Renal Lymphangiectasia Research Articles

Imaging in renal lymphangiectasia: report of two cases and review of literature

Imaging in renal lymphangiectasia: report of two cases and review of literature

Chronic renal insufficiency in a boy with cystic renal lymphangiectasia: morphological findings and long-term follow-up

Cystic renal lymphangiectasia (CRL) is a rare malformation of lymphatics that can present in childhood and adulthood. Symptoms and radiologic features are relatively well defined, but clinical evolution and prognosis remain unclear. We treated a boy with CRL who developed chronic renal insufficiency. The first manifestation was abdominal swelling associated with an umbilical hernia noted incidentally at 1.6 years. Computed tomography with intravenous contrast administration demonstrated perirenal cysts with fluid collection, suggesting CRL. Intractable ascites resisted pharmacologic treatments such as diuretics. After approximately 7 years, the ascites resolved spontaneously, but the perirenal cysts persisted. At 11 years, proteinuria was noted. A renal biopsy specimen showed interstitial abnormalities consistent with CRL, glomeruli showed a focal segmental mesangial increase. Proteinuria persisted despite administration of an angiotensin-converting enzyme inhibitor, increasing as obesity and hypertension worsened. Renal function gradually declined in the ensuing years. Polycythemia coexisted with a normal serum erythropoietin concentration. A follow-up renal biopsy specimen disclosed glomerular enlargement together with focal segmental mesangial expansion, suggesting obesity-related glomerulopathy. Our observation suggest that under some specific circumstances like our patient CRL may exacerbate. Management of complicating obesity and hypertension are likely to be important for maintaining normal renal function, especially in the diffuse bilateral type of CRL present in our patient.

Retroperitoneal lymphangiectasia associated with mediastinal extension: Radiologic findings

Renal lymphangiectasia is a very rare entity. This disorder characterized by developmental malformation of the lymphatic system surrounding the kidneys. A few cases with the retroperitoneal and mediastinal lymphangiectasia reported in the literature. We present the case of an 18-year-old male with retroperitoneal lymphangiectasia and mediastinal extension who presented with abdominal pain. Ultrasonography, computed tomography, and magnetic resonance imaging findings are described.

Renal lymphangiectasia

Renal lymphangiectasia is a benign disorder of renal lymphatics. Seldom observed, the pathophysiology is unclear. The clinical course may vary, and management alternatives range from percutaneous drainage in symptomatic cases to pharmacological substitutes in the form of anti-hypertensives and diuretics. We present a case of bilateral perinephric collections on imaging, which presented with gross ascites, abdominal pain and reversible hypertension. Ultrasound examination indicated ascites. Computerized tomography revealed bilateral symmetrical large perinephric collections. This is consistent with the appearance of renal lymphangiectasis (enlarged kidneys with fluid collections seen to be abutting the surrounding structures) reported in the literature. Needle aspiration of the perinephric fluid was undertaken, and laboratory analysis was carried out, which revealed a protein level of 643 mg dl(-1) and a total leucocyte count of 50, of which 80% were lymphocytes. Thereafter, a diagnosis of renal lymphangiectasia was made, and conservative treatment with diuretics and anti-hypertensives was initiated. Reassessment at subsequent follow up visits showed improvement in the patient's clinical condition.

Unilateral Renal Lymphangiectasia: Imaging Appearance on Sonography, CT and MRI

Lymphangiomas are benign tumors of unknown etiology often seen in children, involving the neck (75%), and axillary (20%) region, but rarely the kidney [Zapzalka et al. (2002) Lymphangioma of the renal capsule. J Urol 168:220.]. We report a case of unilateral renal lymphangiectasia, where the diagnosis was made on imaging.

MRI findings of renal lymphangiectasia

We present the case of a 20-year-old male with renal lymphangiectasia who presented with hypertension and hematuria. We discuss the role of magnetic resonance imaging (MRI) with gadolinium-enhanced MR urography in confirming the diagnosis, extensions of this rare benign entity, and associated conditions.

Cystic renal lymphangiectasia presenting as renal insufficiency in childhood

Cystic renal lymphangiectasia is an unusual cause of cystic renal disease in childhood. We present a case of bilateral cystic renal lymphangiectasia in a 7-year-old boy who presented with asymptomatic renal insufficiency and anemia with decreased erythropoietin production. The clinical features of this condition and the diagnostic approach are reviewed. Although rare, this disorder should be considered in the differential diagnosis of cystic renal disease.

Cystic Renal Lymphangiectasia: A Distinctive Clinicopathologic Entity in the Pediatric Age Group

Cystic Renal Lymphangiectasia: A Distinctive Clinicopathologic Entity in the Pediatric Age Group

Cystic Renal Lymphangiectasia: A Distinctive Clinicopathologic Entity in the Pediatric Age Group

This report describes the features of unilateral cystic renal lymphangiectasia in a 2-year-old child who presented with hypertension, massive ascites, a left flank mass, and no evidence of familial renal cystic disease. The child became normotensive and is now asymptomatic more than 3 years after surgery. The clinical presentation and diffuse pathologic involvement are similar to findings for the few pediatric patients with cystic lymphangiectasia described in the literature and appear distinct from the more localized form of the disease seen in adults.

Unilateral Lymphangiectasis of the Kidney: An Unusual Cause of Renal Enlargement in an Infant

We report on an infant with unilateral renal lymphangiectasia and the absence of small cysts in the renal parenchyma or renal hilum. We suggest that renal lymphangiectasia be part of the differential diagnosis in children who present with unilateral renal enlargement, especially when contrast enhanced computerized tomography shows preservation of a rim of renal cortex with decreased attenuation of the medulla and/or when ultrasound demonstrates increased echogenicity without displacement of the caliceal system.

Renal peripelvic lymphangiectasia: appearance at CT.

In a patient who had undergone emergency nephrectomy during exploratory surgery, a pathologic diagnosis of peripelvic lymphangiectasia was made. Subsequent computed tomographic examination of the remaining kidney showed large multilocular renal sinus cysts, renal capsular cysts, and regions of fluid attenuation surrounding the mesenteric vessels, findings consistent with peripelvic lymphangiectasia. A brief discussion of cystic disease of the renal sinus is presented.