DOI: https://doi.org/10.21203/rs.2.158/v1
Background: To report a case of a cerebral Arteriovenous Malformation(AVM) with eye symptoms firstly and review the characteristic of this case and the main confusing point for diagnosis of such case.
Case presentation: A 58-year-old women presented to the ophthalmology clinic with 1 and an half year of right eye redness, Ocular hypertension and recurrent headache. One and a half year ago she was diagnosed as right eye dry eye and glaucoma and had received treatment according to diagnosis, however all the treatment did not lead to any improvement of redness and headache. On physical examination, it revealed dry eye and severe corkscrew hyperemia with dilated vessels in the right eye. After we consider that the symptom may be related to intracranial abnormal vessels, Computed tomography angiography and venography (CTA+CTV) were performed and the results showed an arteriovenous malformation (AVM) of right parietal-occipital area in the brain. The AVM was definitely located by the further examination of Digital subtraction angiography (DSA). After the AVM endovascular embolism treatment, the conjunctival congestion of the right eye was significantly relieved and the intraocular pressure was decreased to normal.
Conclusion: In clinical practice, when found corkscrew hyperemia accompanied by neurological symptoms, it might be considered as a result from cerebral vessels diseases, so in this case ophthalmologists diagnosis should combine disease history and imaging examination.
Key word: cAVM, DSA, hyperemia, glaucoma
Cerebral arteriovenous malformation(cAVM) is a cluster of vascular masses developed from congenital cerebrovascular dysplasia with dys differentiation at the early stage of embryos. And the acquired factors which lead to cerebral arteriovenous malformation includes specific diseases or external interventions and these could cause the malfunction of cerebral angiogenesis or change the normal physiological structures of cerebral vessels. It’s reported that the cAVM symptoms occurrence rate of the intracranial hemorrhage, headache, dizzy were 43.4%, 17.3% and 24.9% respectively, while headache was the second leading symptom[1]. Furthermore, differences in the part of the cAVM can cause symptoms in the associated organs. Omer Faith Nas[2] had reported one case that a patient with a space occupying lesion compatible with pial AVM in the right occipital region one year ago had symptoms of blurred vision, dizziness, nausea, and headache localized to the occipital region. This reminds us that intracranial vascular lesions can also cause related ophthalmic symptoms.
The superior ophthalmic vein collects the blood from anterior ciliary vein, venae vorticosae, venae centralis retinae. Due to the AVM is directly or indirectly connected to the superior ophthalmic vein, the high pressure of the superior ophthalmic vein prevents the aqueous fluid, blood of sclera and conjunctiva from flowing back. Furthermore, it will lead to severe conjunctival injection, such as increasing intraocular tension, enlarged and tortuous veins of conjunctiva and sclera.
A 58-year-old women presented to the ophthalmology clinic with 1 and an half year of right eye redness and ocular hypertension. Tracing back her history, she got recurrent headache for several years without history of head trauma. She was diagnosed as right eye dry eye and glaucoma and received treatment including NSAID, immunosuppressive therapy, prostaglandin analogue mix β-blocker for eye pressure reducing, even embolization of lacrimal punctum. However all the treatment only lead to slightly decreasing of intraocular pressure with no improvement in eye redness and headache. On examination, visual acuity and intraocular pressure in the right eye of the patient were 20/40 and 20 mmHg after the mentioned treatment, while the other eye were 20/25 and 14mmHg. On physical examination, it revealed exophthalmos and severe corkscrew hyperemia with dilated vessels in right eye. The results of fundus examination of both eye were normal.
After we consider that the symptom may be related to intracranial abnormal vessels, Computed tomography angiography and venography (CTA+CTV) were performed and the results show a arteriovenous malformation (AVM) of right parietal-occipital area in the brain. The AVM was definitely located by further examination Digital subtraction angiography (DSA). It revealed that the AVM is fed by posterior cerebral artery and pericallosal artery and is drained to the medial atrial vein. Then this malformed venousP structure would lead the arterial blood flowing into superior ophthalmic vein(Fig 1).
With the result, patient was ultimately diagnosed with the “Occipital lobe cerebral Arteriovenous Malformation”, and surgical treatment was given.
After general cerebral angiography probe reaching the right occipital lobe AVM embolism, blocking the arteriovenous malformation mass, this lead to restoring normal blood flow of the superior ophthalmic vein, in which the conjunctival congestion of the right eye was significantly relieved(Fig2) and the intraocular pressure was decreased to normal(14-15mmHg).
Aetiology and classification
Cerebral arterial malformations are congenital vascular anomalies. These can present with bleeding and compression effects on surrounding tissues.[2] The authors believe that AVM will gradually cause different degrees of symptoms with age and deterioration of vascular conditions. Due to the absence of capillaries between cerebral veins and cerebral arteries in the area of cerebrovascular diseases, arteriovenous communication and vascular regulation mechanism barriers are caused, leading to cerebral blood vessel flow turbulence. In this case, due to the presence of arteriovenous malformation of right parietal-occipital lobe, the blood flow in arteries passed through local internal atrial veins via superior petrosal sinus then back to jugular veins, then flow backward to the superior ophthalmic vein, causing the high pressure of the superior ophthalmic vein. In this case, early CTA+CTV examination revealed that the right superior ophthalmic veins were enlarged and tortuous, which was in accordance with such change. Since the drainage vessel on both sides were not connected, the affected eye of the patient was on the same side as the malformation mass and only one side was affected.
Clinical presentation and differential diagnosis
The main clinical features of cAVM including intracranial hemorrhage, headache, dizzy and convulsion, as well as neurological dysfunction, brain tissue swelling, etc.. Because the patient didn’t have any history of obvious craniocerebral trauma, it was not considered that the eye disease was caused by intracranial vascular lesions, hence relative imaging examinations were not conducted. Therefore, the patient was once mistakenly diagnosed as "1. Dry Eye in right eyes; 2. Glaucoma in the right eye". Firstly, cAVM and dry eye can be differentiated by corkscrew hyperemia, and the dry eye would not cause recurrent headache. Secondly, cAVM can result in increased intraocular pressure, orbital pain, headache, but without typical keratic precipitates (K.P) and iridociliary disorder, which distinguish this condition from secondary glaucoma. Furthermore, the common anti-glaucoma therapy was not effective for this case. Conjunctival congestion also occurs frequently in various types of conjunctivitis. which is usually characterized by dilation of blood vessels away from the limbus of the cornea. And conjunctivitis are not accompanied by increased intraocular pressure.
After the several ineffective treatment, we considered about intracranial vascular disease such as Carotid-cavernous fistula (CCF). CCF refers to an aberrant connection between the internal carotid artery (ICA), the external carotid artery (ECA) or any of their branches with the cavernous sinus [3]. The symptoms and signs of CCF always include eyelid swelling, proptosis, chemosis, and corkscrew hyperemia, which is similar to this case. At present, the diagnosis of cAVM and CCF mainly depends on digital subtraction angiography(DSA), which is considered to be the golden standard for the diagnosis of cerebral artery malformation and can be used to identify CCF and AVM. But since it’s an invasive examination and costly, it is not appropriate for the requirement of early diagnosis purpose. CTA+CTV can clearly show the 3D structure of the malformation mass, as well as locating the position of the lesions precisely, meanwhile the conditions of cranial arteries and veins as well as the flowing direction of draining veins can be shown clearly[4]. The mature application of combined techniques including CTA, CTV and MRI have provided visualized 3D images of the relation between lesions and their surrounding structures for clinicians, which is more suitable for early diagnosis.
Treatment
At present, the treatment methods of arteriovenous malformation mainly include excision by microsurgery, endovascular embolism, stereotactic radiotherapy. Endovascular embolization has been playing an increasingly important role in AVM therapy due to its convenience and minimally invasive features[5]. Due to it’s less invasive and causes less damage to the brain, the interventional embolization via vessels used in this case has become the main treatment method for arteriovenous malformation[6].
Many patients only present clinically with neurological symptoms, therefore when the ophthalmic symptoms occurred ophthalmologists would not consider intracranial disease. In clinical practice, when found corkscrew hyperemia accompanied by neurological symptoms, it might be considered as a result from cerebral vessels diseases, so in this case ophthalmologists diagnosis should combine disease history and imaging examination.
In conclusion, it’s hard for ophthalmologist to give a diagnosis “cAVM” without any imaging examination, also careful examination of medical history is a necessary part of diagnosing a disease. According to the medical history, ophthalmologist should conduct radiography examination to decide upon conservative treatment or embolization surgery to delay disease development of cAVM, prevent severe complications such as intracranial hemorrhage. Additionally, a cAVM can result in eye symptoms in which situation is very unusual but necessary for ophthalmologist to pay attention to.
cAVM: cerebral Arteriovenous Malformation; CTA: computed tomography angiography; CTV: computed tomography venography; DSA: digital subtraction angiography; K.P: keratic precipitates; CCF: carotid-cavernous fistula; ICA: internal carotid artery; ECA: external carotid artery; MRI: magnetic resonance imaging
Ethics approval and consent to participate
This study followed the tenets of the Declaration of Helsinki and approved by the Ethics Committee of the First Affiliated Hospital of Jinan University. Written informed consent was obtained from the participant.
Consent for publication
Written informed consent was obtained from the patient for publication of this case and any accompanying images. A copy of written consent is available for review by the Executive Editor of this journal.
Availability of data and materials
All data have been presented within the manuscript and in the form of images.
Competing Interest
The authors declare no conflicts of interest.
Funding
Not applicable
Authors’ contributions
SS is responsible for acquisition of the clinical information and writing up of the manuscript. XL and JC are responsible for reviewing the manuscript. CY and CS are responsible for explainations of all the angiographic results, and reviewing the manuscript. Qing Zhou is responsible for reviewing the manuscript. All authors read and approved the final manuscript
Acknowledgements
We would like to thank the patient involed in this study. We also thank to Dr. Chengyou Yang and Changzheng Shi for explainations of all the angiographic results. This work was supported by Department of Ophthalmology, the First Affiliated Hospital, Jinan University, Guangzhou, China.
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