Obstructive uropathy associated with bilateral renal lymphangiomatosis
Jainn-Shiun Chiu1,
Ching-Jiunn Wu2,
Guang-Huan Sun3 and
Shih-Hua Lin1
1 Division of Nephrology, Department of Internal Medicine, 2 Department of Radiology and 3 Division of Urology, Department of Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan
Correspondence and offprint requests to: Shih-Hua Lin, MD, Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, No. 325, Sec. 2, Chenggong Road, Neihu District, Taipei City 114, Taiwan. Email: l521116{at}ndmctsgh.edu.tw
Keywords: bilateral; lymphatic system; obstructive uropathy; renal lymphangiomatosis
Renal lymphangiomatosis (RL) is a rare disorder characterized by developmental malformation of the lymphatic system surrounding the kidneys [1]. Obstructive uropathy is a rarely reported complication of this disorder.
A 55-year-old man presented with abdominal distension and bilateral flank soreness. Blood pressure was 150/94 mmHg. Serum creatinine was 1.9 mg/dl. Abdominal ultrasonography showed bilateral perirenal multiseptated fluid collections. Computed tomography of the kidneys is shown in Figure 1. The cytology from percutaneous drainage exhibited a bloody fluid, filled with lymphocytes, but gave negative cultures for any pathogen. After surgical resection of the masses, the clinical symptoms were relieved and serum creatinine returned to 1.1 mg/dl.

View larger version (70K):
[in this window]
[in a new window]
|
Fig. 1. Computed tomography, with (right) and without (left) intravenous contrast, demonstrates multiple cystic lesions in the parapelvic and perirenal areas of both enlarged kidneys with right hydronephrosis.
|
|
RL is usually bilateral without gender or age differences. Clinically, patients may present with insidious abdominal or flank complaints or a palpable mass. Haematuria, protenuria, hypertension and intracystic haemorrhage may accompany the complaint. The renal parenchyma is spared and renal function is often normal, but may be impaired due to the compressive effects of obstructive uropathy, as in this patient. Although RL is benign in nature, it can be mistaken for malignancy or hereditary/acquired renal cystic disease [2]. For symptomatic patients, percutaneous drainage should be used initially. Surgical wide-excision should be reserved for more complicated conditions, such as obstructive uropathy and secondary hypertension due to page kidney.
Conflict of interest statement. None declared.
References
- Mani NB, Sodhi KS, Singh P, Katariya S, Poddar U, Thapa BR. Renal lymphangiomatosis: a rare cause of bilateral nephromegaly. Australas Radiol 2003; 47: 184187[CrossRef][Medline]
- Varela JR, Bargiela A, Requejo I, Fernandez R, Darriba M, Pombo F. Bilateral renal lymphangiomatosis: US and CT findings. Eur Radiol 1998; 8: 230231[CrossRef][ISI][Medline]