Aseptic thrombosis of orbital veins and cavernous sinus. Clinical symptomatology

Occlusion or thrombosis of the superior ophthalmic vein or of the cavernous sinus is an unspecific finding that may be secondary to different disorders such as tumours of the skull base or nasopharynx. Sometimes, however, no underlying disorder is found in spite of an extensive clinical and radiological evaluation. Eight such cases are here presented. Similar cases have previously been described, both as examples of the Tolosa-Hunt syndrome and as aseptic cavernous sinus thrombosis. The literature on these two disorders is reviewed and different diagnostic criteria discussed.     

Computerised tomography in Tolosa-Hunt syndrome

Twelve patients who satisfied the clinical criteria of Tolosa-Hunt syndrome (THS), underwent axial computerised tomography (CT) scan of the head. Two patients had abnormalities detected in the cavernous sinus on the affected side which supported the clinical diagnosis. This coupled with steroid responsiveness and long-term asymptomatic follow-up firmly established the diagnosis of THS. We discuss the computerised tomographic findings seen in THS and the relevance of using accurate imaging techniques as the first line of investigation in these cases.     

眼上静脉扩张的影像学诊断意义

目的：探讨超声,CT及MRI检查在眼上静脉扩张及其病因诊断中的意义。方法：对1984－2000年临床资料完善的116例（232只眼）伴眼上静脉扩张患者进行回顾性分析。结果：发现多种疾病均可引起眼上静脉扩张,颈动脉－海绵窦瘘是导致眼上静脉扩张的主要疾病。其中颈动脉－海绵窦瘘92例（包括高和低流瘘）,眼型Graves病14例,眶尖炎症2例,Tolosa-Hunt综合征2例,眼部血管畸形2例,眶炎性假瘤、眶内血肿、海绵窦肿瘤及海绵窦血栓形成各1例。扩张的眼上静脉直径为3．5－7．0mm,各病种眼上静脉管径扩张大小无明显差异,但颈动脉－海绵窦瘘眼上静脉最粗7．0mm。影像学检查同时能发现眼外肌肥大、眶部病变及海绵窦膨大等相关影像学表现。结论：超声、CT及MRI检查均能良好显示眼上静脉扩张,综合其他影像学表现,可确定眼上静脉扩张的病因。     

Metastatic B-cell lymphoma of the cecum masquerading as Tolosa-Hunt syndrome

CASE REPORT: Imaging studies of a 41-year-old man who had developed a painful unilateral ophthalmoplegia showed a mass involving the right cavernous sinus region. The systemic workup disclosed a B-cell lymphoma in the cecum. COMMENTS: We report a case of metastatic cecum lymphoma masquerading as Tolosa-Hunt syndrome.     

Steroid-responsive optic neuropathy in a patient with Tolosa-Hunt syndrome: Electrophysiologic findings

We examined visual evoked potentials and pattern electroretinograms in a patient with Tolosa-Hunt syndrome associated with optic nerve involvement. The 82-year-old woman developed unilateral painful ophthalmoplegia and visual loss in the right eye. Magnetic resonance imaging showed an abnormal soft-tissue area in the right cavernous sinus and the right orbital apex. Symptoms responded rapidly to treatment with corticosteroid. Visual evoked potentials to flash and pattern stimuli were both remarkably reduced and delayed in the right eye in the acute stage; however they improved to almost normal after steroid therapy. The pattern electroretinogram recorded in the acute stage was normal bilaterally. These results indicate that optic nerve involvement in Tolosa-Hunt syndrome can be mild and reversible.     

Tolosa-Hunt综合征的临床分析

Tolosa-Hunt综合征(THS)在临床上属于少见病,是由于海绵窦区(有时病变范围波及眶上裂甚至眶尖)非特异性炎性肉芽肿所致的痛性眼肌麻痹。THS临床特征为眶周或眼眶后疼痛同时伴有单根或多根眼外肌麻痹以及同侧三叉神经感觉区障碍。临床上表现为痛性眼肌麻痹的疾病有很多(眼肌麻痹性偏头痛、海绵窦段颈内动脉瘤、脑膜炎、糖尿病致颅神经损伤及鼻咽癌所致的痛性眼肌麻痹等),容易造成诊断上的困难,本文对于THS病因、临床特点、诊断标准、鉴别诊断以及治疗原则做了综合性的论述,同时着重介绍了MRI对于诊断THS的重要性。     

The Tolosa-Hunt syndrome

Painful ophthalmoplegia due to idiopathic granulomatous inflammation of the cavernous sinus/superior orbital fissure has been termed Tolosa-Hunt syndrome (THS). This report is based upon 146 patients with this syndrome published in the English literature. The clinical profile, natural history, diagnostic evaluation, pathologic findings, differential diagnosis, and therapy of THS are presented. Careful initial patient examination and scrupulous follow-up evaluation are essential, as THS is categorically a diagnosis of exclusion. While the etiology of THS is unknown, current pathogenetic theories are reviewed.     

An unusual case of pediatric painless ophthalmoplegia

Acquired unilateral ophthalmoplegia in childhood has many potential causes. Tolosa-Hunt Syndrome is characterized by painful ophthalmoplegia caused by nonspecific inflammation of cavernous sinus or superior orbital fissure. It is rarely present in children. Corticosteroid treatment is the current mainstay of treatment but cases of THS that failed to respond to steroids have been reported. We report a case of cavernous sinus pseudotumour presenting as a painless ophthalmoplegia in a child, in which complete resolution was spontaneous. To our knowledge, such a case has never been reported in the literature.     

An Unusual Case of Pediatric Painless Ophthalmoplegia

Acquired unilateral ophthalmoplegia in childhood has many potential causes. Tolosa-Hunt Syndrome is characterized by painful ophthalmoplegia caused by nonspecific inflammation of cavernous sinus or superior orbital fissure. It is rarely present in children. Corticosteroid treatment is the current mainstay of treatment but cases of THS that failed to respond to steroids have been reported. We report a case of cavernous sinus pseudotumour presenting as a painless ophthalmoplegia in a child, in which complete resolution was spontaneous. To our knowledge, such a case has never been reported in the literature.     

Computed Tomographic Evaluation of the Cavernous Sinus

In evaluating over 300 patients with sellar and parasellar lesions, the computed tomographic (CT) appearance of the cavernous sinus was studied. Optimal visualization of the cavernous sinus was obtained with continuous contrast enhancement of both axial and coronal tomograms. The coronal projection and reformatted views provided imaging of the cranial nerves within the cavernous sinus. In addition to the physiologic CT appearance of the cavernous sinus, a variety of pathologic conditions are illustrated. CT criteria suggesting an abnormal cavernous sinus are: (1) asymmetry of size; (2) asymmetry of shape, particularly the lateral wall; and (3) focal areas of abnormal density within the sinus.     

Abducens nerve palsy and Horner's syndrome revisited

Sympathetic fibers destined for the eye join the abducens nerve for a short distance within the cavernous sinus; thus, a unilateral sixth nerve palsy with an ipsilateral Horner's syndrome is of significant localizing value. We report two cases of cavernous sinus lesions producing an ipsilateral abducens palsy and Horner's syndrome: one case due to an intrinsic and the other to an extrinsic lesion of the sinus region.     

Bilateral cavernous sinus non-Hodgkin's lymphoma as the presenting sign of acquired immunodeficiency syndrome: case report

Case report of bilateral cavernous sinus syndrome due to primary non-Hodgkin lymphoma of the central nervous system in a patient infected by the human immunodeficiency virus. A 51-year-old male patient infected by the human immunodeficiency virus but without antiretroviral treatment developed paralysis of the V and VI cranial nerves. Imaging studies were obtained to investigate an orbital apex and a cavernous sinus syndrome. A computerized tomography scan of the orbit was normal but a high-resolution magnetic resonance imaging demonstrated bilateral enlargement of the cavernous sinus. Although primary lymphoma of the central nervous system is a rare condition, it should be considered in the differential diagnosis in immunocompromised patients who develop ocular motility abnormalities and imaging signs suggestive of infiltrative cavernous sinus lesions.     

Septic thrombosis of cavernous sinus extended to the ipsilateral internal jugular vein and transversal sinus with favorable outcome: Clinical and radiological features of a Lemierre syndrome

An 86-year-old patient was hospitalized for fever and left acute orbital syndrome (red eye with moderate visual impairment, chemosis, ophthalmoplegia without proptosis, or any fundus involvement). CT scan showed superior ophthalmic vein and cavernous sinus enlargement complicating ipsilateral sphenoidal sinusitis. Magnetic resonance imaging (MRI) demonstrated the left cavernous sinus thrombosis extended to the ipsilateral jugular vein and transversal sinus (Lemierre syndrome). Intravenous broad-spectrum antibiotics and curative anticoagulation were prescribed. Blood cultures allowed the identification of Streptococcus intermedius and Staphylococcus warneri species. Evolution was favorable and the patient was discharged 3 weeks after. Antibiotics and anticoagulation were carried out for a total duration of 4 and 12 weeks, respectively. Lemierre syndrome is a potentially life-threatening emergency rarely encountered; thus, ophthalmologists should be cognizant of clinical and radiological features. Broad-spectrum antibiotics are the mainstay of treatment. Curative anticoagulation may be added if no blood coagulation disorder nor bleeding on neuroimaging has been identified.     

Three cases of abducens nerve palsy accompanied by Horner syndrome

PURPOSE: To report three cases of nasopharyngeal carcinoma, exhibiting abducens nerve palsy and Horner syndrome during the medical treatment of the tumor, whose invasive cavernous sinus lesions could be detected. SUBJECTS: The patients were two men and one woman who were referred to our department with complaints of diplopia during the clinical course of nasopharyngeal carcinoma. RESULTS: Right abducens nerve palsy, Horner syndrome, and impairment of the 1st division of the trigeminal nerve were recognized in all patients. Magnetic resonance imaging (MRI) revealed invasive lesions in the right cavernous sinus in all three patients, with iso-intensity on T1-weighted images, which were enhanced by gadolinium. CONCLUSIONS: The abducens and sympathetic nerves run closely together in the cavernous sinus. Nasopharyngeal carcinoma is apt to invade the cavernous sinus and often impairs the abducens nerve. Therefore the presence of Horner syndrome and trigeminal palsy in combination with abducens nerve palsy should be cautiously investigated to confirm the existence of the cavernous sinus lesions.     

ACUTE ORBITAL MYOSITIS: A CASE REPORT

A case of acute orbital myositis is presented in this report. CT scanning provided an immediate diagnosis and hence institution of treatment. There was a typically rapid response to steroids. A previous presentation, prior to the advent of CT, had been misdiagnosed as Tolosa-Hunt syndrome.
Orbital pseudotumor can be acute or chronic, and acute orbital myositis is now regarded as one of five subtypes of the former.     

Acute orbital myositis: a case report

A case of acute orbital myositis is presented in this report. CT scanning provided an immediate diagnosis and hence institution of treatment. There was a typically rapid response to steroids. A previous presentation, prior to the advent of CT, had been misdiagnosed as Tolosa-Hunt syndrome. Orbital pseudotumour can be acute or chronic, and acute orbital myositis is now regarded as one of five subtypes of the former.     

Ophthalmoplegia in carotid cavernous sinus fistula.

The aetiology of ophthalmoplegia in 15 patients with carotid-cavernous sinus fistula is discussed, and the clinical findings are correlated with angiographic and orbital CT appearances. After closure of the fistula the majority of patients with generalised ophthalmoplegia recovered full ocular movements rapidly, while patients with an isolated abduction weakness required much longer to return to normal. Orbital CT studies showed enlarged extraocular muscles in the patients with generalised ophthalmoplegia but muscles of normal size in those with abduction failure alone. After closure of the fistula repeat CT studies of patients with enlarged extraocular muscles showed a diminution in muscle size. We suggest that generalised ophthalmoplegia in carotid cavernous sinus fistula is due to hypoxic, congested extraocular muscles. Isolated abduction weakness is due to a sixth nerve palsy, which probably occurs either in the cavernous sinus or more posteriorly near the inferior petrosal sinus. A combination of these 2 mechanisms may be found in some patients.     

Arterio-venous fistula of cavernous sinus. A comparison of iatrogenic direct fistula with low flow dura shunt syndrome

PURPOSE: Comparison of symptoms of the low-flow dura shunt syndrome as a small arterial anomaly in the cavernous sinus with the direct, traumatic originated internal carotid artery fistula. MATERIAL AND METHODS: The clinical case report for two patients. The first case with a direct cavernous sinus fistula was diagnosed using angiography and the patient was treated with a detachable balloon catheter through the inferior petrousal sinus. The second case after the diagnosis was followed up with doppler sonography. RESULTS: The first case developed the direct cavernous sinus fistula after second thrombarteriotomy of the right internal carotid stenosis. The diagnosis, by typical clinical symptoms, was confirmed through the cavernous sinus angiogram with enlarged superior and inferior ophthalmic veins. The drainage was accomplished through the inferior petrousal sinus and the intercavernous sinus with accompanying signs of cortical drainage. All clinical symptoms, except for the abducens nerve palsy and the incomplete oculomotor nerve palsy, were reduced after neurosurgical occlusion of the fistula. The second case with the low-flow dura shunt syndrome was symptomatic by hypertension crisis and some spontaneous reduction was noticed. CONCLUSIONS: The iatrogenic direct cavernous sinus fistula is seldom but a very dangerous vital complication of the internal carotid arterial stenosis surgery and must be immediately closed through the endovascular embolisation therapy. The low-flow dura shunt syndrome may be in 50% occluded spontaneously.     

痛性眼肌麻痹综合征的MRI诊断

目的 探讨痛性眼肌麻痹综合征在磁共振成像（magnetic resonance inaging,MRI）中的表现,为痛性眼肌麻痹综合征的临床诊断提供客观的影像学依据。方法 对临床确诊的17例痛性眼肌麻痹综合征患者,分别行眶区和（或）海绵窦区MRI平扫和脂肪抑制钆-二乙烯三胺五乙酸（gadolinium-diethylene triamine pentaacetic acid,Gd-DTPA)增强扫描。结果 痛性眼肌麻痹综合征MRI表现为眶上裂区斑片状或小结节状异常信号,患者海绵窦增大、增宽,眶上裂和海绵窦邻近的颞叶脑膜局限性受累。结论 MRI脂肪抑制Gd-DTPA增强扫描可清晰显示痛性眼肌麻痹综合征的病变;MRI检查可为临床诊断痛性眼肌痹综合征提供客观、可靠的影像学依据。     

Pitfalls in computed tomographic evaluation of the cavernous sinus

Computed tomography (CT) is the diagnostic procedure of choice in cavernous sinus evaluation. Two patients are reported with painful ophthalmoplegia due to metastatic carcinoma to the cavernous sinus in which initial CT scanning was considered to be normal. These cases illustrate the necessity of thin tomographic sections (5 mm or less), multiple projections (axial, coronal) and large doses of intravenous contrast medium (bolus and drip infusion). Careful technique is essential in order to thoroughly evaluate the parasellar region and avoid a false-negative CT examination.