CASE 9197 Published on 28.02.2011

Renal lymphangioma: Case report and literature review

Section

Uroradiology & genital male imaging

Case Type

Clinical Cases

Authors

Iqbal S, Jabeen S, Al-Musabbahi, Al-Fatani M

Patient

4 years, male

Categories
Area of Interest Respiratory system, Abdomen, Kidney ; Imaging Technique Digital radiography, Ultrasound, CT
Clinical History
A male Saudi child of 4 years age presented to emergency with severe dyspnoea and abdominal distension. On clinical examination his left chest was silent and stony dull on percussion while abdominal examination showed positive shifting dullness and fluid thrill. Lab findings were within normal limits except for microscopic haematuria.
Imaging Findings
Initial chest X-ray showed opaque left hemi-thorax with contra-lateral shifting of the heart and mediastinum. Abdominal X-ray revealed crowding of the gas filled bowel loops in the centre. Ultrasound abdomen and chest confirmed the presence of massive pleural effusion and ascites. Bilateral kidneys were enlarged and echogenic. Periphery of the kidneys was hypo-echoic and both kidneys had sub-capsular & para-pelvic cysts. However, right kidney showed large sub-capsular cystic mass. On CT scan both kidneys were markedly hypo-attenuated with few distinct cysts. Moderate ascites and marked left sided pleural effusion were present causing contra-lateral shift of heart and the mediastinum. Thoracocentesis was performed to relieve the symptoms. Aspiration cytology confirmed the chylous nature of the fluid. Patient was treated conservatively with satisfactory results.
Discussion
Renal lymphangioma is very rare hamartomatous hyperplasia of renal lymphatics with focal or diffuse involvement. Synonyms include; renal lymphangiomatosis, para-pelvic lymphangiectasia and hygroma renale and polycystic disease of renal sinus. [1] Till now, less than 50 cases of renal lymphangioma have been documented. Among those only 6 were bilateral. The given case is exceptional because the patient had bilateral lymphangioma, ascites and left sided chylothorax. Previously only 1 case of renal lymphangioma associated chylothorax was reported [2]. It can present at any age from 4 months to 80 years with female preponderance of 1.5:1. Generally it is sporadic however familial tendency has been reported [3]. Though it is a benign condition but recent cytogenetic studies have shown its malignant potential by proving anomalous karyotypic involvement of chromosome 7 and the X chromosome [4]. The origin of renal lymphangioma is speculative and is considered to be developmental malformation of lymphatic system which fails to communicate with the draining channels [5]. Majority of patients is asymptomatic but gross haematuria, palpable mass and flank pain may be the presenting symptoms. Sometimes, patient may present with abdominal distension, respiratory distress, and renin-dependent hypertension or pressure symptoms.
Renal lymphangiomas are mainly cystic lesions with relatively normal renal parenchyma. Sonography reveals para-pelvic or sub-capsular cysts. In some instances, echogenic and enlarged kidneys are seen because of small size of innumerable cysts and posterior acoustic enhancement, mimicking the polycystic renal disease [6]. Contrast enhanced CT confirms these findings; however, it is less sensitive than ultrasound to show septations in the cysts. On T1 weighted MRI images, hypo to intermediate signal intensity uni-locular or multi-locular lesions are seen because of fluid nature of the cysts, whereas T2 images are hyper-intense. Cross-sectional imaging can pick up concomitant findings such as ascites and pleural effusion and the involvement of other viscera. X-ray and excretory urography has a limited role. Diagnosis can be confirmed by characteristic features on ultrasound and CT, and fine needle aspiration and cytology of chylous fluid.
Treatment is usually conservative. Per-cutaneous drainage or surgical intervention may be required to alleviate pressure effects. To conclude, renal lymphangioma is an extremely rare disease which should be kept in consideration while evaluating for renal cystic lesions or making diagnosis for fluid collections e.g., ascites & pleural effusion, as prompt diagnosis and early management has an excellent prognosis especially in children.
Differential Diagnosis List
Bilateral renal lymphangioma with chylous abdomen and chylothorax
Benign renal cysts
Polycystic disease of the kidney
Cystic renal cell CA
Multi-loculated cystic nephroma
Final Diagnosis
Bilateral renal lymphangioma with chylous abdomen and chylothorax
Case information
URL: https://www.eurorad.org/case/9197
DOI: 10.1594/EURORAD/CASE.9197
ISSN: 1563-4086