MR imaging of Tolosa-Hunt syndrome

The Tolosa-Hunt syndrome consists of painful ophthalmoplegia caused by cavernous sinus inflammation, which is responsive to steroid therapy. The MR features of 11 patients with the clinical diagnosis of Tolosa-Hunt syndrome were studied. Two patients had normal MR studies of the orbit and cavernous sinuses. In nine patients, abnormal signal and/or mass lesions were seen in the cavernous sinuses; in eight cases, the abnormality was hypointense relative to fat and isointense with muscle on short TR/TE images and isointense with fat on long TR/TE scans. Extension into the orbital apex was seen in eight cases. In six of nine cases the affected cavernous sinus was enlarged; in five of nine it had a convex outer margin. One patient had a thrombosed cavernous sinus and superior ophthalmic vein in addition to a cavernous sinus soft-tissue mass. The signal intensity of Tolosa-Hunt syndrome in this limited series was similar to that of orbital pseudotumor and is confined to a limited differential diagnosis, which includes meningioma, lymphoma, and sarcoidosis.     

MRI findings in Tolosa-Hunt syndrome before and after systemic corticosteroid therapy

Tolosa-Hunt syndrome (THS) is characterized by painful ophthalmoplegia due to a granulomatous inflammation in the cavernous sinus. Corticosteroid therapy dramatically resolves both the clinical and radiological findings of THS. We present MRI findings of six patients with a clinical history of at least one episode of unilateral or bilateral orbital-periorbital pain, clinical findings of associated paresis of one or more of 3rd, 4th, 5th or 6th cranial nerves. All of the patients revealed an enlargement of the symptomatic cavernous sinus on magnetic resonance imaging (MRI) scans. Five patients revealed total resolution of the clinical findings within 1-8 weeks, following systemic corticosteroid treatment. One patient revealed only minor regression of clinical findings within 2 weeks after the initiation of the treatment, so the cavernous sinus lesion was reevaluated as meningioma on MRI, and the patient underwent surgical resection of the mass with resultant histopathological finding of cavernous sinus meningioma. A follow-up MRI scan was performed for five patients at the end of 8-weeks of steroid therapy. Three of these five patients showed total resolution of the cavernous sinus lesions whereas two of them revealed a partial regression of the cavernous sinus lesions. MRI findings before and after systemic corticosteroid therapy are important diagnostic criteria to put the definitive diagnosis of THS and to differentiate it from other cavernous sinus lesions that simulate THS both clinically and radiologically.     

MRI findings in the patients with the presumptive clinical diagnosis of Tolosa-Hunt syndrome

The aim of this study was to present our experience in MRI diagnosis of 23 patients with the clinical findings suggesting Tolosa-Hunt syndrome (THS). Cranial MRI studies of the patients with a clinical history of at least one episode of unilateral or bilateral orbital and periorbital pain, and associated paresis of one or more of third to sixth cranial nerves, were performed on a 1.5-T MRI scanner. Whereas 5 patients had the diagnosis of THS, paracavernous meningiomas in 4 patients, pituitary macroadenomas with cavernous sinus infiltration in 3 patients, Meckel's cave neurinoma in 1 patient, and suprasellar epidermoid in 1 patient were surgically proven MRI findings. Other pathological MRI findings were leptomeningeal metastases in 3 patients, granulomatous pachymeningitis sequelae in 2 patients, and aneurysm with compression on cavernous sinus in 1 patient. Three patients had normal MRI findings. The incidence of radiologically proven diagnosis of THS among the patients with the clinical findings suggesting THS seemed to be low in our study. In conclusion, MRI is the most valuable imaging technique to distinguish THS from other THS-like entities, and permits a precise assessment, management, and therapeutic planning of the underlying pathological conditions. Electronic Publication     

Pseudotumor of the orbit. Clinical, pathologic, and radiologic evaluation

Pseudotumor, Graves' disease, and lymphoproliferative disease are the most common ophthalmologic disease entities requiring evaluation by CT and MR imaging. A history of acute onset, pain, swelling, proptosis, and response to steroids are the classical findings. The radiologic findings are categorized according to location into dacryoadenitis, myositis, and sclerouveitis, with and without associated infiltrations. The inflammatory infiltrate, which is composed of polymorphic leukocytes, lymphocytes, and plasma cells interspersed with a variable amount of fibrovascular tissue, may be diffuse or localized. The preferred radiologic method used for assessment of pseudotumor is CT. MR imaging, however, is indicated for evaluation of the Tolosa-Hunt syndrome, which is characterized by an inflammatory infiltrate in the orbital apex and cavernous sinus leading to cranial nerve involvement with ophthalmoplegia. The clinical and radiologic constellation of findings allows a definitive diagnosis in most cases.     

Tolosa-Hunt syndrome: MR imaging features in 15 patients with 20 episodes of painful ophthalmoplegia

Purpose

(a) To assess MR features in patients with Tolosa-Hunt syndrome (THS) and to (b) correlate MR findings with criteria derived from previously reported pathologic observations.

Methods

Fifteen patients with twenty episodes of painful ophthalmoplegia prospectively selected according to International Headache Society (IHS) standards underwent MR examinations focused on the cavernous sinus. Initial examinations in 20 and follow-up MR images in 17 episodes were retrospectively reviewed by 3 independent observers.

Results

The primary criteria: an enhancing soft tissue lesion within the cavernous sinus, increase in size and lateral bulging of the anterior cavernous sinus contour were consistently present in 15 initial episodes and in 5 recurrences (20/20). Agreement among observers was 100%. The secondary criteria: internal carotid artery narrowing in 7 patients, extension towards the superior orbital fissure in 13 and orbital apex involvement in 8 patients were unanimously agreed upon in 87.5%, 86.6% and 80%. Complete resolution of findings was observed on follow-up studies.

Conclusion

In patients with THS the MR features conform to previously reported pathologic findings. MR features are evocative of THS when an increase in size and bulging of the dural contour of the anterior CS supplemented by carotid artery involvement and extension towards the orbit are present. Resolution of findings within 6 months is required to support the diagnosis.     

Tolosa-Hunt syndrome in a patient with systemic lupus erythematosus

We report a case of Tolosa-Hunt syndrome (THS) in a patient with systemic lupus erythematosus studied with MRI. Magnetic resonance showed enlargement of the cavernous sinus and compression of the carotid syphon by enhancing tissue. In particular, fat-suppressed T1-weighted images before and after contrast agent injection and MR angiography showed extension of the abnormal tissue to the apex of the orbit and narrowing of the internal carotid artery. A presumptive diagnosis of THS was made and steroid treatment was started with rapid relief of symptoms. Follow-up MR study after steroid therapy demonstrated sub-total resolution of the neuroradiological findings. Neuroradiological findings in THS are quite typical but they may be subtle; furthermore, the presence of a systemic disease may suggest secondary involvement of the cavernous sinus. Utilization of the appropriate MR techniques and follow-up exams may contribute to the diagnosis of THS even in the presence of other systemic diseases.     

CT characteristics of orbital pseudotumors and other orbital inflammatory processes

Orbital pseudotumor is a nonspecific inflammatory process of unknown etiology that may mimic a true orbital neoplasm, specifically lymphoma. It exhibits a highly variable clinical and radiographic presentation. Thirty-nine patients with a presumptive diagnosis of orbital inflammatory disease were examined with CT and evaluated with respect to 13 findings associated with inflammatory disease of the orbit. Pseudotumor was included as part of the differential diagnosis in 25 patients. Twenty-one cases received a final (clinical) diagnosis of pseudotumor. The remainder were comprised of a broad range of orbital inflammatory conditions. Key radiographic features required to entertain or exclude the diagnosis of pseudotumor included establishing the presence of an orbital mass, extraocular muscle enlargement, bony erosion, enhancement, and associated paranasal sinus disease.     

Inflammatory myofibroblastic tumor of the orbit with associated enhancement of the meninges and multiple cranial nerves

Inflammatory myofibroblastic tumor (IMT), Tolosa-Hunt syndrome (THS), and idiopathic hypertrophic pachymeningitis (IHP) seem to be part of a spectrum of disorders that have diverse locations but similar histologic and imaging findings. We report a case of a 50-year-old man presenting with multiple progressive cranial nerves palsies with leptomeningeal cranial nerve enhancement on MRI (II, V1-V3, and X), orbital and infraorbital masses, prominence within the left cavernous sinus, and diffuse dural enhancement. Biopsies of the orbital lesion and infraorbital nerve revealed IMT. The patient's lesions, symptoms, and dural enhancement quickly improved with steroid administration and nearly resolved over multiple subsequent scans over the next few months. This case illustrates a rare case of pseudotumor mimicking a more aggressive appearance that would usually portend a case of malignancy. There is a potential association of IMT, THS, and IHP, which may have existed in a concomitant fashion in this patient. The case also describes the unique finding of enhancement of the cisternal segments of multiple cranial nerves (simulating leptomeningeal malignant involvement), which may be related to inflammatory perineural edema or ischemic neuropathy.     

CT of soft tissue injury and orbital fractures

Complex orbital rim and fractures of the internal orbital skeleton (orbital walls) are best analyzed by high-resolution axial CT. Optic canal fractures are optimally visualized with thin 1-mm section high-resolution CT scanning. Spiral CT may be used in the acutely injured patient because of its rapid scan technique. This spiral technique provides smooth data sets for three-dimensional reformations and may demonstrate foreign bodies in more than one plane. Another advantage of spiral CT is the capability of CT angiography. MR imaging usually is not the initial modality for the assessment of orbital trauma, but it is helpful in evaluating vascular injuries such as carotid-cavernous sinus fistulas or post-traumatic pseudoaneurysms. In general, CT with contrast injection is not necessary except when traumatic vascular anomalies, such as carotid cavernous, dural fistulas, or thrombosis of the superior ophthalmic vein, are considered in the differential diagnosis.     

CT observations pertinent to septic cavernous sinus thrombosis

The use of high-resolution computed tomography (CT) is described in four patients with septic cavernous sinus thrombosis. In all patients CT findings included multiple irregular filling defects in the enhancing cavernous sinus. Unilateral or bilateral inflammatory changes in the orbital soft tissues were also present. Enlargement of the superior ophthalmic vein due to extension of thrombophlebitis was noted in three patients.     

MR Imaging of Orbital Inflammatory Pseudotumors with Extraorbital Extension

Objective

To demonstrate a variety of MR imaging findings of orbital inflammatory pseudotumors with extraorbital extension.

Materials and Methods

We retrospectively reviewed the MR features of five patients, who were diagnosed clinically and radiologically as having an orbital inflammatory pseudotumor with extraorbital extension.

Results

The types of orbital pseudotumors were a mass in the orbital apex (n = 3), diffuse form (n = 2), and myositis (n = 1). The extraorbital extension of the orbital pseudotumor passed through the superior orbital fissure in all cases, through the inferior orbital fissure in two cases, and through the optic canal in one case. The orbital lesions extended into the following areas: the cavernous sinus (n = 4), the middle cranial fossa (n = 4), Meckel''s cave (n = 2), the petrous apex (n = 2), the clivus (n = 2), the pterygopalatine fossa and infratemporal fossa (n = 2), the foramen rotundum (n = 1), the paranasal sinus (n = 1), and the infraorbital foramen (n = 1). On MR imaging, the lesions appeared as an isosignal intensity with gray matter on the T1-weighted images, as a low signal intensity on the T2-weighted images and showed a marked enhancement on the post-gadolinium-diethylene triamine pentaacetic acid (post-Gd-DTPA) T1-sequences. The symptoms of all of the patients improved when they were given high doses of steroids. Three of the five patients experienced a recurrence.

Conclusion

MR imaging is useful for demonstrating the presence of a variety of extraorbital extensions of orbital inflammatory pseudotumors.     

Dynamic MR imaging in Tolosa-Hunt syndrome

OBJECTIVE: To evaluate the cavernous sinuses with dynamic magnetic resonance (MR) imaging in patients with Tolosa-Hunt syndrome (THS). METHODS: The sellar and parasellar regions of five patients with THS and 12 control subjects were examined with dynamic MR (1.5 T) imaging in the coronal plane. Dynamic images were obtained with spin-echo (SE) sequences in three patients, and with fast spin-echo (FSE) sequences in two patients and control subjects. Conventional MR images of the cranium including sellar and parasellar regions were also obtained on T1-weighted pre- and post-contrast SE, and T2-weighted FSE sequences in the coronal plane. RESULTS: MR images revealed affected cavernous sinus with bulged convex lateral wall in three patients and concave lateral wall in two patients. In all control subjects, cavernous sinuses were observed with concave lateral wall. The signal intensity on T1- and T2-weighted images and contrast enhancement on post-contrast images of the affected cavernous sinuses in patients were similar to those of the unaffected cavernous sinuses in patients and control subjects. The dynamic images in all patients disclosed small areas adjacent to the cranial nerve filling-defects within the enhanced venous spaces of the affected cavernous sinus, which showed slow and gradual enhancement from the early to the late dynamic images. No such gradually enhancing area was observed in control subjects except one. The follow-up dynamic MR images after corticosteroid therapy revealed complete resolution of the gradually enhancing areas in the previously affected cavernous sinus. CONCLUSION: Dynamic MR imaging may facilitate the diagnosis of THS.     

CT、MRI在眼眶炎性假瘤的诊断价值

目的:探讨CT和MRI对眼眶炎性假瘤的诊断和鉴别诊断价值。方法:回顾性分析经临床、病理证实的63例眼眶炎性假瘤的影像学表现。结果:根据病变累及部位及形态学表现分为:①隔前型2例,表现为眼睑肿胀;②巩膜周围炎型1例,表现为眼球壁增厚;③视神经束膜炎型3例,表现为视神经增粗;④弥漫型10例,病变可同时累及眶隔前组织、眼肌、视神经、眼环、泪腺、眶内脂肪或眶周组织;⑤肿块型12例,表现为边界清楚的肿块;⑥泪腺炎型9例,表现为泪腺肿大;⑦肌炎型26例,表现为1条或数条眼外肌增粗、肥大。结论:CT和MRI能对眼眶炎性假瘤确切定位及分型,为临床治疗方案提供重要依据,二者的结合极大地提高了眼眶炎性假瘤的正确诊断率。     

Tolosa-Hunt syndrome with sellar erosion: case report

The so-called Tolosa-Hunt syndrome consists of painful ophthalmoplegia caused by chronic nonspecific inflammation of the cavernous sinus and/or superior orbital fissure, responsive to steroid therapy. We present a case with the unusual feature of sellar erosion, in which angiography. CT and MRI suggested this idiopathic condition.     

Granulomatous orbital lesions: computed tomographic features.

Orbital lesions exhibiting granulomatous inflammation represent a heterogeneous group of diseases characterized by infiltration with epithelioid cells. The authors retrospectively reviewed the orbital computed tomography (CT) scans of 39 patients who had biopsy-proven lesions with granulomatous inflammation and found that diagnosis on the basis of CT was possible in only a few patients, those with a lesion of characteristic location and attenuation (such as a dermoid cyst) or characteristic distribution (such as bilateral enlargement of the lacrimal gland occurring in orbital sarcoidosis). In patients with multicompartmental disease exhibiting bone and extraorbital involvement the site of origin of the mass, the pattern of bone involvement and the clinical findings helped in classifying the cause of the lesion as Wegener's granulomatosis, a foreign body or mucormycosis. CT was crucial in determining the intraorbital and extraorbital extent of the lesion before excision or biopsy.     

颈动脉海绵窦瘘的临床及MSCT表现特征

目的:分析颈动脉海绵窦瘘(CCF)的临床及MSCT表现特征,提高对CCF的诊断准确率。材料和方法:采用16层螺旋CT对13例CCF病人进行扫描,图像后处理分别行MPR、MIP及VR成像,其中10例外伤性CCF还采用骨窗重建。结果:CCF的主要临床表现为眼球突出、视力减退、结膜充血、血管杂音等,MSCT的直接征象为海绵窦扩大13例(100%),眼上静脉扩张13例(100%),瘘口显示6例(46.2%),大脑中、浅静脉早显8例(61.5%)。间接征象为眼突、眼肌肥厚、眶内软组织肿胀、颅面部骨折等。MSCT检查结合临床病史可以对外伤性CCF作出准确的诊断,自发性CCF临床表现不典型,需与炎性假瘤、眼眶肿瘤、Craves眼病、血管畸形等眼部疾病进行鉴别诊断,本组采用MSCT对CCF的诊断准确率为100%。结论:MSCT具有安全、快速、准确的检查优势,多种成像技术结合使用,能清楚显示CCF的直接征象和间接征象,为临床及介入治疗提供可靠的诊断依据。     

Tributary venosinus occlusion and septic cavernous sinus thrombosis: CT and MR findings.

PURPOSEIn autopsy reports of patients who died of septic cavernous sinus thrombosis, tributary venosinus occlusion has been a common finding related to intracranial inflammatory complications. The purpose of this article is to illustrate the MR and CT appearance of septic cavernous sinus thrombosis and tributary venous occlusion.METHODSOver a period of 7 years, eight patients with septic cavernous sinus thrombosis were examined by contrast-enhanced thin-section CT. The CT scans of these eight patients and those of 30 healthy control subjects were assessed independently and subjectively by two blinded readers to ascertain the presence, size, and density of areas of nonopacification within the cavernous sinus and the presence of filling defects and dilation of tributary veins and venous sinuses. In six subjects, MR images supplemented by a contrast-enhanced spoiled gradient-recalled acquisition in the steady state (SPGR) sequence were assessed with respect to the presence of filling defects, expansion, and signal abnormalities within the cavernous sinus and tributary veins and sinuses. The MR and CT findings were compared.RESULTSThe CT studies of the eight patients were consistently differentiated from those of the control subjects by the two readers. Contrast-enhanced CT findings in patients included areas of nonopacification that were present within the cavernous sinus bilaterally in six cases and unilaterally in two. The size of the filling defects exceeded 7 mm in 76% of thrombosed cavernous sinuses compared with 9% of control subjects. The mean density of filling defects in patients differed significantly from those in control subjects. Comparison of the MR and CT findings in six cases showed the contrast-enhanced SPGR sequence to be equivalent to CT with respect to delineation of filling defects.CONCLUSIONContrast-enhanced high-resolution CT findings indicate that venosinus thrombosis associated with septic cavernous sinus thrombosis is not restricted to the superior ophthalmic vein and is more common than previously assumed. A contrast-enhanced SPGR MR sequence may be used as a reliable alternative to establish the diagnosis of cavernous sinus and tributary venosinus thrombosis.     

Unilateral endocrine exophthalmos. Diagnostic problems in association with computed tomography

Summary Swollen orbital extraocular muscles may mimic an orbital apex tumor on computed tomography. In five of our patients, in spite of highly suggestive findings on CT scanning, indicating the presence of an orbital apex lesion, the correct diagnosis proved to be endocrine exophthalmos. The value of complementary CT projections and of orbital ultrasonography to assist in the differential diagnosis is discussed, and the additional contribution of orbital phlebography for the confirmation of lesions in the orbital apex is emphasized.     

泪腺腺样囊性癌的CT诊断

目的：评价泪腺腺样囊性癌（ACC）的CT影像表现。方法：回顾性分析经手术和病理证实的23例泪腺腺样囊性癌,结合临床和病理,观察CT表现。结果：全部肿瘤均发生在泪腺眶部,扁椭圆形11例,不规则形8例,梭型4例;肿瘤边界清楚15例,边缘不规则8例,其中肿瘤沿肌锥外间隙向眶尖生长10例,肿瘤部分包附眼球1例;肿瘤部分与外直肌和或上直肌和上睑提肌分界不清14例,蔓延到眶上裂和海绵窦1例,颞窝和中颅窝受累2例。11例（包括平扫和增强4例中的平扫）行平扫,显示肿瘤均为较高密度,其中4例密度较均匀,7例病灶内部分见小囊状、斑点状低密度改变;16例（包括平扫和增强4例中的增强）增强扫描大多为中到高度强化,其中10例强化不均匀,5例强化较均匀,1例未见强化;瘤内钙化3例。眶骨受压凹陷变薄13例,其中伴眶上裂扩大1裂;骨质吸收破坏10例。结论：泪腺腺样囊性癌的CT影像表现具有一定的特征,能为诊断定性和临床治疗提供重要依据。     

Computed tomography of cavernous sinus diseases

Summary We retrospectively analyzed CT scans of 21 cavernous sinus lesions in an attempt to discover CT findings helpful to the differential diagnosis. With the integration of various CT observations it was possible to categorize the lesions into inflammatory, vascular, benign neoplastic and malignant metastatic lesions with few exceptions. Four of 5 cases of septic cavernous sinus thrombophlebitis revealed unilateral or bilateral multiple irregular filling defects in the enhancing cavernous sinus with or without orbital inflammatory change. Four of 5 cases of carotid-cavernous fistula demonstrated unilateral or bilateral diffuse bulging and homogeneous enhancement of the cavernous sinus with obliteration of normal low densities of cranial nerves and gasserian ganglion. Dilatation and tortuosity of superior ophthalmic vein were also associated. Four of 5 cases of benign neoplastic lesion showed well-circumscribed enhancing masses confined to the cavernous sinus with pressure erosion or hyperostosis of adjacent bone. Five of 6 cases of malignant metastatic lesion showed changes suggesting malignancy such as destruction of adjacent bone or associated manifestations of intracranial spread. As compared with the axial scan, coronal scans proved to be more sensitive in detection of subtle cavernous sinus expansion, and superior in evaluation of intracavernous neural structures, relationships with the pituitary gland and changes in the skull base. Axial scans, however, were superior in detection of associated orbital and intracranial abnormalities. Scans in both projections are needed in the evaluation of most cavernous sinus diseases.