Intranodal lymphangiography and interstitial lymphatic embolization to treat chyluria caused by a lymphatic malformation in a pediatric patient.

Chyluria is characterized by chyle in the urinary tract and often presents as milky-white urine. We present a case of chyluria from a lymphatic malformation in a 13-year-old boy diagnosed using dynamic intranodal contrast-enhanced magnetic resonance (MR) lymphangiography. This report demonstrates the utility of intranodal lymphangiography and interstitial lymphatic embolization to treat a pediatric patient presenting with persistent chyluria. Glue migration into the urinary collecting system is a potential complication of this procedure that can be mitigated by adjusting the n-butyl cyanoacrylate dilution with Lipiodol.

Milky White Urine After Relief of Urinary Retention.

BACKGROUND: Chyluria is a rare condition where chyle is excreted into the urine. Clinically, most patients manifest with intermittent passage of milky urine. Patients may also present with dysuria, urinary frequency, urgency, retention, or with the sequelae of chronic malnutrition. CASE REPORT: We present a 55-year-old African American man who presented to the emergency department complaining of milky white urine, dysuria, decreased urine output, and suprapubic abdominal pain once a day. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Patients may present to the emergency department complaining of milk-colored urine, hematuria, urinary retention, or the sequelae of malnutrition. Initial evaluations should include laboratory investigations of common causes of chyluria and the severity of the potential malnutrition. If the patient presents with urinary retention, after relieving the obstruction in the emergency department, assessment for clot/chyle burden and likelihood of recurrence of urinary retention should be performed by urology. Arrangements for proper outpatient follow-up should be made if the disease manifestations are not severe enough to warrant admission.

Unipedal Diagnostic Lymphangiography Followed by Sequential CT Examinations in Patients With Idiopathic Chyluria: A Retrospective Study.

OBJECTIVE: The objective of our study was to investigate the clinical value of diagnostic lymphangiography followed by sequential CT examinations in patients with idiopathic chyluria. MATERIALS AND METHODS: Thirty-six patients with idiopathic chyluria underwent unipedal diagnostic lymphangiography and then underwent sequential CT examinations. The examinations were reviewed separately by two radiologists. Abnormal distribution of contrast medium, lymphourinary leakages, and retrograde flow were noted, and the range and distribution of lymphatic vessel lesions were recorded. The stage of idiopathic chyluria based on CT findings and the stage based on clinical findings were compared. Therapeutic management and follow-up were recorded. Statistical analyses were performed. RESULTS: Compared with CT studies performed after lymphangiography, diagnostic lymphangiography showed a unique capability to depict lymphourinary leakages in three patients. Lymphourinary fistulas and abnormal dilated lymphatic vessels were found in and around kidney in all patients. CT depicted retrograde flow of lymph fluid in 47.2% of patients. The consistency in staging chyluria based on CT findings and clinical findings was fair (κ = 0.455). Twenty-nine patients underwent conservative therapy, and seven underwent surgery. Surgical therapy was superior to conservative management (no recurrence, 85.7% of patients who underwent surgery vs 62.1% of patients who underwent conservative therapy; p = 0.025). CONCLUSION: From assessing the drainage of contrast medium on unipedal diagnostic lymphangiography and the redistribution of contrast medium on sequential CT examinations, it is possible to detect the existence of lymphourinary fistulas, the precise location of lymphatic anomalies, the distribution of collateral lymphatic vessels, and hydrodynamic pressure abnormality in the lymph circulation in patients with idiopathic chyluria. CT staging of chyluria provides additional information that can be used to guide therapeutic management.

HIFU, a noninvasive and effective treatment for chyluria: 15 years of experience.

BACKGROUND: The current treatment therapies for chyluria are often invasive and recurrent. Here, we investigated a novel noninvasive treatment of chyluria with high-intensity focused ultrasound (HIFU) and evaluated its clinical efficacy. METHODS: 155 patients with chyluria were treated with HIFU ablation and followed up over a period of 15 years from May 2000 to December 2015. Routine examinations including urine color observation, color Doppler ultrasound examination, blood serum test of Cr, BUN, and albumin, and detection of urinary chyle were performed before and after the treatment, 1 week, 1 and 6 months post-treatment, and followed up via telephone and other forms. We lost contact with 54 patients during the course of the study. RESULTS: In the 101 complete cases, the serum levels of Cr and BUN and the color Doppler ultrasound examination did not reveal significant differences before and after the treatment. However, there was a significant increase in the hemoglobin and albumin levels, as well as the body weight after the HIFU treatment. The other 54 patients also showed an improvement of the symptoms after the HIFU treatment before losing contact. CONCLUSIONS: Our results suggest that the HIFU ablation therapy is a feasible, effective, and noninvasive method for the treatment of chyluria.

Unilateral pedal lymphangiography plus computed tomography angiography for location of persistent idiopathic chyle leakage not detectable by ordinary contrast computed tomography.

BACKGROUND: To identify the value of unilateral pedal lymphangiography (LPG) plus computed tomography angiography (CTA) in accurate depiction of persistent idiopathic chyluria undetectable by ordinary contrast CT. METHODS: Eighteen patients 44-63 years of age with persistent idiopathic chyluria who failed conservative management were included. Ordinary CT had not revealed a chyle leak. Cystoscopy, unilateral LPG, and post-LPG CT angiography (CTA) were sequentially performed. Ligation and stripping of the perirenal lymphatics were subsequently performed guided by lymphangiography and CTA. RESULTS: LPG and post-LPG CTA detected 17 unilateral and one bilateral chyle leaks in the 18 patients, with clear images of the communication of lymphatic vessels and the renal collecting or vascular system. The success rate was significantly better than cystoscopy (100% vs 50.0%, P = 0.005) or LPG alone (100% vs. 72.2%, P = 0.016). Chyluria resolved after surgery in all patients; no relapses were found. CONCLUSIONS: LPG plus post-LPG CTA accurately characterized perirenal lymphangiectasia that was not demonstrated by routine contrast-enhanced CT or not suitable for magnetic resonance imaging. Despite of its invasiveness, this method is a good diagnostic alternative to LPG in patients with persistent chyluria requiring surgery.

Chyluria and chylothorax after posterior selective fusion for adolescent idiopathic scoliosis.

PURPOSE: To describe and discuss the diagnostic and treatment complexity of lymphatic system complications after scoliosis surgery. METHODS: Surgery for adolescent idiopathic scoliosis is very commonly performed with posterior pedicle screw instrumentation. Complications of the anteriorly based lymphatic system are, therefore, rare. We present a case with complications related to the lymphatic system, which have not been reported before after this type of surgery. RESULTS: After standard Th3 to Th12 posterior spinal reduction and fusion of a moderate thoracic curve, chyluria and a chylothorax developed in an adolescent girl. This appeared to be caused by an obstruction of the thoracic duct. Thorax drainage and finally thoracoscopic intervention prevented further pulmonal impairment. The exact cause could not be identified and the persistent lymph drainage problems had to be treated with a medium chain triglyceride diet. CONCLUSION: With this report, we aim to create awareness of the lymphatic system in general and the possibility of severe complications, even after a posterior only approach of the vertebral column.

[Chyluria with "nephrotic syndrome-like" presentation: Diagnostic and therapeutic approach]. / Chylurie révélée par un « pseudo-syndrome néphrotique ¼ : démarche diagnostique et thérapeutique.

Chyluria implies an abnormal communication between the lymphatic system and the urinary tract. It is more frequent in endemic areas of lymphatic filariasis, which constitutes the main cause. Chyluria may mimic a nephrotic syndrome. Diagnosis of chyluria is based on urinary cytological and biocheminal analysis. Localization of the fistula needs medical imaging: cystoscopy, retrograde pyelography, lymphoscintigraphy and more recently magnetic resonance lymphography. Due to frequent spontaneous remissions, traitement can require only dietary measures. In case of malnutrition or clinical complications, sclerotherapy should be rapidly undertaken, before considering surgery of renal lymphatic disconnection.

Factors affecting response to medical management in patients of filarial chyluria: A prospective study.

INTRODUCTION: Filarial chyluria is a common problem in filarial endemic countries. Its management begins with medical therapy but some patients progress to require surgery. The present study aimed to determine factors affecting response to medical management in patients of filarial chyluria. MATERIALS AND METHODS: This prospective study conducted between August 2008 and November 2012, included conservatively managed patients of chyluria. Demographic profile, clinical presentation, treatment history and urinary triglycerides (TGs) and cholesterol levels at baseline were compared between the responders and non-responders. Apart from the clinical grade of chyluria, hematuria was evaluated as an independent risk factor. RESULTS: Out of the 222 patients (mean age, 37.99 ± 13.29 years, 129 males), 31 patients failed to respond while 35 had a recurrence after initial response; the overall success rate being 70.3% at a mean follow-up of 25 months. No difference was observed in demographics, clinical presentation, presence of hematuria, disease duration and mean urinary TGs loss between responders and non-responders. On multivariate analysis, patients with treatment failure were found to have a higher-grade disease (14.3% Grade-I, 36.6% Grades-II and 60% Grade-III), higher number of pretreatment courses (1.59 ± 1.08 vs. 1.02 ± 0.79) and heavier cholesterol (26.54 ± 23.46 vs. 8.81 ± 8.55 mg/dl) loss at baseline compared with responders (P < 0.05). CONCLUSION: Conservative management has a success rate in excess of 70%, not affected by the disease chronicity, previous episodes and recurrent nature. However, higher-grade disease, extensive pre-treatment with drugs and higher urinary cholesterol loss at baseline are the predictors of poor response. Hematuria is not an independent poor risk factor for conservative management.

Magnetic resonance-retrograde pyelography: A novel technique for evaluation of chyluria.

There are no standardized radiological investigations in a patient with chyluria. Retrograde pyelography (RGP) is usually done to demonstrate pyelo-lymphatic reflux before invasive therapy in the form of sclerotherapy and surgery. We describe magnetic resonance-RGP using gadolinium to demonstrate pyelo-lymphatic reflux in addition to the other intra-abdominal details provided by MRI. The advantages of this technique include avoidance of ionic contrast media and radiation exposure and possibility of better understanding of the disease pathophysiology.

Central Lymphatic Imaging in Adults with Spontaneous Chyluria.

Purpose: Chyluria is a rare condition primarily prevalent in developing countries in tropical regions. In chyluria, there exists the communication between lymphatic vessels and the urinary tract, but the specific mechanism of this communication remains undocumented. The objective of this study was to assess the morphology of the main lymphatic vessels including the uro-lymphatic fistula, the thoracic duct using Magnetic Resonance Lymphangiography (MRL) and Intranodal Lymphangiography (IL). Materials and Methods: A retrospective study spanning five years, from January 2020 to January 2024, included 43 patients diagnosed with chyluria through cystoscopy and quantitative urine testing for triglycerides. These patients underwent MRL and then IL for uro-lymphatic fistula embolization. Results: The study involved 43 patients with an average age of 66.1 ± 19.5 years, with a male-to-female ratio of 1:2. Uro-lymphatic fistula occurred predominantly in the left kidney (72.1%), followed by the right kidney (20.9%), and both sides (7%). MRL imaging showed the thoracic duct in 100% of cases but visualized only 84.5% of the uro-lymphatic fistulas. In contrast, IL imaging showed the thoracic duct in 51.5% of patients but visualized uro-lymphatic fistulas in 100% of cases. In the procedure of IL, the average visualization time of the thoracic duct was 45 minutes, with a range of 35 to 69 minutes. Conclusion: MRL and IL complement each other in diagnosing the main lymphatic vessels in chyluria patients. The observed circulatory stasis in the thoracic duct supports the hypothesis that it contributes to increased pressure in the thoracic duct and the formation uro-lymphatic fistula as collateral circulations.

Robot-assisted staged bilateral reno-lymphatic disconnection for massive idiopathic chyluria: A case report.

Chyluria, an abnormal lymphatic disorder, results in excessive abdominal lymph drainage into the urinary system, causing protein loss, nutritional deficiencies, and immune issues. Mainly linked to parasitic infections in developed countries, non-parasitic causes like trauma or tumors are rare. Typically appearing in adults with bilateral involvement, management options include conservative or surgical approaches. We present the case of a 13-year-old with congenital chyluria, treated with robot-assisted staged reno-lymphatic disconnection after failed interventional radiology. Bilateral scleroangiography followed, leading to persistently milky urine for a month. Finally, urine clarity improved, correlating with better urinalysis, emphasizing the need for a comprehensive, multi-disciplinary approach.

Lymphoscintigraphy - Beyond Lymphedema.

Lymphoscintigraphy is an established modality for imaging the lymphatic system using radiocolloids and is routinely indicated to find the cause of limb lymphedema. However, in this case series, we are highlighting other less-known indications of lymphoscintigraphy like chylothorax and chyluria which present as lymphatic leaks in the thorax and abdomen, respectively. Once the site of the lymphatic leak is established by lymphoscintigraphy, definitive management like thoracic duct ligation or sclerotherapy can be done. The other indication discussed is postrenal transplant perinephric fluid collection which can be challenging to confirm whether it is urinoma, lymphocele, or any other collection. And finally, sentinel lymph node localization is another, now, well-established indication of lymphoscintigraphy.

Chromatinless microfilaria.

Herein, we are presenting a case of chromatinless microfilaria in a patient with chyluria.

Chyluria: clearing the 'muddiness' with lipiodol lymphangiography.

Chyluria is a rare entity characterised by the presence of chyle/lymphatic fluid within the urine. It develops following an abnormal communication between the perirenal lymphatics and pelvicalyceal lymphatics. There are multiple causes of chyluria including infective (filariasis), post-traumatic, post-surgical, pregnancy and malignancy. We present a case of a 15-year-old male who presented with a complaint of the intermittent passage of milky urine for the preceding 1 year. Conventional lipiodol lymphangiography followed by cone beam computed tomography was done to look for abnormal fistulous channels. Subsequently, the patient was successfully treated with cystoscopy-guided renal pelvic instillation sclerotherapy of povidone-iodine.

Occlusion of thoracic duct stent resulting in recurrent chyluria: role of renal-lymphatic fistula embolization.

BACKGROUND: Thoracic duct (TD) stenting is considered a treatment option for certain pathological conditions caused by TD obstruction, such as chyluria. Several studies have reported on the efficacy of TD stent treatment for both obstructive and leakage condition of TD, but few have evaluated the stent patency. This report aims to describe the patency of TD stent and the effectiveness of renal-lymphatic fistula embolization in the treatment of chyluria. CASE PRESENTATION: We report a case of chyluria treated by TD stent previously, stent was placed at the TD venous junction four months before the symptoms recurred. At the second intervention we found the stent was obstructed by debris. We recanalized the stent and successfully catheterised the microcatheter through the stent retrograde into the TD then into the renal-lymphatic fistula branch. After embolization of that abnormal branch, the recurrent chyluria was treated and no further episode of chyluria was occurred during 12 months follow up. CONCLUSION: Stent in the TD may be occluded by debris. Embolization of renal-lymphatic fistula might be the most important treatment for spontaneous chyluria.

Chyluria: non-enhanced MR lymphography.

Chyluria is an uncommon medical condition resulting from an abnormal communication between the abdominal lymphatic system and the urinary tract, which results in the presence of chyle in the urine, making it appear milky white. Proper diagnosis is demonstrated by the concentration of urinary lipids. Worldwide, chyluria is most commonly associated with the parasite Wuchereria bancrofti. However, in Europe and North America, where the condition is rare, non-parasitic etiologies predominate. Identifying the cause and location of the uro-lymphatic communication is essential in guiding therapeutic management, but imaging the lymphatic channels remains a challenge. Magnetic resonance (MR) lymphography, a non-invasive free-breathing 3D high-resolution fast-recovery fast spin-echo sequence similar to that used for 3D MR cholangiopancreatography, may demonstrate the cause and location of an abnormal communication between the lymphatic system and urinary tract. In parasitic causes of chyluria, dilated lymphatics vessels communicating with the lymphatic system are demonstrated. In non-parasitic causes of chyluria channel type lymphatic malformations are the most common. Markedly dilated and dysplastic lymphatic vessels communicating with the urinary tract are demonstrated. In addition, other cystic or channel type lymphatic malformations such as thoracic, soft tissue or bone abnormalities may be observed. This review describes the abdominal lymphatic diseases leading to chyluria and presents the technique and images obtained with non-enhanced MR lymphography to enable radiologists in identifying and classifying uro-lymphatic fistulae.Critical relevance statement: Non-enhanced MR lymphography enables the identification and categorization of uro-lymphatic fistulae.

Renal pedicle lymphatic ligation for non-parasitic chyluria via retroperitoneal laparoscopic surgery: a single-center 12-year experience.

Background: Chyluria is a rare disease in which chylous is excreted in the urine. Currently, management of chyluria includes conservative treatments and surgical measures. This study aimed to report our experience in treating non-parasitic chyluria with retroperitoneal laparoscopic ligation of the renal lymphatic vessels. Methods: Data from 52 patients who underwent retroperitoneoscopic ligation of the renal lymphatic vessels for non-parasitic chyluria between December 2009 and May 2022 were reviewed. After general anesthesia, the patients were passively placed in the healthy lateral decubitus position and underwent three-port retroperitoneal laparoscopy. Detailed medical data, including demographic characteristics, intraoperative outcomes, postoperative data, and complications, were reviewed. Results: Fifty-two patients received surgery treatment at our institution. The mean disease course was 89.3 months. The mean age was 58.8 years, with females accounting for 57.7% (30/52); the majority of patients (33/52) had the laterality of chyluria on the left and 9 (17.3%) had a history of previous thoracic or abdominal surgery. Compared with the urine and blood data before the operation and on the first day after the operation, urinary protein, urinary tract infection, urinary red blood cells, hemoglobin, albumin, and serum total protein significantly improved 3 months after the operation. However, there were no significant differences in blood creatinine and blood urea nitrogen levels among the three groups. The mean surgery time was about 110.0 minutes, and the estimated total blood loss was 81.2 mL. The postoperative drainage volume was 229.9 mL. The average time to start a liquid diet and to be out of bed were 1.5 and 1.9 days, respectively. Transient postoperative gross hematuria occurred in eight patients, and complications occurred in five patients after surgery. The mean length of hospitalization was 6.6 days. The follow-up duration ranged from 3 to 152 months, and except for three patients who did not respond to treatment, the remaining patients had no recurrence and did not require reoperation. Conclusions: Our long-term follow-up results showed that renal pedicle lymphatic ligation via retroperitoneal laparoscopic surgery is an effective, safe, and reliable surgical option for patients with non-parasitic chyluria.

A case of chyluria with nephrotic-range proteinuria caused by lymphatic malformation, leading to a diagnosis of Klippel-Trenaunay syndrome.

Klippel-Trenaunay syndrome (KTS) is a rare syndrome, which is clinically diagnosed by the presence of unilateral limb hypertrophy with vascular malformation including cutaneous capillaries, veins and lymphatic vessels. Most cases typically exhibit cutaneous manifestations such as port-wine stains and limb hypertrophy from infancy, but cases with mild manifestations may remain undiagnosed. We here report a case of KTS who was diagnosed by chance chyluria. A 15-year-old girl who exhibited hematochyluria with nephrotic-range proteinuria was referred to our hospital. She had been diagnosed as idiopathic scoliosis accompanied by left lower limb hypertrophy in the past. She noticed her milky urine for the first time two months before. Immediately thereafter, she noticed edema of her left leg. Hematochyluria with nephrotic-range proteinuria was found by our initial urine examination. Magnetic resonance imaging suggested venous or lymphatic malformation along the left common iliac vein at the retroperitoneal side. Lymphoscintigraphy showed congestion of radioisotope around backside of the pancreas to the left renal hilus, suggesting an existence of lymphostasis. Based on the findings, we diagnosed the patient as KTS. After admission, hematochyluria and proteinuria were decreased and became insignificant by three days with bed rest. Her left leg edema was reduced. After taking a guidance to avoid intensive exercise, she was discharged in two weeks. Because the present case exhibited mild manifestations, diagnosis was made by urine abnormalities for the first time. The case suggests that we should be aware of the presence of undiagnosed patients of KTS due to relatively mild manifestations.

Chyluria in a Postpartum Obese Female Patient.

Chyluria characterized by the passage of milky white urine is rarely encountered these days due to the overall reduction in the number of cases of lymphatic filariasis. Though lymphatic filariasis accounts for the majority of cases of chyluria, nonparasitic causes have also been reported. Case reports of chyluria as a complication in pregnancy have been published but chyluria presenting solely as a postpartum complication has rarely been documented. We present a case of a 29-year-old female with no known prior comorbidities, who presented with recurring complaints of the painless passage of milky white urine over the last year. Symptoms seem to have started six months post-delivery of her second child. The patient claimed significant weight gain during an otherwise normal pregnancy. She was well-built and had a BMI of 32 kg/m2. Her systemic examination and baseline laboratory workup were within normal limits. Postprandial urine was milky white, rich in chylomicrons, with urine chylomicrons of 112 mg/dl. The patient was screened for filariasis, which was negative. An ultrasound of the abdomen was done to rule out the presence of a fistula, but no evidence of one was found on imaging. Tc-99m sulfur colloid scintigraphy revealed an area of abnormal tracer accumulation in the abdomen with the passage of the tracer in the urine container, confirming the presence of chyluria. The patient was recommended to undergo conservative management with dietary modification and weight reduction. She has been closely followed up and has achieved spontaneous resolution of the chyluria. Most patients with chyluria show a good response to conservative management alone as in our case. Surgical intervention is usually indicated for cases not responding to conservative management or for refractory chyluria.