ISSN: 2572-3235

Abi-Ramia de Moraes et al. Int J Radiol Imaging Technol 2019, 5:059

DOI: 10.23937/2572-3235.1510059
International Journal of Volume 5 | Issue 2
Open Access

Radiology and Imaging Technology

Case Report

Wünderlich Syndrome: A Rare Syndrome Caused by a Polyar-

teritis Nodosa
Leandro Tristão Abi-Ramia de Moraes1*, Monica Arnaldi2 and Sérgio Furlan3
1
Radiology Resident, Hospital Heliópolis, Brazil
2
Neprology Resident, Santa Casa de Misericórdia de São Paulo, Brazil Check for
updates
3
Attendant at Department of Radiology, Hospital Heliópolis, Brazil
*Corresponding author: Leandro Tristão Abi-Ramia de Moraes, Radiology Resident, Complexo Hospitalar Heliópolis, Rua
Cônego Xavier, 276 - Sacomã, São Paulo - SP, 04231-030, Brazil

Abstract symptoms may start rapid or stealthy. It is classical-

ly associated with symptoms and signals named by
Wünderlich syndrome (WS) is a rare condition, in which
Lenk’s triad. The appropriate treatment depends the
spontaneous nontraumatic renal hemorrhage occurs into
the subcapsular and perirenal spaces and was described accurate diagnoses made by radiological imaging and
for the first time by Bonet in 1700. The etiology of the en- clinical information.
tity is very diverse, including benign or malign neoplasms,
vascular diseases, infectious and inflammatory kidney Case Report
diseases, and could be combined with some diseases.
Clinically the syndrome is characterized by the classic
A 37-years-old single woman who presented to
Lenk’s triad. We describe a case of WS in a 37-years-old emergency department with loss of strength in the
woman with loss of strength in the right superior limb for right superior limb for three months and associated
three months. After admission in the hospital her physical with loss of sensitivity on feet and legs. She was ad-
examination showed up pallor, tachycardia and evolved mitted to the hospital for investigation. In her physical
with diffuse abdominal pain. Fallowing all medical con-
ducts the patient do not resist.
examination was pallor, tachycardia, loss of strength
in the right arm and loss of the pain sensitivity in legs
Keywords and feet. Blood pressure: 140 × 100 mmHg. Evolved
Wünderlich syndrome, Retroperitoneal hemorrhage, Com- with diffuse abdominal pain. Lab results: hemoglobin =
puted tomography 6 (admission); hemoglobin = 3 (before being referred
to the computed tomography), hematocrit = 28%, leu-
Introduction kocytes = 21.610, neutrophil segmented = 59%, eosin-
ophils = 10%, platelets = 898.000, erythrocyte sedi-
Many causes of retroperitoneal hemorrhage have
mentation rate = 105, creatine phosphokinase = 3.396,
been described. The origin may be caused by any
C-reactive protein > 100, urea, creatinine and urinaly-
retroperitoneal structure (renal, vascular, pâncreas,
sis without abnormalities. After all medical conducts
adrenal, etc.). Spontaneous retroperitoneal hemor-
the patient do not resist. The computed tomography
rhage (SRH) of non-traumatic originated from renal
showed following images (Figure 1, Figure 2 and Figure
parenchymal rupture was first described by Bonet in
3).
1700. And in 1856 Carl Wünderlich made the clinical
description. The causes vary a lot: benign or malig- Discussion
nant tumors, vascular diseases, inflammatory pro-
Carl Reinhold August Wunderlich, a German phy-
cess, cystic ruptura, infectious. These illness can lead
sician, in 1856, made the clinical description of SRH of
to a retroperitoneal hemorrhage or bleeding into
non-traumatic originated from renal parenchymal rup-
urinary tract, which could be a life-threatening. The
ture called by ‘spontaneous renal capsule apoplexy’ [1,2].

Citation: Abi-Ramia de Moraes LT, Arnaldi M, Furlan S (2019) Wünderlich Syndrome: A Rare Syn-
drome Caused by a Polyarteritis Nodosa. Int J Radiol Imaging Technol 5:059. doi.org/10.23937/2572-
3235.1510059
Accepted: December 28, 2019: Published: December 30, 2019
Copyright: © 2019 Abi-Ramia de Moraes LT, et al. This is an open-access article distributed under the
terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and
reproduction in any medium, provided the original author and source are credited.

Abi-Ramia de Moraes et al. Int J Radiol Imaging Technol 2019, 5:059 • Page 1 of 4 •
DOI: 10.23937/2572-3235.1510059 ISSN: 2572-3235

Figure 3: Portal phase. Subcapsular hemorrhage displac-

Figure 1: Non-contrast phase. Inflammatory stranding in ing left kidney. Extensive inflammatory stranding is demon-
the left retroperitoneal space. Kidney showing loss contour. strated in the left peri- and para-renal spaces.

Table 1: Causes of spontaneous retroperitoneal hemorrhage.

2A Tumors Vascular
Angiomyolipoma Renal artery aneurysm
Renal cell carcinoma Renal artery ruptura
Pheochromocytoma Thrombosis
Myeloma Renal infarction
Adenoma Abdominal aortic aneurysm ruptura
Hemangioma Aretriovenous fistula/malformation
Metastasis Polyarteritis nodosa
Carcinoma Behçet syndrome
Retroperitoneal tumors
Pancreas

Table 2: Causes of spontaneous retroperitoneal hemorrhage

2B
Systemic Others

Thrombocytopenic purpura Renal cystic ruptura

Leucemia Renal calculi
Hemofilia Nephroesclerosis
Policitemia Pyelonephritis
Anticoagulant therapy Renal tuberculosis
Renal cortical abscesso
Nephritis

Figure 2a: Arterial phase. Large hematoma displacing the The most common causes of the WS are renal neo-
renal cortex from the capsule (blue arrow). Small aneurysms plasms. Angimiolipomas a mesenchymal neoplasm
(red arrow) of small sized arteries in the both kidneys.
composed by adipose tissue, smooth muscle and dys-
Figure 2b: Arterial phase. Striated nephrogram. Small an-
morphic blood vessels is the most common cause of the
eurysms (red arrow) of small sized arteries in the both kid-
neys. WS. Renal cell carcinoma is in second place and is in first
between the malignant tumors that causes WS [3-5,9-
13].
Spontaneous retroperitoneal hemorrhage may
have several origins as well as low back pain. The Vasculitis has different types and have their classifi-
causes could be tumoral, non-tumoral, vascular, in- cation according to the size and type of vessels involved
flammatory or systemic (some of these are exposes and the existence or the lack of associated fibrinoid ne-
on Table 1 and Table 2) and [1-11]. crosis and/or granulomas [14].

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DOI: 10.23937/2572-3235.1510059 ISSN: 2572-3235

Polyarteritis nodosa (PAN) is a rare form of primary blood, therefore identification of etiology may not be
systemic vasculitis with heterogeneous presentations. possible. To document the evolution of hematoma a
PAN is an autoimmune disease characterized by nec- follow-up with ultrasound is advantageous, because
rotizing arteritis of small- and medium-sized muscular it´s cheap and accessible. Color and spectral Doppler
arteries associated with aneurysmal nodules, condition may help in the detection of some vascular and non-
first described by Adolph Kussmaul and Rudolph Maier vascular pathologies responsible for WS. On US, acute
in 1866, and could affect multiple organs [15-19]. hematoma appears as an isoechoic to hyperechoic
collection; subacute and chronic hematomas appear as
Most cases of PAN, primary cases, are idiopathic.
hypoechoic to anechoic [3,4,12].
But several subgroups have already been defined by
their aetiologic association, secondary PAN. It is of- Computed tomography (CT) with multiplanar dis-
ten notice in hepatitis B and hepatitis C infections, play of datasets allows high-resolution imaging of the
malignancies such as hairy cell leukemia, familial renal parenchyma, vasculature, and the collecting sys-
mediterranean fever (FMF), connective tissue dis- tem, is the investigative modality of choice in diagnos-
ease related and deficiency of adenosine deaminase ing WS, its etiology, related complications and severi-
2 (DADA2) [20-26]. ty [1,9,11,30,31]. To demonstration perirenal hemor-
rhage CT is the method of choice (sensitivity of 100%)
PAN is not associate with antineutrophil cytoplasmic
but has sensitivity about 57% in identifying renal neo-
antibodies (ANCA) [20,21,27]. No specific serologic diag-
plasm when performed at the time of hemorrhage and
nostic tests have been developed.
may miss rare cases of segmental arterial medio lysis.
Renal involvement is common. Some authors plead [1,5,9,12,30] Sequential dynamic contrast image sets
approximately 60% of patients with PAN have renal in- should be obtained as corticomedullary, nephrographic
volvement. Rarely complicated by acute kidney injury and excretory phase images [4]. When CT give few infor-
(AKI) in patients with renal involvement, but has high mation’s it has been suggested that angiography should
morbidity. PAN is rare in patients with acute abdomen, be done as it can reveal vascular lesions not shown by
on the other hand, acute abdomen is frequent in cases CT. Renal angiography helps in diagnosing vasculitis, the
of PAN [4,15-19]. most common vascular cause of perinephric hemor-
rhage [5,11,31,32]. If angiography and CT fail because
The American College of Rheumatology proposed
of distortion of renal architecture caused by multiple
classification criteria for PAN in 1990 [28].
renal cysts and hypovascularity of some tumors [32], CT
The diagnosis is made by histology in most cases. could be repeat. Large-volume hematomas could mask
Imaging studies in vasculitis’s are important to deter- hidden neoplasms or the cause of perirenal bleeding
mine disease extension and activity. MRI, CT, and CT remains unclear, therefore follow-up CT is advocate
angiography are the noninvasive techniques to delin- [15,33]. Renal arteriography with selective embolization
eate vasculitis lesions. locates the bleeding site and allows bleeding control
The clinical picture vary hugely depending on the de- avoiding surgery.
gree of the bleeding and duration. Acute lumbo-abdom- If CT failed in initial examination or contrast-en-
inal pain, nausea, vomiting, hematuria, hemodynam- hanced CT is contraindicated, a magnetic resonance
ic instability, anemia and hypovolemic shock may be (MR) may be performed to reveal the cause of bleeding
present [1]. SRH is clinically characterized by the Lenk’s [3,4,30].
triad: Acute flank pain, hemodynamic compromise and
Treatment options depend of the aetiology, the
flank palpable mass. amount of bleeding and how fast the diagnostic are done.
WS is not common but is life-threatening entity. SRH Some cases could be follow closely. Minimally invasive
is a diagnostically challenging condition and need to be procedures as artery embolization is another option. Al-
management rapidly and radiological imaging plays an though, sometimes, nephrectomy could be required.
important role in establishing SRH and causes. WS could be a life threatening, and determining its
Confirmation of the diagnosis by identifying re- etiology of is often a challenge. Nevertheless, when
nal hemorrhage in the subcapsular and/or perirenal Lenk´s triad is present the patient should be managed
space, detecting the possible cause and identify any aggressively. Accurate diagnosis order a combination
underlying pathological process that may result in a of clinical and radiological imaging information’s. All
predisposition to hemorrhage for the bleeding is the imaging radiological methods have their role. CT, an-
aim of the imaging in a suspect case of WS [4,29]. giography and MR perform an important function in
the diagnosis and determine the cause of the syn-
Ultrasonography may be the first tool to detect
drome.
perinephric hematoma, but it has been consider to be
only modest useful in identifying renal hemorrhage Acknowledgement
and in differentiating the renal mass and clotted
Funding: None.

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DOI: 10.23937/2572-3235.1510059 ISSN: 2572-3235

Conflict of Interest: None. J Gastroenterol 19: 2830-2834.

All the authors made substantial contribution to the 17. Ambrosio MR, Rocca BJ, Ginori A, Onorati M, Fabbri A, et
al. (2012) Renal infarction due to polyarteritis nodosa in a
preparation of this manuscript and approved the final patient with angioimmunoblastic T-cell lymphoma: A case
version for submission. report and a brief review of the literature. Diagn Pathol 7:
50.
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