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Successful laparoscopic management of unilateral chyluria in a 5 year old
Journal of Pediatric Urology (2007) 3, 63e65
CASE REPORT
Successful laparoscopic management of unilateral chyluria in a 5 year old K. Senthil Ganesh, S. Rao*, A.J. D’Cruz Division of Pediatric Surgery, Narayana Hrudayalaya Children’s Hospital, No.258/A, Bommasandra Industric Area, Anekal Taluk, Bangalore 560 099, India Received 18 January 2006; accepted 7 February 2006 Available online 17 April 2006
KEYWORDS Chyluria; Lymphorenal disconnection; Laparoscopic surgery
Abstract Chyluria is defined as abnormal passage of chyle in the urine. It is usually seen in adults and is bilateral. The commonest cause is infestation with Wuchereria bancrofti. Other causes include trauma, tumours, tuberculosis and congenital malformations. This report describes the successful management of a 5-year-old child with long-standing, resistant and symptomatic unilateral chyluria. Failure of conservative treatment led to use of laparoscopic techniques to perform a lymphorenal disconnection with excellent outcome. Relevant literature is reviewed. ª 2006 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction
Case report
Chyluria, defined as the passage of milky urine containing fat and triglycerides, is a rare condition. It is usually seen in adults, is frequently due to infestation with Wuchereria bancrofti and is bilateral. We present a 5-year-old child with unilateral chyluria. The child was successfully managed by laparoscopic surgery.
A 5-year-old boy presented with a 2-year history of dysuria and passage of white clots in urine. He had not lived in an area endemic for filariasis. Examination was unremarkable. Urine triglyceride level was 875 mg/dl and no fat globules were demonstrable. Ultrasonogram and MRI scan of the renal tracts and abdomen and a VCUG were all normal. Cystoscopy, on three separate occasions, demonstrated chyluria only from the right ureteric orifice. Retrograde pyelogram did not demonstrate any renolymphatic channels. A trial of conservative treatment was initiated with a diet high in protein and restricted fat. Coconut oil was
* Corresponding author. Tel: þ91 80 7835000; fax: þ91 80 7832648. E-mail address: [email protected] (S. Rao).
1477-5131/$30 ª 2006 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jpurol.2006.02.003
64
K.S. Ganesh et al.
suggested as the cooking medium as it has a highmedium chain triglyceride content. At review 3 months later there was no improvement and laparoscopic lymphorenal disconnection was planned. Under general anaesthesia and with the child placed in a 45 right lateral position, an umbilical port was placed by open technique. Port sites are shown in Fig. 1. The right kidney was exposed by mobilizing the hepatic flexure and right colon and dividing Gerota’s fascia. Several lymphatic channels crossed over the pelvis, upper and mid ureter. The kidney (on all its surfaces and both poles), pelvis, upper ureter and renal pedicle were bared. The kidney was supplied and drained by two pairs of vessels attaching independently to the aorta and inferior vena cava. Dilated perirenal and hilar lymphatics were individually clipped and divided. Dissection was carried out in the perivascular sheath of the renal pedicle. Magnification and illumination provided by the laparoscope greatly facilitated precise identification and division of lymphatic channels. Most of the perirenal fat was removed. The kidney was left attached only by its dual pedicle. The lines of disconnection are shown in Fig. 2. There was a transient venous congestion of the upper pole that cleared by the end of the operation. The double pedicle prevented excessive mobility and precluded a renopexy. A suction drain was placed in the right retroperitoneal space. The postoperative period was uneventful and the child began passing clear urine immediately
Figure 1
Port sites.
Figure 2 Lines of lymphorenal disconnection (Ao, aorta; IVC, inferior vena cava; PUJ, pelviureteric junction).
after surgery. The urinary catheter and drain were removed on the 2nd postoperative day. The urine was clear of triglycerides on testing. The child has been on regular follow up and remains asymptomatic, and the urine remains clear 6 months after surgery.
Discussion Chyluria is an abnormal condition in which chyle appears in urine because of fistulous communication between lymphatics and the urinary tract [1]. Renal lymphatic drainage is abundant and follows blood vessels through the renal columns to exit at the renal parenchyma, and then forms several large trunks at the renal sinus. Communicating lymphatics from the renal capsule and perinephric tissue join these trunks. The foci of rupture of these lymphatic trunks into the renal system may occur at any level, calyces/pelvis/ureter or bladder. The rupture occurs due to high intralymphatic pressure [1,2]. The most common cause is infestation with W. bancrofti [2,3]. The parasite causes inflammation and sclerosis of deep pelvis and retroperitoneal nodes and lymphatic vessels. Non-parasitic chyluria may arise due to thoracic duct strictures, direct injury to the kidney, tuberculosis, abscesses, neoplasms, pregnancy or retroperitoneal lymphangiectasia. Chyluria is usually reported in young adults with or without microfilaremia, earlier in the natural history of filariasis than elephantiasis.
Successful laparoscopic management of unilateral chyluria Chyluria in children, especially in those less than 10 years of age, is rare. Diagnosis is based on a detailed history (including whether filariasis is endemic) and a urine analysis for triglycerides and fat. Cystoscopy and a retrograde pyelogram for detection of collateral lymphatics at the renal pedicle help to localize the leak site. Pedal lymphangiography is deferred in children because of the large amount of contrast required and risk of complications such as pulmonary oil embolism and local infection [1]. Imaging of retroperitoneum and kidney is carried out with ultrasonography, CT scan and MRI. It is important to consider the possibility of contralateral lesions or an incipient stage of bilateral disease when deciding on the treatment of chyluria. Therapeutic options include trial of conservative management with bed rest, fat restriction, high protein diet and coconut oil as cooking medium [4]. Lavage of renal pelvis with various agents (50% glucose, normal saline, 1e2% silver nitrate, 10e25% sodium iodide/bromide, 15% potassium iodide or povidone iodine) has been used for many years. Reported success rates vary from 59% to 68% with 51% recurrence rates among responders [5]. Problems include fatal renal and hepatic failure, arterial haemorrhage following aneurysm formation and pelvicalyceal cyst formation [6,7], and severe back pain [8]. Persistent chyluria, clot colic, urinary retention or weight loss despite conservative management requires surgical intervention. Surgery is also considered for unilateral chyluria. The various surgical methods available are surgical disconnection of lymphorenal channels, renal capsulectomy, lymphaticovenous microsurgical anastomosis and renal autotransplantation. Punekar et al. [9] report 98% success at 1 year after surgery from experience of over 15 years. Surgery seeks to sever the lymphatic communications by stripping lymphatics in the hilar region. Minimally invasive techniques allow achievement of the same objective effectively, as has been demonstrated in this report.
65
Options available to treat recurrence after disconnection include renal autotransplantation and lymphangiovenous anastomosis. Renal autotransplantation carries the risk of renal artery stenosis/renovascular hypertension at a later age. Nephrectomy should be performed only when renal condition has deteriorated and all other attempts have failed [1,8e10]. Our patient did not demonstrate any obvious cause of the chyluria. The presence of double arteriovenous drainage suggests a developmental/ congenital obstruction to lymph drainage of the kidney. This could then lead to high intralymphatic pressure and rupture into the collecting system. Brunkwall et al. [8] describe a similar association of unilateral chyluria with multiple renal vessels.
References [1] Hemal AK, Gupta NP. Retroperitoneoscopic management of intractable chyluria. Br J Urol 2002;167:2473. [2] Yagi S, Goto T, Kawamoto K, Miyawaki I, Tanaka I, Mori K, et al. Endoscopic treatment of refractory filarial chyluria: a preliminary report. J Urol 1998;159:1615. [3] Guermazi A, Brice P, Hennequin C, Sarfati E. Lymphography; an old technique retains its usefulness. Radiographics 2003; 23:1541. [4] Hashim SA, Roholt HB, Babayan VK, Vanitallie TB. Treatment of chyluria and chylothorax with medium chain triglycerides: N Engl J Med 1964;270:756. [5] Sabnis RB, Punekar SV, Desai RM, Bradoo AM, Bapat SD. Instillation of silver nitrate in the treatment of chyluria. Br J Urol 1992;70:660. [6] Kulkarni AA, Pathak MS, Sirsat RA. Fatal renal and hepatic failure following silver nitrate instillation for treatment of chyluria. Nephrol Dial Transplant 2005;20:1276. [7] Srivastava DN, Yadav S, Hemal AK, Berry M. Arterial haemorrhage following instillation of silver nitrate in chyluria: treatment by coil embolization. Aust Radiol 1998;42:234. [8] Brunkwall J, Simonsen O, Bergqvist D, Jonsson K, Bergentz SE. Chyluria treated with renal autotransplantation: a case report. J Urol 1990;143:793e6. [9] Punekar SV, Kelkar AR, Prem AR. Surgical disconnection of lymphorenal communication in chyluria. Br J Urol 1997;80:858. [10] Chang CY, Lue YB, Lapides J. Surgical treatment of chyluria. J Urol 1973;109:299.
CASE REPORT
Successful laparoscopic management of unilateral chyluria in a 5 year old K. Senthil Ganesh, S. Rao*, A.J. D’Cruz Division of Pediatric Surgery, Narayana Hrudayalaya Children’s Hospital, No.258/A, Bommasandra Industric Area, Anekal Taluk, Bangalore 560 099, India Received 18 January 2006; accepted 7 February 2006 Available online 17 April 2006
KEYWORDS Chyluria; Lymphorenal disconnection; Laparoscopic surgery
Abstract Chyluria is defined as abnormal passage of chyle in the urine. It is usually seen in adults and is bilateral. The commonest cause is infestation with Wuchereria bancrofti. Other causes include trauma, tumours, tuberculosis and congenital malformations. This report describes the successful management of a 5-year-old child with long-standing, resistant and symptomatic unilateral chyluria. Failure of conservative treatment led to use of laparoscopic techniques to perform a lymphorenal disconnection with excellent outcome. Relevant literature is reviewed. ª 2006 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction
Case report
Chyluria, defined as the passage of milky urine containing fat and triglycerides, is a rare condition. It is usually seen in adults, is frequently due to infestation with Wuchereria bancrofti and is bilateral. We present a 5-year-old child with unilateral chyluria. The child was successfully managed by laparoscopic surgery.
A 5-year-old boy presented with a 2-year history of dysuria and passage of white clots in urine. He had not lived in an area endemic for filariasis. Examination was unremarkable. Urine triglyceride level was 875 mg/dl and no fat globules were demonstrable. Ultrasonogram and MRI scan of the renal tracts and abdomen and a VCUG were all normal. Cystoscopy, on three separate occasions, demonstrated chyluria only from the right ureteric orifice. Retrograde pyelogram did not demonstrate any renolymphatic channels. A trial of conservative treatment was initiated with a diet high in protein and restricted fat. Coconut oil was
* Corresponding author. Tel: þ91 80 7835000; fax: þ91 80 7832648. E-mail address: [email protected] (S. Rao).
1477-5131/$30 ª 2006 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jpurol.2006.02.003
64
K.S. Ganesh et al.
suggested as the cooking medium as it has a highmedium chain triglyceride content. At review 3 months later there was no improvement and laparoscopic lymphorenal disconnection was planned. Under general anaesthesia and with the child placed in a 45 right lateral position, an umbilical port was placed by open technique. Port sites are shown in Fig. 1. The right kidney was exposed by mobilizing the hepatic flexure and right colon and dividing Gerota’s fascia. Several lymphatic channels crossed over the pelvis, upper and mid ureter. The kidney (on all its surfaces and both poles), pelvis, upper ureter and renal pedicle were bared. The kidney was supplied and drained by two pairs of vessels attaching independently to the aorta and inferior vena cava. Dilated perirenal and hilar lymphatics were individually clipped and divided. Dissection was carried out in the perivascular sheath of the renal pedicle. Magnification and illumination provided by the laparoscope greatly facilitated precise identification and division of lymphatic channels. Most of the perirenal fat was removed. The kidney was left attached only by its dual pedicle. The lines of disconnection are shown in Fig. 2. There was a transient venous congestion of the upper pole that cleared by the end of the operation. The double pedicle prevented excessive mobility and precluded a renopexy. A suction drain was placed in the right retroperitoneal space. The postoperative period was uneventful and the child began passing clear urine immediately
Figure 1
Port sites.
Figure 2 Lines of lymphorenal disconnection (Ao, aorta; IVC, inferior vena cava; PUJ, pelviureteric junction).
after surgery. The urinary catheter and drain were removed on the 2nd postoperative day. The urine was clear of triglycerides on testing. The child has been on regular follow up and remains asymptomatic, and the urine remains clear 6 months after surgery.
Discussion Chyluria is an abnormal condition in which chyle appears in urine because of fistulous communication between lymphatics and the urinary tract [1]. Renal lymphatic drainage is abundant and follows blood vessels through the renal columns to exit at the renal parenchyma, and then forms several large trunks at the renal sinus. Communicating lymphatics from the renal capsule and perinephric tissue join these trunks. The foci of rupture of these lymphatic trunks into the renal system may occur at any level, calyces/pelvis/ureter or bladder. The rupture occurs due to high intralymphatic pressure [1,2]. The most common cause is infestation with W. bancrofti [2,3]. The parasite causes inflammation and sclerosis of deep pelvis and retroperitoneal nodes and lymphatic vessels. Non-parasitic chyluria may arise due to thoracic duct strictures, direct injury to the kidney, tuberculosis, abscesses, neoplasms, pregnancy or retroperitoneal lymphangiectasia. Chyluria is usually reported in young adults with or without microfilaremia, earlier in the natural history of filariasis than elephantiasis.
Successful laparoscopic management of unilateral chyluria Chyluria in children, especially in those less than 10 years of age, is rare. Diagnosis is based on a detailed history (including whether filariasis is endemic) and a urine analysis for triglycerides and fat. Cystoscopy and a retrograde pyelogram for detection of collateral lymphatics at the renal pedicle help to localize the leak site. Pedal lymphangiography is deferred in children because of the large amount of contrast required and risk of complications such as pulmonary oil embolism and local infection [1]. Imaging of retroperitoneum and kidney is carried out with ultrasonography, CT scan and MRI. It is important to consider the possibility of contralateral lesions or an incipient stage of bilateral disease when deciding on the treatment of chyluria. Therapeutic options include trial of conservative management with bed rest, fat restriction, high protein diet and coconut oil as cooking medium [4]. Lavage of renal pelvis with various agents (50% glucose, normal saline, 1e2% silver nitrate, 10e25% sodium iodide/bromide, 15% potassium iodide or povidone iodine) has been used for many years. Reported success rates vary from 59% to 68% with 51% recurrence rates among responders [5]. Problems include fatal renal and hepatic failure, arterial haemorrhage following aneurysm formation and pelvicalyceal cyst formation [6,7], and severe back pain [8]. Persistent chyluria, clot colic, urinary retention or weight loss despite conservative management requires surgical intervention. Surgery is also considered for unilateral chyluria. The various surgical methods available are surgical disconnection of lymphorenal channels, renal capsulectomy, lymphaticovenous microsurgical anastomosis and renal autotransplantation. Punekar et al. [9] report 98% success at 1 year after surgery from experience of over 15 years. Surgery seeks to sever the lymphatic communications by stripping lymphatics in the hilar region. Minimally invasive techniques allow achievement of the same objective effectively, as has been demonstrated in this report.
65
Options available to treat recurrence after disconnection include renal autotransplantation and lymphangiovenous anastomosis. Renal autotransplantation carries the risk of renal artery stenosis/renovascular hypertension at a later age. Nephrectomy should be performed only when renal condition has deteriorated and all other attempts have failed [1,8e10]. Our patient did not demonstrate any obvious cause of the chyluria. The presence of double arteriovenous drainage suggests a developmental/ congenital obstruction to lymph drainage of the kidney. This could then lead to high intralymphatic pressure and rupture into the collecting system. Brunkwall et al. [8] describe a similar association of unilateral chyluria with multiple renal vessels.
References [1] Hemal AK, Gupta NP. Retroperitoneoscopic management of intractable chyluria. Br J Urol 2002;167:2473. [2] Yagi S, Goto T, Kawamoto K, Miyawaki I, Tanaka I, Mori K, et al. Endoscopic treatment of refractory filarial chyluria: a preliminary report. J Urol 1998;159:1615. [3] Guermazi A, Brice P, Hennequin C, Sarfati E. Lymphography; an old technique retains its usefulness. Radiographics 2003; 23:1541. [4] Hashim SA, Roholt HB, Babayan VK, Vanitallie TB. Treatment of chyluria and chylothorax with medium chain triglycerides: N Engl J Med 1964;270:756. [5] Sabnis RB, Punekar SV, Desai RM, Bradoo AM, Bapat SD. Instillation of silver nitrate in the treatment of chyluria. Br J Urol 1992;70:660. [6] Kulkarni AA, Pathak MS, Sirsat RA. Fatal renal and hepatic failure following silver nitrate instillation for treatment of chyluria. Nephrol Dial Transplant 2005;20:1276. [7] Srivastava DN, Yadav S, Hemal AK, Berry M. Arterial haemorrhage following instillation of silver nitrate in chyluria: treatment by coil embolization. Aust Radiol 1998;42:234. [8] Brunkwall J, Simonsen O, Bergqvist D, Jonsson K, Bergentz SE. Chyluria treated with renal autotransplantation: a case report. J Urol 1990;143:793e6. [9] Punekar SV, Kelkar AR, Prem AR. Surgical disconnection of lymphorenal communication in chyluria. Br J Urol 1997;80:858. [10] Chang CY, Lue YB, Lapides J. Surgical treatment of chyluria. J Urol 1973;109:299.