Bilateral renal lymphangiectasia- an unusual case of page kidney

Abstract

Renal lymphangiectasia is a rare benign condition caused by failed renal lymphatic drainage. A 36-year-old female presented with bilateral flank pain, pedal oedema, and secondary hypertension due to Page kidney. Imaging revealed large bilateral perirenal fluid collections, and aspiration confirmed chylous fluid. Despite temporary relief, reaccumulation and worsening renal function necessitated bilateral cyst deroofing, allowing drainage into the peritoneum. Postoperatively, blood pressure and renal function normalized, with complete resolution on follow-up. This case highlights the importance of recognizing renal lymphangiectasia as a cause of secondary hypertension and renal impairment to ensure timely and effective management.

1 Introduction

Renal lymphangiectasia is a rare benign condition that happen due to the failure of the renal lymphatics to join with the larger retroperitoneal lymphatics. Renal lymphangiectasia contributes to up to 1 % of all diagnosed lymphoma. It can present across all age groups and both sexes and could be unilateral or bilateral. Owing to its rarity it is quite usual for this condition to be misdiagnosed. Here we present the case of a 36 years old female who presented with secondary hypertension – page kidney and bilateral renal lymphangiectasia who was treated with full recovery of renal function and resolution of hypertension. This case underlines the importance of aptly diagnosing such rare conditions that can completely turn the tide in the life of patients.

2 Case history

A 36 years old female presented to the Emergency room with complaints of bilateral flank pain and pedal oedema for 5 days duration. On examination she was found to have blood pressure of 180/90 mmHg. Abdominal examination revealed bilateral flank swellings −10 × 8 cm mass on the right side and 8 × 6 cm mass on the left side. Both lumps were firm in consistency moving with respiration and were not crossing midline. They were bimanually palpable and ballotable. No evidence of renal bruits bilaterally. Her bloodwork revealed a raised creatinine of 2.4 mg/dL. She underwent Ultrasound scanning of the abdomen which revealed bilateral large fluid collection surrounding the kidneys. For further characterisation of these, she underwent a CT Urogram ( Fig. 1 ) which revealed bilateral cystic collections around both the kidneys which showed no contrast extravasation in the urogram phase. Thus, an active connection to the Pelvicalyceal system was ruled out. A USG guided aspiration of the collection was planned in order to further analyse the nature of the fluid and to give a therapeutic trial. She underwent aspiration of bilateral collections. 860 ml of straw-coloured fluid was aspirated from the left side and 740 ml of fluid was aspirated from the right side. The fluid was sent for analysis which was showing high levels of chylomicrons and triglycerides. 3 days after the aspiration, her abdominal pain subsided, Serum creatinine level fell to 1.3 mg/dL and pedal oedema subsided. 9 days after aspiration, she underwent a repeat ultrasound examination which was showing reaccumulation of the fluid. This was associated with an increase in the blood pressure to 164/86 mm Hg and Serum creatinine worsened to 1.9 mg/dL. During this time her plasma renin activity increased to 39.1 ng/ml/hr.

3 Surgical intervention

She was taken up for surgery in view of recurrence. Prior to surgery, she underwent an RGP ( Fig. 2 ) which demonstrated no dye extravasation to the collection, the pelvicalyceal system appeared to be compressed extrinsically. She underwent bilateral deroofing of the cysts. A 2 × 2 cm widow was created on both the cysts in order to drain into peritoneum ( Fig. 3 ). Post operatively she improved, abdominal pain subsided, blood pressure normalized and serum creatinine dropped to 0.9 mg/dL. She was discharged on POD 4.