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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2014  |  Volume : 25  |  Issue : 2  |  Page : 385-389
Giant renal artery pseudoaneurysm caused by rupture of renal angiomyolipoma following pregnancy: Endovascular treatment and review of the literature

Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey

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Date of Web Publication11-Mar-2014


Renal angiomyolipoma is a hamartomatous, benign tumor composed of blood vessels, fatty tissue and smooth muscle cells, and is often detected incidentally. It can also be associated with the tuberous-sclerosis complex (TSC). Pregnancy and use of oral contraceptives are known to be associated with an increased risk of tumoral rupture and bleeding. Herein, we report a unique case of renal angiomyolipoma associated with TSC who presented with hypovolemic shock as a result of spontaneous rupture of a giant renal pseudoaneurysm, immediately after pregnancy. Emergency endovascular treatment was successful with sparing of most of the affected kidney as demonstrated by follow-up computed tomography imaging.

How to cite this article:
Idilman IS, Vesnic S, Cil B, Peynircioglu B. Giant renal artery pseudoaneurysm caused by rupture of renal angiomyolipoma following pregnancy: Endovascular treatment and review of the literature. Saudi J Kidney Dis Transpl 2014;25:385-9

How to cite this URL:
Idilman IS, Vesnic S, Cil B, Peynircioglu B. Giant renal artery pseudoaneurysm caused by rupture of renal angiomyolipoma following pregnancy: Endovascular treatment and review of the literature. Saudi J Kidney Dis Transpl [serial online] 2014 [cited 2020 Sep 26];25:385-9. Available from: http://www.sjkdt.org/text.asp?2014/25/2/385/128570

   Introduction Top

Renal angiomyolipoma (AML) is a benign neo-plasm composed of thick blood vessels, fatty tissue and smooth muscle cells. [1],[2] Most cases of renal AML present as an isolated lesion while approximately 20% of patients have an association with the tuberous-sclerosis complex (TSC) and other phacomatoses such as neurofibromatosis. [1],[3],[4] Renal AMLs asso­ciated with TSC tend to be multifocal, bilateral and larger. These lesions are more prone to rupture and tend to be symptomatic rather than the isolated lesions. [4],[5] Renal AMLs are hor­mone-sensitive tumors. [4],[5],[6],[7] Moreover, pregnancy induces tumor growth and rupture. [8] In this report, we report a case of renal AML asso­ciated with TSC who was admitted to the hospital for spontaneous rupture of a giant pseudoaneurysm, which had occurred as a complication of AML, following pregnancy. The patient was successfully managed with emergency endovascular embolization.

   Case Report Top

A 25-year-old puerperal female patient was admitted to an outside hospital with a sudden onset of abdominal pain, dizziness and fatigue. She had delivered a baby two weeks earlier. She was hypotensive (around 70/40 mm Hg) and had tachycardia with a heart rate over 120/min. A diagnosis of hypovolemic shock due to blood loss was made on the basis of a drop in hemoglobin level to 8 g/dL from 14 g/dL two to three weeks earlier. Abdominal ultra­sonography (US) revealed bilateral renal le­sions suspicious of AMLs and a giant cystic mass originating from the lower pole of the left kidney. Subsequent computed tomography (CT) of the abdomen revealed a giant pseudoaneurysm originating from the lower pole of the left kidney with a diameter of 12 cm × 15 cm [Figure 1]. The patient was immediately referred to our hospital for possible endovascular treatment.
Figure 1: Abdominal computed tomography images obtained at an outside hospital showing giant pseudo-aneurysm originating from the left kidney. Multiple hypodense lesions are also seen suggestive of renal angiomyolipomas.

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In our hospital, the patient was taken to the angiography unit and abdominal aorta and selective renal artery injections were obtained. Abdominal aortography demonstrated contrast extravasation from the left segmental renal artery [Figure 2]. Subsequent selective left renal artery injections confirmed the diagnosis of giant pseudoaneurysm arising from the segmental branch of the renal artery [Figure 3]. Using a micro-catheter, this segmental artery was embolized with 33% diluted n-butyl cyanoacrylate (nBCA), avoiding filling the sacitself [Figure 4]. Folio-wing this, control angiography documented no further filling of the pseudoaneurysm sac and approximately 90% of the left renal parenchyma was pre­served [Figure 5]. Six weeks after the proce­dure, follow-up abdominal CT showed throm­bosed pseudoaneurysm with no evidence of active bleeding. Also, the diameter of the sac had decreased from 15 cm to 10 cm [Figure 6]. The patient is on regular follow-up and is doing very well.
Figure 2: Abdominal aortogram showing filling of the giant pseudoaneurysm located in the left lower quadrant of the abdomen. Also, note the intestinal gas displacement to the right lower quadrant.

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Figure 3: Selective injection into the left renal artery showing the segmental renal artery branch directly opening into the giant pseudoaneurysm (arrows).

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Figure 4: Scout film after nBCA embolization demonstrating the glue cast filling the segmental artery (arrows).

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Figure 5: Selective left renal artery injection after endovascular treatment demonstrating the mostly preserved left kidney with other smaller AMLs (arrows).

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Figure 6: Six-week follow-up computed tomography image showing significant decrease in diameter of the totally thrombosed pseudoaneurysm in the left kidney. Also, note some hyperdense glue foci (arrows) within the aneurysm sac representing distal emboli during proximal parent artery embolization.

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   Discussion Top

Renal AMLs are generally benign and clini­cally silent lesions and diagnosed incidentally on US or CT examination performed for other clinical reasons. [9] However, they also tend to grow and be complicated with aneurysm for­mation and rupture, [10] which may cause life-threatening hemorrhage. In symptomatic pa­tients, the classic triad of renal AML is flank pain, a palpable tender mass and hematuria due to intra-capsular or retroperitoneal blee­ding. [11] There was no retro-peritoneal bleeding in our case as documented by CT imaging prior to the endovascular treatment. Although the patient did not have any baseline CT imaging prior to the bleeding, we believe that the rupture was totally intra-tumoral, which led to the formation of a pseudoaneurysm. CT imaging provides a more definitive and accu­rate assessment of the exact site and extent of hemorrhage in these cases. [12]

The risk of rupture of a renal AML increases with tumor size, presence of symptoms and pre-sence of TSC. [10],[13] Several authors agree that lesions with a diameter of 4 cm or more have a high risk of hemorrhage. [1],[13],[14],[15] The treatment of asymptomatic lesions larger than 4 cm is debatable, and some authors have sug­gested that they need not be treated. [14] How­ever, some other authors have suggested that AMLs larger than 4 cm should be treated even if patients are asymptomatic; in symptomatic patients, AML of any size needs treatment. [16],[17] There are two different options for treatment of renal AML. One of them is the classical surgical approach with a total or partial neph­rectomy and the other is selective emboliza­tion. [5],[18] Surgery should be reserved as a last measure, particularly in patients with multiple renal AMLs in both kidneys (e.g., TSC). Today, prophylactic embolization is recom­mended in high-risk patients including not only tumors large in size but also multiple lesions, ones associated with TSC and those seen in younger women who desire having children. [9]

The patient in this report was completely asymptomatic until the time of pregnancy. Sebaceous adenomas on her face did not draw any attention until the diagnosis of bilateral renal masses was made. Therefore, the diag­nosis of TSC was made only after bleeding occurred from the renal AML, most likely triggered by pregnancy.

Several renal lesions such as AML and renal cysts are well recognized in patients with TSC. [19] Renal AML is the most common renal lesion occurring in up to 80% of adult patients with TSC. [20],[21] Renal AMLs tend to increase in size over time and can distort the renal archi­tecture, which can cause renal dysfunction and lead to renal failure in such patients. [19],[20],[21] In addition, renal AMLs contain abnormal blood vessels and aneurysms that may cause spon­taneous rupture and bleed. In the present case, there were two additional risk factors such as pregnancy and TSC that increased the risk of rupture and bleeding. The life-threatening giant renal artery pseudo-aneurysm seen in our pa­tient as a result of rupture of renal AML, which was treated by an endovascular approach, is unique and should draw attention to early diagnosis and treatment of these so-called benign lesions. [22],[23]

In contrast to surgery, transcatheter emboli­zation is minimally invasive without the need for general anesthesia, can preserve renal functions more effectively, is cheap and can be performed multiple times, as required. [24] The shorter recovery time and hospitalization are additional benefits of embolization over sur­gery. Several investigators have suggested that transcatheter embolization should be reserved for acute bleeding and multiple lesions in order to avoid life-threatening hemorrhage and preserve renal parenchyma. [14],[18] In a patient with ruptured AML who presents with hypo­volemic shock, the surgical option is usually radical nephrectomy due to impossibility to differentiate normal renal parenchyma from the huge hemorrhagic AML. [15]

There are different types of embolic agents that can be used for embolization of renal AMLs, including liquid agents such as nBCA (Onyx), polyvinyl alcohol particles, gelfoam and metallic coils. There is no consensus regarding the embolic agent of choice in the literature. [2] In the present case, nBCA was used as the embolic agent to embolize the parent artery from which the aneurysm originated, without filling the aneurysm sac itself. By this method, we aimed to achieve complete occlu­sion of the ruptured vessel with maximal nephron sparing and also to help thrombosis of the aneurismal sac and reduction in size with time. Reduction of size of the AML after embolization is an expected result and goal; however, efficacy should not be evaluated by only tumor size. The absence of bleeding epi­sodes in a long-term follow-up period and the disappearance or at least reduction of the vas­cular component should be taken into account. [14]

In conclusion, spontaneous rupture of renal AML should be suspected in a puerperal patient with sudden onset of abdominal pain and hypovolemic shock. CT imaging should be the diagnostic tool of first choice. We suggest that trans-catheter embolization should be considered in high-risk patients with renal AML as the first therapeutic option to prevent the growth and spontaneous rupture of the lesions. Because renal failure is the most common cause of death in patients with the TSC, selective transcatheter embolization can be helpful both for preserving the renal functions and for preventing catastrophic complications.

Conflict of interest: None

   References Top

1.De Luca S, Terrone C, Rocca Rosseti S. Manage­ment of renal angiomyolipoma: A case report of 53 cases. BJU Int 1999;83:215-8.  Back to cited text no. 1
2.Jou YC, Chen WP, Huang CL. Urgent angioembolization with early elective nephron-sparing surgery for spontaneously ruptured renal angio­myolipoma. J Chin Med Assoc 2009;72:450-2.  Back to cited text no. 2
3.Bora A, Soni A, Sainani N, Patkar D. Emergency embolization of bleeding renal angiomyolipoma using polyvinyl alcohol particles. Diagn Interv Radiol 2007;13:213-6.  Back to cited text no. 3
4.Steiner MS, Goldman SM, Fishman EK, Marshall FF. The natural history of renal angio­myolipoma. J Urol 1993;150:1782-6.  Back to cited text no. 4
5.Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma. J Urol 2002;168:1315-25.  Back to cited text no. 5
6.Blute ML, Malek RS, Segura JW. Angyomyolipoma: Clinical metamorphosis and concerns. J Urol 1988;139:20-4.  Back to cited text no. 6
7.Harabayashi T, Shinohara N, Katano H, Nonomura K, Shimizu T, Koyanagi T. Management of renal angiomyolipomas associated with tuberous sclerosis complex. J Urol 2004;171:102-5.  Back to cited text no. 7
8.Kontos S, Politis V, Fokitis I, Lefakis G, Koritsiadis G, Simaioforidis V. Rapture of renal angio­myolipoma during pregnancy: A case report. Cases J 2008; 17:245  Back to cited text no. 8
9.Wright T, Sooriakumaran P. Renal angiomyolipoma presenting with massive retroperitoneal haemorrhage due to deranged clotting factors: A case report. Cases J 2008;4:213.  Back to cited text no. 9
10.Yamakado K, Tanaka N, Nakagawa T, Kobayashi S, Yanagawa M, Takeda K. Renal angio­myolipoma: Relationships between tumor size, aneurism formation, and rupture. Radiology 2002;225:78-82.  Back to cited text no. 10
11.Unlu C, Lamme B, Nass P, Bolhuis HW. Retroperitoneal haemorrhage caused by a renal angiomyolipoma. Emerg Med J 2006;23:464-5.  Back to cited text no. 11
12.Yip KH, Peh WC, Tarn PC. Spontaneous rupture of renal tumours: The role of imaging in diagnosis and management. Br J Radiol 1998;71:146-54.  Back to cited text no. 12
13.Rimon U, Duvdevani M, Garniek A, et al. Ethanol and polyvinyl alcohol mixture for transcatheter embolization of renal angiomyolipoma. AJR Am J Roentgenol 2006;187:762-8.  Back to cited text no. 13
14.Dabbeche C, Chaker M, Chemali R, et al. Role of embolization in renal angiomyolipomas. J Radiol 2006;87:1859-67.  Back to cited text no. 14
15.Han YM, Kim JK, Roh BS, et al. Renal angiomyolipoma: Selective arterial embolization-effectiveness and changes in angiomyogenic components in long-term follow-up. Radiology 1997; 204:65-70.  Back to cited text no. 15
16.van Baal JG, Smith NJ, Keeman JN, Lindhout D, Verhoef S. The evolution of renal angiomyolipoma in patients with tuberous sclerosis. J Urol 1994;52:35-8.  Back to cited text no. 16
17.Kennelly MJ, Grossman HB, Cho KJ. Outcome analysis of 42 cases of renal angiomyolipoma. J Urol 1994;152:1988-91.  Back to cited text no. 17
18.Earthman W, Mazer M, Winfield A. Angiomyo­lipomas in tuberous sclerosis: Subselective embolotherapy with alcohol, with long-term follow-up study. Radiology 1986;160:437-41.  Back to cited text no. 18
19.Casper KA, Donnelly LF, Chen B, Bissler JJ. Tuberous sclerosis complex: Renal imaging findings. Radiology 2002;225:451-6.  Back to cited text no. 19
20.Chonco AM, Weiss SM, Stein JH, Ferris TF. Renal involvement in tuberous sclerosis. Am J Med 1974;56:124-32.  Back to cited text no. 20
21.Narla LD, Slovis TL, Watts FB, Nigro M. The renal lesion of tuberosclerosis (cist and angiomyolipoma); screening with sonography and computerized tomography. Pediatr Radiol 1988; 18:205-9.  Back to cited text no. 21
22.Martinez Barcina MJ, Quintian Schwieters C, Bonnin Sanchez D, Alberola Bou JM. Intratumoral pseudoaneurysm: Atypical radiologic manifestation of bleeding from an angiomyolipoma. Radiologia 2008;50:79-81.  Back to cited text no. 22
23.Gaikwad AB, Madathil MB, Kothari AS. Giant renal angiomyolipoma with fatal hemorrhage due to a large pseudoaneurysm. J Clin Ultrasound 2008;36:174-6.  Back to cited text no. 23
24.Ewalt DH, Diamond N, Rees C, et al. Long term outcome of transcatheter embolization of renal angiomyolipomas due to tuberous sclerosis complex. J Urol 2005;174:1764-6.  Back to cited text no. 24

Correspondence Address:
Ilkay S Idilman
Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara, 06100
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DOI: 10.4103/1319-2442.128570

PMID: 24626009

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


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