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Wunderlich's syndrome, an unusual cause of acute abdomen in tuberous sclerosis.

INTRODUCTION: Wunderlich's syndrome is one of the most feared complications of renal angiomyolipoma (AML) & should be managed aggressively (1). Wunderlich's syndrome refers to spontaneous, non-traumatic renal bleeding into the subcapsular and/or perirenal space (1,2). 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 proportion (2).

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 life (2).

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 & 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 1856 (2,4). 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 angiomyolipoma (1,5). 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 lesion (7). 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 rupture (2,8). 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 increases (2). 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 decade (1).

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 AML (2,5,8). However, it has been found to identify all cases of Wunderlich's syndrome due to AML8.A confident diagnosis of AML can be made by CT by demonstrating the fat content of these lesions (5,8,9). 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 lesion (10,11).

Ultrasonography has been found to be only moderately useful in identifying renal hemorrhage and in differentiating the renal mass and clotted blood (5,8,9,13). 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 AML (8), 9the benign nature of AML supports a partial nephrectomy or other nephron sparing surgery (9). 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 deferred (12,13).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 demonstrated (14).

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.

Wunderlich's syndrome- an unusual cause of acute abdomen in Tuberous sclerosis.

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.

(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: CT-pathology 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 of 7 cases. ActasUrolEsp 1995; 19(10):772-6.

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
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Article Details
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Title Annotation:CASE REPORT
Author:Parthasarathi, A.; Gautam, M.; Kishor, V.H.
Publication:Journal of Evolution of Medical and Dental Sciences
Article Type:Case study
Geographic Code:9INDI
Date:Dec 23, 2013
Words:1630
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