The disease of chyluria has a unique unpredictable course associated with remission and exacerbation. It can lead to considerable weakness and weight loss especially when it is chronic and recurrent.[12345] Recurrence is defined as the relapse of chyluria after a period of initial clearance.[6] Recurrence of chyluria may be on account of the leakage of chyle from the contralateral side or from the same unit previously treated.[678] Recurrence of chyluria is highest with the conservative treatment, i.e. dietary modification and antifilarial drugs which have been reported as high as 80%.[7] Recurrence after renal pelvic instillation resclerotherapy (RPIS) has been reported between 13 and 41% at a follow-up of 2-115 months.[67910] Remission is the period of lessening of intensity of chyluiria and this period may last for few weeks to few months. Failure of treatment is defined as the persistence of chyluria from the treated unit. Immediate failure has been reported between 10 and 20% after the first course of RPIS.[67] Occurrence of chyluria from the contraletral site in immediate postintervention period should not be mistaken with the failure of the treatment. Some of the important causes of recurrent chyluria are described in Table 1.
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Table 1: Causes of recurrence of chyluria
Indications for surgical intervention
Patients having persistent chyluria, clot colic, retention of urine, hypoprotenemia, progressive weakness and weight loss despite conservative management and sclerotherapy need surgical intervention.[5131415] Recurrent chyluria is a disturbing situation as this not only affects the psyche of the patient but also affects the economy depending on the severity of the disease. The main stays of the treatment are resclerotherapy, open or laparocopic chylolymphatic disconnection, microsurgical lympho-venous anastomosis and autotransplantation [Table 2]. [91011121314151617181920] A simple algorithm for the treatment of recurrent chyluria is given [Figure 1].
Table 2: Treatment options for recurrent chyluria
Figure 1: Algorithm for the treatment of recurrent chyluria
Renal pelvic re-instillation sclerotherapy
Renal pelvic instillation resclerotherapy (RPIS) along with diethyl carbamazine is the treatment most frequently used. In sclerotherapy the various agents used are 10-25% bromide, 50% glucose-saline, 1-2% silver nitrate or 0.2% povidone iodine. The cumulative success rate after two courses of RPIS has been reported up to 82% in the silver nitrate and 83% in the povidone group.[6] Second course should be repeated after a gape of 3 months. Second time instillation may be of the same scloresant or a different agent, i.e. povidone iodine followed by povidone iodine or silver nitrate followed by povidone iodine or vice versa. Patients failing second course of RPIS with significant chyluria should be treated with chylolymphatic disconnection or microsurgical procedures like lympho-venous or lympmhnodo-venous anastomosis.[68]
Chylolymphatic disconnection
The procedure of open chylolymphatic disconnection was first reported by Dr. Phani Bhushan Prasad.[11] Now the same procedure is commonly known as Patna operation, in which the areolar tissue containing dilated lymphatics traveling to the kidney in the perirenal and hilar region is dissected and divided between ligatures, thus stripping these structures completely.
The kidney is exposed through an extraperitoneal flank approach. The renal fascia containing fat, areolar tissue and lymphatic plexus is dissected and stripped from entire kidney surface dividing it in between ligatures. Lymphatics along the renal artery and vein are also ligated and divided. The renal pelvis and upper ureter are bared in the similar manner. In cases of bilateral disease only one side is operated at a time. The overall success rate was up to 98% at a follow-up of 1-15 years.[511] Now a days open chylolymphatic disconnection is less commonly practiced as the same procedure is now done by less invasive retroperitoneoscopic technique with equally impressive results.[8]
Lympho-venous anastomosis
This is the most physiological method of surgical correction for recurrent chyluria.[17181920] The procedure increases the drainage of lymph into venous system, which rapidly decreases the intralymphatic pressure. Thus facilitating the healing of pyelo-lymphatic fistulae. The procedure is technically cumbersome as lymphatics are difficult to identify, lymphatic channels are thin, brittle and liable to collapse, which requires microsurgical expertise.
Retro-peritoneal lympho-venous anastomosis
The technique was described by Cockett and Goodwin.[20] Jiang and Hu (1982) treated 29 patients of chyluria with retroperitoneal lympho-venous anastomosis (RPLVA). At a follow-up of 1-14 years, 24 patients were cured and four were improved. The shortcomings of RPLVA are deep seated operative field, risk of renal pedicle injury, risk of renal artery stenosis and renovascular hypertension.[20]
Trans-inguinal spermatic lympho-venous anastomosis
The procedure has been described in male patients with recurrent chyluria. Inguinal hernia incision is used.[1920] About 1 ml of 1% methylene blue + 1% procaine injected in testis. Three large blue stained lymphatics and spermatic veins (of similar caliber) are dissected out. End-to-end interrupted anastomosis is done by 10-0 nylon. Xu et al.[17] described 64 patients in whom transinguinal spermatic lymphangiovenous anastomosis was performed [Figure 2]. Follow up was 6 months to 11 years in 50 of the patients. Chyluria disappeared completely in 30 (60%). In another study by Zhao et al.[19] reported a success rate of 76.3%. The procedure has the advantage of a superficial operative field and is simple and less traumatic.
Figure 2: Transinguinal lymphovenous anastomosis
Inguinal lymph node-saphenous vein anastomosis
Here, a lymphnodo-venous anastomoses is made according to the principles of lymphovenous shunt. A conical tissue of lymph node close to the greater saphenous vein in the inguinal region is removed and the remaining tunnel-shaped node is anastomosed to the vein to drain the lymph into the venous system. Houet al[15] treated 30 cases of chyluria and 21 cases were followed up for 6 months after the operation. Among them, 16 (76.2%) showed disappearance of chyluria and 2 (9.5%) were improved, giving an effective rate of 85.7%. The operation avoids damage to both the afferent and efferent lymphatic vessels and affords a large anastomotic stoma for free passage of the lymph into the vein. In another study by Jiet al[16] where in males a lymphatico-venous anastomosis of the spermatic cord was performed and in females the lower inguinal lymph nodes were anastomosed to the branches of the greater saphenous vein. In patients with scrotal lymphangial fistulae, bilateral lymphatico-venous anastomosis was carried out after excision of the fistula and scrotoplasty. Thirty-seven patients were followed up for 1-9 years, 36 being cured by a single operation.
Autotransplantation or nephrectomy
Brunkwallet al[13] reported a case of recurrent chyluria after failed initial surgical treatment, which consisted of stripping of the renal pedicle. Patient was successfully managed by renal autotransplantation. Before the advent of sclerotherapy and chylolymphatic disconnection more formidable procedure like nephrectomy has also been described for recurrent chyluria.[3]
CONCLUSION
Recurrent chyluria is a significant health problem in certain parts of the world, which are known for the endemicity of the filarial disease. The most common procedure practiced is the resclerotherapy. Patients failing to second courses of sclerothrapy with significant chyluria (weight loss, heavy proteinuria and anemia) should be treated with chylolymphatic disconnection. In India, procedures like microsurgical lymph venous anatomists or autotransplantation have rarely been described.
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