Basilar aneurysm and coarctation of the aorta

Shrikant K Kalbagwar; Pandurang S Barve; Trimurti D Nadkarni

doi:10.4103/jcvs.jcvs_27_20

CASE REPORT

Year : 2020 | Volume : 8 | Issue : 2 | Page : 123-125

Basilar aneurysm and coarctation of the aorta

Shrikant K Kalbagwar, Pandurang S Barve, Trimurti D Nadkarni
Department of Neurosurgery, Topiwala National Medical College, B.Y.L. Nair Ch. Hospital, Mumbai, Maharashtra, India

Date of Submission	07-Nov-2020
Date of Acceptance	28-Nov-2020
Date of Web Publication	3-Feb-2021

Correspondence Address:
Prof. Trimurti D Nadkarni
Department of Neurosurgery, Topiwala National Medical College, B.Y.L. Nair Ch. Hospital, Mumbai, Maharashtra
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcvs.jcvs_27_20

Abstract

A 31-year-old male presented with subarachnoid haemorrhage due to a basilar tip aneurysm associated with an incidental coarctation of the aorta (CoA). The aneurysm was treated with endovascular coiling. Patients with CoA frequently harbour intracranial aneurysms at a young age. The occurrence of basilar tip aneurysm associated with CoA is extremely rare. The relevant literature on the subject is discussed.

Keywords: Basilar tip, coarctation of the aorta, intracranial aneurysm

How to cite this article:
Kalbagwar SK, Barve PS, Nadkarni TD. Basilar aneurysm and coarctation of the aorta. J Cerebrovasc Sci 2020;8:123-5

How to cite this URL:
Kalbagwar SK, Barve PS, Nadkarni TD. Basilar aneurysm and coarctation of the aorta. J Cerebrovasc Sci [serial online] 2020 [cited 2023 Feb 4];8:123-5. Available from: http://www.jcvs.in/text.asp?2020/8/2/123/308632

Introduction

Coarctation of the aorta (CoA) is a disorder accounting for 10% of the cases of congenital heart disease, and the most common site of narrowing is distal to the left subclavian artery. Due to the mechanical outflow obstruction and extensive collateral formation, a number of haemodynamic changes are seen in CoA. Intracranial aneurysms (IAs) are associated with CoA in 2%–10% of the cases, of which 2.7% of the patients die from aneurysmal rupture.^[1] The frequency of IA amongst patients with CoA is approximately 5-fold that of the general population, and these IAs occur most commonly in the anterior circulation.^[2]

Case Report

A 31-year-old male with no comorbidities presented with the chief complaint of sudden onset of severe headaches for 2 days, associated with multiple episodes of vomiting. On examination, the patient had no neurological deficit except terminal neck stiffness. Multiple blood pressure readings confirmed that the patient had hypertension, which was controlled with anti-hypertensive medications. Two-dimensional echocardiography revealed chronic changes of hypertensive heart disease with a left ventricular ejection fraction of 60%. Computed tomography (CT) images of the brain revealed subarachnoid haemorrhage (SAH) in the basal cisterns bilaterally including both Sylvian fissures and posterior fossa cisterns [Figure 1]. An intraventricular extension of blood was noted causing acute non-obstructive hydrocephalus. Three-dimensional (3D) reconstructed images on CT angiogram showed a saccular aneurysm measuring 5.4 mm × 5.7 mm at the basilar artery bifurcation [Figure 2]. It was also noted that all pulsations in the lower limbs were feeble as compared to those of the upper extremities. During the endovascular procedure, it was difficult to negotiate the angiocatheter through the descending aorta into the proximal arch of the aorta. An aortogram demonstrated an incidental CoA distal to the left subclavian artery [Figure 3]. The patient underwent endovascular coiling of the basilar artery aneurysm with complete obliteration of the aneurysm [Figure 4] and [Figure 5]. The patient made an uneventful recovery.

Figure 1: Plain computed tomography scan of the brain showing subarachnoid haemorrhage in both Sylvain fissures and basal cisterns

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Figure 2: Three-dimensional reconstructed computed tomography angiogram demonstrating a basilar tip aneurysm

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Figure 3: Aortogram performed during endovascular procedure. There is a narrowing noted that obstructed the smooth passage of angiocatheter into the arch of the aorta suggestive of coarctation of the aorta

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Figure 4: Right vertebral artery angiogram (a) lateral view, (b) Towne's view that defines the basilar tip aneurysm

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Figure 5: Post-coiling right vertebral artery angiogram (a) lateral view, distal to the left subclavian artery (b) Towne's view that shows complete obliteration of the basilar tip aneurysm from circulation

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Post endovascular procedure, a 3D CT aortogram confirmed the CoA [Figure 6]. A short-segment narrowing was noted in the descending thoracic aorta distal to the left subclavian artery. The diameter of the aorta proximal to the narrowing measured 2 cm, that distal to narrowing measured 2.4 cm and at the aortic narrowing (CoA) measured 9 mm. The patient was planned for the treatment of coarctation of the aorta.

Figure 6: Three-dimensional computed tomography aortogram that demonstrates the coarctation of the aorta distal to the left subclavian artery

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Discussion

IA rupture with coarctation occurs much earlier in life (mean: 25 years) than without coarctation (mean: 50–54 years).^[3] The incidence of SAH from a cerebral aneurysm in young patients with coarctation ranges from 12% to 23% as compared to an occurrence of 49.4 per 100,000 per year in patients without coarctation.^[3] This suggests that CoA is an important cause of subarachnoid haemorrhage in young patients. The incidence of aneurysms observed in patients with coarctation was 5-fold more than that expected in the general population.^[2]

There are two theories proposed regarding the pathophysiology of association of aortic coarctation and IAs. The first suggests that there is a common inherited pathogenic factor responsible for these two lesions^[4] and the second is inadequately controlled hypertension as the main cause.^[5]

The myocardial and cervicocephalic arteries and the aortic arch all arise from embryonic neural ridge tissue called the neural crest. An error in neural crest development may be the common factor in the association between IA and CoA.^[4]

The degree and duration of high blood pressure affect cerebral anatomy and autoregulation of cerebral blood flow increasing the likelihood that IAs will develop. In CoA patients, narrowing of the aortic lumen usually occurs distal to the origin of the left subclavian artery, leading to upper extremity and intracranial hypertension. Spikes in blood pressure can spur the IAs' growth. A study done by Curtis et al. showed that most IAs in adult patients with CoA were small and at low risk of rupture.^[4]

Stehbens was the strongest proponent of theory of the importance of hypertension in development of IAs based on two observations.^[5] Hypertension is often a constant feature in CoA patients, and it has been considered as a major risk factor for the development of IAs even without CoA.

Cerebral arteries have direct origin from the pre-coarctation segment of the aorta; therefore, these arteries are affected by the haemodynamic changes occurring in this part of the aorta. There are increased aortocranial pressure gradients due to the increased resistance to outflow which correlates to the severity of stenosis.^[1] The increase in pressure gradient causes dilatation of cervicocephalic arteries in CoA.^[1]

The location of IAs is variable with CoA, and anterior circulation aneurysms are relatively more common than posterior circulation. Our literature search has yielded only 10 patients who had basilar tip aneurysms associated with CoA.^{[2],[3],[4],[6],[7],[8],[9],[10]}

The treatment strategy of repairing the CoA first has a high risk of perioperative rebleeding from the cerebral aneurysm. In addition, aggressive hypertensive therapy for vasospasm was not possible. Recent advances in anaesthesia, perioperative management and endovascular neurosurgical techniques allow treatment of the ruptured cerebral aneurysm in the acute phase in the presence of untreated coarctation with relative safety as noted in our case. The authors recommend that the ruptured aneurysm should be treated as early as possible before correcting the coarctation. Even incidental unruptured aneurysms should also be treated before aortic repair. However, in case of severe heart failure, the coarctation may be repaired first.

Conclusion

IAs are commonly associated with CoA in a younger age group. The occurrence of basilar tip aneurysms is rare. In known patients of coarctation, imaging of cerebral vasculature is recommended to rule out intracranial aneurysm.

Cerebral aneurysm should be treated first unless patients had heart failure due to coarctation.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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