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

2. 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

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

4. 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

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

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

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

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

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

10. 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.

11. 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.

12. 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.

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

14. 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.

15. 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.

16. 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

17. 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

18. 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

19. Thank you …!
References
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Int Urol Nephrol. 2007;39:361-4.
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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: