British Journal of Radiology (2007) 80, e117-e118
© 2007 British Institute of Radiology
doi: 10.1259/bjr/16931054
Renal lymphangiectasia
K Ashraf, FCPS, FRCR 2A
1
S S Raza, MBBS
2
O Ashraf, MBBS
3
W Memon, FCPS
2
A Memon, FRCS
4 and
T A Zubairi, MBBS
2
1 Cross-sectional Imaging Section, James Paget University Hospitals, Norfolk NP31 6LA, UK, 2 Department of Radiology, Aga Khan University Hospital, 3 Medical College, Aga Khan University, 4 Department of Urology, Aga Khan University Hospital, Stadium Road, Karachi 74800, Pakistan
Correspondence: Kashif Ashraf, Consultant Radiologist, Cross-sectional Imaging Section, James Paget University Hospitals, Norfolk NR31 6LA, UK. E-mail: kashif.ashraf{at}jpaget.nhs.uk
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Abstract
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Renal lymphangiectasia is a benign disorder of renal lymphatics. Seldom observed, the pathophysiology is unclear. The clinical course may vary, and management alternatives range from percutaneous drainage in symptomatic cases to pharmacological substitutes in the form of anti-hypertensives and diuretics. We present a case of bilateral perinephric collections on imaging, which presented with gross ascites, abdominal pain and reversible hypertension. Ultrasound examination indicated ascites. Computerized tomography revealed bilateral symmetrical large perinephric collections. This is consistent with the appearance of renal lymphangiectasis (enlarged kidneys with fluid collections seen to be abutting the surrounding structures) reported in the literature. Needle aspiration of the perinephric fluid was undertaken, and laboratory analysis was carried out, which revealed a protein level of 643 mg dl–1 and a total leucocyte count of 50, of which 80% were lymphocytes. Thereafter, a diagnosis of renal lymphangiectasia was made, and conservative treatment with diuretics and anti-hypertensives was initiated. Reassessment at subsequent follow up visits showed improvement in the patient's clinical condition.
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Introduction
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Renal lymphangiectasia is a very rare benign disorder of renal lymphatics that has at times been confused with other cystic diseases of the kidney [1]. Knowledge of the condition is based mostly on solitary case reports. Approximately 40 cases have been reported since 1890 [2]. The largest study that was carried out on retroperitoneal lymphangiectasia consisted of 19 patients [3]. We present the clinical course of a patient with renal lymphangiectasis and discuss the imaging features and management alternatives.
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Case history
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A 23-year-old female presented with a history of progressive abdominal distension and vague abdominal pain for 1 month. There was no history of any urinary complaints. She did have a previous history of high-grade fever 1 month previously. However, at the time of presentation, she was afebrile. She was also diagnosed as having a paraumbilical hernia 3 months earlier. She had no other significant past or family history. On examination, she was pale, and her blood pressure was 170/110 mmHg. She also had a positive shifting dullness suggesting ascites. Baseline investigations revealed urinary proteins 24 mg, haemoglobin level 8.1 mg dl–1, urea 20.8 mg dl–1, creatinine 0.8 mg dl–1, serum potassium 3.9 mEq l–1 and serum albumin 3.9 mg dl–1. An ultrasound examination indicated gross ascites and bilateral hydronephrosis.
To determine the cause of ascites and hydronephrosis, a CT scan was carried out on a GE helical scanner. Interestingly, what was thought of as hydronephrosis on ultrasound turned out to be large renal sinus fluid collections. The renal pelvis and calyces appeared normal in calibre. No calculi were found in either kidney or ureter, and the renal veins were also seen to be patent.
The most significant finding on the CT scan was the presence of large bilateral perinephric fluid collections, which were almost symmetrical (Figure 1
). Gross ascites was also present. No cystic areas were seen in the pancreas or liver. No other abnormality was found on the CT scan. CT density of the fluid was 15 Hounsfield units.
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Figure 1. Computerized tomographic section of two images showing bilateral perinephric and renal sinus fluid collections, along with ascites.
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In the absence of cardiovascular, renal or hepatic pathology, normally visualized renal vessels and characteristic CT appearance, a diagnosis of renal lymphangiectasia was made.
Needle aspiration of the perinephric fluid was carried out, and laboratory analysis showed a protein level of 643 mg dl–1 and a total leucocyte count of 50, of which 80% were lymphocytes. No growth was found on culture of the fluid.
A diagnosis of renal lymphangiectasia was made. Conservative treatment with diuretics and anti-hypertensives was carried out. She was reassessed on a follow up visit. Her blood pressure had returned to 130/70 mmHg. A repeat ultrasound showed decreasing ascites.
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Discussion
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Renal lymphangiectasia is a very rare benign disorder of the renal lymphatics. It is known by many different names including renal lymphangiomatosis [1], peripelvic lymphangiectasia, hygroma renale [2] and polycystic disease of the renal sinus [4]. The origins and cause of the condition are unclear, with it now generally being regarded as a developmental malformation that follows failure of the lymphatics to make communication with the rest of the lymph trunks. The abnormal lymphatics thus dilate and form cystic spaces at the renal sinus and in the perinephric space [3].
Clinically, it is usually asymptomatic and incidentally diagnosed. When symptomatic, the most common presentations are abdominal pain (42%), abdominal distension (21%), followed by fever, haematuria, fatigue, weight loss and hypertension and occasional deterioration in renal function (mostly reversible) [2, 3].
Imaging features are simple to understand. The appearance is of soft tissue masses on plain radiographs, displacing the surrounding structures such as bowel loops. No fat lucency is seen within the mass, and no bone erosions are found. On intravenous urographic examination, displacement and distortion of urinary structures is seen without invasion [1, 3].
On ultrasound and CT, features depend on the site and extent of lymphatic obstruction. If only smaller intrarenal lymphatics are blocked, either a solid renal lesion or diffuse enlargement of the kidneys may be seen, without any cystic spaces [5]. On sonography, characteristics of simple cysts are seen. Cysts are seen to be anechoic, with enhancement through transmission and a sharply defined far wall [3]. Kidneys may appear enlarged, and cortico-medullary differentiation may be lost [1]. On CT, similar appearances of fluid collections are seen, but the septa may not be very conspicuous. The attenuation within the cysts lies in the range of fluid [3, 6]. Surrounding structures are not seen to be invaded, but only abutted or displaced. Ascites may be found, as in our case, and is a known complication. However, features on ultrasound and CT are known to be diagnostic of the condition [6].
The distinguishing laboratory features of these renal fluid collections from normal lymph include the presence only of sporadic cells, small amounts of fat globules, proteins and high renin levels in the fluid, suggesting the renal origin of the lymph [2, 7]. Differentials of renal lymphangiectasis include polycystic diseases, nephroblastomatosis, lymphoma and multilocular cystic nephroma depending on the age and appearance of the disease [4, 6]. Other causes of perinephric collections have also to be ruled out, e.g. urinoma or abscess.
The complications of the disease can include haematuria, ascites, occasional deterioration in renal function and renin-dependent hypertension [2, 4].
Treatment of asymptomatic cases is not required. When collections are very large and causing pressure symptoms, percutaneous drainage may be carried out. Exacerbations, as encountered during pregnancy, may necessitate drainage [1, 8]. Marsupialization has been reported as a treatment option where a connection is made with the peritoneal cavity, draining the fluid into it [2]. Diuretics can then be used to control the ascites. Anti-hypertensive medication is used to control hypertension, when present. In severe uncontrollable cases, nephrectomy may be performed.
In summary, we present a rare case of renal lymphangiectasia, which was also complicated by ascites. Awareness of this condition will be helpful in differentiating it from other cystic diseases of the kidney and other causes of perirenal fluid collections.
Received for publication August 18, 2005.
Revision received November 1, 2005.
Accepted for publication November 4, 2005.
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References
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Abstract
Introduction
