Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 8  |  Issue : 5  |  Page : 80-82

Role of digital subtraction angiography in diagnosis of fibromusculardysplasia


1 Faculty of Medicine, University of Western Sydney; Vascular Surgery Department, Royal North Shore Hospital, Sydney, NSW
2 Vascular Surgery Department, Royal North Shore Hospital, Sydney, NSW

Date of Submission15-Dec-2020
Date of Acceptance31-Dec-2020
Date of Web Publication30-Aug-2021

Correspondence Address:
Animesh Singla
Vascular Surgery Department, Royal North Shore Hospital, Sydney
NSW
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_170_20

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  Abstract 


Fibromuscular dysplasia (FMD) is a vascular disease characterized by abnormal arterial wall architecture, usually visualized angiographically as the classic “string of beads” sign. We present the diagnostic dilemma of a 51-year-old woman admitted for a spontaneous renal infarct, with initial examination and investigations equivocal. She was consequently assessed for thromboembolic and vasculitic disease, before proceeding to digital subtraction angiography (DSA). This invasive method elucidated an area of focal FMD, previously not seen on computed tomography angiography (CTA). This is unusual due to CTA's high sensitivity and specificity but could be attributed to the less common subtype of FMD which requires combined imaging modalities to arrive at a diagnosis. Hence, there may be a role for the dual use of CTA and DSA in young patients presenting with a spontaneous renal infarct in the absence of other diagnoses.

Keywords: Digital subtraction angiography, fibromuscular dysplasia, renal artery dissection, renal infarction


How to cite this article:
Wang C, Singla A, Kotecha K, Nguyen D. Role of digital subtraction angiography in diagnosis of fibromusculardysplasia. Indian J Vasc Endovasc Surg 2021;8, Suppl S1:80-2

How to cite this URL:
Wang C, Singla A, Kotecha K, Nguyen D. Role of digital subtraction angiography in diagnosis of fibromusculardysplasia. Indian J Vasc Endovasc Surg [serial online] 2021 [cited 2021 Nov 30];8, Suppl S1:80-2. Available from: https://www.indjvascsurg.org/text.asp?2021/8/5/80/324940




  Introduction Top


Renal artery dissection is an uncommon clinical phenomenon arising from etiologies including traumatic or iatrogenic vessel injury, connective tissue disorders, or underlying arterial disease such as fibromuscular dysplasia (FMD).[1] FMD, a nonatherosclerotic arterial wall disease, is diagnosed radiographically with computed tomography angiography (CTA) being the initial test of choice.[2] This case report details a middle-aged woman who presented with a spontaneous renal infarct secondary to FMD, the radiographic features of which were not detected on CTA, highlighting the role for digital subtraction angiography (DSA) when diagnosis is elusive.


  Case Report Top


A 51-year-old woman presented with a 4-day history of colicky lower abdominal pain radiating to the back, which had worsened and become constant. The pain was associated with fluctuating nausea, left-sided migraine, and a loss in appetite. There were no changes to urinary or bowel function. Her father previously passed away in his 60s from a dissecting thoracic aortic aneurysm. On admission, she was afebrile with a blood pressure of 173/62 and heart rate of 60. Abdominal examination revealed diffuse abdominal tenderness. Laboratory testing and renal duplex ultrasound were otherwise unremarkable [Figure 1]. The subsequent CTA revealed a left renal infarct with occlusion of the segmental branch artery supplying the infarcted parenchyma [Figure 2]. Incidental findings included a right ovarian cyst, multifibroid uterus, and giant hemangioma on the posterior aspect of her liver.
Figure 1: (a) Color duplex of the left renal artery showing normal caliber and flow. (b) Spectral analysis showing peak systolic velocity of 58 cm/s with normal low resistance arterial waveform

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Figure 2: Computed tomography imaging (maximum intensity projection) reconstruction showing normal caliber and lumen of the left main renal artery

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The patient was commenced on anticoagulation and assessed for exclusion of thromboembolic disease. While transthoracic echocardiogram and 24-h Holter monitoring were negative, the CT aortic angiogram demonstrated a cuff-like soft-tissue density around the segmental renal branch and new vessel wall changes, supportive of dissection or vasculitis rather than embolic disease. Autoimmune vasculitis and thrombophilia screens returned unremarkable. After multidisciplinary discussion, the decision was made to carry out further investigation. A digital subtraction angiogram was performed via a left radial approach. Selective cannulation of the left renal artery identified a focal area of dissection with patent false lumen [Figure 3]. This had not been appreciated on previous renal artery duplex or serial CTA. There was no evidence of involvement of the contralateral renal artery or other arterial beds. These findings in conjunction with the patient's demographics and symptoms suggested FMD as the likely diagnosis. The patient was discharged a few days later on oral anticoagulation and antihypertensives with outpatient arterial duplex ultrasound follow-up.
Figure 3: Digital subtraction angiography with cannulation of the left renal artery and visualization of focal area of intimal dissection. Lack of contrast filling seen in the mid-portion of the left kidney (and associated lobar/interlobar arteries)

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  Discussion Top


FMD is a nonatherosclerotic, noninflammatory vascular disease, originally described by Leadbetter and Burkland in 1938 which can clinically manifest as dissection, stenosis, aneurysm, or arterial tortuosity.[3],[4] It is most prevalent in females, with a mean age at diagnosis of 51.9 years.[3] It can affect any arterial segment, though most commonly involves the renal, extracranial carotid, and vertebral arteries.[4] Histopathologic classifications of FMD have been replaced by angiographic diagnosis of two discrete types: (1) multifocal FMD (typically medial and perimedial fibroplasia), with alternating regions of stenosis and dilation typified by its “string of beads” appearance usually localized to the mid and distal portion of the artery, and (2) focal FMD (typically adventitial and intimal fibroplasia), with focal tubular or concentric stenosis which can occur in any portion of the artery.[2],[5] In our patient, an area of dissection in the left renal artery was imaged, representing a potential source of embolism and a diagnosis of the less common focal FMD.

As per the 2019 International Consensus on FMD, radiographical investigation of FMD begins with CTA as the initial imaging modality of choice, or magnetic resonance angiography (MRA) if CTA is contraindicated.[2] CTAs render high-quality images with spatial resolution superior to that of MRA in that visualization of renal parenchyma and vessel wall and lumen is possible, with added benefits of being noninvasive, readily available, and time effective.[2],[5] However, disadvantages of CTA include its radiation dose and use of iodinated contrast, both of which MRA successfully avoids as an imaging choice.[5] Typical computed tomography (CT) features of FMD include stenoses with poststenotic dilatation, dissection, or aneurysmal change.[2] The presence of vessel tortuosity or elongation may also be suggestive of FMD.[2] Pathology typically involves the mid to distal portion of the vessel, with more frequent involvement of the right renal artery (thought to be vessel length and congenital abnormality in vasovasorum).[6],[7] These are most easily detected with thin slices (<1 mm), with a sensitivity/specificity approaching 100%.[8] The presence of duplex criteria for renal artery stenosis with involvement beyond the renal artery orifice can also support CT findings. This includes a peak systolic velocity > 200 cm/s, acceleration times > 0.7 s, and renal-aortic ratio > 3.5.[9] However, it is strongly operator dependent, with sensitivity/specificity varying among institutions.[2],[5] In this patient, initial noninvasive imaging did not find any pathology. It is uncommon to have no abnormality detected on either CTA or duplex imaging in a vascular accredited laboratory.[9]

DSA is still considered a gold standard for diagnosis of most vascular pathologies.[2] Its role in therapy is well established, including in treatment of renal FMD.[2] However, in the modern era of multidetector CT, its role as a diagnostic aid in daily clinical practice has greatly diminished.[5] In this case, evidence of renal artery abnormality was only visible with selective vessel cannulation and direct interrogation, clearly identifying a focal area of dissection. This most likely represented an intimal form of FMD, for which noninvasive imaging may be less accurate.[10] It is clear from previous correlation studies, intimal and adventitial FMD is more likely to lead to focal lesions, which may decrease the sensitivity/specificity of CTA.[8],[10] As such, DSA was critical in establishing the underlying diagnosis, and deciding on long-term management for this young patient.


  Conclusion Top


While DSA's role in FMD imaging has diminished in the face of noninvasive testing, there remains a role for DSA in patients who have pathology undetectable on CTA, MRA, or duplex ultrasound. In particular, less common FMD subtypes may require combined imaging modalities (including DSA) to adequately confirm diagnosis. Hence, in female middle-aged patients presenting with renal infarction with no clear inciting event, consideration for DSA is warranted in the absence of other diagnoses.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Renaud S, Leray-Moraguès H, Chenine L, Canaud L, Vernhet-Kovacsik H, Canaud B. Spontaneous renal artery dissection with renal infarction. Clin Kidney J 2012;5:261-4.  Back to cited text no. 1
    
2.
Gornik HL, Persu A, Adlam D, Aparicio LS, Azizi M, Boulanger M, et al. First International consensus on the diagnosis and management of fibromuscular dysplasia. Vasc Med 2019;24:164-89.  Back to cited text no. 2
    
3.
Krittanawong C, Kumar A, Johnson KW, Kaplin S, Virk HU, Wang Z, et al. Prevalence, presentation, and associated conditions of patients with fibromuscular dysplasia. Am J Cardiol 2019;123:1169-72.  Back to cited text no. 3
    
4.
Narula N, Kadian-Dodov D, Olin JW. Fibromuscular dysplasia: Contemporary concepts and future directions. Prog Cardiovasc Dis 2018;60:580-5.  Back to cited text no. 4
    
5.
Lewis S, Kadian-Dodov D, Bansal A, Lookstein RA. Multimodality imaging of fibromuscular dysplasia. Abdom Radiol (NY) 2016;41:2048-60.  Back to cited text no. 5
    
6.
Sanidas EA, Seferou M, Papadopoulos DP, Makris A, Viniou NA, Chantziara V, et al. Renal fibromuscular dysplasia: A not so common entity of secondary hypertension. J Clin Hypertens (Greenwich) 2016;18:240-6.  Back to cited text no. 6
    
7.
Varennes L, Tahon F, Kastler A, Grand S, Thony F, Baguet JP, et al. Fibromuscular dysplasia: What the radiologist should know: A pictorial review. Insights Imaging 2015;6:295-307.  Back to cited text no. 7
    
8.
Sabharwal R, Vladica P, Coleman P. Multidetector spiral CT renal angiography in the diagnosis of renal artery fibromuscular dysplasia. Eur J Radiol 2007;61:520-7.  Back to cited text no. 8
    
9.
Schäberle W, Leyerer L, Schierling W, Pfister K. Ultrasound diagnostics of renal artery stenosis: Stenosis criteria, CEUS and recurrent in-stent stenosis. Gefasschirurgie 2016;21:4-13.  Back to cited text no. 9
    
10.
Vuong PN, Desoutter P, Mickley V, Bültmann B, Rothenberger-Janzen K, Guyot H, et al. Fibromuscular dysplasia of the renal artery responsible for renovascular hypertension: A histological presentation based on a series of 102 patients. Vasa 2004;33:13-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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