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Abstract
Lymphangiomatosis is a rare and benign mesenchymal disorder where there is developmental malformation and dilatation of lymphatic channels. The most common sites for lymphangiomatosis are the cervical neck and axilla, but it can also occur in the mediastinum, retroperitoneum, mesentery, omentum, colon, and pelvis. Renal lymphangiomatosis is an even rarer multicystic disorder which may be hilar, pericalyceal, paracalyceal, peripelvic, or para pelvic. We report a case of 24-year-old female with bilateral perirenal lymphangiomatosis. Her complain was bilateral flank pain and bilateral perinephric fluid collection with multiple sepatations was noted on radiological examination.
Introduction 
Renal lymphangiomatosis, also called lymphangiectasis is a rare congenital disorder characterized by the dilatation of lymphatic channels. The lymphangiectasis can be unilateral or bilateral, and it can have focal as well as diffuse involvement of lymphatics. It may be limited to the renal hilum or extend from the renal parenchyma up to the corticomedullary junction. Renal functions remain normal in this condition.
Case Report A 24-year-old female patient presented with vague history of bilateral flank pain. There was no significant history or family history of any diseases. She was not taking any medications previously. On examination, her blood pressure was 110/60 mm Hg. The baseline investigations revealed normal serum crea tinine and urea. Urine analysis was also normal.
Ultrasound showed a large perinephric collection with septations in it, having a thickness of 34 mm and involving both kidneys circumferentially [Figure 1] and [Figure 2].
Figure 1: Ultrasound: Significant perinephric collection with multiple internal septations involving right kidney circumferentially.
Figure 2: Ultrasound: Significant perinephric collection with multiple internal septations involving the left kidney circumferentially.The computed tomography (CT) scan of the abdomen showed bilateral hypodense nonenhancing collections with an average density of 8 HU and normal contrast enhancement of cortex with mild pelvic ascites [Figure 3] and [Figure 4].
Figure 3: Computed tomography with contrast: Axial section showing bilateral nonenhancing collections with normal contrast enhancement of cortex.
Figure 4: Computed tomography with contrast: Sagittal section showing bilateral nonenhancing collections with normal contrast enhancement of cortex.Needle aspiration of perinephric fluid was done under ultrasound guidance. The laboratory analysis revealed chylous fluid having abundant proteins.
Informed oral consent was obtained from the patient for reporting of this case.
Discussion Renal lymphangiomatosis is a rare and benign developmental malformation of lymphatic channels where there is abnormal communication between peri-nephric lymphatics and the rest of the lymphatic system.[1] It usually involves one kidney, and bilateral form is rare.[2] The failure of renal lymphatics to drain into the retroperitoneal lymphatic system results in the dilatation of lymphatic channels and the formation of multiple cystic lesions in the perinephric space and renal sinus. This benign entity has also been named as renal lymphangioma, renal lymphangiectasia, and renal hygroma.[3] The underlying pathogenesis of this condition is unclear.[4] It can affect any age group and has no sex predilection. It can be asymptomatic but can also present with hypertension, hematuria, and proteinuria.[5] The renal functions remain normal. Ultrasound and CT findings are quite specific for the diagnosis of renal lymphangiomatosis, but diagnosis is usually confirmed by needle aspiration of cystic fluid. Contrary to other renal cystic lesions, it is chylous and rich in proteins.
According to the literature review, renal lymphangiomatosis has two radiological patterns. The first pattern is of a cystic lesion in the renal sinus that can be either a small peripelvic lesion originating from the renal sinus or a large single parapelvic cyst that originates in the medial renal parenchymal tissue. The second pattern shows perinephric fluid collection with multiple septations, which has resemblance to our case.[6]
The differential diagnosis of renal lymphangiomatosis includes all the conditions which have clinical features of renal mass. These conditions are hydronephrosis, polycystic kidney disease, abscess, urinomas and lymphoma and nephroblastomoatosis. In hydronephrosis, there is distended collecting system, but in renal lymphangiomatosis, splaying of calyces is seen. In polycystic kidneys, there are noncommunicating cysts in renal parenchyma, whereas in lymphangiomatosis, renal parenchyma is normal, and the lesion is perirenal or pararenal.[7] Urinoma and abscess can be differentiated from lymphangiomatosis based on proper history, whether the disease is unilateral or bilateral, the condition of the underlying parenchyma, and the attenuation and enhancement pattern of the collection.[1] Lymphoma and nephroblastomatosis usually present as a soft-tissue mass with increased vascularity on ultrasound and contrast enhancement on CT.
The radiological findings are sufficient to make the diagnosis of renal lymphangiomatosis, but the diagnosis can be confirmed on needle aspiration of perinephric fluid.[8] The characteristic ultrasound and CT findings and fluid analysis in our case confirmed the diagnosis of bilateral renal lymphangiomatosis.
Conclusion Bilateral renal lymphangiomatosis is a rare condition which can affect any age group and has no sex predilection. It is a benign condition and usually asymptomatic but can cause mild symptoms due to the mass effect of the cysts. Complications of this entity include cyst rupture, hemorrhage, and hypertension. Usually, there is no need of treatment as long as the patient is asymptomatic. The patient was reassured, and conservative management for flank pain was advised in our case. The patient has been on regular follow-up in nephrology.
Conflict of interest: None declared.
References 
Correspondence Address:
Muhammad Tanzeel Abbasi
Department of Nephrology, Multan Institute of Kidney Diseases, Multan, Punjab
Pakistan
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/1319-2442.367815
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