endovascular embolization of a dissected external carotid...

Case ReportEndovascular Embolization of a Dissected External CarotidArtery Pseudoaneurysm in a Young Female withNeurofibromatosis Complicated by Preeclampsia

Sasha Lalla1 Rajeev Seecheran2 Valmiki Seecheran3 Sangeeta Persad3

Ronald Henry1 and Naveen Anand Seecheran 2

1Advanced Cardiovascular Institute Port of Spain Trinidad And Tobago2University of the West Indies St Augustine Trinidad And Tobago3North Central Regional Health Authority Mt Hope Trinidad And Tobago

Correspondence should be addressed to Naveen Anand Seecheran nseecherangmailcom

Received 25 November 2018 Revised 16 April 2019 Accepted 16 May 2019 Published 12 June 2019

Academic Editor Ayman El-Menyar

Copyright copy 2019 Sasha Lalla et al This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use distribution and reproduction in any medium provided the original work is properly cited

Carotid artery pseudoaneurysms are infrequently encountered in clinical practice Major contributory etiologies include blunttrauma infections cystic medial necrosis fibromuscular dysplasia arteriosclerosis and congenital abnormalities The authorsreport an exceedingly rare case of a dissected external carotid artery pseudoaneurysm in a 26-year-old female patient withneurofibromatosis complicated by preeclampsia at 28-week period of gestation safely and successfully treated by coil embolization

1 Introduction

Neurofibromatosis type-1 (NF-1) is an autosomal dominantgenetic disorder that results from a mutation of the neu-rofibromin one gene located on chromosome 17 (17q112)It is a multisystemic disorder with complete penetrance andan estimated prevalence of 1 in 3500 births The prevalenceof NF-1-associated vascular lesions ranges from 04 to 64according to a previous clinical series [1]

The spectrum of vasculopathies includes stenosesaneurysms and arteriovenous malformations involvingthe aortic renal mesenteric carotid-vertebral subclavian-axillary iliofemoral intracerebral and coronary arteriesRenal artery involvement is the most common [1]

Due to the rarity of NF-1-associated vasculopathy clinicalcharacteristics and management strategies have not yet beenwell ascertained The authors report an exceedingly rarecase of a dissected carotid artery pseudoaneurysm in a 26-year-old female patient with neurofibromatosis complicatedby preeclampsia at 28-week period of gestation safely andsuccessfully treated via an endovascular coil embolizationtechnique

2 Case Report

A 26-year-old female with a medical history of neurofi-bromatosis type-1 and 28-week gestational age complicatedby preeclampsia was referred to the cardiovascular centerfor evaluation of an expanding pulsatile tender mass onthe right neck On admission her vital signs reflected ahypertensive emergency with systolic blood pressures of 200smmHg tachycardia of 112 beats per minute tachypnea of 20breaths per minute with oxygen saturation of 98 on roomair On physical examination there were several featuresconsistent with her preexisting diagnosis of NF-1 whichincluded multiple ldquocafe-au-laitrdquo macules and neurofibromasthroughout her chest and abdomen with both axillary andinguinal freckling [2]

Routine blood investigations were normal 12-lead elec-trocardiography indicated a sinus tachycardia with leftventricular hypertrophy which was also visualized on aninpatient transthoracic echocardiogram A computerizedtomography scan revealed a dissected pseudoaneurysm ofthe right external carotid artery (ECA) In the interim shewas treated with an intravenous nitroglycerin infusion and

HindawiCase Reports in Vascular MedicineVolume 2019 Article ID 6020393 4 pageshttpsdoiorg10115520196020393

2 Case Reports in Vascular Medicine

Figure 1 Facial depiction of the hematoma Frontal series of the right neck hematoma (eclipsed in white) resulting from dissection of theright external carotid pseudoaneurysm

Figure 2 Right carotid artery cineangiography series (a) Preintervention thewhite ellipse encircles the external carotid artery dissectionwithcontrast extravasation (b) InterventionThewhite arrow indicates successful coil embolizationwith 0018rdquo and 0035rdquo Tornado embolizationcoils (CookMedical LLC Bloomington IN USA) (c) Postintervention the white arrow indicates complete occlusion of the external carotidartery distal to the superior thyroid branch with no antegrade flow within the aneurysm

hydralazine to achieve near-normotensive pressures overthe ensuing 12 hours (see Figure 1) Subsequently on thesecond day of hospitalization selective carotid angiographydemonstrated a dissected pseudoaneurysm of the right ECAmeasuring 27 cm arising in association with the occipitalbranch with contrast extravasation (see Figure 2(a)) Ad-hoc successful coil embolization was achieved with 0018rdquoand 0035rdquo Tornado embolization coils (CookMedical LLCBloomington IN USA) (see Figure 2(b)) At the conclusionof the procedure cineangiography revealed complete occlu-sion of the vessel distal to the superior thyroid branch withno further opacification of the aneurysm (see Figure 2(c))

3 Discussion

NF-1 diagnostic criteria include the presence of two ormore of the following criteria six or more ldquocafe-au-laitrdquomacules two or more neurofibromas of any type or oneplexiform neurofibroma axillary or inguinal freckling twoor more Lisch nodules optic pathway gliomas distinctivebone lesions such as sphenoid dysplasia or thinning of thelong bone cortex with or without pseudoarthrosis and a firstdegree relative diagnosed with NF-1 (see Table 1) [2]

ECA pseudoaneurysms are exceedingly rare within thegeneral population as evidenced by the paucity of casereports and consequently optimal management strategiesare not well establishedThe estimated prevalence is approxi-mately 007 with a resultant mortality rate of 33 and only

a handful of cases specifically involving the external carotidartery [3 4]

Generally these lesions accounted for only 14 ofaneurysms in a recent review [5] Intracranial aneurysms arealso rare in cases with NF-1 Baldauf et al identified 28 casesof intracranial aneurysms associated with neurofibromatosispredominantly located in the left internal carotid arterycirculation with a female preponderance [6] These clinicalfeatures were diametrically opposite in our patient as shedisplayed right external carotid artery involvement

The pathophysiology of carotid pseudoaneurysm forma-tion is usually attributed to penetrating or blunt traumausually from motor vehicle accidents Other postulatedmechanisms include direct compression of the neck hyper-extensionrotation injury and injury to the base of theskull and anastomotic disruption following carotid vascularinterventions [1] Predisposing conditions include infectionradiotherapy poor nutritional status and neoplasia Headand neckmalignancies treated with radiotherapy can developmultifocal iatrogenic arteriopathy in the radiation fieldleading to carotid ldquoblow-outrdquo [4 7]

NF-1 is caused by a heterozygous mutation in the NF1gene which results in a loss of functional protein neurofi-bromin which is implicated in cell growth and differentia-tion The mechanisms for the vasculopathy are complex andmultifactorial including smooth muscle dysplasia and directvascular invasion by neurofibromatosis tissue [6]This resultsin intimal proliferation of spindle cells with subsequent

Case Reports in Vascular Medicine 3

Table 1 Clinical criteria for Neurofibromatosis Type-1 (NF-1) ge 2 or more of the following [2]

ge 6 ldquocafe-au-laitrdquo maculesge 2 neurofibromas of any type or 1 plexiform neurofibromaaxillary or inguinal frecklingoptic pathway gliomasge 2 Lisch nodulesdistinctive bone lesions such as sphenoid dysplasia or thinning of the long bone cortexwith or without pseudoarthrosisfirst-degree relative diagnosed with NF-1

degenerative changes in the small vessel including fibrosisloss of the smooth muscle media and elastin fragmentationAdditionally in larger caliber vessels neurofibromas or gan-glioneuromas invade and weaken the arterial wall leading toaneurysm formation [8 9]

The most common clinical presentation for NF-1 asso-ciated vasculopathy is renal artery stenosis with secondaryhypertension [10ndash12] Hypertension is present in 1 ofNF1 patients and is significantly associated with mortalityand morbidity [13] Our patient did indeed present with ahypertensive crisis however renal artery stenosis was notdiagnosed with Doppler ultrasonography

Patients with dissected ECA pseudoaneurysms typicallypresent with a tender pulsatile neck mass There may be anassociated palpable thrill audible bruit or focal neurologicaldeficits Rapid enlargement of external carotid aneurysmsmay even result in rupture or cranial nerve entrapment [1415]

Diagnostic investigations include duplex ultrasonogra-phy with a sensitivity of 92 with the significant caveatthat it may incompletely visualize the distal internal carotidarteries Computerized tomography imaging as a stand-alonemodality may not be sensitive enough and often appearsnormal initially whereas digital subtraction angiographyprovides high-fidelity enhancement of the pseudoaneurysmwhich may guide management strategies Magnetic reso-nance angiography accurately demonstrates carotid dissec-tion with sensitivity and specificity of 95 and 99 respec-tively as compared to 84 and 99 for magnetic resonanceimaging alone [16]

Generally the management of carotid pseudoaneurysmsis not well delineated and more so focused on the internalcarotid vasculature Strategies include observation anticoag-ulation ligation of the carotid artery with or without a bypassprocedure and arterial reconstruction With the advent andevolution of endovascular techniques many periproceduralsurgical complications such as cerebrovascular events (5-15)and operative mortality (2-4) have substantially decreased[17] Of the transcatheter armamentarium parent vesselocclusion with coil placement is currently in vogue withcovered stent grafts becoming a viable alternative In thisclinical scenario there was also the added complexity of thepatient being of 28-week gestational age and fetal exposureto radiation was a significant concern during fluoroscopy Arecent study indicated that the average radiation dose wascirca 4100 mGy with fluoroscopy duration of 98 minutes and

expended contrast media volume of approximately 130mL[18]The risk-benefit analysis of the alternative ldquoopenrdquo vascu-lar surgery and its attendant risks were considered and thusthe endovascular approach was favored A systematic reviewindicated an endovascular procedural success of 928 within-hospital mortality stroke and cranial nerve injury of41 18 and 05 respectively These results suggest thatthis approach has achieved comparable clinical outcomes toconventional surgery in patients with extracranial carotidartery aneurysms [17]

The outcome of carotid artery pseudoaneurysms is mostaptly summarized byWinslow in 1926 who stated that ldquobut itis not so much the rarity of this lesion when it does occurthat commands our attention as its propensity to imitateperitonsillar abscess which habit ofmimicry has lent onmorethan one occasion to lancing with a mortal hemorrhageThe majority of the patients should recover if an aneurysmis promptly recognized and treated but an overwhelmingproportion dies under dilatory blundering or pernicioustacticsrdquo [19]

4 Conclusion

ECA pseudoaneurysms are exceedingly rare and should beconsidered when a patient presents with a tender pulsatileneck mass Early diagnostic imaging and invasive manage-ment should be immediately pursued with a high index ofclinical suspicion as illustrated in this case where endovas-cular embolization proved to be a safe and effective treatmentmodality

Data Availability

All available data can be obtained by contacting the corre-sponding author

Additional Points

Key Clinical Message The clinician should be cognizant thata pulsatile neck mass can be a dissected aneurysm in apatient with neurofibromatosis type-1 which can be safely andsuccessfully treated via endovascular embolization

Ethical Approval

All procedures performed in studies involving human par-ticipants were in accordance with the ethical standards of


the institutional and national research committee and withthe 1964 Helsinki Declaration and its later amendments orcomparable ethical standards

Consent

The patient has provided written informed consent to havethe details of her case published

Conflicts of Interest

The authors declare that they have no competing interestsand there are no financial disclosures

Authorsrsquo Contributions

Sasha Lalla Rajeev Seecheran Valmiki Seecheran SangeetaPersad Ronald Henry and Naveen Anand Seecheran allcontributed equally in writing the manuscript All authorsread and approved the final manuscript

Acknowledgments

The authors acknowledge Professor Michael Ramdass Uni-versity of the West Indies St Augustine

References

[1] J M Friedman J Arbiter J A Epstein et al ldquoCardiovasculardisease in neurofibromatosis 1 report of the NF1 cardiovasculartask forcerdquo Genetics in Medicine vol 4 no 3 pp 105ndash111 2002

[2] G Jentarra S L Snyder and V Narayanan ldquoGenetic aspectsof neurocutaneous disordersrdquo Seminars in Pediatric Neurologyvol 13 no 1 pp 43ndash47 2006

[3] S Nadig S Barnwell and M K Wax ldquoPseudoaneurysm of theexternal carotid artery-review of literaturerdquo Head amp Neck vol31 no 1 pp 136ndash139 2009

[4] D J Minion T G Lynch B T Baxter and R LiebermanldquoPseudoaneurysm of the external carotid artery followingradical neck dissection and irradiation a case report and reviewof the literaturerdquo Cardiovascular Surgery vol 2 no 5 pp 607ndash611 1994

[5] Z Li G Chang C Yao et al ldquoEndovascular stenting ofextracranial carotid artery aneurysm a systematic reviewrdquoEuropean Journal of Vascular and Endovascular Surgery vol 42no 4 pp 419ndash426 2011

[6] J Baldauf J Kiwit andM Synowitz ldquoCerebral aneurysms asso-ciated with von Recklinghausen1015840s neurofibromatosis Report ofa case and review of the literaturerdquo Neurology India vol 53 no2 p 213 2005

[7] J C Yuen and D J Gray ldquoEndovascular treatment of apseudoaneurysm of a recipient external carotid artery followingradiation and free tissue transferrdquo Annals of Plastic Surgery vol44 no 6 pp 656ndash659 2000

[8] J L FalconeM R Go D T Baril G J OakleyM SMakarounand R A Chaer ldquoVascular wall invasion in neurofibromatosis-induced aortic rupturerdquoVascular andEndovascular Surgery vol44 no 1 pp 52ndash55 2010

[9] S Saitoh and S Matsuda ldquoAneurysm of the major vesselsin neurofibromatosisrdquo Archives of Orthopaedic and TraumaSurgery vol 117 no 1-2 pp 110ndash113 1998

[10] E Criado L Izquierdo S Lujan E Puras and M Mar EspinoldquoAbdominal aortic coarctation renovascular hypertension andneurofibromatosisrdquoAnnals of Vascular Surgery vol 16 no 3 pp363ndash367 2002

[11] G R Craddock V R Challa and R H Dean ldquoNeurofibro-matosis and renal artery stenosis A case of familial incidencerdquoJournal of Vascular Surgery vol 8 no 4 pp 489ndash494 1988

[12] M Halpern and G Currarino ldquoVascular lesions causing hyper-tension in neurofibromatosisrdquo The New England Journal ofMedicine vol 273 no 5 pp 248ndash252 1965

[13] S Kothari and I Malav ldquoRenal artery stenosis due to neurofi-bromatosisrdquo Annals of Pediatric Cardiology vol 2 no 2 p 1672009

[14] C Rhee T Jinn H Jung M Sung K H Kim and YMin ldquoTraumatic pseudoaneurysm of the external carotidartery with parotid mass and delayed facial nerve palsyrdquoOtolaryngologyndashHead and Neck Surgery vol 121 no 1 pp 158ndash160 2016

[15] V M Pereira S Geiprasert T Krings et al ldquoExtracranial verte-bral artery involvement in neurofibromatosis type I Report offour cases and literature reviewrdquo Interventional Neuroradiologyvol 13 no 4 pp 315ndash328 2007

[16] C Levy J P Laissy V Raveau et al ldquoCarotid and verte-bral artery dissections Three-dimensional time-of- flight MRangiography and MR imaging versus conventional angiogra-phyrdquo Radiology vol 190 no 1 pp 97ndash103 1994

[17] G S Oderich T M Sullivan T C Bower et al ldquoVascularabnormalities in patients with neurofibromatosis syndrometype I clinical spectrum management and resultsrdquo Journal ofVascular Surgery vol 46 no 3 pp 475e1ndash484e1 2007

[18] G P Colby L-M Lin N Nundkumar et al ldquoRadiation doseanalysis of large and giant internal carotid artery aneurysmtreatment with the pipeline embolization device versus tra-ditional coiling techniquesrdquo Journal of NeuroInterventionalSurgery vol 7 no 5 pp 380ndash384 2015

[19] H W Pia C Langmaid and J Zierski Cerebral AneurysmsAdvances inDiagnosis andTherapy Springer ScienceampBusinessMedia 2012

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Volume 2018Hindawiwwwhindawicom

Submit your manuscripts atwwwhindawicom


Figure 1 Facial depiction of the hematoma Frontal series of the right neck hematoma (eclipsed in white) resulting from dissection of theright external carotid pseudoaneurysm

Figure 2 Right carotid artery cineangiography series (a) Preintervention thewhite ellipse encircles the external carotid artery dissectionwithcontrast extravasation (b) InterventionThewhite arrow indicates successful coil embolizationwith 0018rdquo and 0035rdquo Tornado embolizationcoils (CookMedical LLC Bloomington IN USA) (c) Postintervention the white arrow indicates complete occlusion of the external carotidartery distal to the superior thyroid branch with no antegrade flow within the aneurysm

hydralazine to achieve near-normotensive pressures overthe ensuing 12 hours (see Figure 1) Subsequently on thesecond day of hospitalization selective carotid angiographydemonstrated a dissected pseudoaneurysm of the right ECAmeasuring 27 cm arising in association with the occipitalbranch with contrast extravasation (see Figure 2(a)) Ad-hoc successful coil embolization was achieved with 0018rdquoand 0035rdquo Tornado embolization coils (CookMedical LLCBloomington IN USA) (see Figure 2(b)) At the conclusionof the procedure cineangiography revealed complete occlu-sion of the vessel distal to the superior thyroid branch withno further opacification of the aneurysm (see Figure 2(c))

3 Discussion

NF-1 diagnostic criteria include the presence of two ormore of the following criteria six or more ldquocafe-au-laitrdquomacules two or more neurofibromas of any type or oneplexiform neurofibroma axillary or inguinal freckling twoor more Lisch nodules optic pathway gliomas distinctivebone lesions such as sphenoid dysplasia or thinning of thelong bone cortex with or without pseudoarthrosis and a firstdegree relative diagnosed with NF-1 (see Table 1) [2]

ECA pseudoaneurysms are exceedingly rare within thegeneral population as evidenced by the paucity of casereports and consequently optimal management strategiesare not well establishedThe estimated prevalence is approxi-mately 007 with a resultant mortality rate of 33 and only

a handful of cases specifically involving the external carotidartery [3 4]

Generally these lesions accounted for only 14 ofaneurysms in a recent review [5] Intracranial aneurysms arealso rare in cases with NF-1 Baldauf et al identified 28 casesof intracranial aneurysms associated with neurofibromatosispredominantly located in the left internal carotid arterycirculation with a female preponderance [6] These clinicalfeatures were diametrically opposite in our patient as shedisplayed right external carotid artery involvement

The pathophysiology of carotid pseudoaneurysm forma-tion is usually attributed to penetrating or blunt traumausually from motor vehicle accidents Other postulatedmechanisms include direct compression of the neck hyper-extensionrotation injury and injury to the base of theskull and anastomotic disruption following carotid vascularinterventions [1] Predisposing conditions include infectionradiotherapy poor nutritional status and neoplasia Headand neckmalignancies treated with radiotherapy can developmultifocal iatrogenic arteriopathy in the radiation fieldleading to carotid ldquoblow-outrdquo [4 7]

NF-1 is caused by a heterozygous mutation in the NF1gene which results in a loss of functional protein neurofi-bromin which is implicated in cell growth and differentia-tion The mechanisms for the vasculopathy are complex andmultifactorial including smooth muscle dysplasia and directvascular invasion by neurofibromatosis tissue [6]This resultsin intimal proliferation of spindle cells with subsequent











4 Conclusion


Data Availability


Additional Points


Ethical Approval




Consent






Acknowledgments


References
























of




Disease Markers



OncologyJournal of





PPAR Research



Volume 2018


Journal of

ObesityJournal of





























4 Conclusion


Data Availability


Additional Points


Ethical Approval




Consent






Acknowledgments


References
























of




Disease Markers



OncologyJournal of





PPAR Research



Volume 2018


Journal of

ObesityJournal of





















Consent






Acknowledgments


References
























of




Disease Markers



OncologyJournal of





PPAR Research



Volume 2018


Journal of

ObesityJournal of























of




Disease Markers



OncologyJournal of





PPAR Research



Volume 2018


Journal of

ObesityJournal of



















endovascular embolization of a dissected external carotid...

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