Analysis of clinical features of ocular presentation in cranial venous sinus thrombosis
© I. Holzapfel Publishers 2011
Received: 23 March 2011
Accepted: 25 May 2011
Published: 25 July 2011
To recognize ocular presentations in cranial venous sinus thrombosis (CVST) which were easy to be misdiagnosis.
Review clinical informations including general informations, general performances, and ocular presentations of 118 inpatients with CVST in the general hospital of chinese people's liberation army during 2005-2009.
Main Outcome Measures
The ocular symptoms as the initial onset presentations or simultaneous phenomenon among different onset type patients were analyzed.
Of all the CVST patients, 21.2% (25/118) presented with ocular symptom as the initial presentation, 30.5% (36/118) presented with ocular symptom as well as the other symptoms, and 48.3% (57/118) presented with non-ocular symptoms as the initial onset. The CVST patients were divided into 3 groups according to the onset type. There was no marked statistical significance among groups. The most common major complaints were blurring and degeneration of acute vision, accounting for 85.9% (61/71) of all abnormal ocular chief complaints. The most common objective sign in eyes was papilloedema, accounting for 48.3% (57/118) in this group of CVST patients. About 22.4% (13/58) showed acute vision deterioration at 1-year follow-up, due to optic atrophy.
As ophthalmologists, we should master the onset characteristics and clinical manifestations of CVST. Early diagnosis and treatment is very important for the prevention of vision deterioration, especially for patients with ocular syndrome as the initial onset syndrome. For isolated agnogenic intracranial hypertension, we should consider the possibility of CVST.
Cranial venous sinus thrombosis (CVST) is a specific type of cerebrovascular disease. Patients present signs of intracranial hypertension, such as headache, vomiting, and papilloedema, which are common clinical manifestations. Because ocular onset presentation is the most common symptom, some patients may be misdiagnosed or a missed diagnosis may occur if only ocular onset presentation occurs as the initial onset symptom. In recent years, the popularity of some technical applications, including magnetic resonance imaging (MRl), magnetic resonance venography (MRV), and digital subtraction angiography (DSA) has resulted in ongoing improvements in diagnosis and increased levels of awareness of this disease. Although there are many reported clinical observations of CVST [1–4], there are few reports on the study of the interrelationship between the clinical manifestations and ocular onset presentation. To this end, we collected the information of 118 inpatients with CVST and treated the data with a retrospective analysis for the period 2005-2009. We expected that it would provide more clinically significant data for the early diagnosis and treatment of CVST.
Initial presentation with different onset style of 118 patients
Ocular with other Number
DSA with high sensitivity is the key standard of diagnosis of CVST: Accuracy ranges from 75% to 100% . Although MRI and MRV have some advantages for checking venous sinus thrombosis, such as being non-invasive, having repeatability and high-resolution, they are not effective with smaller vascular thromboses. Diagnosis using MRI and MRV is difficult also because of the anatomical variants of venous sinus . DSA not only displays the filling state of venous sinus (including symptoms of venous sinus filling defect, underdevelopment, backflow, and anastomosed varicose vein), but also can analyze the cycle time of cerebral arteriovenous cycles in the arterial phase, capillary phase, venous phase, and sinus period to confirm which period is delayed when there is an extension of the total cycle time. Because all the patients received the DSA examination to make a definite diagnosis, so the patients we enrolled were accurate and credible. The average age of onset for these patients is 34.9 ± 10.6 years, and it affects slightly more females than males-match with the reported literatures [7, 8]. Females aged from 20 to 35 are most commonly affected which may relate to pregnancy, childbirth, and use of oral contraceptive according to other reports . In this study, we do not make any further analysis of the relevant factors of this phenomenon, but there was a study showed that CVST was related to antiphospholipid antibody syndrome .
The clinical manifestation of CVST is complex and non-specific. Its main symptoms are intracranial hypertension and cortical damage. Headache is the most common symptom, accounting for 74-90% of CVST patients; 70-75% of this group presented with headache as the initial onset symptom [3, 10]. However, 20%-40% of the CVST patients only presented with benign intracranial hypertension . over 75% of patients in this group mainly complained of serious headache, and this corresponds to the reports. It should be noted that 21.2% of CVST patients presented with ocular syndrome as the initial onset and 30.5% of the CVST patients presented with ocular syndrome simultaneously with other syndromes in this group. The ocular onset presentation has no relationship to the onset symptoms. So the ophthalmologist must pay attention to, and be familiar with CVST to avoid misdiagnosis and a missed diagnosis when patients present with ocular symptom as the initial onset symptom.
At present, it is believed that underlying ocular symptom of CVST are venous obstruction and intracranial hypertension. The most common ocular symptom is papilloedema. In this group of patients, 48.3% had papilloedema; this corresponds to the reported 41% . Unless the macular is affected by retinal hemorrhage, retinal edema, or exudation, there are no identifiable symptoms of early papilloedema, not even vision changes or impaired vision. If the papilloedema caused by intracranial hypertension lasted too long, it would cause progressively deteriorating vision in narrow irregular concentric circles - a result of optic nerve fiber damage. This would eventually result in seriously impaired vision or blindness caused by optic atrophy .
The pathogenesis of papilloedema by intracranial hypertension is unclear and controversial. The generally accepted mechanism is that blockage in axoplasmic flow of the optic nerve causes papilloedema . The prognosis for long-term and serious papilloedema is very poor, so we must actively treat the patient before the papilloedema develops and optica trophy occurs. Once the opportunity for treatment is missed, it would be difficult to retrieve lost vision.
In addition, the intracranial hypertension leads to eighth cranial nerve paralysis and mild cognitive impaired. Some patients presented with diplopia and strabismus because of intracranial hypertension. The cause may due to the eighth cranial nerve having a long itinerary in the cranial cavity, it is easily damaged. This group had 6 patients with diplopia and strabismus. There is a complex relationship between CVST and dural arteriovenous fistula. According to some reports, there may be a dural arteriovenous fistula in the terminal stage of CVST [15, 16]. In this group, there are 5 patients with dural arteriovenous fistula. These patients were diagnosed with CVST via DSA 6 months to 3 years ago. Each patient presented with the related symptoms of arteriovenous fistula. In addition, the dural arteriovenous fistula may have already existed before thrombosis, so it might be the basic cause of CVST .
We have analyzed and summarized the data of 118 patients with CVST over 5 years. This has allowed a deepened recognition of ocular onset presentation of CVST but, in this study, we have not analyzed the relationship between the severity level of ocular onset presentation and the site of thrombosis which need further study.
Conflict of interests
The authors declare that they have no competing interests.
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