Document

General Urology
Arch. Esp. Urol. 2013; 66 (10): 925-929
SPONTANEOUS RETROPERITONEAL HAEMORRHAGE OF RENAL ORIGIN
(WUNDERLICH SYDROME): ANALYSIS OF 8 CASES
Roberto Molina Escudero and Octavio A. Castillo1.
Urology Department. Hospital Universitario de Fuenlabrada. Madrid. Spain.
1
Urology Department. Clínica INDISA y Facultad de Medicina. Universidad Andrés Bello. Santiago de Chile.
Summary.- OBJECTIVES: To analyze the characteristics, etiology and treatment of a series of patients
with spontaneous retroperitoneal hemorrhage from renal
causes.
METHODS: We retrospectively reviewed patients
diagnosed of spontaneous retroperitoneal hemorrhage
between 2006 and 2011. All patients consulted for
back pain and the diagnosis was made by computed
tomography (CT) and / or magnetic resonance (MR). All
patients were treated surgically.
RESULTS: The series includes 8 patients. Six cases had
renal mass and associated hematoma and 2 presented
only perirenal hematoma. Six patients underwent total
nephrectomy, one underwent partial nephrectomy, and
one just drainage of the hematoma.
The pathological study showed 4 cases of renal
angiomyolipoma (one associated with multiple small
renal carcinomas), 2 cases of renal carcinoma and 1
case of hemorrhagic renal infarction.
CONCLUSION: Renal masses are the main cause
of Wunderlich syndrome and CT is the diagnostic
procedure of choice. Surgical treatment is preferred
in patients with renal mass diagnosed and cases of
hemodynamic compromise.
Keywords: Wunderlich syndrome. Retroperitoneal
bleeding. Renal tumor. Angiomyolipoma. Renal
cancer.
@
Resumen.- OBJETIVO: Analizar las características,
etiología y tratamiento en una serie de pacientes con
hemorragia retroperitoneal espontánea de causa renal.
CORRESPONDENCE
Octavio A. Castillo
Departamento de Urología
Clínica INDISA
Av. Santa María 1810
Providencia 7520440
Santiago Chile. (Chile)
octavio.castillo@indisa.cl
Accepted for publication: June 28th, 2013
MÉTODOS: Revisamos retrospectivamente los pacientes diagnosticados de hemorragia retroperitoneal espontanea entre 2006 y 2011. Todos los pacientes
consultaron por dolor lumbar y el diagnostico se realizó
mediante Tomografía computada (TC) y/ o Resonancia
magnética (RM). Todos los pacientes fueron tratados quirúrgicamente.
RESULTADOS: La serie está compuesta por 8 pacientes.
Seis casos presentaron masa renal y hematoma asociado y en 2 solo se apreció un hematoma perirenal. Seis
pacientes fueron tratados mediante nefrectomía total,
uno mediante nefrectomía parcial y otro solo con drenaje del hematoma. El estudio anátomo-patológico demostró 4 casos de Angiomiolipoma renal (uno asociado
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R. Molina Escudero and O. A. Castillo.
a múltiples carcinomas renales pequeños), 2 casos de
Carcinoma renal y 1 caso de infarto renal hemorrágico.
CONCLUSIÓN: Las masas renales constituyen la principal causa de síndrome de Wünderlich y la TC es la
técnica diagnostica de elección. El tratamiento quirúrgico es de elección en pacientes con masa renal diagnosticada y en casos de compromiso hemodinámico.
Palabras clave: Síndrome de Wünderlich.
Hemorragia retroperitoneal. Tumor renal. Angiomiolipoma. Cáncer renal.
INTRODUCTION
Wünderlich syndrome is defined as a nontraumatic spontaneous retroperitoneal hemorrhage. It
is an uncommon situation and its etiology is varied, it
can be caused by renal causes (vascular, inflammatory
tumors) or extrarenal (adrenal disease, aortic, etc...)
Its characteristic presentation is Lenk´s triad, clinical
sudden onset including presence of a palpable mass,
acute back pain and hemodynamic compromise (1).
The aim of this paper is to analyze the demographic,
medical causes and management of a series of
patients diagnosed of Wünderlich syndrome.
MATERIAL AND METHODS
initially evaluated by abdominal ultrasonography, the
diagnosis was confirmed in all cases by CT. MRI was
performed only in the last 3 cases in the series, since
this technology was incorporated only from the year
2008. We found a renal mass and renal or perirenal
hematoma in six cases (Figure 1), while in 2 cases
showed only the presence of unexplained perirenal
hematoma.
Surgery was performed as soon patients
achieved hemodynamic stabilization, due to the
likelihood of re-bleeding. Treatment in 6 cases was
the total nephrectomy, perdorming three of them by
transperitoneal laparoscopic way, a patient was
treated by laparoscopic partial nephrectomy for a
3.5 cm lesion and one patient underwent exclusively
drainage of the hematoma by retroperitoneal way.
There were no perioperative complications. Pathology
was obtained in all cases, 3 of them were renal
angiomyolipoma, two were clear cell renal carcinoma
limited to the kidney, one hemorrhagic renal infarction
associated with vasculitis and another case was renal
angiomyolipoma with 20 foci of carcinoma cells
kidney between 0.5 and 1 cm. (case 3). In the patient
in which only the hematoma drainage was performed,
subsequent radiological monitoring revealed a renal
cell carcinoma of 2 cm, so laparoscopic partial
nephrectomy was performed 3 months later (case 4).
This patient had late tumor recurrence in ports, dying
of peritoneal carcinomatosis (2).
With a minimum follow-up of six years, none
of the remaining patients had recurrence of the treated
disease (Table I).
We performed a retrospective review of
a series of patients diagnosed of spontaneous
retroperitoneal hemorrhage in our department between
2006 and 2011. In all cases the patients consulted by
back pain of sudden onset and diagnosis was made
by computed tomography (CT). After establishing the
responsible cause, all patients underwent definitive
surgical treatment.
We analyze the demographics, etiology,
treatment performed and pathology of the patients in
the series.
RESULTS
We included 8 patients, 5 women and 3
men, with a mean age of 54 years. The left side was
affected in 6 cases and the right in two. No patient
required urgent treatment, we found only one case of
hemodynamic compromise that was initially controlled
with medical management. Three patients were
FIgure 1. Renal mass and renal hematoma.
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SPONTANEOUS RETROPERITONEAL HAEMORRHAGE OF RENAL ORIGIN (WUNDERLICH SYDROME): ANALYSIS OF 8 CASES
Bonet first described in 1700 the spontaneous
retroperitoneal hemorrhage from renal parenchymal
rupture. In 1856, Wunderlich performed the first
clinical description, defining this situation as a
spontaneous renal capsule stroke. Coenen in
1910 used the term Wunderlich syndrome to the
retroperitoneal hemorrhage without prior trauma,
urological or vascular manipulation (3).
angiomyolipomas, followed by vascular causes
(18%) and infectious (10%) (4). The most recent metaanalysis conducted by Zhang in 2002 reviewed 165
patients with a diagnosis of spontaneous perirenal
hemorrhage between 1985 and 1999. 70% of cases
were benign, including vascular diseases, infections
and benign tumors, the latter corresponding to 61%
of the causes (5). These data are consistent with those
observed in our series, where the cause in six of the
patients was a renal mass.
Its etiology is varied, being causes
kidney the most common: angiomyolipoma, renal
carcinoma, renal artery aneurysms, hydronephrosis
or caliceal rupture. As extra-renal causes include
pheochromocytomas,
adrenal
myelolipoma,
retroperitoneal tumors and systemic diseases such
as coagulopathies and vasculitis. A review of the
English literature in 1975 by Mcdugal identified as
first cause renal tumors (57%) being the most common
Regarding angiomyolipomas, these are
sporadic in 80% of cases, while 20% are associated
with tuberous sclerosis. Sporadic angiomyolipomas
are usually unilateral, solitary and occur most frequently
in the right side and middle aged women. The
Tuberous Sclerosis related occur at earlier ages tend
to be larger and bilateral, usually requiring surgical
management. Thus, approximately 64% to 77% of
angiomyolipomas under 4 cm are asymptomatic,
DISCUSSION
Tabla I. Clinico-pathological characteristics of the series.
Case
Age
Sex
Side
Ct
Treatment
Pa
1
47
Female
Left
Tumor + hematoma
Open Nefrectomy
AML 6 cm
2
57
Female
Left
Hematoma
Open Nefrectomy
hemorrhagic
renal infarction
3
60
Male
Left
Tumor + hematoma
Laparoscopic Nefrectomy
AML+ RCC
Hematoma drainage +
4
74
Female
Right
Perirenal colection
Laparoscopic Nefrectomy
RCC
5
53
Female
Left
Tumor + hematoma
Partial Nephrectomy
AML3.5 cm
6
63
Male
Right
Tumor + hematoma
Open Nefrectomy
AML4.5 cm
7
37
Female
Left
Tumor + hematoma
Laparoscopic Nefrectomy
RCC 9 cm
8
45
Male
Left
Tumor + hematoma
Laparoscopic Nefrectomy
RCC
AML: Angiomyolipoma RCC: Renal cell carcinoma, PA: Pathological anatomy
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R. Molina Escudero and O. A. Castillo.
while between 82% and 90% of lesions larger than 4
cm produced symptoms (6). In our series none of the
patients had tuberous sclerosis and angiomyolipomas
were lonely and over four centimeters in diameter.
Wunderlich syndrome can occur abruptly
or insidiously, and as discomfort or forming isolated
Lenk’s triad, characterized by pain, palpable mass
and hemodynamic compromise. Clinical suspicion
is essential for diagnosis, however this is done by
imaging. Computed tomography is the examination
of choice and gives us information about the etiology,
and severity of the associated disease, demonstrating
the presence of perirenal hematoma with a sensitivity
of 100% (7).
In the case of tumor etiology, computed
tomography can characterize and differentiate
angiomyolipoma and renal cell carcinoma allowing
treatment planning strategy. However the initial CT
may miss the presence of renal cell carcinoma in up
to 60% of the cases (6). In cases that there is not
responsible mass for the bleeding, arteriography may
demonstrate vascular lesions that are not appear on
CT, and facilitate the embolization (8). In our series,
all patients were diagnosed by CT without performing
angiography in any case. Abdominal ultrasonography
did not allow define definitive etiology it the three
cases where was conducted as initial test.
Initial management of this disease depends
on the patient’s hemodynamic status. Initially the
measures should aim to maintain hemodynamic
stability. In cases where the patient has hemodynamic
instability refractory to medical treatment, emergency
surgery is necessary or selective embolization of
active bleeding point if is possible to locate it.
Definitive treatment will depend on the
responsible cause, possible therapeutic options are
expectant management, management by endovascular
embolization of the bleeding vessel, nephron sparing
surgery and radical nephrectomy, no guidelines exist
to recommend one treatment over another (9-11).
Of note in our series, is the highest number of total
nephrectomies performed, considering that in no case
was severe hemodynamic compromise that forced
emergency surgery. The only reasonable explanation
is the difficulty of interpretation of imaging in the event
of a renal carcinoma could go unnoticed.
In patients in whom embolization is chosen as
treatment and in which the first CT revealed no renal
mass as the cause of the bleeding, after stabilization
of the patient is necessary to reassess the imaging or
perform a new CT scan to avoid overlooking tumoral
cause requiring surgical management.
While this a retrospective series of clinical
cases, and since most of the published data refers
only to isolated clinical cases (no more than 20 cases
reported in the Spanish literature as Rey Rey J, et al.
11), we can draw some conclusions that will help the
proper management of this disease:
1. Only occasionally, renal spontaneous retroperitoneal hemorrhage requires immediate or urgent surgical management, as shown in this series.
2. The current imaging studies gives more precise
diagnosis, avoiding unnecessary total nephrectomy.
3. The main cause of renal spontaneous retroperitoneal
hemorrhage is Angiomyolipoma; therefore nephronsparing surgery can be safely performed in most
cases.
CONCLUSION
Spontaneous retroperitoneal bleeding of renal
origin is uncommon and usually associated with benign
tumor lesions, such as renal Angiomyolipoma.
Currently, imaging studies allow accurate
diagnosis, and once the patient is stabilized,
conservative renal surgery can be done in most
cases. In case of hemodynamic instability, selective
embolization is the initial treatment of choice.
A proper understanding of this condition will
avoid unnecessary total nephrectomy.
REFERENCES AND RECOMMENDED READINGS
(*of special interest, **of outstanding interest)
1. Vega Molina F, Albión Tristán A, Blasco de Villalonga M “Hemorragia retroperitoneal espontánea”. Arch. Esp. Urol. 1994, 47: 129-133
*2. Castillo OA, Vitagliano G, Díaz M, SánchezSalas R. Port-Site Metastasis after Laparoscopic
Partial Nephrectomy: Case Report and Literature
Review. J Endourol 2007; 21: 404-407
**3. García Rodríguez J, Fernández Gómez JM, Rodríguez Martínez JJ, Rodríguez Faba O, Regadera
Sejas J, Escaf Barmadah S. “Hemorragia retroperitoneal espontánea por Feocromocitoma”. Arch.
Esp. Urol. 2002; 55: 955-958.
4. McDougal W, Kursh E, Persky L “Spontaneous
rupture of kidney with perirenal hematoma” J
Urol. 1975; 114: 181_184.
*5. Zhang JQ, Fielding JR, Zou KH. “Etiology of
spontaneous perirenal hemorrhage: a meta-analysis. J. Urol. 2002; 167: 1593-1596.
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6. Beaumont-Caminos C, Belzunegui-Otano T, Fenandez-Esain B, Martinez-Jarauta J, Garcia -Sanchotena JL. “Wunderlich syndrome: an unusual
cause of flank pain”. Am J of Emerg Med. 2011;
29:474.
7. Parameswarma B, Khalid M, Malik N. “Wunderlich syndrome following rupture of a renal angiomyolipoma. Ann Saudi Med. 2006; 26: 310-312.
8. Dominguez, C.; Boronat, F.; Broseta, E: Hematoma perirrenal por rotura espontanea calicial superior. Arch. Esp. Urol. 1990, 43: 59-62
*9. Ikari O, D’Ancona C, Prando A, Rodrigues N Dilema en el tratamiento del angiomiolipoma.Arch.
Esp.Urol. 2000; 53: 425-429.
10. Peña J, Serrano M, Cosentino M, Rosales A, Algaba F, Palou J, Villavicencio H. Laparoscopic
management of spontaneous retroperitoneal hemorrhage. Urol Int 2011;87:114-116.
11. Rey Rey J, Lopez Garcia S, Dominguez Freire
F, Alonso Rodrigo A, Rodriguez Iglesias B, Ojea
Calvo A. Sindrome de Wünderlich: importancia
del diagnostic por imágen. Actas Urol Esp. 2009;
33(8): 917-919