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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 29  |  Issue : 1  |  Page : 66-72

Endovascular management of auricular arteriovenous malformations


Department of Diagnostic and Interventional Neuroradiology, Lahore General Hospital, Lahore, Pakistan

Date of Submission22-Jan-2020
Date of Acceptance05-Feb-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Dr. Saima Ahmad
Department of Diagnostic and Interventional Neuroradiology, Pakistan Institute of Neurosciences, Lahore General Hospital, Ferozpur Road, Lahore
Pakistan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjps.tjps_95_19

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  Abstract 


This is a retrospective review of eight patients with extracranial arteriovenous malformations (AVMs) of the auricular region. All cases presented with significant hemorrhages or cosmetic defects. Highflow AVMs of the auricular are quite rare in contrast to low flow vascular lesions of the head and neck, making early diagnosis and treatment critical as incidences of recurrence are high if not managed properly. The purpose of this study is to present our experience of experimental endovascular treatment in a series of eight patients with auricular AVMs. From January 2015 to December 2017, eight patients with auricular AVM were put through transcatheter arterial embolization and percutaneous direct puncture embolization with polyvinyl alcohol (PVA) and bleomycin in five patients, glue embolization in one patient and coiling along with PVA in two patients to occlude high-flow fistula. Clinical outcomes were obtained through physical examination, and symptoms and a degree of devascularization on follow-up angiography. We performed 10 therapeutic embolization procedures. Of these, 3 resulted in absolute devascularization while 5 resulted in 75% obstruction. In 5 patient's embolization was performed as a palliative procedure. The other 3 were embolized as a sole mean of treatment for occluding the AVM. Only two patients developed postembolization necrosis and infection, and there has been no reexpansion of lesions to date. While thus far only having been used as percutaneous local sclerotherapy, this study found that bleomycin and PVA mixture, if used intraarterially can be more effective in the management of auricular AVM's.

Keywords: Arteriovenous malformations, bleomycin, endovascular procedures, polyvinyl alcohol


How to cite this article:
Ahmad S. Endovascular management of auricular arteriovenous malformations. Turk J Plast Surg 2021;29:66-72

How to cite this URL:
Ahmad S. Endovascular management of auricular arteriovenous malformations. Turk J Plast Surg [serial online] 2021 [cited 2021 Jan 27];29:66-72. Available from: http://www.turkjplastsurg.org/text.asp?2021/29/1/66/305915




  Introduction Top


Head and neck make up almost half of all arteriovenous malformations (AVMs). The ear being the second-highest affected location with the cheek being first among extracranial AVMs in head and neck.[1],[2]

While the pathogenesis of lesions is still elusive, the most common hypothesis is that primary abnormal capillary communication between arteries and veins is the cause. It is theorized that uptake in blood flow to the central section or nidus of these abnormal connections results in a secondary phenomenon of hypertrophied arteries and dilated veins.[3] Mostly growing during puberty, the lesions never regress on their own and only ever expand.

The Schobinger classification is used to define the clinical stages of AVMs, and their severity is classified through the table.[4],[5] Stage 1 has quiescent lesions that remain stable for years. In stage two, there is a rapid expansion that follows either trauma, hormonal changes of pregnancy and puberty or failed attempts at excision. In the third stage, the lesions become destructive and lead to bleeding, necrosis, ulceration, or persistent pain and this stage can be life-threatening.

The fourth and final stage has lesions that cause hemodynamic decompensation, possible coagulopathy, or even cardiac failure. The Schobinger classification is used to anticipate the progression of lesions as well as indicate the need for intervention.

The signs and symptoms presenting that correlate with the stage of AVM are most commonly pulsations (51.2%), bleeding (41.5%), and pain (29.3%).[6] Hearing deterioration has also been noted in certain cases presumably because the bruit is audible.[7],[8]

There is no federal drug authority (FDA) approved gold standard for treatment in this regard, but various methods and drugs have been used to try and treat this anomaly with varying degrees of success. Since this is a rare condition, and no universal standard has been adopted, endovascular treatment has shown great progress and can be considered a potentially safe and effective approach. N-Butyl cyanoacrylate (NBCA), polyvinyl alcohol (PVA), and other embolic agents can now be used for the treatment of AVM nidus since the advancements in microcatheter techniques (Pham, Wong, and Allison, 2001).

Percutaneous treatment with antiangiogenic drugs like bleomycin is another treatment option. Bleomycin is a sclerosing agent that affects vascular endothelium. Bleomycin has rationally been intralesionaly administrated, showing a success rate of over 90%.[9],[10],[11] Its effectiveness, ease of use, and safety make it the ideal therapeutic modality in complicated vascular lesions. In this study, we have used, bleomycin and PVA as a composition to treat auricular AVMs in eight patients as a sole treatment, however, in three patients with high-flow fistulas and dominant outflow vein we use pushable coils and NBCA as well, during 2 years' period.


  Methods Top


Patients

We obtained approval from the institutional review board of our hospital for a retrospective review of patients' medical records and imaging records from their respective wards. We also obtained written informed consent from all the patients for the procedure after a discussion about the advantages and complications of the procedure.

From September 2015 to December 2017, about eight patients who presented with auricular AVM, and embolized, were reviewed. The study group consisted of eight patients (7 males and 1 female) with a mean age of 15 years, age range (2.5 years to 30 years). Two patients had previously undergone through surgeries with incomplete excision, and further deterioration occurred. These patients were treated with NBCA and coils. In the remaining five patients, embolization with PVA mixed with bleomycin was decided as their primary treatment modality. All patients were assessed initially by thorough clinical examination. Patients presented with multiple presentations at different stages. Four of them gave a history that dates back to their childhood when lesion presented as a vascular birthmark on-ear which expanded with the passage of time. Rest of them presented with swelling after ear trauma. The most common sign and symptom was ear swelling (all eight patients), redness (seven patients), warmth (eight patients), pulsations (all eight patients), bruit (eight patients), pain, ulceration and hemorrhage (five patients), and blisters (one patient). The dilatation of the external jugular vein was seen in five patients (62%). The tissue adjacent to the lesion was erythematous and edematous in five patients. The audiological assessment was done in all patients and hearing was disturbed in one patient. The stage of AVM was evaluated according to the clinical staging system described by Schobinger. Selective and super-selective digital subtraction angiography done in all patients to accurately outline the feeding arteries, draining vein, and nidus of the AVM. It was performed in all patients prior to embolization. The data of the patients are shown in [Table 1], while the results and outcome of their therapy are shown in [Table 2].
Table 1: Patient demographics, clinical characteristics, and location

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Table 2: Angioarchitecture and clinical outcomes for auricular arteriovenous malformation emobolizations

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Embolization technique

Two interventional neuroradiologists with experience of 20 and 4 years, respectively, performed all the procedures and patients were embolized under general anesthesia through nasal tube.

Internal carotid and external carotid artery angiograms were done for all patients. Those whose external carotid artery had been ligated in prior surgeries had additional angiograms done of their common carotid, thyrocervical trunk, contralateral external carotid artery, and vertebral artery. This was done so that the angioarchitecture of the AVMs could be fully determined, and then selective angiographies of the occipital, posterior auricular, and superficial temporal artery were done.

After the right groin region was sterilized and draped, an Angiographic needle was used to percutaneously puncture the right femoral artery. Once the routes of endovascular access to the lesions were identified, a microcatheter was introduced into the nidus. At times, due to the tortuosity of vessels, direct puncture of the nidus was required. Super-selective placement of catheter tip was a requirement only then PVA particles and bleomycin be injected into the nidus with sparing of all other normal vascular structures. PVA particles were injected after several arteriograms to determine an appropriate volume and rate of injection on the basis of flow characteristics of the AVMs. Arteriography was performed after each PVA particle mixed with bleomycin injection to determine whether thrombosis has occurred. The total amount of bleomycin used per session was between 10 and 15 units and PVA particle size was (150–250 μm) [Figure 1].
Figure 1: Schematic depiction of the treatment of auricular extracranial arteriovenous malformations

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Postoperative management consisted of steroids and intravenous infusion of fluids. The subsequent medication usually included a tapering dose of oral steroids for 2 weeks to reduce swelling. The clinical outcome of the patients was evaluated in regular follow-ups.

Follow-ups and evaluations

One month intervals were the benchmark for follow-up evaluation through physical examination and all patients underwent follow-up, but the duration varied from as low as 1 month all the way to 26 months' post procedure. Two patients also had follow-up angiograms done. We only did a second session for patients in whom signs and symptoms of the AVM remained and were present on angiograms. Two interventional radiologists and a maxillofacial surgeon evaluated through general consensus the clinical outcomes of the patients. Patients who saw complete resolution of symptoms were considered to be cured; partial palliation was when there was a significant improvement in clinical signs and symptoms. Effective outcome of the procedure using PVA particles and bleomycin embolization was considered to be the partial palliation or cure of patients who underwent the procedures.

In the rare case of a complication arising, additional angiograms as well as past angiograms were carefully reviewed to do a root cause analysis and help the patient.


  Results Top


We found through clinical examination that patients were in various stages of Schobinger stage at the initial presentation [Table 3]. One patient had stage 1 [Figure 2] (12.5%), 2 patients had state 2 [Figure 3] (25%) [Figure 4], four patients had stage 3 (50%), and finally, only one patient had stage 4 (12.5%) [Figure 5]. We found through super-selective angiography that the superficial temporal artery (n = 2), the occipital artery (n = 2), the superficial temporal artery (n = 2), and the posterior auricular artery (n = 8) on the ipsilateral side of the lesions were the main feeders of the AVMs. Those patients who underwent the previous ligation of external carotid artery and AVM worsened and high-flow fistula formed with dominant dilated veins. The AVMs of the superior helices were commonly supplied by superficial temporal artery.
Table 3: Schobinger's classification of arteriovenous malformations

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Figure 2: (a) The lateral view of the left ear before treatment shows arteriovenous malformations.(b) Photograph 10 months after polyvinyl alcohol particle embolization of arteriovenous malformations, showing shrinkage of the ear with no symptoms. (c) Lateral view of an angiogram of left external carotid artery showing extracranial auricular arteriovenous malformation around the left ear.(d) Final lateral view of left external carotid artery showing obliteration of arteriovenous malformations left ear. (e) The lateral view of external carotid artery angiogram 10 months after embolization of arteriovenous malformations showing complete obliteration

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Figure 3: (a) A 30-year-old man with right auricular arteriovenous malformations causing pulsating red mass, ulceration, bleeding, and pustule formation. (b) Lateral digital subtraction angiography image obtained after the right external carotid artery injection demonstrates high-flow AV fistula. (c) Lateral digital subtraction angiography image showing the deployment of coil through microcatheter. (d) Lateral digital subtraction angiography image obtained after embolization with coil and polyvinyl alcohol particles reveals a significant reduction in the flow toward AV fistula. (e) The lateral view of the right ear after 1 month later post treatment shows shrinkage of swelling, ulceration, and redness of ear

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Figure 4: (a) The lateral view of the right ear before treatment. (b) The lateral view of the right ear after 6 months. (c) The lateral view of the right ear after 6 months later after second treatment shows shrinkage of swelling. (d) A lateral supra-selective digital subtraction angiography image demonstrating arteriovenous malformations nidus with micro-catheter during the first session.(e) A lateral digital subtraction angiography image demonstrates nidus involving the right ear getting supply from posterior auricular artery before embolization in the second session. (f) Lateral digital subtraction angiography image obtained after polyvinyl alcohol particle and bleomycin embolization reveal total obliteration of nidus

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Figure 5: (a) Pre-operative photographs show enlarged left ear with pulsatile mass and disfigurement. (b) The lateral view of left ear posttreatment shows marked necrosis. (c) The lateral view of the left ear after wedge resection and debulking post selective embolization.(d) Preembolization lateral angiogram of the left external carotid artery in arterial phase demonstrated flow toward auricle arteriovenous malformations with supply from posterior auricular artery.(e) Lateral digital subtraction angiography image showing supra-selective catheterization of the left posterior auricular artery. (f) Postembolization angiogram demonstrates significant reduction of flow toward auricular arteriovenous malformations

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Central and inferior portions of the ears (the concha and lobule) were fed by the posterior auricular artery. The AVMs of the pinna and retro auricular region were fed by the occipital artery. Examinations showed that contagious soft tissue was often located in the retro auricular skin (n = 4), occipital scalp (n = 3), temporal scalp (n = 4), and cheek/parotid gland (n = 1). There was never any evidence to show mandible involvement nor was the external auditory canal inner or middle ear ever involved. 8 out of the 10 embolized patients had bleomycin as the agent of choice with a range from 10 to 15 units and PVA particles size range from 150 to 250 um in one session. Eight patients with ten sessions underwent transcatheter arterial embolization and one went through percutaneous embolization. The eight exhibited focal swelling around the AVMs post procedure that resolved in one to 2 weeks.

The results of our study showed hemorrhage (5/5 patients), infections (5/5) patients, bruits (8/8 patients), pulsation (8/8) patients, pain (5/5 patients), and ulceration (5/5 patients). All swelling and redness were alleviated in patients and warmth was achieved.

(37.5%) of the eight patients, 3 had complications, only one patient had infection and necrosis, one patient had ulceration and necrosis, needs wedge resection and debunking surgery, one patient develop headache and hypertension. Three patients were totally cured. In five patients' partial palliation done with stable disease.


  Discussion Top


”AVMs are congenital structural anomalies resulting from arrest in the development of or the failure of orderly resorption of primitive embryologic vascular elements. In AVMs, the endothelial cells demonstrate a normal rate of cellular division and do not regress at the histologic level.” (Pham, Wong and Allison, 2001).

A multidisciplinary approach is needed for the treatment of auricular AVMs and the involvement of plastic surgeons is necessary in this regard in the surgery after embolization, which involves reconstruction of local free flaps (Panda et al., 2016). In cases where plastic surgery is a difficult intervention, free tissue transfer is an added and current technique for large volume and complex reconstructions in alleviating this ailment.[12] This study is different from previous similar studies since it used bleomycin percutaneously as an adjunct therapy along with intra-arterial embolization in those lesions that are complete embolization is not possible through transarterial route. In one or two studies, they have also used a similar methodology.[5]

Even though lesions are congenital, mostly, they remain dormant in childhood and show an increase in size as puberty hits.[13] Almost 80% are detected in childhood[10] and all do progress ultimately.[11] Regression is almost never seen.[11] On the cellular level, these lesions do not grow abnormally as endothelial cells are seen in these tumors, along with angiogenesis and vasculogenesis these are thought to account for their expansion. An increase in blood flow cause collateralization, dilation of existing vessels and thickening of adjacent vessels[11] and increased pressure in thought to stimulate cellular hypertrophy.[11]

It has been found that there is currently no cure treatment modality available that is FDA approved or recognized by any other international body for the treatment of auricular AVMs. The current palliative treatment is only symptomatic in nature and ends with the necrosis and surgical removal of an ear. Even though surgery is the primary recognized treatment modality it often times fails, with the main reason being that when ligation of proximal feeding arteries is performed, there is only a partial resection and over time symptoms recur and worsen as the primary problem of abnormal vascular elements being very aggressive remains. The biggest recanalization reason is that the AVM is not properly managed through embolizing agents that do not completely destroy the endothelial cells, decreasing oxygen tension and sending out angiogenesis factors that stimulate neovascular formation.[13]


  Conclusion Top


This study in spite of a small study sample demonstrates that the usage of PVA particles along with bleomycin if used during presurgical embolization with surgical excision can have greatly superior results, with far more positive outcomes and the palliation of said AVMs. It also leads the way for further reconstructive plastic surgery with the usage of flapping and a potential endovascular treatment modality for palliative and/or complete devascularization for an issue that currently does not have a set standard. This paper and study can lead the way for others like it with proper funding and on a larger scale to fully gauge the effectiveness of such a treatment modality and pave the way for the development of an internationally agreed-upon treatment method for auricular AVMs.

Acknowledgments

I would also like to thank Mian Faizan and the team online for their continued support in proofreading the content and generating images legends and record-keeping system for all supplementary data of this paper.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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Frieden IJ, Haggstrom AN, Drolet BA, Mancini AJ, Friedlander SF, Boon L, et al. Infantile hemangiomas: Current knowledge, future directions. Proceedings of a research workshop on infantile hemangiomas, April 7-9, 2005, Bethesda, Maryland, USA. Pediatr Dermatol 2005;22:383-406.  Back to cited text no. 4
    
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Muir T, Kirsten M, Fourie P, Dippenaar N, Ionescu GO. Intralesional Bleomycin Injection (IBI) treatment for haemangiomas and congenital vascular malformations. Pediatr Surg Int 2004;19:766-73.  Back to cited text no. 7
    
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Song JJ, An GS, Choi I, De Ridder D, Kim SY, Choi HS, et al. Objectification and differential diagnosis of vascular pulsatile tinnitus by transcanal sound recording and spectrotemporal analysis: A preliminary study. Otol Neurotol 2016;37:613-20.  Back to cited text no. 9
    
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Hartzell LD, Stack BC Jr., Yuen J, Vural E, Suen JY. Free tissue reconstruction following excision of head and neck arteriovenous malformations. Arch Facial Plast Surg 2009;11:171-7.  Back to cited text no. 12
    
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    Figures

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

  [Table 1], [Table 2], [Table 3]



 

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