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| CASE REPORT |
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| Year : 2017 | Volume
: 14
| Issue : 3 | Page : 176-178 |
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Bilateral renal lymphangiectasia: A case of cystic rarity
Alka Rani T Patil1, Vinayaka Hosadurga Sathyanarayana2, Venkatesh Murthy Dammaningala Venkataramaiah1
1 Department of Paediatrics, Indira Gandhi Institute of Child Health, South Hospital Complex, Dharmaram College Post Near Nimhans, Bengaluru, Karnataka, India
2 Department of Pediatric Nephrology, Indira Gandhi Institute of Child Health, South Hospital Complex, Dharmaram College Post Near Nimhans, Bengaluru, Karnataka, India
| Date of Web Publication | 27-Oct-2017 |
Correspondence Address:
Vinayaka Hosadurga Sathyanarayana
Indira Gandhi Institute of Child Health, South Hospital Complex, Dharmaram College Post Near Nimhans, Bengaluru - 560 029, Karnataka
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/am.am_28_17
Renal lymphangiectasia is a benign, rare disorder of lymphatic system of the kidneys, characterized by dilatation of the perirenal lymphatics. This condition closely mimics other cystic lesions of the kidney such as polycystic kidney disease. The diagnosis can be made with classic radiological finding and other laboratory analysis. We report a 3-year-old male boy, a case of bilateral renal lymphangiectasia, diagnosed on ultrasonography and computed tomography typical imaging findings and the laboratory analysis of aspirated fluid.
Keywords: Computed tomography, cyst, perirenal lymphatics, renal lymphangiectasia, ultrasonography
How to cite this article:
Patil AT, Sathyanarayana VH, Venkataramaiah VM. Bilateral renal lymphangiectasia: A case of cystic rarity. Apollo Med 2017;14:176-8 |
| Introduction | |  |
Renal lymphangiectasia is a rare disorder of kidney secondary to developmental malformation of the perirenal lymphatic system. It occurs due to the failure of the perirenal lymphatic tissue to form normal communication with the other lymphatics.[1] This results in dilatation of perirenal lymphatics and formation of unilocular or multilocular cystic masses in the perirenal area.[1] It can occur in both adults and children with no sex predilection.[1] This condition is commonly misdiagnosed as a cystic disease of the kidney.[2] There are only a few reported cases of renal lymphangiectasia in pediatric age group because of its rarity we are reporting this case.
| Case Report | | |
A 3-year-old male child, second-born child to non-consaguinously married couple, presented with a history of abdominal distension since birth. On clinical examination, his abdomen was uniformly distended with bilateral flank mass which was bimanually palpable and ballotable. His blood pressure was found to be normal and no other positive findings.
His routine laboratory investigation revealed that hemoglobin was 11 gm/dl, total count 8700 was cells/mm 3, packed cell volume was 32.5, and platelet counts were 275,000 cells/mm 3, suggestive of normal hemogram findings, blood urea was 16 mg/dl, and serum creatinine 0.4 mg/dl. His routine urine examination findings were within normal limits. We initially thought of cystic disease of the kidney, i.e., autosomal recessive polycystic kidney disease and nephroblastomatosis. We investigated further with imaging to know the cause for his abdominal mass.
Ultrasonography of abdomen demonstrated bilateral multiple perinephric cysts and the largest cyst measuring 8.2 cm × 7.5 cm × 10.3 cm in the right kidney and 10.2 cm × 7 cm × 10.9 cm in the left kidney. Prominent bilateral renal pelvis was seen with increased renal parenchymal echogenicity. There was no hydronephrosis or calculus. Corticomedullary differentiation was not well made out [Figure 1]. | Figure 1: Ultrasonography of abdomen showing bilateral multiloculated perinephric collection
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The perinephric fluid aspirated under ultrasound guidance was found to be straw colored. Laboratory analysis of aspirated fluid revealed creatinine of 0.4 mg/dl, protein 0.6 mg/dl, triglycerides 4 mg/dl, and the presence of few scattered lymphocytes. Culture was sterile.
A contrast-enhanced computed tomography (CECT) of the abdomen revealed well-circumscribed perirenal fluid attenuation insinuating into the renal pelvis, filling up the entire abdomen, and displacing the bowel anteriorly. Thin septations were seen within the cyst. Renal parenchyma was poorly enhanced. There was no hydronephrosis and no evidence of ascites. The fluid attenuation extended superiorly through esophageal hiatus along the mid and lower esophagus [Figure 2]. | Figure 2: Contrast-enhanced computed tomography abdomen showing bilateral perinephric collection with fluid attenuation and no post contrast enhancement
Click here to view |
A final diagnosis of bilateral renal lymphangiectasia was made based on typical imaging findings and laboratory analysis of aspirated fluid.
As the child was asymptomatic, child was discharged and advised for regular follow-up. Child is now on regular follow-up.
| Discussion | | |
Renal lymphangiectasia, also known as renal lymphangiomatosis, is a rare benign disorder characterized by lymphatic malformation of kidney (perirenal, peripelvic, and intrarenal lymphatics). It occurs secondary to failure of renal lymphatic ducts to drain into larger retroperitoneal lymphatics, further leading to dilatation of ducts and formation of unilocular or multilocular cystic spaces in the perinephric space and pelvic sinus.[3]
It can occur at any age with no sex predilection.[1] Familial association has also been reported.[4] The pathophysiology of this condition is still not clear.
Clinical symptoms may vary from asymptomatic to nonspecific symptomatology. When symptomatic, they usually present with abdominal pain, abdominal mass, edema, symptoms of hypertension, hematuria, and can progress to renal failure. Some cases have been presented with renal vein thrombosis and renal insufficiency.[3] Exacerbation during pregnancy has also been reported.[4]
Differential diagnosis for renal lymphangiectasia includes:
- Autosomal dominant polycystic kidney disease
- Lymphoma
- Nephroblastomatosis
- Von Hippel-Lindau syndrome
- Tuberous sclerosis.[1]
Complication of lymphangiectasia described in the literature includes secondary infection intracystic hemorrhage, severe and prolonged hypertension, obstructive uropathy, and renal vein thrombosis.[5]
The diagnosis of renal lymphangiectasia is made by clinical history with typical imaging finding from ultrasonography and CT. The diagnosis can be confirmed by analysis of perinephric fluid which also excludes the presence of urinoma and abscess.
Renal lymphatic aspirates surprisingly not milky or chylous. This is because renal lymphatic ducts are outside the mesenteric drainage pathway. It contains only sporadic cells, mostly lymphocytes and small amounts of fat and protein.[6]
Imaging (ultrasonography and CT) plays an important role in the diagnosis of renal lymphangiectasia. With the classical imaging finding, we can make out the diagnosis of renal lymphangiectasia confidently.
In ultrasonography, there will be perirenal and parapelvic hypo or anechoic lesions. There may be debris or fine echoes with in the cyst which likely indicates intracystic hemorrhage. The cystic lesion may extend into the parapelvic region where the renal pedicle is drained by large perirenal lymphatics.[7] The kidney size may be enlarged with increased renal cortical echogenicity and loss of corticomedullary differentiation, particularly in pediatric patients.[7],[8]
CT demonstrates thin walled, multiloculated cystic lesions filled by material with fluid attenuation with no postcontrast enhancement.[7] There may be increased attenuation in case of cystic hemorrhage.[7]
Asymptomatic patients of renal lymphangiectasia are managed conservatively, whereas the symptomatic patients due to large collection causing pressure symptoms and other complications are treated with percutaneous drainage.[1] Partial regression and deterioration during pregnancy have also been reported in some cases.[4] Recurrent collection can be treated by marsupialization. Ascites and hypertension can be treated with diuretics and antihypertensives. Nephrectomy is indicated only for uncontrollable collection with complications.[9]
| Conclusion | | |
Renal lymphangiectasia is a rare benign disorder of kidney secondary to lymphatic malformation can occur at any age group. It can be differentiated from other cystic diseases of kidney by sonography or CT. It is diagnosed based on typical radiological images (ultrasonography and CT scan). There are only a few reported cases. We are reporting this case of a 3-year-old male child with bilateral renal lymphangiectasia because of its rarity; diagnosis can be missed if not evaluated in the midst of more common renal cystic conditions and imaging can be a diagnostic tool in such scenarios.
Acknowledgment
We would like to acknowledge Dr. Ramesh R L, MBBS, MD, Associate professor, consultant pediatric radiologist, Indira Gandhi Institute of Child Health, South Hospital, for his help in providing the images pertaining to this case.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Valerio M, Meuwly JY, Tawadros C, Jichlinski P. Percutaneous drainage and sclerotherapy as definitive treatment of renal lymphangiomatosis. Can Urol Assoc J 2012;6:E3-7.  [ PUBMED] |
| 2. | Lindsey JR. Lymphangiectasia simulating polycystic disease. J Urol 1970;104:658-62. [ PUBMED] |
| 3. | Davidson AJ, Hartman DS. Lymphangioma of the retroperitoneum: CT and sonographic characteristic. Radiology 1990;175:507-10. [ PUBMED] |
| 4. | Meredith WT, Levine E, Ahlstrom NG, Grantham JJ. Exacerbation of familial renal lymphangiomatosis during pregnancy. AJR Am J Roentgenol 1988;151:965-6. [ PUBMED] |
| 5. | Riehl J, Schmitt H, Schäfer L, Schneider B, Sieberth HG. Retroperitoneal lymphangiectasia associated with bilateral renal vein thrombosis. Nephrol Dial Transplant 1997;12:1701-3. |
| 6. | Llorente JG, García AD, Sacristan JS, Chicharro GN. Renal lymphangiectasia: Radiologic diagnosis and evolution. Abdom Imaging 2002;27:637-9. |
| 7. | Pianezza ML, Mokhtassi A, Wu L, D'A Honey RJ. Case report: Renal lymphangiectasia. Can J Urol 2006;13:3204-7. |
| 8. | Ratti M, Ammar L, Zennaro F, Guastalla P, Marchetti F, Lazzerini M, et al. Renal lymphangiectasia. Pediatr Radiol 2004;34:669-70. [ PUBMED] |
| 9. | Ashraf K, Raza SS, Ashraf O, Memon W, Memon A, Zubairi TA, et al. Renal lymphangiectasia. Br J Radiol 2007;80:e117-8. |
[Figure 1], [Figure 2]
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