Renal lymphangiectasia presented by pleural effusion and ascites

Abstract

A young male patient complaining of vague abdominal pain, dyspnea, generalized weakness and abdominal distension for 6 months was referred for abdominal ultrasound. Ultrasound showed enlarged echogenic kidneys, perinephric and renal sinus cystic fluid collections bilaterally with ascites and right pleural effusion. The ultrasound findings were confirmed by abdomen CT scan. Renal function test was within normal. Laboratory analysis of aspirated perinephric fluid revealed abundant lymphocytes. The radiological findings and perinephric fluid aspiration analysis are consistent with renal lymphangiectasia. Pleural effusion, in addition to ascites and perinephric fluid collections, is a new presentation of the disease. Ascites and pleural effusion were improved by diuretics.

CASE REPORT

A 22-year-old male presented with a history of generalized weakness, progressive abdominal distension, vague abdominal pain and dyspnea for 6 months. He had no other significant past or family history. On examination, his blood pressure was 120/70 mmHg. Urine analysis was normal, hemoglobin level 15 mg dl-1(13–18 dl-1), urea 20.8 mg dl-1(10–50 dl-1), creatinine 1.37 mg dl-1(1.6 mg dl-1), serum potassium 3.9 mEq l-1(3.3–5.5 mEq l-1), serum albumin 3.9 mg dl-1(3.5–5.0 mg dl-1), SGPT 24 IU/L (5 to 40 IU/L), SGOT 27 IU/L (10 to 40 IU/L) and ALP 90 IU/L (30 to 120 IU/L).

Ultrasound revealed enlarged echogenic kidneys, each measured about 14 cm long with perinephric and renal sinus cystic fluid collections bilaterally (figure 1) as well as right pleural effusion (figure 2) and ascites (figure 3).

Figure 1.

Abdomen ultrasound of a 22 male patient with renal lymphangiectasia, done by Siemens-sienna machine using a convex transducer of 3.5 MHz. The grayscale coronal view at the right flank shows enlarged right kidney, about 14 cm long, with renal sinus cysts (asterisks) and perinephric collections (arrows).

Figure 2.

Abdomen ultrasound of a 22 male patient with renal lymphangiectasia, done by Siemens-sienna machine using a convex transducer of 3.5 MHz. The grayscale coronal oblique view at the right hypochondrial region shows massive right pleural effusion (asterisk).

Figure 3.

Abdomen ultrasound of a 22 male patient with renal lymphangiectasia, done by Siemens-sienna machine using a convex transducer of 3.5 MHz. The grayscale sagittal view in the suprapubic region shows ascites (asterisk) with contracted urinary bladder (arrow).

Abdomen CT scan showed bilateral perinephric fluid collections, which were almost symmetrical (figure 4), right pleural effusion (figure 5) and gross ascites (figure 5 and 6). No cystic areas were seen in the pancreas or liver. No other abnormality was found on the CT scan. The mean CT density of the renal sinus cysts was 3 Hounsfield units. The mean CT density of the perinephric fluid collection was 2, of the pleural effusion; 3 and that of ascites; 3 Hounsfield units, suggesting transudate.

Figure 4.

Abdomen CT scan of a 22 male patient with renal lymphangiectasia. The examination was done by Brilliance 64 Philips machine; kV 120.0, mAs 246 and 3 mm slice thickness. Oral contrast and 70 ml IV contrast (ultravist) were given. The axial contrast enhanced section in the excretory phase at level of the upper abdomen shows renal sinus cysts (asterisks) and perinephric fluid collections (arrows) bilaterally.

Figure 5.

Abdomen CT scan of a 22 male patient with renal lymphangiectasia. The examination was done by Brilliance 64 Philips machine; kV 120.0, mAs 246 and 3 mm slice thickness. Oral contrast and 70 ml IV contrast (Ultravist) were given. The axial contrast enhanced section in the excretory phase at level of the upper abdomen shows Rt. pleural effusion (thick arrow) and ascites (thin arrows).

Figure 6.

Abdomen CT scan of a 22 male patient with renal lymphangiectasia. The examination was done by Brilliance 64 Philips machine; kV 120.0, mAs 246 and 3 mm slice thickness. Oral contrast and 70 ml IV contrast (Ultravist) were given. The axial contrast enhanced section in the excretory phase at level of the pelvis shows ascites (asterisks). Ureters are filled with contrast (arrows).

Needle aspiration of the perinephric fluid was carried out, and laboratory analysis revealed abundant lymphocytes. This patient underwent anti TB treatment for one year with no improvement and the pleural effusion and ascites were improved by diuretics only (figures 7 and 8).

Figure 7.

Follow up abdomen CT scan of a 22 male patient with renal lymphangiectasia. The examination was done by Brilliance 64 Philips machine; kV 120.0, mAs 246 and 3 mm slice thickness. Oral contrast and 70 ml IV contrast (Ultravist) were given. The axial contrast enhanced section in the excretory phase at level of the upper abdomen shows relative improvement in the Rt. pleural effusion (arrow).

Figure 8.

Follow up abdomen CT scan of a 22 male patient with renal lymphangiectasia. The examination was done by Brilliance 64 Philips machine; kV 120.0, mA 246 and 3 mm slice thickness. Oral contrast and 70 ml IV contrast (Ultravist) were given. The axial contrast enhanced section in the excretory phase at level of the pelvis shows relative improvement in the ascites (asterisk).

DISCUSSION

Renal lymphangiectasia is a rare benign disorder of renal lymphatics that has been confused with other cystic disease of the kidney (1). Knowledge of the condition is based mostly on solitary case reports. Approximately 40 cases have been reported since 1890 (2). It is known by many different names including renal lymphangiomatosis (1), renal lymphangioma (3), renal peripelvic multicystic lymphangiectasia" (4), peripelvic lymphangiectasia (5), hygroma renale (2) and polycystic disease of the renal sinus (6). The origins and cause of the condition are unclear (7).

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) (2,7).

Imaging findings of renal lymphangiectasia include peripelvic cysts and perirenal fluid collections (1,5,6,8). 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 (7,9). Surrounding structures are not seen to be invaded, but only abutted or displaced. Ascites may be found, and is a known complication. However, features on ultrasound and CT are known to be diagnostic of the condition (9).

Differentials of renal lymphangiectasia include polycystic renal disease, nephroblastomatosis, lymphoma and multilocular cystic nephroma depending on the age and appearance of the disease (6,9). The diagnosis of renal lymphangiectasia can be confirmed with needle aspiration of chylous fluid from the perinephric fluid collections (6).

Causes of ascites (as shown in table 1) can be classified according to if associated with peritoneal diseases or not (10).

Table 1.

Causes of ascites according to if associated with peritoneal disease or not.

Without Peritoneal Disease:

Portal hypertension

Cirrhosis

Alcoholic hepatitis

Hepatic congestion

Congestive heart failure

Tricuspid insufficiency

Constrictive pericarditis

Inferior vena cava obstruction

Hepatic vein obstruction (Budd-Chiari syndrome)

Cardiomyopathy

Portal vein occlusion

Thrombosis

Tumor

Idiopathic tropical splenomegaly

Partial nodular transformation

Hypervitaminosis A

Fulminant hepatic failure

Idiopathic

Hypoalbuminemia

Cirrhosis

Nephrotic syndrome

Protein-losing enteropathy

Lymphangiectasia

Severe malnutrition

Miscellaneous

Myxedema

Hepatocellular carcinoma (usually with cirrhosis)

Ovarian disease

Tumor (Meigs' syndrome)

Struma ovarii

Ovarian overstimulation syndrome

Pancreatic ascites

Rupture of pseudocyst

Leak from pancreatic duct

Bile ascites

Gallbladder rupture

Traumatic bile leak

Chylous ascites

Rupture (traumatic, surgical) of abdominal lymphatics

Congenital lymphangiectasia

Obstructed lymphatics (especially secondary to malignancy, tuberculosis, filariasis)

Constrictive pericarditis

Cirrhosis

Sarcoidosis

With Peritoneal Disease:

Infection

Mycobacterial

Bacterial

Primary (spontaneous bacterial peritonitis in cirrhosis)

Secondary (ruptured viscus)

Fungal (rare, especially candidiasis, histoplasmosis, cryptococcosis)

Parasitic (rare, especially schistosomiasis, ascariasis, enterobiasis)

AIDS

Neoplasm

Primary mesothelioma

Metastatic carcinomatosis

Ovarian

Pancreatic

Gastric

Colonic

Lymphoma

Miscellaneous

Peritoneal vasculitides

Systemic lupus erythematosus

Henoch-Schönlein purpura

Köhlmeier-Degos disease

Eosinophilic peritonitis

Familial Mediterranean fever

Pseudomyxoma peritonei

Whipple's disease

Granulomatous peritonitis

Foreign bodies (especially starch)

Sarcoidosis

Gynecologic lesions (especially endometriosis, ruptured dermoid cyst)

Peritoneal lymphangiectasis

The CT density of the pleural fluid is in our case 3 Hounsfield units suggesting transudate. The causes of pleural transudate can be also cardiac, renal, hepatic or due to thrmboembolic diseases (11).

Complications of renal lymphangiectasia can include haematuria, ascites, occasional renal venous thrombosis, deterioration in renal function and renin-dependent hypertension (2,6). Treatment is not usually necessary. Conservative treatment with diuretics and anti-hypertensives may be initiated for symptomatic patients (10). Complicated cases may be treated with nephrectomy (if unilateral), percutaneous drainage, or marsupialization (6).

TEACHING POINT

Renal lymphangiectasia is a rare disease and diagnosis is based primarily on the radiological findings of renal sinus cysts and aspiration of perinephric fluid collections. This case presents with pleural effusion in addition to ascites and perinephric fluid collections, which is a new finding of the disease and should be considered in the differential diagnosis of pleural effusion.

Table 2.

Differential diagnoses of pleural transudate.

Cardiac disease

Congestive heart failure

Fluid overload

Constrictive pericarditis

Obstruction of superior vena cava or azygos vein

Renal disease

Nephrotic syndrome

Acute glomerulonephritis

Urinary tract obstruction

Peritoneal dialysis

Liver disease

Cirrhosis with ascites

Thromboembolic disease

Pulmonary embolism

Others

Meigs' syndrome

Myxedema

Sarcoidosis

Severe malnutrition (with hypoalbuminemia)

Iatrogenic (e.g., venous catheter in pleural space)

After lung transplantation

ABBREVIATIONS

mmHg

millimeter mercury

mg dl⁻¹

milligram per deciliter

mEq l⁻¹

milli-equivalent per liter

cm

centimeter

CT

computed tomography

TB

tuberculosis

ALP

Alkaline phosphatase

SGPT

Serum Glutamic Pyruvate Transaminase

SGOT

Serum Glutamic Oxaloacetic Transaminase

IU

International unit

IV

intravenous