Bilateral Wünderlich Syndrome Caused by Spontaneous Rupture of Renal Angiomyolipomas

Sotošek, Stanislav; Markić, Dean; Španjol, Josip; Krpina, Kristian; Knežević, Siniša; Maričić, Anton

doi:10.1155/2015/316956

Cited by 13 publications

(16 citation statements)

References 9 publications

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“…1,2,5 As neoplasias benignas são a causa mais comum, sendo o AML a mais frequente, com uma probabilidade de hemorragia até 50% em tumores com mais de 40 mm. 80% dos AML são esporádicos sendo os restantes 20% relacionados com esclerose tuberosa.…”

Section: Discussionunclassified

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Síndrome de Wunderlich, uma causa rara de dor abdominal

Oliveira¹,

Torres-Ramalho²,

Lopes³

2017

Gal. Clin.

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RESUMOA síndrome de Wunderlich é uma doença rara que se caracteriza por hemorragia espontânea nos espaços subcapsular e perirrenal. Apresentamos dois casos de síndrome de Wunderlich. Uma mulher de 38 anos com esclerose tuberosa que se apresentou com dor abdominal e hipotensão. A tomogra a computadorizada (TC) revelou um hematoma peri-renal. Uma mulher de 86 anos, anticoagulada com varfarina por tromboembolismo pulmonar, apresentou-se com dor abdominal nos quadrantes inferiores. A TC abdominal revelou uma massa renal com hemorragia no espaço peri--renal. Foram ambas submetidas a embolização super-seletiva da artéria renal, com boa evolução posterior. Palabras clave: síndrome de wunderlich, angiomiolipoma, femorragia renal espontánea ABSTRACTWunderlich syndrome is a rare disease characterized by spontaneous renal hemorrhage into the subcapsular and perirenal spaces. We report two cases of Wunderlich syndrome. A 38-year-old woman with tuberous sclerosis presented with abdominal pain and hypotension, perinephric hematoma was diagnosed by computerized tomography (CT) scan. A 86-year-old woman, treated with warfarin for pulmonary embolism, presented with abdominal pain in the lower quadrants. Abdominal CT scan revealed a renal mass with recent hemorrhage to perinephric space. Renal artery highly selective embolization was performed with good outcome in both cases.

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Section: Discussionunclassified

“…[1][2][3][4][5][6] A síndrome de Wunderlich é a complicação mais letal do AML, 1 relacionando-se com o seu tamanho. 5 Nestes doentes a radiologia teve um papel fundamental no diagnóstico e tratamento. A TC abdominal é o melhor exame para o diagnóstico desta doença.…”

Section: Discussionunclassified

Síndrome de Wunderlich, uma causa rara de dor abdominal

Oliveira¹,

Torres-Ramalho²,

Lopes³

2017

Gal. Clin.

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“…Acute bleeding of AML manifest as Lenk's triad, including acute flank pain, abdominal tenderness, and signs of internal bleeding such as hematuria; this urological emergency is also called Wünderlich syndrome. [ 7 , 8 ] Currently, the risk of tumor rupture is usually determined by tumor size and surgical approach is necessary for tumor size >4 to 8 cm. [ 9 , 10 ] However, these criteria have been challenged by a series of clinical cases and cohort studies.…”

Section: Introductionmentioning

confidence: 99%

An update on recent developments in rupture of renal angiomyolipoma

Wang

Peng

et al. 2018

Medicine

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Background:Renal angiomyolipoma (AML) is a common benign tumor of the kidney. The main complication of AML is retroperitoneal hemorrhage caused by AML rupture, which can be severe and life threatening. The risk of AML rupture used to be determined by tumor size. However, these criteria have been challenged by series of clinical studies and case reports, suggesting prediction AML rupture based on tumor size is not always reliable.Methods:The authors searched PubMed using “angiomyolipoma,” “AML,” and “rupture” and reviewed relevant studies. The authors investigated the risk factors of AML rupture using the retrieved literature. The authors also summarized current modalities to evaluate and manage AML.Results:It is established that risk of AML rupture is associated with lesion size. However, genetic abnormality, aneurysm formation, and pregnancy are also risk factors for tumor rupture. Thus, the prediction of AML rupture should be based on a more comprehensive risk assessment system. The management of renal AML and tumor rupture was also discussed in the present paper.Conclusion:The risk of AML rupture is associated with but not exclusive to lesion size. Any decision to intervene AML must be based on multiple factors including risk, symptoms, and auxiliary findings.

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“…The tendency for bleeding is multifactor and includes foci deficiency of the elastic tissue in abnormally rigid and thick blood vessels, hypervascularization and venous invasion. 3,5,7 The spontaneous rupture of an angiomiolipoma is an infrequent urological emergency, although presenting a potential risk to life. 8 The risk of bleeding is associated with a large tumor diameter.…”

Section: Introductionmentioning

confidence: 99%

“…Many authors have argued that the WS can be treated in a conservative way if the hemorrhage is self-limited and the patient responsive to resuscitation fluids. 5 Others recommend conservative treatment for RAML of less than 6cm suggesting that only 34% of the patients with tumors bigger than 4 cm require intervention. 9 In our case, the conservative treatment was our choice and is under serial and outpatient monitoring, with benign development for 1year.…”

Section: Introductionmentioning

confidence: 99%

Wunderlich Syndrome - Unusual Complication of Spontaneous Renal Haemorrhage

Nacif¹

2017

IJRRT

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A 50year old man went to the emergency service with an unspecific abdominal pain complaint. We performed a multidetector computed tomography (MDCT) of the abdomen, with and without iodinated intravenous contrast, that showed a hypodense nodule formation with regular and well delimited outlines, few heterogeneous foci and fat density (-35 HU), sizing about 1.8cm from the inferior pole of the left kidney (Figure 1). The patient was relieved of the pain and was discharged from hospital in the same day. After a month, the patient returned to the emergency service with a worse abdominal pain and an altered hemodynamic state. We performed another MDCT of the abdomen which detected the same nodule spontaneously dense and larger, with 2.2cm. We also detected an extend subcapsular renal and retroperitoneal hematoma, all aspects related to a spontaneous renal bleeding (Figure 2). We choose a more conservative treatment with hemodynamic support and stabilization in an intensive care unit. The patient improved in the hemodynamic parameters, after a month of hospitalization and then hospital discharge, and remains in outpatient treatment with no complaints for 1year. DiscussionThe display of fat in a kidney tumor by MDCT, as described in the case, is very suggestive of a RAML. 3 Other kidney tumors can also psent high levels of fat, such as the clear cell renal cell carcinoma, liposarcomas, atypical Wilms' tumor and teratomas. However, a cortical renal mass manly composed of fat (less than -20 HU) can be diagnosed as a RAML. 4-6Int J Radiol Radiat Ther. 2017;2(1):6-7.6

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Bilateral Wünderlich Syndrome Caused by Spontaneous Rupture of Renal Angiomyolipomas

Cited by 13 publications

References 9 publications

Síndrome de Wunderlich, uma causa rara de dor abdominal

Síndrome de Wunderlich, uma causa rara de dor abdominal

An update on recent developments in rupture of renal angiomyolipoma

Wunderlich Syndrome - Unusual Complication of Spontaneous Renal Haemorrhage

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