Bilateral renal lymphangiectasia mimicking renal cystic disease and literature review

TL;DR

This paper discusses a rare kidney condition called bilateral renal lymphangiectasia that can be mistaken for other cystic kidney diseases, emphasizing accurate diagnosis to avoid unnecessary treatments.

Contribution

The paper provides a comprehensive review of 56 cases and highlights diagnostic imaging features to improve recognition of bilateral renal lymphangiectasia.

Findings

Bilateral renal lymphangiectasia is often misdiagnosed as other cystic kidney diseases due to similar imaging appearances.

Conservative treatment is commonly used, while interventional procedures and nephrectomy are reserved for symptomatic cases.

Imaging modalities like CT and MRI are crucial for accurate diagnosis and avoiding unnecessary surgeries.

Abstract

Bilateral renal lymphangiectasia is a rare, benign lymphatic disorder that causes the perirenal, parapelvic, and intrarenal lymphatic channels to dilate. Because it is rare and has varying radiologic appearances, it might be confused with other cystic renal illnesses, such as polycystic kidney disease, which can lead to misdiagnosis and unnecessary treatments. In this case review, a 51-year-old female patient who presented with abdominal pain was diagnosed with bilateral renal lymphangiectasia based on imaging findings. Ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) revealed bilateral intrarenal and perirenal cysts. We comprehensively reviewed 56 cases of bilateral renal lymphangiectasia in adults published in the literature until June 2025. Conservative treatment was the preferred approach in about half of the cases. In cases where there were symptoms or complications, treatments such as percutaneous drainage, marsupialization, or nephrectomy were done. While aggressive treatment methods such as nephrectomy were commonly used in the 1980s, percutaneous interventional procedures and conservative treatments began to be used more frequently starting in the 1990s. Haematuria, ascites, hypertension, polycythaemia, pleural effusion, and renal vein thrombosis are rare complications reported. The aim of this case and literature review is to improve diagnostic accuracy by highlighting the clinical spectrum and imaging findings of bilateral renal lymphangiectasia. Accurate diagnosis based on imaging modalities is important to avoid unnecessary interventional and surgical procedures.

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