Bilateral renal lymphoangiomatosis

Abstract

HighlightsRenal lymphangiomatosis is a rare congenital benign disease of renal lymphatic system.Here we are presenting a very rare form of disease, which is bilateral form.Disease can be found incidentally or with different signs and symptoms.Initial radiological work up will raise suspicion of renal tumor.Disease can be diagnosed and followed by imaging with an expert radiologist.Management usually analgesia unless a complex case or directed to the complications.

Bilateral Renal Lymphangiomatosis: A Case Report and Literature Review

“…Mani et al documented a case involving an eight-year-old male patient with renal lymphangiomatosis who presented with gradually progressive bilateral flank swelling but who was otherwise asymptomatic [ 2 ]. The clinical presentation of renal lymphangiomatosis varies, ranging from asymptomatic cases incidentally discovered to those presenting with abdominal pain, abdominal mass, hematuria, flank or back pain, proteinuria, renin-dependent hypertension, fatigue, and weight loss​ [ 9 , 10 ]. Abdominal pain, abdominal distension (ascites), and hypertension are the most commonly observed symptoms in pediatric patients ​[ 3 ].…”

“…Complications that can be encountered include cyst-related complications such as rupture, hemorrhage, and infection​ [ 6 ]. Additionally, complications related to mass effects can occur, such as renal vein thrombosis, obstructive uropathy, and deterioration of renal function ​[ 3 , 10 ]. It is important to note that pregnancy can worsen the disease by increasing the fluid content within cysts ​[ 11 ].…”

“…Diagnosing renal lymphangiomatosis involves a combination of clinical evaluation and imaging studies, such as ultrasound, CT, and magnetic resonance imaging (MRI), which demonstrate classical features that enable a confident diagnosis of renal lymphangiomatosis ​[ 10 ]. In some cases, needle aspiration of chylous fluid from perinephric collections may be necessary for confirmation ​[ 12 ].…”

“…In some cases, needle aspiration of chylous fluid from perinephric collections may be necessary for confirmation ​[ 12 ]. Ultrasound is often used as a primary diagnostic tool due to its ease of use and lack of side effects ​[ 10 ]. In the majority of cases, contrast-enhanced computerized tomography (CECT) is diagnostic ​[ 13 ].…”

“…On CECT, lymphatic dilations appear as hypodense structures (0-10 HU), non-enhancing, non-communicating structures surrounding the kidneys and renal pelvis ​[ 13 ]. MRI can provide additional information regarding tumor size and extension ​[ 10 ]. MRI reveals a hypointense appearance in T1-weighted images and a hyperintense appearance in T2-weighted images, with a reversal of the cortico-medullary intensity ​[ 13 ].…”