Journal of Emergencies, Trauma, and Shock
Home About us Editors Ahead of Print Current Issue Archives Search Instructions Subscribe Advertise Login 
Users online:381   Print this pageEmail this pageSmall font sizeDefault font sizeIncrease font size   


 
SHOCK SCENARIOS Table of Contents   
Year : 2009  |  Volume : 2  |  Issue : 3  |  Page : 203-205
Wunderlich's syndrome and hemorrhagic shock


1 Cardiovascular Interventional Radiology Service, University of Milan, San Donato Milanese, Milan, Italy
2 Urology Department, IRCCS Policlinico San Donato, University of Milan, San Donato Milanese, Milan, Italy

Click here for correspondence address and email

Date of Submission19-Apr-2009
Date of Acceptance10-May-2009
Date of Web Publication31-Aug-2009
 

   Abstract 

We report a case of Wunderlich's syndrome in an obese woman associated with massive retroperitoneal hemorrhage. Stable hemodynamic patient condition was obtained by selective arterial embolization. Since the first embolization of a renal angiomyolipoma in 1976 by Moorhead et al., highly selective renal arterial embolization of angiomyolipoma with rupture has become a procedure that offers greater efficacy, particularly in life-threatening cases.

Keywords: Angiomyolipoma, embolization, kidney, massive hemorrhage, renal tumor

How to cite this article:
Medda M, Picozzi SC, Bozzini G, Carmignani L. Wunderlich's syndrome and hemorrhagic shock. J Emerg Trauma Shock 2009;2:203-5

How to cite this URL:
Medda M, Picozzi SC, Bozzini G, Carmignani L. Wunderlich's syndrome and hemorrhagic shock. J Emerg Trauma Shock [serial online] 2009 [cited 2020 Jun 1];2:203-5. Available from: http://www.onlinejets.org/text.asp?2009/2/3/203/55346



   Introduction Top


Classic angiomyolipoma is considered to be benign mixed mesenchymal tumors 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 proportions. A second type of angiomyolipoma is described containing a large fourth component, the perivascular epithelioid cells, making it more aggressive than the classical form. The incidence in the general population is between 0.07 and 0.3%. [1] Approximately 80% of renal angiomyolipoma 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.

Several studies have demonstrated that the frequency of symptoms and the risk of bleeding increase with the size of the lesion. Approximately 64-77% of tumors <40 mm in diameter are asymptomatic, although 82-90% of angiomyolipoma >40mm produce symptoms. [2]

In symptomatic patients, the classic presentation includes flank or abdominal pain, a palpable tender mass and gross hematuria (Lenk's triad). Other symptoms as nausea, vomiting, fever, anaemia, 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 associated with hemorrhagic shock.


   Case Report Top


An obese 50-year-old Caucasian woman (body mass index 35.2) was admitted to our Emergency Department with right-sided abdominal pain of sudden onset at 4.50 pm. There was no significant past medical or family history. General examination was normal except for pallor. Her hemodynamic parameters were stable; her blood pressure was 135/80 mmHg, pulse was 96/min and O 2 saturation was 98% in room air. There was no fever. Cholecystitis was suspected, because Murphy's sign was positive, and analgesic treatment was administered at 5.15 pm. Initial blood test revealed a hemoglobin level of 13.5 g/dl, a white blood cell count of 11300/ul, a platelet count of 256.000/ul, liver and renal function in the normal range, C-reactive protein of 9 mg/dl, normal coagulation tests and negative urinalysis. Abdomen ultrasonography was requested to the Radiological Department.

At 6.50 pm, the patient's symptoms worsened and she developed nausea, vomiting, hypotension (80/50 mmHg), tachycardia (125 bpm), confusion and diaphoresis. Her hemoglobin dropped to 8.5 g/dl. Urgent ultrasonography and computed tomography showed a 22 ×15 cm right renal fat mass associated with massive recent hemorrhage (findings suspicious of a bleeding renal angiomyolipoma) [Figure 1].

The patient's condition precipitated. Supportive therapy with ventilation, monitoring and establishment of a central venous access in addition to two large-bore catheters in peripheral lines was initiated. Fluid treatment included repeated aliquots of 250ml of Ringer's solution and 6% hydroxyethyl starch 130/0.4 in 0.9% sodium chloride injection solutions, administered with continuous monitoring, with re-establishment of a systolic blood pressure of 70 mmHg and appearance of a radial pulse.

The decision-making process performed by the urologist resulted in alerting the Emergency Cardiovascular Interventional Radiology Service. At 7.15 pm, after resuscitation, selective embolization of the upper pole branch of the renal artery (the lesion-supplying artery) was performed with 2/20 mm and 3/20 mm coils [Figure 2]. After the procedure, the patient's hemodynamic condition was stable and she was transferred to the intensive care unit where she received a total of 8 units of packed red blood cells and 2 units of fresh frozen plasma. Following embolization, the patient's general condition improved and an elective exploratory laparotomy was performed 3 days after embolization. At surgery, a huge retroperitoneal hematoma extending to the pelvis was found. The size of the tumor was of importance and a right nephrectomy was performed [Figure 3]. The postoperative recovery was uneventful. Histology confirmed a renal angiomyolipoma [Figure 4].


   Discussion Top


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. [3] 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. Several causes are involved, including benign and malignant renal neoplasms, vascular disease (vasculitis, renal artery arteriosclerosis and renal artery aneurism rupture), nephritis, infections, undiagnosed hematological disorders and anatomical lesions. Wunderlich's syndrome is one of the most feared complications of renal angiomyolipoma and can be fatal if not treated promptly and aggressively. It occurs in up to 50% of patients with tumors larger than 40 mm because of the association with an increased risk of intralesional aneurismal formation and, therefore, a greater possibility of rupture. [4] In fact, having abnormal elastin-poor vascular structures, angiomyolipomas are likely to form aneurisms as they grow and as the blood flow entering them increases.

Computerized tomography 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 angiomyolipoma. [4],[5]

In 1986, Oesterling et al . proposed a treatment protocol based on size and symptoms: asymptomatic tumors < 40 mm should be observed regularly with computerized tomography or ultrasound whereas those > 40 mm should be studied by angiography and considered for arterial embolization or surgery. [2]

Since the first embolization of a renal angiomyolipoma in 1976 by Moorhead et al. , highly selective renal arterial embolization of angiomyolipoma with rupture has become a procedure that offers greater efficacy in life-threatening cases. [6] The major advantages of embolization in these cases include its minimal invasiveness, the capacity of stopping acute hemorrhaging in emergencies, relieving patient symptoms, preventing further hemorrhaging, feasibility of retreatment and preservation of renal function in preventing a nephron-sparing surgery.


   Conclusion Top


A case of Wunderlich's syndrome associated with massive retroperitoneal hemorrhage is reported. Wunderlich's syndrome is one of the most feared complications of renal angiomyolipoma and can be fatal if not treated promptly and aggressively. The appropriate treatment for this condition depends on the accuracy of diagnosis and the determination of its cause. Highly selective renal arterial embolization of angiomyolipoma with rupture permits to obtain stable hemodynamic patient condition in emergency. This procedure has become a procedure that offers greater efficacy, particularly in life-threatening cases.

 
   References Top

1.Hao LW, Lin CM, Tsai SH. Spontaneous hemorrhagic angiomyolipoma present with massive hematuria leading to urgent nephrectomy. Am J Emerg Med 2008;26:249.   Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Oesterling JE, Fishman EK, Goldman SM, Marshall FF. The management of renal angiomyolipoma. J Urol 1986;135:1121-4.  Back to cited text no. 2  [PUBMED]    
3.Wunderlich CR. Handbuch der Pathologie und Therapie. 2 nd ed. Stuttgart: Ebner and Seubert; 1856.  Back to cited text no. 3      
4.Albi G, Del Campo L, Tagarro D. Wunderlich's syndrome: causes, diagnosis and radiological management. Clin Radiol 2002;57:840-5.  Back to cited text no. 4      
5.Zhang JQ, Feilding JR, Zou KH. Etiology of spontaneous perirenal hemorrhage: a meta-analysis. J Urol 2002;167:1593-6.   Back to cited text no. 5      
6.Moorhead JD, Fritzsche P, Hadley HL. Management of hemorrhage secondary to renal angiomyolipoma with selective arterial embolization. J Urol 1977;117:122-3.  Back to cited text no. 6      

Top
Correspondence Address:
Stefano CM Picozzi
Urology Department, IRCCS Policlinico San Donato, University of Milan, San Donato Milanese, Milan
Italy
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2700.55346

Rights and Permissions


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

This article has been cited by
1 A Cough Deteriorating Gross Hematuria: A Clinical Sign of a Forthcoming Life-Threatening Rupture of an Intraparenchymal Aneurysm of Renal Artery (Wunderlichćs Syndrome)
Ioannis Anastasiou,Ioannis Katafigiotis,Christos Pournaras,Evangelos Fragkiadis,Ioannis Leotsakos,Dionysios Mitropoulos,Constantinos A. Constantinides
Case Reports in Vascular Medicine. 2013; 2013: 1
[Pubmed] | [DOI]
2 Wunderlichćs Syndrome in a Tuberous Sclerosis Patient
Surya Prakash Vaddi,Vedamurthy Pogula Reddy,Rahul Devraj
Indian Journal of Surgery. 2011; 73(3): 227
[Pubmed] | [DOI]
3 Wünderlich syndrome: an unusual cause of flank pain
Carlos Beaumont-Caminos,Clint Jean-Louis,Tomás Belzunegui-Otano,Bernabé Fenández-Esain,Javier Martínez-Jarauta,José L. García-Sanchotena
The American Journal of Emergency Medicine. 2011; 29(4): 474.e1
[Pubmed] | [DOI]



 

Top
 
  Search
 
  
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
    Introduction
    Case Report
    Discussion
    Conclusion
    References
    Article Figures

 Article Access Statistics
    Viewed4232    
    Printed166    
    Emailed1    
    PDF Downloaded107    
    Comments [Add]    
    Cited by others 3    

Recommend this journal