Renal Lymphangiectasia: An Unusual Mimicker of Cystic Renal Disease – A Case Series and Literature Review

Umapathy, S.; Alavandar, Ezhilmathi; Renganathan, R.; Thambidurai, S; Arunachalam, Venkatesh Kasi

doi:10.7759/cureus.10849

Cited by 13 publications

(34 citation statements)

References 14 publications

(20 reference statements)

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“…These fluid-containing lesions correspond to the accumulation of lymph in the renal lymph ducts, which become ectatic and form simple or multiloculated cystic lesions around the kidney (perinephric lymphangiectasia), inside the kidney parenchyma (intrarenal and renal parapelvic lymphangiectasia) or around the calices and pelvis (RPL) 3…”

Section: Discussionmentioning

confidence: 99%

Renal peripelvic lymphangiectasia after bilateral renal vein thrombosis

Villard¹,

Meuwly²,

Righini³

et al. 2022

BMJ Case Rep

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Renal peripelvic lymphangiectasia (RPL) is one of the rare conditions that mimic renal cysts. Its physiopathology remains unknown, but an association with renal vein thrombosis has been reported. We share the case of a male patient in his 20s suffering from antiphosphlipid syndrome. The patient was hospitalised for thrombosis of the inferior vena cava (IVC) extending from the iliac veins to the level of renal veins. Consecutive CT and clinical follow-up over the course of 14 years showed the development of numerous retroperitoneal venous collaterals and the apparition of several bilateral peripelvic cystic lesions after extensive thrombosis of the IVC and both renal veins. The renal function remained normal throughout the follow-up. We suggest that the development of RPL is secondary to bilateral renal vein thrombosis. The presumed mechanism would be an increased hydrostatic pressure in the kidney capillaries leading to a more important interstitial fluid drainage by the lymphatic system. To our knowledge, this is the first well-documented case of renal vein thrombosis followed by RPL, contrasting with the previous hypothesis that compression by the lymphangiectasia could cause the thrombosis.

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Section: Discussionmentioning

confidence: 99%

Renal peripelvic lymphangiectasia after bilateral renal vein thrombosis

Villard¹,

Meuwly²,

Righini³

et al. 2022

BMJ Case Rep

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“…Symptoms of this disease are: abdominal pain, flank pain, bloating of the stomach, fatigue, fever, weight loss, hematuria, proteinuria [3,4].…”

Section: Discussionmentioning

confidence: 99%

“…In the most severe cases it is manifested with high blood pressure and ascites, renal insufficiency and renal vein thrombosis or these can be complications when the disease will not be very well threated [4,5]. But in literature hypertension associated with renal lymphangiectasia is rarely reported [10].…”

Section: Discussionmentioning

confidence: 99%

Renal Lymphangiectasia- An Unusual Mimicker of Renal Hydronephrosis

Tashkova

Arifi

Jovanovska

et al. 2021

JMS

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Renal lymphangiectasia is a rare malformation of the renal lymphatics. It occurs in both children and adults and can be unilateral or bilateral with no gender predilection. It is a condition characterized by different degrees of dilatation of the lymphatic ducts. Because of the rarity, it can be easily misdiagnosed for other cystic masses, most commonly peripelvic cysts, renal cysts or hydronephrosis. Usually is asymptomatic and incidental finding, but in severe cases can lead to hypertension and renal failure.We report a case of unilateral renal lymphangiectasia in adult male patient with medical history of hydronephrosis and characteristic radiologic CT findings. From imaging methods, we conducted ultrasound (US), contrast enhanced computed tomography (CT) and CT angiography, because they have an important role as diagnostic procedures to recognize renal lymphangiectasia.Kidney cystic lesions revealed on ultrasound and confirmed on CT as hypodense intrarenal multiloculated findings, as well as fluid attenuation collections, not always go in favor of hydronephrosis. Knowledge of the radiological findings associated with renal lymphangiectasia can contribute to better differentiation from other conditions and with the right diagnosis, successful management and treatment can be provided for the patient.

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“…The differential diagnosis of this condition includes autosomal dominant polycystic kidney disease, although in this condition the renal parenchyma is diffusely abnormal; tumors such as liposarcoma, leiomyosarcoma, fibrosarcoma, malignant teratomas, and multilocular cystic nephroma, which are predominantly cystic or necrotic, but these malignancies usually contain substantial solid components; abscess and urinoma may also produce a similar appearance, but almost all these conditions can be differentiated with the help of clinical history, normal biochemical parameters and typical imaging findings of perirenal and parapelvic involvement sparing the renal parenchyma [ 2 , 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…There are no clear guidelines as to when conservative management, percutaneous drainage, or surgery should be used; however, treatment is generally conservative for asymptomatic patients. Complicated cases are treated with nephrectomy, percutaneous drainage, and marsupialization, however, there is a risk of an increase in the size of cysts in the contralateral kidney when a nephrectomy is performed in patients with bilateral involvement [ 1 , 3 , 4 , 5 ].…”

Section: Discussionmentioning

confidence: 99%

Bilateral Renal Lymphangiectasia Associated With Polycythemia: A Rare Case Report

Jokar¹,

Chidobem²,

Khan³

2021

Cureus

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Renal lymphangiectasia is a rare benign mesenchymal tumor of unclear etiology resulting from dilatation of perinephric lymphatic channels and formation of cystic masses. Polycythemia is a rarely associated finding with only five cases reported in the literature. We report a case of bilateral renal lymphangiectasia associated with polycythemia in a 38-year-old man who was managed conservatively with pain control. There are no clear guidelines for the management of renal lymphangiectasia; although most patients can be treated conservatively, some cases, whose diagnosis is unclear or develop complications, require definitive surgical excision.

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Renal Lymphangiectasia: An Unusual Mimicker of Cystic Renal Disease – A Case Series and Literature Review

Cited by 13 publications

References 14 publications

Renal peripelvic lymphangiectasia after bilateral renal vein thrombosis

Renal peripelvic lymphangiectasia after bilateral renal vein thrombosis

Renal Lymphangiectasia- An Unusual Mimicker of Renal Hydronephrosis

Bilateral Renal Lymphangiectasia Associated With Polycythemia: A Rare Case Report

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