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 Table of Contents  
Year : 2020  |  Volume : 53  |  Issue : 5  |  Page : 202-204

Scalp cirsoid aneurysm

1 Department of Surgery, Chia-Yi Christian Hospital, Chiayi City, Taiwan
2 Department of Surgery, Chia-Yi Christian Hospital; Chung-Jen Junior College of Nursing, Health Sciences and Management, Chiayi City, Taiwan
3 Division of Neurosurgery, Department of Surgery, Sijhih Cathay General Hospital; Department of Medicine, School of Medicine, Fu Jen Catholic University, New Taipei City; Department of Neurological Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan
4 Department of Surgery, Chia-Yi Christian Hospital, Chiayi City; Department of Neurological Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei; Department of Biotechnology, Asia University, Taichung City, Taiwan

Date of Submission20-Feb-2020
Date of Decision30-Apr-2020
Date of Acceptance29-Jun-2020
Date of Web Publication19-Oct-2020

Correspondence Address:
Jui-Ming Sun
Department of Surgery, Chia-Yi Christian Hospital, No. 539, Zhongxiao Road, East District, Chiayi City 600
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/fjs.fjs_19_20

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Scalp cirsoid aneurysm, also known as arteriovenous fistula, is a rare condition that commonly presents with a pulsatile cutaneous mass. Angiography is the gold standard for diagnosing this rare lesion. Here, we report the case of a 21-year-old woman who presented with a 7-year subcutaneous pulsatile mass over the suboccipital region. Computed tomographic angiography and conventional angiography confirmed the diagnosis of scalp cirsoid aneurysm. No residual or recurrent fistulas were observed after treatment with only endovascular embolization.

Keywords: Arteriovenous fistulas, cirsoid aneurysm, embolization, scalp, suboccipital

How to cite this article:
Huang KC, Chen YH, Hsieh CT, Sun JM. Scalp cirsoid aneurysm. Formos J Surg 2020;53:202-4

How to cite this URL:
Huang KC, Chen YH, Hsieh CT, Sun JM. Scalp cirsoid aneurysm. Formos J Surg [serial online] 2020 [cited 2021 May 7];53:202-4. Available from: https://www.e-fjs.org/text.asp?2020/53/5/202/298498

  Introduction Top

Scalp cirsoid aneurysm is a rare condition that is involved in the vascular structure connecting feeding arteries and drainage veins without intervening capillaries. It was previously referred to as aneurysm serpentinum, aneurysm racemosum, plexiform angioma, scalp arteriovenous fistula, and scalp arteriovenous malformation.[1],[2] The majority of scalp cirsoid aneurysms present with a subcutaneous pulsatile mass, which may be misdiagnosed preoperatively as a benign scalp lesion, leading to devastating surgical complications.[3] Here, we report a case of suboccipital scalp cirsoid aneurysm characteristically presenting with a subcutaneous pulsatile mass and treated only with endovascular embolization.

  Case Report Top

The informed consent was obtained from the patient for the case report. A 21-year-old woman presented with a 7-year progressive enlargement of subcutaneous mass over the suboccipital region, to which there had been no previous trauma. The subcutaneous mass, measuring approximately 6.5 cm in maximal length, was soft with a pulsatile sensation. An audible bruit was also discovered. Neurological function was normal. Laboratory examination yielded results within normal limits. An enhanced computed tomography (CT) scan of the brain revealed an abnormal vascular structure primarily located in the left suboccipital region [Figure 1]a. CT angiography revealed that this pathologic vascular structure was scalp arteriovenous fistula [Figure 1]b. Digital subtraction angiography confirmed the diagnosis of scalp arteriovenous fistulas with a feeding artery from the occipital artery and the distal branch of the thyrocervical trunk [Figure 2]a and [Figure 2]b. Venous drainage was mainly into the suboccipital venous plexus and internal vertebral venous plexus. No communication with the intracranial circulation was discovered.
Figure 1: (a) Sagittal computed tomographic scan with contrast revealed an enhanced subcutaneous mass located over the suboccipital scalp region (white arrow). (b) Computed tomographic angiography revealed the angioarchitecture of the suboccipital arteriovenous fistulas

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Figure 2: Conventional digital subtraction angiography of the brain. (a) These scalp arteriovenous fistulas were primarily fed by the occipital artery (white arrow). (b) The distal branch of the thyrocervical trunk (white arrow) was also a feeder. (c) Endovascular embolization for these scalp fistulas was performed with microcoils, onyx, and poly (glycolide-co-lactide)

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Endovascular embolization was selected for treatment with consideration of the diagnosis because the patient concerned with the cosmetic outcome. After general anesthesia, the Seldinger technique was performed through the right femoral artery and vein under fluoroscopy. A guide catheter was placed in the left occipital artery through the left external carotid artery. Three vials of onyx were injected into the arterial feeders from the left external carotid artery through the occlusion balloon catheter after microcoil embolization of the distal branch of the left external carotid artery by using a microcatheter. Poly (glycolide-co-lactide) and microcoils were also used for the embolization of the venous sac. After the endovascular treatment, no residual arteriovenous fistulas or perioperative complications were identified [Figure 2]c.

No neurological deficits were observed during a 1-year follow-up after endovascular embolization treatment. CT angiography did not reveal recurrent arteriovenous fistulas [Figure 3].
Figure 3: Computed tomographic angiography revealed no residual or recurrent scalp arteriovenous fistulas following endovascular embolization therapy

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  Discussion Top

Scalp cirsoid aneurysm, which was first described by Brecht et al. in 1833, is a misnomer for scalp subcutaneous arteriovenous fistulas with a connection to the artery feeders and the venous drainage system without an intervening capillary bed.[4] The term cirsoid (varix in Greek) describes the phenomenon of dilation of draining vessels.[5] The incidence of scalp cirsoid aneurysm has been estimated to account for approximately 8% of all arteriovenous malformations.[6] A systematic review of the literature revealed that only 242 cases of scalp cirsoid aneurysms were reported in 74 articles.[2]

Scalp cirsoid aneurysm can have congenital, iatrogenic, or traumatic etiology.[2] Congenital lesions are more common than traumatic cirsoid aneurysms, with an incidence of nearly 80% in a case series.[1],[5] Most congenital scalp cirsoid aneurysms become symptomatic in the third decade of life, and men are primarily affected.[2],[4],[7],[8] Although the exact mechanism of its congenital occurrence remains unclear, the persistence of primitive arteriovenous fistulas is the primary hypothesis.[4],[9]

A pulsatile mass is the most common presentation, followed by headache and tinnitus.[1],[2] The duration of symptoms prior to presentation varies, with a median of 3 years.[2] The superficial temporal artery is the most common main afferent vessel to supply the scalp cirsoid aneurysm, followed by the occipital artery.[5] The majority of fistulas have one dominant feeding artery.[1] Preoperative radiologic imaging plays a crucial role in the exact diagnosis. CT or magnetic resonance angiography provides the contour, extension, and location of scalp fistulas.[10] However, conventional digital subtraction angiography remains the gold standard for diagnosing scalp cirsoid aneurysm and investigating its detailed angioarchitecture.[3]

Multidisciplinary approaches such as endovascular embolization, direct puncture embolization, percutaneous injection of sclerosing agents, surgical excision, and the combination of embolization and excision are recommended for managing scalp cirsoid aneurysms according to the complexity of their angioarchitecture.[4],[6],[11] Surgical intervention with total resection of arteriovenous fistulae is a common method for definitively managing abnormal vascular circulation.[6] El Shazly and Saoud demonstrated that well-planned surgery of these lesions without preoperative embolization could achieve complete excision of fistulas without any residual abnormal vascular structures or recurrence and with a low incidence of complications during the follow-up period with a mean period of 34.1 months.[7] However, when a fistula is small with few feeding arteries, endovascular embolization alone is considered an effective alternative method.[4],[12]

Our case with a subcutaneous pulsatile mass exhibited typical features of scalp cirsoid aneurysm. The audible bruit was a crucial sign during physical examination. Despite surgical excision being a common method for limiting recurrence, endovascular embolization was chosen because of the absence of clinical neurological deficits. No recurrent lesion was observed during a 1-year follow-up. Therefore, endovascular treatment appears to be an effective alternative method for treating scalp cirsoid aneurysm.

Although scalp cirsoid aneurysm is a rare condition, this diagnosis should be considered when a patient presents with a progressive pulsatile scalp mass. Detailed preoperative angiography is the gold standard for confirming the diagnosis of this rare disease.

Declaration of patient consent

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

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Albuquerque Sousa LH, Maranha Gatto LA, Demartini Junior Z, Koppe GL. Scalp cirsoid aneurysm: An updated systematic literature review and an illustrative case report. World Neurosurg 2018;119:416-27.  Back to cited text no. 1
Sofela A, Osunronbi T, Hettige S. Scalp cirsoid aneurysms: Case illustration and systematic review of literature. Neurosurgery 2020;86:E98-107.  Back to cited text no. 2
Özkara E, Özbek Z, Özdemir AÖ, Arslantaş A. misdiagnosed case of scalp arteriovenous malformation. Asian J Neurosurg 2018;13:59-61.  Back to cited text no. 3
Gupta AK, Purkayastha S, Bodhey NK, Kapilamoorthy TR, Krishnamoorthy T, Kesavadas C, et al. Endovascular treatment of scalp cirsoid aneurysms. Neurol India 2008;56:167-72.  Back to cited text no. 4
[PUBMED]  [Full text]  
Gurkanlar D, Gonul M, Solmaz I, Gonul E. Cirsoid aneurysms of the scalp. Neurosurg Rev 2006;29:208-12.  Back to cited text no. 5
Li D, Heiferman DM, Rothstein BD, Syed HR, Shaibani A, Tomita T. Scalp arteriovenous malformation (cirsoid aneurysm) in adolescence: Report of 2 cases and review of the literature. World Neurosurg 2018;116:e1042-6.  Back to cited text no. 6
El Shazly AA, Saoud KM. Results of surgical excision of cirsoid aneurysm of the scalp without preoperative interventions. Asian J Neurosurg 2012;7:191-6.  Back to cited text no. 7
[PUBMED]  [Full text]  
Khodadad G. Familial cirsoid aneurysm of the scalp. J Neurol Neurosurg Psychiatry 1971;34:664-7.  Back to cited text no. 8
Chowdhury FH, Haque MR, Kawsar KA, Sarker MH, Momtazul Haque AF. Surgical management of scalp arterio-venous malformation and scalp venous malformation: An experience of eleven cases. Indian J Plast Surg 2013;46:98-107.  Back to cited text no. 9
[PUBMED]  [Full text]  
Goel V, Verma AK, Singh S, Puri SK. Cirsoid aneurysm of scalp: Demonstration on CT angiography (CTA). BMJ Case Rep 2013;2013:1-2.  Back to cited text no. 10
Hage ZA, Few JW, Surdell DL, Adel JG, Batjer HH, Bendok BR. Modern endovascular and aesthetic surgery techniques to treat arteriovenous malformations of the scalp: Case illustration. Surg Neurol 2008;70:198-203.  Back to cited text no. 11
Wajnberg E. Endovascular treatment of scalp cirsoid aneurysms. Radiol Bras 2010;43:224-8.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3]


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