CASE REPORT
WUNDERLICH’S SYNDROME, AN UNUSUAL CAUSE OF ACUTE ABDOMEN IN
TUBEROUS SCLEROSIS
Parthasarathi A1, Gautam M2, Kishor V.H3
HOW TO CITE THIS ARTICLE:
Parthasarathi A, Gautam M, Kishor V.H. “Wunderlich’s Syndrome, an unusual cause of acute abdomen in
tuberous sclerosis”.Journal of Evolution of Medical and Dental Sciences 2013; Vol2, Issue 51, December 23;
Page: 10060-10065.
ABSTRACT: Wunderlich’s syndrome refers to spontaneous non-traumatic renal bleeding into the
subcapsular and/or perirenal space1, 2. We present a patient of Tuberous sclerosis complex with
bilateral renal angiomyolipomas and spontaneous retroperitoneal hemorrhage following rupture of
aneurismal component of angiomyolipoma from right kidney (Wunderlich’s syndrome) along with a
short review of this syndrome.
INTRODUCTION: Wunderlich’s syndrome is one of the most feared complications of renal
angiomyolipoma (AML) & should be managed aggressively1.Wunderlich’s syndrome refers to
spontaneous, non-traumatic renal bleeding into the subcapsular and/or perirenal space1, 2. Classic
angiomyolipoma is considered to be a benign mixed mesenchymal tumor that occurs predominantly
in the kidney.These tumors consist of a collection of thick-walled blood vessels, smooth muscle and
mature adipose tissue in varying proportion2.
The incidence of Wunderlich’s syndrome in the general population is between 0.07 and 0.3%
2, 3. Approximately 80% of renal AML’s occur sporadically and 20% are associated with tuberous
sclerosis. In the sporadic cases, these lesions are found usually larger, single and unilateral, with a
female preponderance (approximately 4:1) in the fourth to sixth decade of life2.
In symptomatic patients, the classic presentation includes flank or abdominal pain, a
palpable tender mass and gross hematuria (Lenk's triad)2. Other symptoms as nausea, vomiting,
fever, anemia, renal failure and hypotension are observed less frequently.Three types of
hemorrhagic etiology exist, including Wunderlich's syndrome (spontaneous retroperitoneal
hemorrhage of nontraumatic origin), bleeding or rupture after trauma and rupture during
pregnancy (secondary to a rapid hormonal-related growth). We present a case of Wunderlich's
syndrome in an obese woman with tuberous sclerosis.
CASE REPORT: A 25-year-old unmarried obese female patient, a known case of tuberous Sclerosis
was brought to our emergency department in a state of shock following sudden severe right flank
pain. On examination, the patient was drowsy, pale, cold and sweating, with a pulse of >100 and low
blood pressure. She had severe tenderness in the right flank with guarding. In addition to this,
adenoma sebaceum, hypomelanoticpatches("ash leaf spots") and shagreen patches [Fig.1] were
detected on her physical examination. The patient was rapidly resuscitated with IV fluids. Her blood
hemoglobin and hematocrit levels were below the normal limits. The coagulation profile was
normal. A urine test for pregnancy was negative. An initial ultrasound examination of the abdomen
revealed angiomyolipomas (AML) involving both kidneys with large perinephric hematoma on right
side and multiple small hyperechoic lesions of various sizes involving both lobes of the liver [Fig.2].A
provisional diagnosis of AML in both kidneys with right perinephric hematoma
Journal of Evolution of Medical and Dental Sciences/Volume 2/Issue 51/ December 23, 2013
Page 10060
CASE REPORT
&nonrenalhamartomas in liverwas made and a computed tomography (CT) of the abdomen was
deemed necessary for further evaluation.
The nonenhaned CT (NECT) [Fig.3] and contrast enhanced CT of the abdomen done soon
after revealed a hypodense mass of fat densitywithHounsefield units (HU) of -50 to -100 without
calcifications in right kidney and large rightperinephric hematoma [Fig.4] The perinephric
hematoma is seendisplacing the intestines anteroinferiorly and crossing the midline with
compression over inferior venacava (IVC). The left kidney also revealed a similar hypodense mass of
fat density with HU of -50 to –100 without calcifications predominantly in the mid and lower pole
without perinephric collection. Liver showed multiple nonenhancinghypodense lesions involving
both lobes of liver. The CT findings were thus consistent with perinephric hematoma secondary to
rupture of a renal AML. Multiple hypodenselesions were seen involving both lobes of liver consistent
with biliary hamartomas[Fig.5]. A diagnosis of Tuberous sclerosis with bilateral renal
angiomyolipomas (AML) with spontaneous rupture of right renal AML (Wunderlich’s syndrome)
was made. CT scan brain of the same patient, revealed multiple subependymal calcifications [Fig.6]
consistent with tuberous sclerosis.
DISCUSSION: Spontaneous nontraumatic bleeding confined to the subcapsular and/or perinephric
space in patients with no known underlying cause was first described as “spontaneous renal capsule
apoplexy” by Carl Reinhold August Wunderlich in 18562, 4. Radiology plays an important role in the
evaluation of patients with this clinical problem.
Presentation of this clinical picture may vary greatly depending on the degree and duration
of the bleeding. Commonly acute lumbo-abdominal pain, nausea, vomiting, hematuria, hemodynamic
instability, anemia and hypovolemic shock are present. Common causes of Wunderlich’s syndrome
including benign and malignant renal neoplasms, vascular disease (vasculitis, renal artery
arteriosclerosis and renal arteryaneurysm rupture), nephritis, infections, undiagnosed
hematological disorders, anatomical lesions like cysts &hydronephrosis.Wunderlich’s syndrome is
one of the most feared complications of renal angiomyolipoma and can be fatal if not treated
promptly and aggressively.
The commonest cause of spontaneous renal hemorrhage in most series is angiomyolipoma1,
5. It has an incidence of about 0.3-3%.Approximately 20% to 30% of AMLs are found in patients with
tuberous sclerosis syndrome (TS), an autosomal dominant disorder characterized by mental
retardation, epilepsy & adenoma sebaceum, a distinctive skin lesion7. Presence of hepatic
hamartomas,is a minor feature in patients with tuberous sclerosis. Wunderlich’s syndrome occurs in
up to 50% of patients with tumors larger than 40 mm because of the association with an increased
risk of intralesional aneurysmal formation and, therefore, a greater possibility of rupture2, 8. In fact
theabnormal elastin and poor vascular structures make angiomyolipomas more likely to form
aneurysms as they grow and as the blood flow entering those increases2. In contrast, of the 70% to
80% of patients with AML who do not have TS, a more pronounced female predominance is found &
most patients present later in life during the fifth or sixth decade1.
Computerized tomography (CT) is the method of choice for the demonstration of perirenal
hemorrhage (sensitivity of 100%) and, if performed during the time of hemorrhage, it has been
found to identify all cases of Wunderlich’s syndrome due to AML2, 5, 8. However, it has been found to
identify all cases of Wunderlich's syndrome due to AML8.A confident diagnosis of AML can be made
Journal of Evolution of Medical and Dental Sciences/Volume 2/Issue 51/ December 23, 2013
Page 10061
CASE REPORT
by CT by demonstrating the fat content of these lesions5, 8, 9. Other renal tumors like renal cell
carcinoma, liposarcoma, myolipoma, lipoma, oncocytoma, and Wilm's tumor may also show fat
content. However, it is felt that a renal cortical mass showing predominantly fat attenuation of less
than -20 HU can be diagnosed as an AML, particularly if there is no or little calcification in the
lesion10, 11.
Ultrasonography has been found to be only moderately useful in identifying renal
hemorrhage and in differentiating the renal mass and clotted blood5, 8, 9, 13. Biopsy is only rarely
useful in the diagnosis of renal AML9. Once a patient is diagnosed with spontaneous perinephric
hemorrhage due to AML, the treatment options are either surgery or therapeutic embolisation.
Embolisation is extremely useful in the setting of acute hemorrhage due to rupture of renal AML8,
9the benign nature of AML supports a partial nephrectomy or other nephron sparing surgery9.
Surgery also facilitates a pathological diagnosis. However, it is felt that if the patient can be stabilized
medically during the acute phase of spontaneous perinephric hemorrhage, a nephrectomy can be
deferred12, 13.In a review of the diagnosis and management of 7 cases of Wunderlich syndrome,
Cubillana et al also found conservative management to be the most acceptable option, unless a
malignant pathology could be demonstrated14.
In conclusion, we have presented a case of Wunderlich syndrome due to rupture of a renal
AML in a patient with tuberous sclerosis, which is the commonest cause reported in most series. CT
plays an important role in the management of patients presenting with this syndrome, not only by
demonstrating the perirenal hematoma, but has illustrated in our patient, by revealing the
underlying cause as well.
REFERENCES:
1. Mongha R, Bansal P, Dutta A, Das RK, Kundu AK. Wunderlich’s syndrome with hepatic
angiomyolipoma in tuberous sclerosis. Indian journal of cancer 2008;45:64-66.
2. Massimo Medda, Stefano CM Picozzi, Giorgio Bozzini, Luca Carmignani.Wunderlich’s syndrome
and hemorrhagic shock. Journal of Emergencies, Trauma, and Shock I 2:3 I Sep - Dec 2009.
3. Hao LW, Lin CM, Tsai SH. Spontaneous hemorrhagicangiomyolipoma present with massive
hematuria leading to urgent nephrectomy. Am J Emerg Med 2008;26:249.
4. Wunderlich CR. Handbuch der Pathologie und Therapie. 2nd ed. Stuttgart: Ebner and Seubert;
1856.
5. Zhang JQ Feilding JR, Zou KH. Etiology of spontenousperirenalhemorrhage; Ameta analysis. J
Urol 2002;167:1593-6.
6. Rakowski SK, Winterkorn EB, Paul E, Steele DJ, Halpern EF, Thiele EA. Renal manifestations of
tuberous sclerosis complex: Incidence, prognosis & predictive factors. Kidney Int
2006;70:1777-82.
7. Text book of Diagnostic Neuroradiology Anne G Osborn. 1994, ISBN 0-8016-7486-7, Indian
Reprint ISBN 978-81-8147-047-8: 93-98.
8. Albi G, Del Campo L, Tagarro D. Wunderlich’s syndrome: causes, diagnosis and radiological
management. ClinRadiol 2002;57:840-5.
9. Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma.
J.Urol 2002; 168:1315-1325.
Journal of Evolution of Medical and Dental Sciences/Volume 2/Issue 51/ December 23, 2013
Page 10062
CASE REPORT
10. Lloyd G. Logue, Robin E. Acker, and Anna E. Sienko. Best Cases from the AFIP:
Angiomyolipomas in Tuberous Sclerosis. RadioGraphics 200323: 241-246.
11. Merran S, Vieillefond A, Peyromaure M, Dupuy C. Renal angiomyolipoma with calcification: CTpathology correlation. Br J Radiol 2004; 77: 782-783.
12. Zagoria RJ, Dyer RB, Assimos DG, Scharling ES, Quinn SF. Spontaneous perinephrichemorrhage:
imaging and management. J. Urol 1991; 145: 468-471
13. Yip KH, Peh WC, Tam PC. Spontaneous rupture of renal tumours: the role of imaging in
diagnosis and management. Br J Radiol 1998; 71:146-154.
14. Cubillana LP, Rosino EH, Egea AL, Montiel MR, Villaplana GH, Albacete PM. Wunderlich
syndrome. Review of its diagnosis and therapy. Report of7 cases. ActasUrolEsp 1995;
19(10):772-6.
Wunderlich’s syndrome- an unusual cause of acute abdomen in Tuberous sclerosis.
FIG. 1. Pictures showing Adenoma sebaceum, hypomelanotic
patches and shagreen patches of tuberous sclerosis patient.
Fig. 2: Non contrast axial CT scan showing Angiomyolipoma in
bilateral kidneys with large perinephric hematoma on right
side displacing intestines anteroinferiorly and crossing midline.
Journal of Evolution of Medical and Dental Sciences/Volume 2/Issue 51/ December 23, 2013
Page 10063
CASE REPORT
Fig. 3: Contrast enhanced axial CT scan showing Angiomyolipoma in bilateral
kidneys with large perinephric hematoma & extravasation of contrast
on right side displacing intestines anteroinferiorly and crossing midline.
Fig. 4: Transabdominal ultrasound of liver showing hyperechoic
lesions in right lobe of liver consistent with biliary hamartomas.
Fig. 5: Contrast enhanced CT showing non enhancing hypodense
lesion in right lobe of liver consistent with hamartoma
Journal of Evolution of Medical and Dental Sciences/Volume 2/Issue 51/ December 23, 2013
Page 10064
CASE REPORT
Fig. 6: Non contrast axial CT scan brain of same
patient showing subependymal calcifications.
AUTHORS:
1. Parthasarathi A.
2. Gautam M.
3. Kishor V.H.
PARTICULARS OF CONTRIBUTORS:
1. Assistant
Professor,
Department
of
Radiodiagnosis,
Rajarajeshwari
Medical
College.
2. Assistant
Professor,
Department
of
Radiodiagnosis,
Rajarajeshwari
Medical
College.
3. Professor, Department of Radiodiagnosis,
Rajarajeshwari Medical College.
NAME ADRRESS EMAIL ID OF THE
CORRESPONDING AUTHOR:
Dr. Gautam M,
Assistant Professor,
Department of Radiology,
Rajarajeshwari Medical College,
Mysore Road, Bangalore.
Email –drgautampes@gmail.com
Date of Submission: 11/11/2013.
Date of Peer Review: 13/11/2013.
Date of Acceptance: 05/12/2013.
Date of Publishing: 23/12/2013
Journal of Evolution of Medical and Dental Sciences/Volume 2/Issue 51/ December 23, 2013
Page 10065