Renal Lymphangiectasia

Renal Lymphangiectasia

Lorenz T. Ramseyer, MD

1 From Bass Baptist Memorial Hospital, 600 S Monroe,
Enid, OK 73701. Received May 28, 1999; revision requested July 20;
revision received August 26; accepted August 30. Address

correspondence to the author (e-mail: lramseyer@peakonline.com

Index terms: Diagnosis Please • Kidney, CT, 81.12112 • Lymphatic system, abnormalities, 994.829 • Lymphatic system, CT, 994.12912 • Urography, 81.11

History

A 28-year-old obese man had microscopic
hematuria. Physical examination results were
normal. His medical history was unremarkable,
and there was no pertinent family history. Excretory
urography (Fig 1) and computed tomography

(CT) of the abdomen (Fig 2) were
performed after oral and intravenous
administration of contrast material.

Imaging Findings

The excretory urogram demonstrated
bilateral nephromegaly with distortion of
the pelvocaliceal systems (Fig 1). The CT
scan of the abdomen (Fig 2) showed fluid
collections in the perinephric space bilaterally,
surrounding the renal cortex. In addition, there
were peripelvic fluid collections bilaterally,
with distortion of the pelvocaliceal systems.

There were fluid collections in the
retroperitoneum that crossed the midline
at the level of the renal hila, adjacent to the
abdominal aorta and the inferior vena cava.

Discussion

Several differential diagnostic possibilities are to
be considered with bilateral nephromegaly with
pelvocaliceal splaying and distortion. Adult polycystic
kidney disease, lymphoma, nephroblastomatosis,
and other causes of multiple renal masses, such as
von Hippel-Lindau disease and tuberous sclerosis,
are considerations. Adult polycystic kidney disease
has characteristic findings of numerous bilateral renal
cysts, with or without hepatic or pancreatic cysts.

The cysts typically vary in size and are scattered
throughout the parenchyma. Lymphoma and other
malignancies can demonstrate soft-tissue masses
involving the kidneys, pelvocaliceal systems, or
retroperitoneum. The fluid attenuation of the perinephric
collections in the test case, as evidenced by attenuation
measurements of 0–10 HU in Figure 2c, exclude
lymphoma and other soft-tissue masses. Perinephric and
retroperitoneal soft-tissue masses can be seen in

retroperitoneal fibrosis but are again excluded by the
fluid attenuation in the test case. Nephroblastomatosis
is a cause of nephromegaly in children and is characterized
by multiple subcapsular and parenchymal soft-tissue nodules
composed of metanephric blastema (1).

Renal lymphangiectasia is a rare disorder.
Patient symptoms described in the literature (2–4)include
hematuria, flank pain, and abdominal pain. The
condition has been found in children and in adults
(2,4–7). The origin of this disorder is speculative.

There is a familial association in some cases,
which argues for a congenital cause (6). There was
no known family history of renal lymphangiectasia
in the test case. Others argue for an acquired cause,
which suggests that the lymphatic vessels may become
blocked owing to inflammation or other obstruction
and so cause lymphatic ectasia (8). Others have
suggested that these lesions may be a true
neoplasm (9). The nomenclature of this disorder
is confusing and has evolved in recent years. Other
names have included "renal lymphangiomatosis"

(3,6), "renal lymphangioma" (5), "peripelvic
lymphangiectasia" (2), and "renal peripelvic multicystic
lymphangiectasia" (8). "Renal lymphangiectasia" is the
preferred name, given that the disorder is characterized
by ectatic perirenal, peripelvic, and intrarenal
lymphatic vessels (10,11).

Imaging findings of renal lymphangiectasia include
peripelvic cysts and perirenal fluid collections. The
finding of retroperitoneal fluid collections, presumably
dilated lymphatic vessels, is a more variable finding
but has been noted in multiple cases in the literature (2–4,6).

Renal lymphangiectasia has been found associated with
renal venous thrombosis and hypertension (4,6,12).
Ascites and large perinephric fluid collections have
been found and are exacerbated by pregnancy (6).

The natural history of this disorder is not completely
understood. In a neonatal case, partial regression
was reported (7).

The diagnosis of renal lymphangiectasia can be
confirmed with needle aspiration of chylous fluid
from the perinephric fluid collections (4). However,
the ultrasonographic and CT findings are characteristic
for this disease and allow the diagnosis to be made
confidently (2,6). Treatment is not usually necessary.

Complicated cases may be treated with nephrectomy,
percutaneous drainage, or marsupialization (4).

In the case presented here, the diagnosis of renal
lymphangiectasia was based on the characteristic
CT findings. The perirenal, peripelvic, and retroperitoneal
collections had attenuation measurements consistent
with fluid (0–10 HU) rather than with the soft-tissue
attenuation seen with lymphoma or other causes of
bilateral renal soft-tissue masses (Fig 2c). No invasive
procedures to confirm diagnosis were deemed
appropriate for this patient.

Our congratulations to the 26 individuals who
submitted the most likely diagnosis (renal lymphangiectasia)
for Diagnosis Please, Case 34. The names and locations
of the individuals, as submitted, are as follows:

Marc P. Banner, Philadelphia, Pa
Lawrence R. Bigongiari, MD, Hope, Ark
Marc G. de Baets, MD, Lugano, Switzerland
Kemal Demir, MD, Ataköy,stanbul, Turkey
Giovanna Demurtas, MD, Cagliari, Italy
Luis E. Fajre, MD, Tucuman, Argentina
Sandra K. Fernbach, Chicago, Ill
Milton R. Fuentealba, MD, General Roca, Rio Negro, Argentina
Celso Ichihara, Brazil
Kartik Jhaveri, MD, Mumbai, India
Douglas S. Katz, MD, Mineola, NY
Glenn Krinsky, MD, New York, NY
Prof. Dr. Luis Mendez Uriburu, Tucuman, Argentina
Sergio J. Moguillansky, MD, Cipolletti, Rio Negro, Argentina
Adalberto Montanhini Júnior, Brazil
Steven Perlmutter, MD, Mineola, NY
Timothy J. Phalen, MD, Cincinnati, Ohio
Dr. Arturo Ramos-Pablos, Cd. Obregón, Son., México
Luiz Antonio Rossi, São Paulo, Brazil
Anthony J. Scuderi, MD, Johnstown, Pa
Matt Shapiro, MD, Lowell, Mass
Paolo Siotto, MD, Cagliari, Italy
Douglas L. Teich, MD, Brookline, Mass
Christopher Vittore, MD, Rockford, Ill
Keith Wittenberg, MD, Rochester, Minn
Joe Yut, Olathe, Kan

Footnotes

Part 1 of this case appeared 4 months previously
and may contain larger images.

Article