Bilateral peri-renal lymphangiomatosis
Rajeev TP1, Barua S1, Deka PM1, Hazarika S21 Departments of Urology, Gauhati Medical College Hospital, Guwahati, Assam, India2 Departments of Radiology, Gauhati Medical College Hospital, Guwahati, Assam, India
Correspondence Address:Rajeev T P"Sadananda", House No-9, Bye Lane No-7, Natun Sarania, Gandhi Basti, Guwahati - 781003, Assam Indiarajeevtp@hotmail.com
A 30 yrs old female with non-specific abdominal pain is presented. Ultrasound, computerised tomography abdomen were performed. A finely septated fluid collection surrounding both kidneys were found. Ultrasound guided fluid aspiration; chemical and cytological evaluation of the fluid was done. Exploration was done on the right side with the idea of removing the peri- renal cystic lymphatic collection. The entire cystic collection was removed. Patient followed up for the last two years and there is no recurrence of the cystic collection and the kidney function is preserved.
Keywords: Kidney, lymphangioma, peri-renal cysts
Renal lymphangiomatosis is a very rare disorder. Only a few cases are reported in the literature. Lymphangiomatosis are regarded as developmental malformations in which lymphangiectasia follows the failure of developing lymphatic tissue to establish normal communication with the remainder of the lymphatic system. Abnormal lymphatic channels may dilate to form a cystic mass that may be uni-locular or multi-locular.
A 30 year-old woman was referred for bilateral non-specific abdominal pain of 5 months duration. Previous history was unremarkable, except for a normal delivery 5 months back. Per abdominal examination revealed a bilateral vague, non-tender flank mass (right > left). Abdominal ultrasound showed a bilateral, peri-renal, septated, anechoic mass with increased through-transmission [Figure - 1]. Both kidneys were otherwise normal. Computed tomography with and without intravenous contrast, confirmed bilateral, peri-renal, multiple thin walled fluid collections surrounding both kidneys [Figure - 2]. The peri-renal fascia was thickened bilaterally. Ultrasound guided fluid aspiration was done bilaterally, which revealed a straw coloured fluid and examination of the fluid showed 100% lymphocytes. Exploration was done on the right side, as the right side seemed to be more affected, from physical findings and investigation reports. On exploration, the peri-renal fat was found to be thickened and studded with multiple cysts of varying sizes. The entire peri-renal fat with cysts was excised. On histological examination, each cyst was found to be lined with a single layer of flattened cells and filled with homogenous eosinophillic substance [Figure - 3]. The lining cells were focally positive for factor VIII-related antigen and negative for CD34, strongly suggesting lymphatic endothelial cells. The final diagnosis was peri-renal lymphangiomatosis.
Lymphangiomas are rare, benign malformations of the lymphatics. Retroperitoneal lymphangiomas are usually of the cystic type and may be unilateral or bilateral. Peri-renal lymphangiomatosis is a very rare disorder. This entity is due to obstruction of larger lymphatics that drain the kidney through the renal pedicle. Retroperitoneal lymphangiomatosis is detected incidentally by ultrasound or computed tomography, in patients with non-specific abdominal complaints. Exacerbation of retroperitoneal lymphangiomatosis may occur during pregnancy, as in our case. The only possible way of diagnosis in retroperitoneal lymphangiomatosis is bilateral peri-renal distribution of thin walled fluid density collections depicted on ultrasound, or computed tomography coupled with evaluation of the aspirated fluid. This condition must be distinguished from autosomal dominant polycystic kidney disease, where there are innumerable parenchymal cysts, whereas the cysts in retroperitoneal lymphangiomatosis are perinephric with normal renal parenchyma. In the earlier case reported, the diagnosis was made after nephrectomy. In our case, we explored with a suspicion of the diagnosis and excised the entire peri-renal cystic collection and were able to preserve the kidney.
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Indian Journal of Urology