Bilateral peri-renal macrocystic lymphatic malformation in pediatric age-unusual presentation

Lymphatic malformations are benign lesions, found in children, most common locations are neck and axillary regions. We report here a case of bilateral Peri-renal macrocystic lymphatic malformation in a 12-year-old female, masquerading as bilateral renal cysts. A 12-year-old female presented with complaints of pain in right flank for one week associated with fullness in right flank. On evaluation a provisional diagnosis of right perirenal lymphangioma was made and ultrasound guided pigtail drainage done. The pigtail output was 200-300ml daily for a week and it was removed and child was discharged. However, on follow up, CT was done and bilateral peri-renal lymphatic malformation(right>left) was diagnosed. Patient was then planned for exploratory laparotomy which revealed bilateral perirenal cyst, circumscribing both the kidneys completely. Excision of bilateral cysts was done and sent for histopathological evaluation. Cyst Fluid analysis revealed normal creatinine, LDH, total cholesterol, total protein, triglycerides, urea levels. Post-operative period was uneventful. Histopathological examination revealed cystic lesions formed by endothelial cells with focal inflammation, no signs of malignancy were seen. The child was followed up post-operatively and there was no evidence of recurrence. Perirenal lymphatic malformation is a rare entity. The entity is thought to be the result of the obstruction of the perirenal lymphatics. We are presenting this case for its rarity and unusual presentation. Macro cystic Lymphatic malformations are common diagnosis but around the kidneys, there are only very few cases reported in literature. Prognosis of cystic lymphatic malformation is excellent.