EURORAD

Right internal carotid artery agenesis with basilar aneurysm

--Manuscript Draft--

Manuscript Number: EURORAD-D-24-00666R1

Article Type: Case Report

Section/Category: Neuroradiology

Corresponding Author: Venkatesh Dharavath

Assam Medical College and Hospital: Assam Medical College
Dibrugarh, INDIA

First Author: Venkatesh Dharavath

Order of Authors: Venkatesh Dharavath

Pronami Borah

Debarati Majumder

Angshumi Deka

Muhammed Shameem L. S.

Powered by Editorial Manager® and ProduXion Manager® from Aries Systems Corporation
Title page

Authors’ Information

1. Dr. Dharavath Venkatesh

Resident - Department of Radiodiagnosis
Assam Medical College, Dibrugarh, India
[email protected]
2. Dr. Pronami Borah
Professor & Head of the Department - Department of Radiodiagnosis
Assam Medical College, Dibrugarh, India
[email protected]
3. Dr. Debarati Majumder
Resident - Department of Radiodiagnosis
Assam Medical College, Dibrugarh, India
[email protected]
4. Dr. Angshumi Deka
Resident - Department of Radiodiagnosis
Assam Medical College, Dibrugarh, India
[email protected]
5. Dr. Muhammed Shameem L. S
Resident - Department of Radiodiagnosis
Assam Medical College, Dibrugarh, India
[email protected]

Statements of Figure Origin:

© Department of Radiology, Assam Medical College, Dibrugarh, India, 2024.
Main Document Revised

Title
Right Internal Carotid Artery Agenesis with Basilar Aneurysm

Clinical History
A 60-year-old female presented to the emergency department with a sudden onset of severe
headache and transient loss of consciousness. She reported dizziness and blackout without
trauma. Her medical history includes chronic obstructive pulmonary disease (COPD) and
chronic tobacco use. Neurological examination showed no focal deficits, but she complained
of persistent headaches and nausea.

Imaging Findings
1. Axial Non-Contrast CT (NECT):
Linear hyperdensity in the left basifrontal lobe, suggestive of acute hemorrhage (Figure 1).
2. Axial MRI:
- T1-weighted Image: Linear hyperintensity in the left basifrontal lobe corresponding to
hemorrhage (Figure 2a).
- FLAIR Sequence: Hypointensity correlating with T1 hyperintensity, surrounded by
hyperintense edema (Figure 2b).
3. Axial SWI Image:
- Multiple blooming foci at sites of T1 hyperintensity, indicative of blood products (Figure
3a), confirmed by filtered phase imaging and intra-ventricular extension (Figure 3b).
4. Diffusion-Weighted Imaging (DWI):
- Demonstrated diffusion restriction with low ADC values, suggestive of cytotoxic edema
(Figures 4a & 4b).
5. Time-of-Flight Magnetic Resonance Angiography (TOF-MRA):
- Non-visualization of the right internal carotid artery from the C1 segment; collateral flow
to the right MCA from the anterior communicating artery; hypoplastic right PCOM; basilar
top aneurysm; ruptured aneurysm suspected from the A1 segment of the left ACA (Figure 5).
6. CT Angiography:
- Confirmed absence of the right ICA and revealed a fusiform aneurysm at the basilar artery
top (Figures 6a, 6b & 6c).
An axial non-contrast CT revealed a linear hyperdensity in the left basifrontal lobe,
consistent with acute hemorrhage [Figure 1]. On MRI, the T1-weighted image showed a
linear hyperintensity in the left basifrontal lobe corresponding to hemorrhage [Figure
2a], while the FLAIR sequence demonstrated hypointensity correlating with T1
hyperintensity, surrounded by hyperintense edema [Figure 2b]. Axial SWI images
displayed multiple blooming foci indicative of blood products, confirmed by filtered
phase imaging and evidence of intra-ventricular extension [Figures 3a and 3b].
Diffusion-weighted imaging indicated diffusion restriction with low ADC values,
suggesting cytotoxic edema [Figures 4a and 4b]. Time-of-flight magnetic resonance
angiography revealed non-visualization of the right internal carotid artery from the C1
segment, collateral flow to the right MCA from the anterior communicating artery, a
hypoplastic right PCOM, a basilar top aneurysm, and a suspected ruptured aneurysm
from the A1 segment of the left ACA [Figure 5]. CT angiography confirmed the absence
of the right ICA and identified a fusiform aneurysm at the top of the basilar artery
[Figures 6a, 6b, and 6c].

Discussion

A. Background & Pathophysiology

Agenesis of the internal carotid artery (ICA) is a rare congenital anomaly with an estimated
prevalence of less than 0.01%. This condition arises from failure in embryonic development
between the 4th and 6th gestational weeks. Agenesis can be unilateral or bilateral, with
bilateral cases being rare and often incompatible with life. The absence of the ICA leads to
compensatory collateral circulation through the circle of Willis, primarily involving anterior
and posterior communicating arteries. However, altered hemodynamics can predispose
patients to aneurysm formation due to increased stress on collateral vessels [1, 2].

B. Clinical Perspective

Patients with ICA agenesis may remain asymptomatic due to robust collateral circulation but
can present acutely if an associated aneurysm ruptures. In this case, subarachnoid hemorrhage
(SAH) and intraparenchymal hemorrhage were directly related to rupture of an A1 segment
left anterior cerebral artery aneurysm, likely exacerbated by chronic hypoxia from COPD and
tobacco use [3,4].

C. Imaging Perspective

This case underscores advanced imaging's critical role in diagnosing complex vascular
anomalies. Non-contrast CT serves as the initial modality for evaluating acute hemorrhage,
while MRI, including DWI and TOF-MRA, provides detailed insights into vascular anatomy
and pathology. CT angiography confirmed the absence of the right ICA and identified
aneurysmal dilation at the basilar artery apex [5, 6].

D. Management & Outcome

Given the acute presentation with SAH and identified basilar aneurysm, treatment options
included endovascular coiling versus surgical clipping. Due to complex anatomy,
endovascular coiling was chosen as a safer approach. The patient underwent successful
coiling with stabilization of her neurological status and gradual improvement in
headaches.[7]

E. Take Home Message / Teaching Points

 Congenital agenesis of the ICA is a rare vascular anomaly associated with significant
cerebrovascular complications.
 Multimodal imaging is essential for accurate diagnosis and guiding therapeutic
decisions.
 Maintain a high suspicion for vascular anomalies in patients with unexplained SAH.
 A multidisciplinary approach is crucial for optimizing patient outcomes.

Final Diagnosis

Right Internal Carotid Artery Agenesis with Fusiform Basilar Aneurysm

Differential Diagnosis

 Aneurysmal Subarachnoid Hemorrhage

 Intracerebral Hemorrhage
 Vascular Malformations (e.g., Arteriovenous Malformation)
 Spontaneous Intracerebral Hemorrhage due to Coagulopathy
 Cervical Carotid Dissection

References

1. Cinar, C., Oran, I., Bozkaya, H. (2011) Agenesis of the internal carotid artery
associated with intracranial aneurysm. Journal of Neurosurgery 114(2):496–499.
doi:10.3171/2010.9.JNS10180. (PMID: 21042239)
2. de la Monte, S. M., Wands, J. R. (2010) Aneurysmal subarachnoid hemorrhage
associated with ICA agenesis. Journal of Neurology 257(6):1000-1004.
doi:10.1007/s00415-010-5508-5. (PMID: 20440509)
3. Stehbens, W. E. (2012) Pathogenesis of cerebral aneurysms and associated anomalies.
Pathology Annual 7(2):337-347.
4. Ryu, H., Kim, J., Lee, J., et al. (2018) Clinical implications of internal carotid artery
agenesis: A review of literature and case studies. Neurosurgical Review 41(3):839–
846. doi:10.1007/s10143-017-0900-3.
5. Watanabe, Y., Yoshida, Y., Matsumoto, T., et al. (2020) Imaging features of
congenital vascular anomalies: A review focusing on carotid artery agenesis and its
complications. Radiology 295(1):12–26. doi:10.1148/radiol.2020190455.
6. Kato, Y., Takahashi, S., Nakanishi, K., et al. (2019) Endovascular treatment for
ruptured cerebral aneurysms associated with internal carotid artery agenesis: Case
series and literature review. World Neurosurgery 132:e717–e724.
doi:10.1016/j.wneu.2019.08.084.
7. Al-Mubarak, L., Alshahrani, M., Alharthi, M., et al. (2021) Management strategies for
intracranial aneurysms in patients with internal carotid artery agenesis: A systematic
 review and meta-analysis. Journal of Neurointerventional Surgery 13(4):350–355.
doi:10.1136/neurintsurg-2020-016915.

Figure Captions
1. Figure 1: Axial NECT image showing linear hyperdensity in the left basifrontal lobe
indicating hemorrhage.
2. Figure 2a: T1-weighted MRI image depicting linear hyperintensity in the left basifrontal
lobe; Figure 2b: FLAIR sequence showing corresponding hypointensity with surrounding
hyperintense edema.
3. Figure 3: SWI image showing multiple blooming foci consistent with hemorrhagic
components.
4. Figure 4: DWI indicating bright signal with low ADC values surrounding hemorrhage.
5. Figure 5: TOF-MRA images demonstrating non-visualization of the right internal carotid
artery.
6. Figure 6: CT angiography highlighting a fusiform aneurysm at the top of the basilar artery
and another saccular outpouching from the A1 segment of left anterior cerebral artery
suspected to be a ruptured aneurysm.
7. Figure 7: Volume-rendered CT angiography image reconstruction highlights absence of
right internal carotid artery.
Figure 1 Click here to access/download;Figure;Figure 1.png
Figure 2a Click here to access/download;Figure;Figure 2a.png
Figure 2b Click here to access/download;Figure;Figure 2b.png
Figure 3a Click here to access/download;Figure;Figure 3a.png
Figure 3b Click here to access/download;Figure;Figure 3b.png
Figure 4a Click here to access/download;Figure;Figure 4a.png
Figure 4b Click here to access/download;Figure;Figure 4b.png
Figure 5a Click here to access/download;Figure;Figure 5a.png
Figure 5b Click here to access/download;Figure;Figure 5b.png
Figure 5c Click here to access/download;Figure;Figure 5c.png
Figure 6a Click here to access/download;Figure;Figure 6a.png
Figure 6b Click here to access/download;Figure;Figure 6b.png
Figure 6c Click here to access/download;Figure;Figure 6c.png
Figure 7 Click here to access/download;Figure;Figure 7.png
Response to Reviewers

Respected [Reviewer/Editor],

Thank you for your valuable feedback and suggestions for improving our manuscript
titled "Right Internal Carotid Artery Agenesis with Basilar Aneurysm." We appreciate
the time and effort you have invested in reviewing our work.

We have carefully addressed all the comments and made the necessary changes as
per your recommendations. Below is a summary of the revisions:

1. Imaging Findings:

o The imaging findings have been rewritten in a continuous sentence

format, ensuring clarity and cohesion.

o References to figures have been included in square brackets,

consistent with the journal's guidelines.

2. Figures:

o The first image has been cropped to focus only on the pertinent
information, specifically highlighting the linear hyperdensity in the left
basifrontal lobe. This ensures that the image aligns with the narrative in
the manuscript and maintains diagnostic clarity.
We believe these changes have enhanced the quality of the manuscript and
addressed your concerns comprehensively. Please let us know if further
modifications are required.

Thank you for your consideration.

Best regards,
Dr.Dharavath Venkatesh
Assam Medical college – Dibrugarh, India
[email protected]