Advertisement

Internal Carotid Artery Aneurysm: Large Cavernous Carotid Artery Aneurysm Causing Compression of the Internal Carotid Artery in a Young Woman with Ehlers-Danlos Syndrome with Segmental Dissections of the Carotid and Vertebral Arteries; Complete Reconstruction of the Internal Carotid Artery with Five Pipeline Embolization Devices; Complete Aneurysm Resolution and Good Clinical Outcome

  • Carlos Bleise
  • Rene Viso
  • Ivan Lylyk
  • Jorge Chudyk
  • Pedro LylykEmail author
Living reference work entry
  • 53 Downloads

Abstract

A 47-year-old female presented with intense headaches and oculomotor, trochlear, and abducens nerve palsy. A dissecting internal carotid artery (ICA) cavernous aneurysm associated with a dissection of the left proximal M1 segment from the proximal segment of the left ICA as well as segmental dissections of the right-hand ICA and both vertebral arteries was found. The cavernous ICA aneurysm associated with the dissection of the left ICA was treated by the endovascular implantation of five Pipeline Embolization Device (PED) flow diverters with good angiographic and clinical outcome. Placing flow diverter stents in a dissected artery allows the lacerated vessel segment to reconstruct. Vascular Ehlers-Danlos syndrome and its cerebrovascular complications is the main topic of this chapter, together with using flow diversion to treat dissected vessel segments in an acute setting.

Keywords

Internal carotid artery Flow diverter Ehlers-Danlos syndrome Telescoping flow diverters Carotid cavernous aneurysm Carotid artery dissection 

Patient

A 47-year-old female patient with a family history of cerebral aneurysms, who presented with thunderclap headache and oculomotor, trochlear, and abducens nerve palsy during a vacation abroad.

Diagnostic Imaging

On a non-contrast CT (NCCT), a hyperdense mass with erosion of the sphenoidal bone and the dorsum sellae was seen. On CT angiography (CTA), a partially thrombosed carotid cavernous aneurysm was diagnosed. Subsequent evaluation of the aneurysm by MRI and MRA was done. On axial T2WI and contrast-enhanced T1WI MRI, the thrombosed portion of the aneurysm was better visible. On the TOF MRA, slow blood flow from the left ICA could be seen, although the supraclinoid carotid artery was not shown. In the subsequent DSA examination, poor filling of the left ICA beyond the cavernous segment was seen, attributed to both compression from the partially thrombosed aneurysm to the left ICA and segmental dissection (similar to fibromuscular dysplasia) of the left cervical and supraclinoid ICA. Injecting contrast medium into the right-hand ICA revealed similar segmental dissections in the cervical segment of said artery. The right-hand intracranial ICA was supplying the left anterior circulation via the anterior communicating artery (AcomA). However, a significant delay of the arterial phase of the left anterior circulation was obvious. Injecting contrast into both vertebral arteries also revealed segmental dissections (Fig. 1).
Fig. 1

Diagnostic imaging in a female patient presenting with sudden-onset severe headache and oculomotor, trochlear, and abducens nerve palsy. NCCT shows a hyperdense mass with erosion of the sphenoidal bone and the dorsum sellae (a). On CTA a partially thrombosed carotid cavernous aneurysm becomes visible (b). T2WI axial MRI shows the intra-aneurysmal thrombus with heterogeneous signal intensity (c) with contrast enhancement of the aneurysm wall on the axial post-gadolinium T1WI (red arrow (d)). No arterial blood flow is visible on TOF MRA in the left ICA distal to the aneurysm, with preserved flow in the left ACA and MCA (e). DSA in posterior-anterior (f) and lateral (g) projection showing dysplasia of the left ICA with a partially thrombosed carotid cavernous aneurysm and reduced flow in the supraclinoid ICA and the MCA. The cervical segment of the right ICA shows multiple focal dissections, similar to “fibromuscular dysplasia” (lateral projection (h)). The left anterior circulation has some collateral supply from the right ICA via the anterior communicating artery (i) as well as through the posterior communicating artery (not shown). Both vertebral arteries share similar features to both the ICAs with multiple segmental dissections

Treatment Strategy

The aims of the treatment were to prevent further growth of the cavernous aneurysm and to reduce the mass effect of the cavernous ICA aneurysm on the adjacent cranial nerves. Given the segmental dissections of the right ICA and of both VAs, preserving the patency of left ICA was deemed crucial in order to maintain the brain supply and to avoid future ischemic events. Both ICAs and VAs appeared fragile as well as thrombogenic.

Treatment

Procedure, 15.10.2017: endovascular reconstruction of the left intracranial internal carotid artery with telescoping flow diverters and endovascular treatment of a dissecting carotid cavernous aneurysm

Anesthesia: general anesthesia; 10,000 IU unfractionated heparin (Riveparin, Ribero) IV

Premedication: 1× 100 mg ASA (Aspirin, Bayer Vital) daily for 3 days prior intervention

Access: right femoral artery, 8F sheath (Terumo); guide catheter: 6F Shuttle (Cook) and Navien A+ 058 (Medtronic); microcatheters: Excelsior SL-10 (Stryker), Marksman 0.027″ (Medtronic); microguidewire: Synchro2 0.014″ (Stryker), Chikai Black 0.014″ (Asahi Intecc), Transend 0.014″ 300 cm (Stryker)

Implants: 5× Pipeline Embolization Device (PED) – 3.25/20 mm, 3.5/20 mm, 4.5/20 mm, 4/30 mm, 5/35 mm (Medtronic)

Balloon: Minitrek 1.5/12 mm (Abbott)

Course of treatment: After the diagnostic DSA, the left ICA was catheterized with a 6F Shuttle catheter. The ICA was then catheterized with an Excelsior SL-10 microcatheter over a Synchro2 0.014″ microguidewire. After several failed attempts to pass the aneurysm neck, the Synchro2 microguidewire was replaced by a Chikai Black 0.014″ microguidewire. The aneurysm neck was crossed, and the microcatheter was placed into the left MCA (M1 segment). The Excelsior SL-10 was removed over a Transend 0.014″ 300 cm microguidewire, and a Marksman 0.027″ microcatheter was placed in the first third of the M1 segment. After the placement of said microcatheter, a DSA with contrast medium injection through the microcatheter was done to confirm the patency of the MCA. A Pipeline Embolization Device (PED) 3.5/20 mm was inserted and deployed from the left proximal M1 segment to the distal supraclinoid segment of the ICA. After deploying the first PED, the reconstruction of the ICA was carried out by telescopically placing another four PEDs from distal to proximal (3.5/20 mm, 4.5/20 mm, 4/30 mm, 5/35 mm). The subsequent DSA revealed a partial opening of the distal PED. A Mini Trek balloon catheter was inserted into the distal ICA, and angioplasty of the most distal PED was carried out. DSA then confirmed that the reconstruction of the ICA was complete and that there were both reduced flow inside the aneurysm and significant improvement of the distal flow in the left ICA and MCA (Fig. 2).
Fig. 2

Treatment of a dissecting carotid cavernous aneurysm attributed to an ICA dissection in a patient with Ehlers-Danlos syndrome with endovascular reconstruction of the left ICA. After inserting the microcatheter into the left M1 segment, contrast medium injection confirmed the position of this microcatheter in the true lumen of the left MCA/M1 (a). The first PED 3.5/20 mm was deployed from the M1 segment to the distal supraclinoid ICA (b). The endovascular reconstruction of the left ICA was continued toward the cervical segment, finishing with a PED 5/35 mm (c). A DSA run showed the complete reconstruction of the ICA (d). VasoCT confirmed the complete opening and correct wall apposition of the five PEDs (e). DSA eventually showed the improved blood flow to the left MCA and the dependent vessels (posterior-anterior projection (f)

Duration: 1st–30th DSA run: 130 min; fluoroscopy time: 60 min

Complications: none

Postmedication: 1× 100 mg ASA PO daily for life and 1× 10 mg prasugrel PO daily for 1 year

Clinical Outcome

The procedure was well tolerated, and the patient was discharged home 5 days later. The cranial nerve palsies partially resolved during the following months although diplopia persisted.

Follow-Up Examinations

Cranial MRI performed 6 months after the endovascular procedure showed shrinkage in the cavernous aneurysm and a change of the signal characteristics of the intra-aneurysmal thrombus. Follow-up DSA at 12 months showed a complete reconstruction of the left ICA with a minor neck remnant of the cavernous aneurysm and normalized distal flow in the left MCA and the ACA. Genetic studies for connective tissue disorders were carried out. A mutation in the COL3A1 coordinates NM_000090.3:c.952G > T Cp.(Gly318Cys) was detected, in line with the diagnosis of the vascular type of the Ehlers-Danlos syndrome. This genetic disorder causes a generalized fragility of arteries and is notorious for a high rate of vascular complications in catheter angiography. Therefore, no further follow-up DSA examinations were carried out. The 1-year follow-up MRI in October 2018 did not show any other manifestations of this disease, and the patient is scheduled for annual follow-up MRI/MRA examinations (Fig. 3).
Fig. 3

Follow-up MRI 6 months after the treatment of a dissecting aneurysm of the left cavernous ICA with 5 PEDs. Axial T2WI showed an increase of the signal intensity of the aneurysm (a). On post-contrast T1WI, no contrast medium accumulation of the aneurysm was seen (b). The image quality of TOF MRA was impaired due to an artifact, but no supraclinoid mass was seen, with normal signal intensity in the MCA and ACA (c). At the 12-month follow-up MRI (d), the axial T2WI showed a decreased thrombus signal with normal signal intensity in the TOF MRA (e). A 1-year follow-up DSA showed adequate distal flow in the MCA with complete reconstruction of the supraclinoid ICA and a small neck remnant of the cavernous aneurysm (f). The left cervical, petrous, and cavernous ICA was well reconstructed (g). VasoCT showed adequate apposition of the PEDs to the left ICA (h, i). 3D DSA showed the adequate vascular diameter of the complete ICA with a small neck remnant of the cavernous aneurysm (j)

Discussion

Ehlers-Danlos Syndrome (EDS) is a clinically and genetically heterogeneous group of disorders secondary to alteration in collagen metabolism, with an estimated prevalence of 1:500 to 1:250.000 births (Germain 2007). This alteration is characterized by friable soft connective tissues manifesting with alteration in the skin, ligaments, joints, blood vessels, and organs. Clinical manifestations include hyperextensibility of the skin, hypermobility of joints, atrophic scar formation after superficial injury, and premature rupture of membrane during pregnancy; however the clinical findings depend on the subtype of EDS (Eagleton 2016).

EDS classification is based on the clinical characteristics and the genetic defect (Malfait et al. 2017) and summarized in Table 1.
Table 1

Classification of the EDS

EDS type

Genetic defect

Protein affect

Clinical manifestation

Classic (I/II)

COL5A1/COL5A2

Procollagen type V

Skin and joint hypermobility, atrophic scars, easy bruising

Hypermobility (III)

Not known

Not known

Joint hypermobility and dislocations

Joint pain

Vascular (IV)

COL3A1

Procollagen type III

Thin skin. Arterial, hollow organ, and uterine rupture, small joint hyperextensibility

Kyphoscoliosis (VI)

PLOD1

Lysyl-hydroxylase-1

Hypotonia, joint laxity, congenital scoliosis, and ocular fragility

Arthrochalasia (VII a,b)

COL1A1/COL1A2

Procollagen type I

Severe joint hypermobility and scoliosis

Dermatosparaxis (VIIc)

COL1A1/COL1A2

Procollagen

N-peptidase

Severe skin fragility, cutis laxa, and easy bruising

Familial joint hypermobility syndrome

Not known

Not known

Joint hypermobility with the absence of skin hyperextensibility and atrophic scarring, excluding type I EDS

Tenascin X deficiency

TNX-B

Tenascin-X

Joint hypermobility and skin hyperextensibility; increased risk of postpartum hemorrhage

EDS progeroid form

4GALT

Galactosyltransferase I

Progeroid appearance, curly and fine hair, and periodontitis

EDS cardiac valvular form

COL1A2

Deficiency of a2

Joint hypermobility, skin hyperextensibility, and cardiac valvular defects

Vascular like

COL1A1

Procollagen type I

Classic EDS presentation with propensity for arterial rupture in adulthood

The main clinical characteristics of the classic EDS are present in varying degrees in each subtype of EDS, and the most common feature is skin hyperextensibility. However, this is not seen in the vascular type. Vascular EDS is an autosomal dominant defect in type III collagen synthesis and represents about 5% of all EDS cases (Bergqvist et al. 2013). The patients are at risk of arterial dissection, rupture, and aneurysm formation with a reduced median life expectancy of 40–50 years only due to the vascular complication (Pepin et al. 2000).

In patients with vascular complications associated with the EDS, several technical considerations have to be taken into account. The extreme fragility of the vessels is the underlying reason for further damage from medical procedures. In general, vascular clamps should be avoided because they may induce vessel transection. The use of endovascular balloon catheters can cause a vessel rupture. Bergqvist et al. (2013) reported on a total of 231 EDS patients. Half of these patients had aneurysms, and one third presented with spontaneous arterial rupture in the absence of an aneurysm. Open surgical repair was done in 44 patients with a mortality of 30%. Endovascular procedures were performed in 33 patients with a mortality rate of 24%. The complications in the surgical group were caused by intra- or postoperative bleedings. The complications of the endovascular group occurred at sites remote from the intervention.

Oderich et al. (2005) reported on 31 patients with vascular EDS, observed during 30 years (1971–2001). An angiography was performed in 42% of these patients (in 70% performed on an emergency basis), with a complication rate of 23% and with a mortality rate of 20%. In the series of Brooke et al. (2010), 40 patients with EDS had a total of 45 endovascular procedures, and 18 underwent open surgical procedures. The 5- and 10-year survival rate free of complications was 54% and 42%, respectively. However, only three of these patients had a vascular type of EDS. In the report by Cikrit et al. (1987), complications associated with angiographic procedures caused a morbidity of 67% and a mortality of 10%.

The disease frequently involves the proximal branches of the aortic arch, the descending thoracic aorta, and the abdominal aorta (Germain 2007). Possible cerebrovascular manifestations include carotid cavernous sinus fistulae, dissections of the vertebral and the carotid arteries in their extra- and intracranial segments, and intracranial aneurysms (Schievink 2004). In a report by Pepin et al. (2000), 11% of patients with EDS type IV presented with cerebrovascular complications (six carotid cavernous sinus fistulae, four intracranial aneurysms, four intracranial hemorrhages suspected to be due to intracranial aneurysms, four spontaneous internal carotid artery or vertebral artery dissections, and one suspected vertebral artery dissection). The most frequent location of the intracranial aneurysm formation is the cavernous segment of the ICA (Kim et al. 2016). North et al. (1995) reviewed the clinical data of 202 individuals with EDS type IV. A total of 19 patients presented with neurovascular complications, including 6 patients with carotid cavernous sinus fistulae, 4 ruptured intracranial aneurysms, and 4 intracranial hemorrhage of uncertain etiology.

Incidentally discovered, unruptured intracranial aneurysms are generally managed conservatively because of the extremely fragile arteries. Sultan et al. (2002) reported the case of a 46-year-old patient with an extracranial vertebral artery aneurysm, treated with a common carotid artery to V3 bypass, using reversed saphenous vein graft with incidental avulsion of the V2 segment of the vertebral artery. Since no proximal flow control was achieved, endovascular coil occlusion of the vertebral artery was required to control the bleeding with complete postoperative recovery of the patient. Schievink et al. (2002) reported on four patients with EDS who underwent open surgery. One patient died as a direct result of the operation.

The incidence of cervical carotid and vertebral artery dissection in EDS patients has been addressed in only a few studies. Brandt et al. (2001) reported on 65 patients with non-traumatic spontaneous cervical artery dissection. Skin biopsies were done on a total of 36 patients with connective tissue alterations found compatible with EDS type II or III. In a previous report on 25 patients with non-traumatic cervical dissection, 68% of the abnormalities were associated with EDS type II or III (Brandt et al. 1998).

An internal carotid artery occlusion due to a giant cavernous carotid artery aneurysm is infrequently reported in the literature. Whittle et al. (1982) reported on a patient with ophthalmoplegia and proptosis with unilateral headache due to a thrombosed cavernous ICA aneurysm and occlusion of the same ICA. The patient underwent microsurgery. Opening the aneurysm and removing the clot resulted in no improvement in the ophthalmoplegia. In a review by Sastri et al. (2013), two patients with ICA occlusion due to cavernous ICA aneurysms were presented, and a total of 15 cases from the literature were reviewed. The majority had ophthalmoparesis, facial pain, or hypoesthesia. Several pathomechanisms have been proposed in order to explain the ICA occlusion: (1) direct stretching and compression of the ICA by the giant aneurysm (Whittle et al. 1982), (2) a proximal propagation of the intramural thrombus (Inagawa 1991), or (3) compression of the ICA against the anterior clinoid process (Perrini et al. 2005). In the report of Sastri et al. (2013), the authors recommended the clinical and radiological follow-up in patients who are not acutely or severely symptomatic. The risk of ischemia is below 1% per patient year (Kupersmith et al. 2002). The term matricidal carotid artery aneurysm refers to carotid cavernous aneurysms that cause external compression and stenosis of the adjacent ICA. In a multicenter retrospective case series of Dacus et al. (2019), flow diversion was the most commonly attempted single treatment modality used to treat those aneurysms (20/40 patients) with a treatment failure rate of 30%. The parent vessel occlusion in 12 patients (4 with bypass and 8 without bypass) had a failure rate of 17%. Coil occlusion was carried out in seven patients with a failure rate of 29%. Three patients died, and two patients clinically deteriorated. The morbidity and mortality rates were 5.4% and 7.5%, respectively.

The medical management of traumatic cervical ICA dissections with anticoagulation or antiplatelet therapy yields good clinical outcomes in 75% of cases with a complete recanalization rate of 50%. In a subset of patients, however, the dissection will progress despite the medical therapy (Amuluru et al. 2017). Endovascular treatment is indicated after failure of conservative management (e.g., progressive pseudoaneurysm enlargement, acute hemodynamic infarcts). A wide variety of stents have been used to open and reconstruct the dissected segment of the ICA. The usage of dedicated carotid stents for that purpose can be technically challenging due to the rigidity of the stent delivery system. These stents can be used for proximal ICA dissections. For the petrous segment of the ICA and beyond, self- expanding stents for assisted coiling (e.g., Neuroform; Stryker) have been used prior to the availability of flow diverters (Ecker et al. 2007). Both the radial force and the hemodynamic effect of these stents may be insufficient to both keep the dissected vessel patent and to obliterate the aneurysm. Coronary balloon expandable stents have been used successfully to prevent vessel occlusion. Their wall apposition in dissected arteries is, however, poor after the resolution of the intramural hematoma. Flow diverter stents (e.g., PED, Medtronic; p64, phenox) combine advantageous flexibility, sufficient radial force, good wall apposition, and high coverage. They have become the preferred device for the endovascular treatment of dissecting ICA aneurysms (Brzezicki et al. 2016).

When a flow diverter is implanted into an acutely dissected artery, the vessel is narrow due to the intramural hematoma. The resulting vessel diameter once this hematoma is resolved is impossible to predict. A potential issue in the treatment of ICA dissections with flow diverter stents is the migration of the implanted device (Amuluru et al. 2017). Brzezicki et al. (2016) treated 11 patients with 9 traumatic and 4 spontaneous high cervical and skull base ICA dissections with the PED. They achieved complete revascularization in 91% of the treated vessels, and 75% of the aneurysms were completely obliterated at follow-up. One PED was found partially collapsed without neurological sequelae.

Cross-References

References

  1. Amuluru K, Al-Mufti F, Roth W, Prestigiacomo CJ, Gandhi CD. Anchoring pipeline flow diverter construct in the treatment of traumatic distal cervical carotid artery injury. Interv Neurol. 2017;6(3–4):153–62.  https://doi.org/10.1159/000457836.CrossRefPubMedPubMedCentralGoogle Scholar
  2. Bergqvist D, Björck M, Wanhainen A. Treatment of vascular Ehlers-Danlos syndrome: a systematic review. Ann Surg. 2013;258(2):257–61.  https://doi.org/10.1097/SLA.0b013e31829c7a59.CrossRefPubMedGoogle Scholar
  3. Brandt T, Hausser I, Orberk E, Grau A, Hartschuh W, Anton-Lamprecht I, Hacke W. Ultrastructural connective tissue abnormalities in patients with spontaneous cervicocerebral artery dissections. Ann Neurol. 1998;44(2):281–5.  https://doi.org/10.1002/ana.410440224.CrossRefPubMedGoogle Scholar
  4. Brandt T, Orberk E, Weber R, Werner I, Busse O, Müller BT, Wigger F, Grau A, Grond-Ginsbach C, Hausser I. Pathogenesis of cervical artery dissections: association with connective tissue abnormalities. Neurology. 2001;57(1):24–30.CrossRefGoogle Scholar
  5. Brooke BS, Arnaoutakis G, McDonnell NB, Black JH 3rd. Contemporary management of vascular complications associated with Ehlers-Danlos syndrome. J Vasc Surg. 2010;51(1):131–8; discussion 138–9.  https://doi.org/10.1016/j.jvs.2009.08.019.CrossRefPubMedGoogle Scholar
  6. Brzezicki G, Rivet DJ, Reavey-Cantwell J. Pipeline embolization device for treatment of high cervical and skull base carotid artery dissections: clinical case series. J Neurointerv Surg. 2016;8(7):722–8.  https://doi.org/10.1136/neurintsurg-2015-011653.CrossRefPubMedGoogle Scholar
  7. Cikrit DF, Miles JH, Silver D. Spontaneous arterial perforation: the Ehlers-Danlos specter. J Vasc Surg. 1987;5(2):248–55.CrossRefGoogle Scholar
  8. Dacus MR, Nickele C, Welch BG, Ban VS, Ringer AJ, Kim LJ, Levitt MR, Lanzino G, Kan P, Arthur AS; Endovascular Neurosurgery Research Group (ENRG). Matricidal cavernous aneurysms: a multicenter case series. J Neurointerv Surg. 2019; pii: neurintsurg-2018-014562.  https://doi.org/10.1136/neurintsurg-2018-014562.CrossRefGoogle Scholar
  9. Eagleton MJ. Arterial complications of vascular Ehlers-Danlos syndrome. J Vasc Surg. 2016;64(6):1869–80.  https://doi.org/10.1016/j.jvs.2016.06.120.CrossRefPubMedGoogle Scholar
  10. Ecker RD, Levy EI, Hopkins LN. Acute Neuroform stenting of a symptomatic petrous dissection. J Invasive Cardiol. 2007;19(5):E137–8.PubMedGoogle Scholar
  11. Germain DP. Ehlers-Danlos syndrome type IV. Orphanet J Rare Dis. 2007;2:32.  https://doi.org/10.1186/1750-1172-2-32. Review. PubMed PMID: 17640391CrossRefPubMedPubMedCentralGoogle Scholar
  12. Inagawa T. Follow-up study of unruptured aneurysms arising from the C3 and C4 segments of the internal carotid artery. Surg Neurol. 1991;36(2):99–105.CrossRefGoogle Scholar
  13. Kim ST, Brinjikji W, Lanzino G, Kallmes DF. Neurovascular manifestations of connective-tissue diseases: a review. Interv Neuroradiol. 2016;22(6):624–37.  https://doi.org/10.1177/1591019916659262.CrossRefPubMedPubMedCentralGoogle Scholar
  14. Kupersmith MJ, Stiebel-Kalish H, Huna-Baron R, Setton A, Niimi Y, Langer D, Berenstein A. Cavernous carotid aneurysms rarely cause subarachnoid hemorrhage or major neurologic morbidity. J Stroke Cerebrovasc Dis. 2002;11(1):9–14.  https://doi.org/10.1053/jscd.2002.123969.CrossRefPubMedGoogle Scholar
  15. Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, Bloom L, Bowen JM, Brady AF, Burrows NP, Castori M, Cohen H, Colombi M, Demirdas S, De Backer J, De Paepe A, Fournel-Gigleux S, Frank M, Ghali N, Giunta C, Grahame R, Hakim A, Jeunemaitre X, Johnson D, Juul-Kristensen B, Kapferer-Seebacher I, Kazkaz H, Kosho T, Lavallee ME, Levy H, Mendoza-Londono R, Pepin M, Pope FM, Reinstein E, Robert L, Rohrbach M, Sanders L, Sobey GJ, Van Damme T, Vandersteen A, van Mourik C, Voermans N, Wheeldon N, Zschocke J, Tinkle B. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175(1):8–26.  https://doi.org/10.1002/ajmg.c.31552.CrossRefPubMedGoogle Scholar
  16. North KN, Whiteman DA, Pepin MG, Byers PH. Cerebrovascular complications in Ehlers-Danlos syndrome type IV. Ann Neurol. 1995;38(6):960–4.  https://doi.org/10.1002/ana.410380620.CrossRefPubMedGoogle Scholar
  17. Oderich GS, Panneton JM, Bower TC, Lindor NM, Cherry KJ, Noel AA, Kalra M, Sullivan T, Gloviczki P. The spectrum, management and clinical outcome of Ehlers-Danlos syndrome type IV: a 30-year experience. J Vasc Surg. 2005;  https://doi.org/10.1016/j.jvs.2005.03.053.CrossRefGoogle Scholar
  18. Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342(10):673–80. Erratum in: N Engl J Med 2001 Feb 1;344(5):392.  https://doi.org/10.1056/NEJM200003093421001.CrossRefPubMedGoogle Scholar
  19. Perrini P, Bortolotti C, Wang H, Fraser K, Lanzino G. Thrombosed giant intracavernous aneurysm with subsequent spontaneous ipsilateral carotid artery occlusion. Acta Neurochir. 2005;147(2):215–6; discussion 216–7.  https://doi.org/10.1007/s00701-004-0403-4.CrossRefPubMedGoogle Scholar
  20. Sastri SB, Sadasiva N, Pandey P. Giant cavernous carotid aneurysm with spontaneous ipsilateral ICA occlusion: report of 2 cases and review of literature. J Neurosci Rural Pract. 2013;4(Suppl 1):S113–6.  https://doi.org/10.4103/0976-3147.116439.CrossRefPubMedPubMedCentralGoogle Scholar
  21. Schievink WI, Link MJ, Piepgras DG, Spetzler RF. Intracranial aneurysm surgery in Ehlers-Danlos syndrome type IV. Neurosurgery. 2002;51(3):607–11; discussion 611–3.CrossRefGoogle Scholar
  22. Schievink WI. Cerebrovascular involvement in Ehlers-Danlos syndrome. Curr Treat Options Cardiovasc Med. 2004;6(3):231–6.CrossRefGoogle Scholar
  23. Sultan S, Morasch M, Colgan MP, Madhavan P, Moore D, Shanik G. Operative and endovascular management of extracranial vertebral artery aneurysm in Ehlers-Danlos syndrome: a clinical dilemma–case report and literature review. Vasc Endovasc Surg. 2002;36(5):389–92.  https://doi.org/10.1177/153857440203600510.CrossRefGoogle Scholar
  24. Whittle IR, Williams DB, Halmagyi GM, Besser M. Spontaneous thrombosis of a giant intracranial aneurysm and ipsilateral internal carotid artery. Case report. J Neurosurg. 1982;56(2):287–9.  https://doi.org/10.3171/jns.1982.56.2.0287.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Carlos Bleise
    • 1
  • Rene Viso
    • 1
  • Ivan Lylyk
    • 1
  • Jorge Chudyk
    • 1
  • Pedro Lylyk
    • 1
    Email author
  1. 1.Clinica La Sagrada Familia, ENERIBuenos AiresArgentina

Personalised recommendations