ABSTRACT ID : IRIA -1090

Bilateral Renal Lymphangiectasia - Case Report

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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

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Ultrasound abdomen done Ultrasound examination revealed dilated

pelvicalyceal system without ureteric dilatation and diagnosis of pelviureteric junction obstruction was considered

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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

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

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PLAIN CONTRAST

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

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PLAIN CONTRAST

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

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T1 WI Axial STIR COR

Volume rendered image showed mild streaching & distorted pelvicalyceal system

No hydroureteronephrosis

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Differential diagnosis

DDx for filling defects in renal sinus/Distortedcollecting system

Renal lymphangiectasia Urinoma Polycystic kidney disease Lymphoma.

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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.

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WHY NOT..

POLYCYSTIC KIDNEY DISEASE 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.

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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.

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Renal lymphangiectasia

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

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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.

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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.

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IMAGING FEATURES..

The imaging characteristics depend on the site and extent of the lymphatic involvement.

USG may showo Perirenal collection & peripelvic cysts Retroperitoneal fluid collection Ascites Echogenic renal parenchyma

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IMAGING FEATURES..

CT demonstrates Perinephric fluid collection Parapelvic /perirenal multiloculated cystic

lesions with septationsIf 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

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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

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R E F E R E N C E S1 . A S H R A F K , R A Z A S S , A S H R A F O , E T A L . R E N A L LY M P H A N G I E C T A S I A . B R JR A D I O L . 2 0 0 7 ; 8 0 : E 1 1 7 - 8

2 . G U P T A R , S H A R M A R , G A M A N A G A T T I S , E T A L . U N I L A T E R A L R E N A L LY M P H A N G I E C T A S I A : I M A G I N G A P P E A R A N C E O N S O N O G R A P H Y, C T A N D M R I .I N T U R O L N E P H R O L . 2 0 0 7 ; 3 9 : 3 6 1 - 4 .

3 . K E V I N K M , G A R E Y L M . R E N A L P E R I P E LV I C LY M P H A N G I E C T A S I A : A P P E A R A N C EA T C T. R A D I O L O G Y. 1 9 9 1 ; 1 8 0 : 4 5 5 - 6 .

4 . D O B R E M E Z E , L L A N A S B , H A R P E R L , E T A L . T H E P A R A P E LV I C R E N A LC Y S T. A R A R E A E T I O L O G Y O F B L O O D H Y P E R T E N S I O N I N C H I L D R E N . E U R JP E D I A T R S U R G . 2 0 0 6 ; 1 6 : 6 1 - 3 .

5 . C A D N A P A P H O R N C H A I M A , F O R D D M , T Y S O N R W , E T A L . C Y S T I C R E N A LLY M P H A N G I E C T A S I A P R E S E N T I N G A S R E N A L I N S U F F I C I E N C Y I N C H I L D H O O D .P E D I A T R N E P H R O L . 2 0 0 0 ; 1 5 : 1 2 9 - 3 1 .

Thank you …!

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