Renal lymphangiomatosis

Last revised by Ismael J Nassar on 1 Jan 2024

Renal lymphangiomas, which can also include renal lymphangiectasia are a rare disorder, where there is dilatation of perirenal, parapelvic, and intrarenal lymphatics. The diagnosis can be suggested by imaging, and aspiration of chylous fluid is usually confirmatory.

For a broader discussion, please refer to the parental article on lymphangioma.

It is important to note that there is significant variation in the nomenclature of this disorder. Some authors use the term as a synonym for peripelvic cysts (also known as renal sinus cysts), and as such, it would be common and found predominantly in adults 3,4. Instead, if the term is restricted to patients with transrenal chylous disease (which may include peripelvic cysts) then it is much rarer and most frequently seen in children 1,2.

This narrower definition is used in this article. For a discussion of the more common entity, refer to the article on peripelvic cysts.

It is important to know that lymphangioma is a benign condition that can occur at almost any location in the body.

The condition can be found primarily in children, although adult cases have been described 1,2.

Hematuria, flank pain and abdominal pain are usually the presenting symptoms 1. Ascites, renal mass, pyelonephritis and renal insufficiency have also been described 2. Compression of the renal parenchyma by subcapsular fluid accumulation can result in high renin hypertension due to relative ischemia, and secondary hypertension.

Pathologically, renal lymphangiectasia is characterized by cortical dilated endothelial-lined spaces, without glomerular or tubular abnormalities. The medulla is usually spared 2. Although the disease may be bilateral, the involvement of extra-renal tissues is usually not encountered 2.

Some familial associations have been described. Acquired causes such as renal or perinephric inflammation, blocking the lymphatics have also been suggested 1.

Ultrasound appearances are variable 1,2:

  • perirenal collection and peripelvic cysts

  • retroperitoneal fluid collection

  • ascites

  • poor corticomedullary differentiation and diffuse echogenic renal parenchyma

CT may show a perinephric fluid attenuation collection of usually 0-10 HU. No other renal pathology is usually found apart from peripelvic cysts in a few cases. Fluid collection in peritoneal or retroperitoneum compartments may also be noted. Perinephric collection may give a Page kidney like appearance.

Treatment depends on symptoms and whether or not the condition is bilateral. Often renal impairment is mild, and treatment with antihypertensives suffices. If mass symptoms are significant, and the condition is unilateral, then nephrectomy may be indicated 2.

It should be noted that biopsy can be complicated by persistent lymphatic leakage 2.

Due to the variable imaging features the differential diagnosis can be broad and includes other causes of bilateral renal enlargement and cystic renal disease:

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