ESPN 51th Annual Meeting

ESPN 2018


 
RENAL LYMPHANGIOMATOSIS PRESENTING WITH ASCITES AND PLEURAL EFFUSION
AYŞE AĞBAŞ 1 BAĞDAGÜL AKSU 1 BERNA BAL 1 GÜZIDE DOĞAN 1 TÜRKAN IKIZCELI 2 HATICE NILGÜN SELÇUK DURU 1 MURAT ELEVLI 1

1- UNIVERSITY OF HEALTH SCIENCES, HASEKI EDUCATION AND RESEARCH HOSPITAL, DEPARTMENT OF PEDIATRICS, ISTANBUL
2- UNIVERSITY OF HEALTH SCIENCES, HASEKI EDUCATION AND RESEARCH HOSPITAL, DEPARTMENT OF RADIOLOGY, ISTANBUL
 
Introduction:

 Renal lymphangiomatosis is a benign, rare entity characterized by developmental malformation of the renal lymphatics. Presenting symptoms and signs are abdominal pain, flank pain, abdominal mass, hypertension, haematuria or proteinuria. 

Material and methods:

A 4-year-old girl patient presented with abdominal pain and distension with a duration of 2 months. 

Results:

 On clinical examination, she had abdominal distension with a level two ascites. Blood pressure was 96/60 mmHg. Urine analysis, liver and kidney function tests (AST: 31 IU/L, ALT: 20 IU/L, albumin: 4.5 g/dl, urea: 18 mg/dl, creatinin: 0.3 mg/dl) were normal. Abdominal ultrasound revealed ascites. Size and structure of liver and spleen were normal. Right kidney was 82 mm, left kidney was 88 mm, parenchymal thicknesses were 10 mm. Renal echogenicity was increased. There were bilateral subcortical multiple cysts with varying sizes, maximum cyst size was 40 mm. Paracentesis was performed for differential diagnosis of the ascites. Ascites was consistent with transudate. Adenosine deaminase activity was (1.1 U/L) normal. There was no cell in the fluid and culture was negative. Viral serology was unremarkable. Echocardiography was normal. Abdominal MRI (Figure-1) revealed that hepatobiliary system was normal. There was perirenal uncomplicated cysts without contrast enhancement. Cysts were causing indentation of the renal parenchyma. Pleural effusion with a 13 mm thickness was detected in the lower thoracic sections of the abdominal MRI. Radiologic images were consistent with renal lymphangiomatosis. Spironolactone was administered. In the 3rd month of the treatment there were no pleural effusion and ascites but subcortical cysts were similar in size. 

Conclusions:

  Renal lymphangiomatosis is a rare, benign disease and should be keep in mind especially in the differential diagnosis of cystic diseases of the kidneys. Diagnosis of renal lymphangiomatosis is done with specific radiological findings. Diuretics, surgery or sclerotherapy are the treatment options. Although the recurrence is frequent, renal function is usually preserved.