Qims 2016 07 04
Qims 2016 07 04
Qims 2016 07 04
Correspondence to: Richa Arora, MD, FRCR, MMed. Nizams Institute of Medical Sciences, Hyderabad, India. Email: dr.richaarora@gmail.com.
Submitted Jul 01, 2016. Accepted for publication Jul 14, 2016.
doi: 10.21037/qims.2016.07.04
View this article at: http://dx.doi.org/10.21037/qims.2016.07.04
© Quantitative Imaging in Medicine and Surgery. All rights reserved. qims.amegroups.com Quant Imaging Med Surg 2016;6(4):466-469
Quantitative Imaging in Medicine and Surgery, Vol 6, No 4 August 2016 467
Figure 2 Plain computed tomography (CT) axial sections of the Figure 4 Coronal reconstructed contrast enhanced CT (CECT)
abdomen showing septated fluid collection in bilateral perinephric abdomen showing multiseptated collections seen involving bilateral
and peripelvic space along with ascites. perinephric and peripelvic space (left bigger than right) with
scalloping of right renal cortex and gross ascites.
A B
Figure 3 Contrast enhanced CT (CECT) abdomen axial sections at the level of kidneys showing multiseptated collections seen involving
bilateral perinephric and peripelvic space (left bigger than right) (A,B) with scalloping of right renal cortex (B) and gross ascites with central
displacement of bowel loops.
and dilatation of lymphatics. Few suggest it as a distinct ascites and flank mass. Subsequently, another case was
neoplastic entity (2,8,9). added by Cadnapaphornchai et al. in a 7-year-old child with
Literature search on PubMed showed five cases of RL in bilateral RL presenting as renal insufficiency. Furthermore,
pediatric age group. Merguerian et al. reported first case of Sanchez et al. published another case of unilateral RL in
unilateral RL in an infant in 1995 and concluded that RL a 4-month-old female as an incidental finding. Lastly,
should be considered as differential diagnosis of unilateral Vasquez et al. reported a pediatric patient with bilateral RL
renal enlargement in children. Two years later, Simonton in 2012 and emphasized the importance of identifying this
et al. reported another similar case of unilateral RL in a pathology by characteristic radiological findings (3-7).
2-year-old child presenting with hypertension, massive Most of the patients are asymptomatic and it is detected
© Quantitative Imaging in Medicine and Surgery. All rights reserved. qims.amegroups.com Quant Imaging Med Surg 2016;6(4):466-469
468 Arora. Bilateral renal lymphangiectasia in a child
as an incidental finding on various imaging studies done for or without dilated ureter. Polycystic kidney disease shows
an unrelated cause. Abdominal pain, flank pain, haematuria, multiple non-communicating cysts scattered in renal
fatigue and weight loss are the usual presenting symptoms in parenchyma with or without cysts in other solid organs like
symptomatic cases. Advanced cases manifest with ascites and liver, spleen and pancreas. Multiloculated cystic nephroma
renal insufficiency (2,10,11). The complications include renal is characterized by focal multiseptated cystic mass arising
vein thrombosis, hypertension (due to compressive effects from kidney with claw shape of adjacent normal renal
of perirenal and peripelvic cysts causing renin-dependent parenchyma. Urinomas and abscesses can be differentiated
arterial hypertension), superimposed infection, intracystic on the basis of proper history and imaging appearances of
hemorrhage, obstructive uropathy. Partial regression during thick enhancing wall in abscess and contrast extravasation
neonatal period has been described (11-15). on delayed phase CT in urinoma (2,13,16).
Imaging features on various diagnostic modalities No treatment is usually required in most asymptomatic
like US, CT and magnetic resonance imaging (MRI) are patients with no complications. Nevertheless, periodic
classical and allow for a confident diagnosis, although, it clinical and imaging follow up is recommended in all
can be further confirmed with aspiration of chylous fluid patients due to risk of complications especially renal failure
from perirenal fluid collections in cases of diagnostic and hypertension. Percutaneous aspiration of lymphatic
dilemma. The distribution and extent of the lymphatics collections is the first line of management in less severe
involved determines the imaging characteristics. Cases with symptomatic cases, but, the success rate is less in larger
involvement of just small intra-renal lymphatics are seen as lesions with multiseptation. Laparoscopic ablation and
mild diffuse enlargement of the kidney with no cystic space. nephrectomy are reserved for advanced complicated cases
These cases show diffusely echogenic renal parenchyma and cases with multiple recurrences (2,13,16).
with poor corticomedullary differentiation on US and RL remains an elusive disorder due to its rarity and
hyperintense signal in the cortex & hypointense signal in unclear etiopathogenesis. This case emphasizes the need
medulla with corticomedullary inversion on T2 weighted to be familiar with its radiological features on the various
sequences of MR due to renal edema. Only peripelvic cysts imaging modalities to allow prompt diagnosis due to its
are seen in cases where lymphatics in the renal sinus are potential complications of renal vein thrombosis and
involved. These peripelvic cysts with thin walls are better hypertension and prevent misdiagnosis with close mimics
appreciated on US and T2 weighted sequences of MR like hydronephrosis, peripelvic cysts etc. This would also
and they may also show enhancement on T1 weighted aid in avoiding unnecessary investigations and planning
sequences acquired after gadolinium administration. appropriate management.
Larger cysts can cause obstruction of collecting system
leading to hydronephrosis. Capsular lymphatic dilatation
Acknowledgements
leads to septated perinephric fluid collections which show
attenuation values of 0–10 Hounsfield units on CT. Rarely, None.
multiple tortuous structures distended with clear fluid are
seen in retroperitoneum surrounding great vessels in a linear
Footnote
pattern due to stasis of lymph channels. Fluid collections in
peritoneal cavity or ascites are also sometimes seen. Al-Dofri Conflicts of Interest: The author has no conflicts of interest to
et al. has also reported presence of pleural effusion in a case declare.
of RL (10). The lymphatic fluid collections in all the spaces
show features of clear fluid on all the imaging modalities
References
unless complicated by hemorrhage (11,13-18).
Differential diagnosis includes hydronephrosis, polycystic 1. Wani NA, Kosar T, Gojwari T, Qureshi UA. Perinephric
kidney disease, multiloculated cystic nephroma, urinomas, fluid collections due to renal lymphangiectasia. Am J
abscesses, lymphoma and nephroblastomatosis. The last Kidney Dis 2011;57:347-51.
two entities display features of soft tissue masses on all 2. Restrepo JM, Amaya JE, Sepúlveda NA, Vélez MU,
modalities with internal vascularity on US and enhancement Massaro M. Renal lymphangiectasia. MDCT and MRI
on CT and MR, unlike fluid collections in cases of RL. findings. Rev Colomb Radiol 2011;22:1-8.
Hydronephrosis shows distended collecting system with 3. Merguerian PA, Sargent SK, Dunn JL. Unilateral
© Quantitative Imaging in Medicine and Surgery. All rights reserved. qims.amegroups.com Quant Imaging Med Surg 2016;6(4):466-469
Quantitative Imaging in Medicine and Surgery, Vol 6, No 4 August 2016 469
© Quantitative Imaging in Medicine and Surgery. All rights reserved. qims.amegroups.com Quant Imaging Med Surg 2016;6(4):466-469