the success of operation. Other difficulty is to find a vein of adequate diameter and in an adequate place.
The procedure is indicated in the treatment of persistent or recurrent chyluria and is also suitable for those older and weaker patients who could not tolerate ligation and division of lymphatics. In men, the lymphangio-venous anastamosis was done in the inguinal region and in the women it was done either in the dorsum of the foot or in the leg and thigh using end-to-end anastamosis. A pre requisite for the success of the operation is the patency of the anastamosis. To increase the drainage of the lymph into the venous system and to allow rapid fall in intralymphatic pressure two or more anastamosis are preferable. If the diameter of the lymphatics are < 0.1 mm then a bundle of lymph vessel is cut and inserted into a vein of corresponding caliber with few anchoring sutures.
Ji et al. did end to side anastamosis of two or more lymphatics to one vein when the caliber was very small. In females they performed lymphnode-saphenous vein anastamosis where in they made a 2-3 mm diameter hole into the medulla of the lymph gland and after confirmation with efflux of lymph, it was anastamosed to the obliquely cut end of the vein with ligation of the distal end. Success rates with these procedures vary from 60 to 83%.
Brunkwall et al. reported renal autotransplantation in a 21-year-old male with Klippel-Trenaunay-Weber syndrome who had failed stripping of renal pedicle 3 months earlier. Following autotransplantation he was symptom free for more than 12 months. They recommended this procedure only when lymphovenous disconnection had failed.
Kekre et al. reported bilateral nephroureterolysis with omental wrapping for Chyluria of nonfilarial origin. Recently Dalela et al. suggested the use of omentum to wrap the renal vessels after stripping. They claimed that it reduces lymphocele by absorbing lymph and obviates use of drain, decreases recurrence and ease of re-operation if recurrence occurs.
Nephrectomy should never be the first option. It is rarely needed except for Kidneys rendered nonfunctional following lymphovenous disconnection or as a life saving measure with severe surgically refractory chyluria.
CAUSES OF RECURRENCE AND WAYS TO TACKLE IT
Recurrence could be due to incomplete stripping, recanalisation, reflux from the contralateral side or at the terminal ureter or bladder. Radioimaging and lymphangiography helps in recognising leak at the bladder level and helps to tackle it at the initial surgery. Similarly cystoscopy findings like blebs under the mucosa or loosely hanging chylous clots from the bladder wall provides clues about the leak at the bladder.
If there is a recurrence after lymphovenous disconnection lymphangiography is mandatory before re-surgery. Operating microscope or magnifying loops helps in identifying minor leak points and helps in reducing the recurrence. Use of omentum may reduce the recurrence.
Open surgery is indicated only in unrelenting chyluria. Lymphovenous disconnection consisting of hilar stripping and ureterolymphatic disconnection is the treatment of choice. Although microsurgical procedure is most physiological it is technically demanding and there are issues to be addressed. Renal decapsulation, fasciectomy and nephrectomy are obsolete procedures and renal autotransplantation is seldom required. Omental wrap is only complementary and the success of the procedure depends on the completeness of the clearance of lymphovenous communication. Minimally invasive procedures like retroperitoneoscopy or laparoscopy make this operation less morbid. Although surgery has 98% success it is important to remember that recurrences can still occur especially in long term.
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