Bilateral Renal Lymphangiectasia - Case Report Abstract Id : IRIA -1090

Case history.. A 48 year old female came with the complaints of mild dull aching lower abdomen pain for past 2 months Past history: No history of SHT/ Epilepsy/ TB / DM No significant history in the past

Ultrasound abdomen done Ultrasound examination revealed dilated pelvicalyceal system without ureteric dilatation and diagnosis of pelviureteric junction obstruction was considered RIGHT KIDNEY LEFT KIDNEY

CECT -KUB Computed tomography (CT) revealed large hypodense non enhancing cystic lesions with lobulated margins noted filling the renal sinus region of both kidneys The lesion was exerting the mass effect and stretching the pelvicalyceal systems of both kidneys Otherwise both kidneys showed normal enhancement and excretion

Large hypodense cystic lesions with lobulated margins noted filling the renal sinus region of both kidneys PLAIN CONTRAST

The lesion was exerting the mass effect and stretching the pelvicalyceal systems of both kidneys PLAIN CONTRAST

MRI revealed multi lobulated cystic lesion appears hypointense on T1 & hyperintense on T2WI lesion seen filling both renal parenchyma T1 WI Axial STIR COR

Volume rendered image showed mild streaching & distorted pelvicalyceal system No hydroureteronephrosis

Differential diagnosis DDx for filling defects in renal sinus/Distorted collecting system Renal lymphangiectasia Urinoma Polycystic kidney disease Lymphoma.

WHY LYMPHANGEICTASIA USG demonstrates anechoic lesion in the renal pelvis with out ureteric dilatation On CT, several lesions with fluid attenuation found in the renal sinus;. Characteristically, these lesions do not invade the adjacent structures, although they cause displacement On MR, cystic lesions appearead hypointense in T1 sequences,and hyperintense in T2 with the presence of fine thin septations.

WHY NOT.. POLYCYSTIC KIDNEY DISEASE URINOMA LYMPHOMA Polycystic kidneys show evidence of scattered parenchymal cysts that may vary in size and may or may not be associatedwith pancreatic or hepatic cysts. URINOMA Its bilateral, symmetricl,Asymtomatic & not obstructing the collecting system. LYMPHOMA Masses are observed with greater levels of attenuation than those found in renal lymphangiectasia.

Renal lymphangiectasia Renal lymphangiectasia is a rare entity of the renal lymphatics that occurs in both children and adults. It may be unilateral or bilateral and has no gender predilection. It is caused by an abnormal development of the lymphatic structures, with single or multilocular fluid-filled cavities, usually found in the neck (75%-80%) and axillary (20%) regions.

Renal lymphangiectasia other names such as Renal lymphangiomatosis Renal lymphangioma Parapelvic lymphangiectasia, Polycystic renal sinus Renal hygroma

Renal lymphangiectasia Pathophysiology.. Pathophysiology of this is unclear, The lymphatic drainage of the kidneys, the renal capsule and the perirenal tissues is interconnected through several large lymphatic trunks located inside the renal sinus. These lymphatic trunks drain into the para-aortic, para-caval and inter-aorto-caval lymph nodes.

Renal lymphangiectasia Pathophysiology.. There may be a developmental malformation and derangement of the drainage of these lymphatic trunks, leading to their dilatation and the creation of cystic voids adjacent to the renal sinus Some have suggested that it may represent the benign neoplastic process.

IMAGING FEATURES.. The imaging characteristics depend on the site and extent of the lymphatic involvement. USG may show Perirenal collection & peripelvic cysts Retroperitoneal fluid collection Ascites Echogenic renal parenchyma

IMAGING FEATURES.. CT demonstrates Perinephric fluid collection Parapelvic /perirenal multiloculated cystic lesions with septations If only the small intra-renal lymphatics are compromised, the lesion may appear as a solid renal lesion or with slight diffuse kidney enlargement with no cystic space The diagnosis can be conformed with needle aspiration chylous fluid from the perinephric fluid collecition

Summary Renal lymphangiectasia is a rare entity; consequently, it is important for radiologists to be familiar with its characteristics and different forms of imaging appearance to diffentiate it from pelviureteric junction obstruction. In that way, we will be able to provide clinicians with the appropriate information required to determine the most adequate treatment for each individual patient

Thank you …! References 1.Ashraf K, Raza SS, Ashraf O, et al. Renal lymphangiectasia. Br J Radiol. 2007;80:e117- 8 2. Gupta R, Sharma R, Gamanagatti S, et al. Unilateral renal lymphangiectasia:imaging appearance on sonography, CT and MRI. Int Urol Nephrol. 2007;39:361-4. 3. Kevin KM, Garey LM. Renal Peripelvic Lymphangiectasia: Appearance at CT. Radiology. 1991;180:455-6. 4.Dobremez E, Llanas B, Harper L, et al. The parapelvic renal cyst. A rare aetiology of blood hypertension in children. Eur J Pediatr Surg. 2006;16:61-3. 5. Cadnapaphornchai MA, Ford DM, Tyson RW, et al. Cystic renal lymphangiectasia presenting as renal insufficiency in childhood. Pediatr Nephrol. 2000;15:129-31.