Renal lymphangiectasia (or renal lymphangioma), is a rare disorder, where there is dilatation of perirenal, peripelvic and intrarenal lymphatics. The diagnosis can be suggested by imaging, and aspiration of chylous fluid is usually confirmatory.
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, 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.
- renal vein thrombosis
- pregnancy-related veno-lymphatic obstruction
Ultrasound appearances are variable 1,2.
- perirenal collection and peripelvic cysts
- retroperitoneal fluid collection
- 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 few cases. Fluid collection in peritoneal or retroperitoneum compartments may also be noted. Perinephric collection may give a Page kidney like appearance.
Treatment and prognosis
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.
- cystic renal dysplasia
- hereditary polycystic kidney disease
familial renal lymphangiomatosis 2
- poorer prognosis
- multiorgan involvement (including osseous) more common
- 1. Ramseyer LT. Case 34: renal lymphangiectasia. Radiology. 2001;219 (2): 442-4. Radiology (full text) - Pubmed citation
- 2. Stevenson RE, Hall JG. Human malformations and related anomalies. Oxford University Press, USA. (2006) ISBN:0195165683. Read it at Google Books - Find it at Amazon
- 3. Dähnert W. Radiology Review Manual. Lippincott Williams & Wilkins. (2011) ISBN:1609139437. Read it at Google Books - Find it at Amazon
- 4. Silverman SG, Cohan RH. CT urography, an atlas. Philadelphia, PA : Lippincott Williams & Wilkins, c2007. (2006) ISBN:0781787548. Read it at Google Books - Find it at Amazon