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
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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.
KeywordsInternal carotid artery Flow diverter Ehlers-Danlos syndrome Telescoping flow diverters Carotid cavernous aneurysm Carotid artery dissection
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.
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.
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)
Duration: 1st–30th DSA run: 130 min; fluoroscopy time: 60 min
Postmedication: 1× 100 mg ASA PO daily for life and 1× 10 mg prasugrel PO daily for 1 year
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.
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).
Classification of the EDS
Procollagen type V
Skin and joint hypermobility, atrophic scars, easy bruising
Joint hypermobility and dislocations
Procollagen type III
Thin skin. Arterial, hollow organ, and uterine rupture, small joint hyperextensibility
Hypotonia, joint laxity, congenital scoliosis, and ocular fragility
Arthrochalasia (VII a,b)
Procollagen type I
Severe joint hypermobility and scoliosis
Severe skin fragility, cutis laxa, and easy bruising
Familial joint hypermobility syndrome
Joint hypermobility with the absence of skin hyperextensibility and atrophic scarring, excluding type I EDS
Tenascin X deficiency
Joint hypermobility and skin hyperextensibility; increased risk of postpartum hemorrhage
EDS progeroid form
Progeroid appearance, curly and fine hair, and periodontitis
EDS cardiac valvular form
Deficiency of a2
Joint hypermobility, skin hyperextensibility, and cardiac valvular defects
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.
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