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。     

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.     

Angiographic Findings in the Tolosa–Hunt Syndrome and Resolution after Corticosteroid Treatment

The Tolosa–Hunt syndrome is a rare clinical condition characterized by painful opthalmoparesis associated with idiopathic granulomatous inflammation of the orbital apex and cavernous sinus. Historically, this condition was thought to result from arteritic changes in the internal carotid artery and cavernous sinus. Modern digital angiographic techniques were unavailable when THS was initially described, and few reports exist on its high-resolution angiographic findings. Painful ophthalmoparesis, especially of the oculomotor nerve, warrants vascular imaging because of the concern for an underlying aneurysm. Here, we describe angiographic findings of THS which may be useful for clinicians when encountering patients presenting with painful ophthalmoplegia.     

Pupil-Sparing Inferior Divisional Palsy of Third Cranial (Oculomotor) Nerve in Tolosa-Hunt Syndrome

Pupil-sparing inferior divisional oculomotor nerve palsy is rare and has not been described in Tolosa-Hunt syndrome (THS). A 24-year-old man with right supraorbital pain and binocular vertical diplopia for 2 weeks underwent full neuro-ophthalmolgical evaluations including brain magnetic resonance imaging (MRI). The patient showed right exo- and hypertropia with impaired depression and adduction of the right eye. The pupils were normal. Other findings of neurological examination and brain MRI were normal. The headache and ophthalmoplegia improved within 2 days of corticosteroid treatment. In view of the pupil sparing, the patient may have a lesion selectively involving the shorter branch of the oculomotor inferior division in the orbit. THS may manifest with pupil-sparing inferior divisional oculomotor palsy.     

Dysmorphology and the orbital region: a practical clinical approach

Dysmorphology is the field of medicine focusing on congenital developmental abnormalities due to exogenous teratogens, chromosomal anomalies, or to a defect in a single gene. Numerous syndromes have been reported and a growing number of genes or chromosomal anomalies are identified. The clinical observation of the face remains an essential part of the clinical evaluation of the patients. The orbital region, as other regions of the face, should be systematically evaluated. Orbital malformations can be isolated or part of a syndrome. In the diagnostic process, the orbital anomaly can be classified as a major feature (essential for the diagnosis), a moderate feature (important but not essential for the diagnosis), or a minor feature (contributing weakly to the diagnosis). The diagnoses of the main orbital anomalies in dysmorphology are reviewed and illustrated with relevant examples of syndromes that are presented as well as the usual landmarks used in clinical practice. Abnormal position of the eyes in syndromes such as hypertelorism, hypotelorism, primary or secondary telecanthus, asymmetry, and proptosis are discussed. Eyelid anomalies, such as cryptophthalmos, ablepharon, blepharophimosis, euryblepharon, or anomalies at the level of the eyelashes and eyebrows are described.     

Orbital Compartment Syndrome: The Ophthalmic Surgical Emergency

Orbital compartment syndrome is an uncommon, ophthalmic surgical emergency characterized by an acute rise in orbital pressure. When intraorbital tension rises, damage to ocular and other intraorbital structures, including irreversible blindness, may occur if not promptly treated. The diagnosis of orbital compartment syndrome is completely clinical and early recognition and emergent orbital decompression (even prior to imaging) is essential in preventing permanent vision loss. Lateral canthotomy and inferior cantholysis remain the mainstays of management. More extensive incision of the orbital septum and orbital bony decompression may be necessary in unresponsive cases. This review discusses the various etiologies and mechanisms resulting in orbital compartment syndrome, clinical features, imaging findings, treatment, and prognosis.     

痛性眼肌麻痹的研究进展

痛性眼肌麻痹综合征（painful ophthamoplegia syndrome）亦称Tolosa-Hunt综合征（THS）,是一种缓解和复发的一侧Ⅲ,Ⅳ,Ⅵ脑神经之一或同时受累,从而造成眼肌麻痹,并伴有眼眶部疼痛的一组症候群。其病因不明,可能为免疫缺陷性反应,也有学者认为其是眶上裂的非特异性炎症或与血管性病变有关。该病依神经受影响之范围大小、程度轻重,其眼部症状有各种表现。痛性眼肌麻痹综合征在临床上少见,易与眼肌麻痹性头痛相混淆,临床诊断有一定的困难,治疗上对肾上腺皮质激素有效。本文旨在对痛性眼肌麻痹综合征的病因、临床特点、诊断、鉴别诊断和治疗进行综述如下。     

Ocular coloboma

Ocular coloboma is common malformation which includes a spectrum of anomalies that ranges from iris coloboma to clinical anophthalmos. Coloboma is etiologically heterogeneous. As an isolated defect, it is usually inherited as an autosomal dominant disorder, although autosomal recessive inheritance also occurs. Patients with multiple malformations and coloboma may have a recognized malformation syndrome of unknown etiology, a single gene disorder, or chromosomal abnormality. Prognosis and recurrence risk can be determined only after complete evaluation of the patient and other family members.     

Lax eyelid syndrome or ‘progeria’ of eyelid tissues

PURPOSE The Lax Eyelid Syndrome was described by Van den Bosch and Lemij 1 as an uncommon disorder seen in non-obese elderly people and characterised by chronic ocular surface irritation symptoms and a “floppy upper eyelid”. The authors present some new features of the lax eyelid syndrome. METHODS The authors report five patients, belonging to a younger age group, who presented with premature laxity of all the eyelid tissues. This caused medial and lateral canthal dystopia and eyelid malposition including ptosis, entropion and ectropion. Initial surgical correction was often followed by recurrence after some time. CONCLUSIONS The authors highlight the differences between lax eyelid syndrome, cutis laxa, floppy eyelid syndrome and the blepharochalasis syndrome and suggest that lax eyelid syndrome can be thought of as “progeria” or premature ageing of the eyelid tissues to distinguish it clearly from these other conditions.     

Canthal cutdown for emergent treatment of orbital compartment syndrome

This article evaluates the use of a “canthal cutdown” technique in orbital compartment syndrome in a cadaveric model. Twelve cadaver orbits were used to simulate orbital compartment syndrome using a blood analog solution. Two pressure probes, in different orbital locations, were used to monitor orbital pressure. Pressure was monitored during successive procedures: canthotomy, cantholysis, and canthal cutdown. Orbits were then re-injected with solution, simulating an active orbital hemorrhage, and pressure measurements were recorded over a 10-minute duration. No statistically significant difference was found between the two orbital pressure monitoring devices at each measurement point (p = 0.99). Significant pressure reductions, for both probes, were observed after canthal cutdown compared to initial measurement after injection of 20 mL blood analog (p < 0.001 and p = 0.005). When comparing the orbital pressure following canthotomy and inferior cantholysis versus canthal cutdown, the cutdown procedure provided an additional 74% in orbital pressure reduction (p =0.01). After re-injection of 10 mL of solution and 10 minutes of egress, pressure returned to baseline (probe 1: baseline 7 mm Hg vs. post-cutdown at 10 minutes 7 mm Hg; p = 0.83; and probe 2: 5 mm Hg vs. 5 mm Hg; p = 0.83). The canthal cutdown technique provides further reduction in orbital pressure versus canthotomy and cantholysis alone. The technique may be effective for treatment of static orbital compartment syndrome and temporizing treatment of compartment syndrome from active orbital hemorrhages.     

非特异性炎症致眶尖综合征临床特征分析

目的 总结眶尖非特异炎症导致眶尖综合征患者的临床特征。设计 回顾性病例系列。研究对象 2016年6月至2019年5月北京同仁医院神经眼科及北京市普仁医院眼科眼眶非特异性炎症致眶尖综合征15例（16眼）。方法 回顾患者的临床症状、体征、化验及影像学检查、激素治疗效果及病情转归。主要指标 临床表现、眼眶及头颅CT、MRI表现。结果 15例中男性8例，平均年龄（53±16）岁。14/15例单眼发病。病灶均局限于眶尖区，7例同时累及海绵窦。首先出现患侧眼眶疼痛，相继出现眶尖综合征表现。眼眶MRI均显示眶尖部条片状不规则实性病灶，呈等T1WI、等T2WI信号，均匀强化。经全身或眼球局部糖皮质激素治疗后14眼（87.5%）眼痛完全消失，13眼（81.3%）上睑下垂改善，11眼（68.8%）眼球运动及复视症状改善。3个月后复查眼眶MRI病灶均缩小。仅一半患者治疗后视力提高，其余8眼（50.0%）视力无改善。随访过程中2例（12.5%）病变复发。结论 局限于眶尖的非特异炎症可导致严重的眶尖综合征。早期无眼红肿等炎症表现，眼球突出亦不明显，表现为眶周疼痛、眼球运动障碍，继而视力进行性损伤，易被误诊。临床表现和神经影像学是诊断主要依据。糖皮质激素治疗能有效改善眼球运动障碍和疼痛，但视力恢复困难，且病变有复发可能。（眼科，2021，30： 56-61）     

Epithelioid Angiosarcoma of the Orbit Presenting as Tolosa-Hunt Syndrome: A Clinicopathologic Case Report with Review of the Literature

The clinical diagnosis of Tolosa-Hunt syndrome was first considered in a 66-year-old man with facial pain and diplopia. A complete neuroradiologic evaluation as well as an oncologic work-up yielded normal results. Several courses of oral prednisone provided no significant benefit. Within a year the patient became clinically worse and a CT scan disclosed an abnormal area of enhancement at the left orbital apex. An orbital exploration was performed elsewhere and a histologic diagnosis of myositis was obtained. Because of further worsening the patient was re-evaluated 3 months later and a CT scan showed a mass in the left orbital apex and superior orbital fissure. A second orbital exploration was performed and a sausage-shaped mass encompassing the optic nerve was excised. By light microscopy a poorly differentiated malignant tumor was infiltrating the orbital tissues with areas of intra- and perineural invasion. The tumor cells were arranged in strands and tubules with a definite tendency to form lumens that often contained red blood cells. Electron microscopic studies disclosed features consistent with a neoplasm of endothelial cell origin displaying a polarized basal lamina and rare micropinocytotic vesicles on the luminal side. The presence of multiple, slender microvilli and sometimes tonofilaments as well as desmosomes were interpreted as epithelioid metaplasia of an angiosarcoma.     

Neuro-Sweet Disease Causing Orbital Inflammation

Neuro-Sweet disease is a rare condition causing encephalitis or meningitis in addition to the erythematous skin plaques of Sweet syndrome. Neuro-Sweet disease has been associated with several ocular manifestations, including ocular movement disorders, episcleritis, conjunctivitis, uveitis, and optic disc oedema. The author reports a patient with orbital inflammation, cranial neuropathies, and a skin rash in the setting of myelodysplastic syndrome. Biopsy of her skin lesion confirmed the diagnosis of neuro-Sweet disease. To the author’s knowledge, this is the first reported case of neuro-Sweet disease causing orbital inflammation. Her ocular inflammation resolved with the use of systemic corticosteroid treatment.     

Neuro-Sweet Disease Causing Orbital Inflammation

Abstract

Neuro-Sweet disease is a rare condition causing encephalitis or meningitis in addition to the erythematous skin plaques of Sweet syndrome. Neuro-Sweet disease has been associated with several ocular manifestations, including ocular movement disorders, episcleritis, conjunctivitis, uveitis, and optic disc oedema. The author reports a patient with orbital inflammation, cranial neuropathies, and a skin rash in the setting of myelodysplastic syndrome. Biopsy of her skin lesion confirmed the diagnosis of neuro-Sweet disease. To the author’s knowledge, this is the first reported case of neuro-Sweet disease causing orbital inflammation. Her ocular inflammation resolved with the use of systemic corticosteroid treatment.     

Orbital Inflammatory Syndrome and Anterior Uveitis: A Case Series

Purpose: To describe four cases of orbital inflammatory syndrome (OIS) with associated anterior uveitis that have presented within 2 years to our practice.

Methods: Charts of patients diagnosed with OIS from June 2013 to May 2015 were reviewed.

Results: Four patients, three children and one adult, presented with orbital swelling, pain, and varying degrees of vision loss. Treatment with intravenous methylprednisolone resulted in significant symptomatic improvement in all cases initially; when symptoms recurred, the patients had evidence of anterior uveitis. With continued systemic therapy and the addition of topical prednisolone, the patients all achieved control of their uveitis and OIS and are well controlled with regular outpatient follow-up.

Conclusions: Reports of OIS-associated with uveitis are relatively rare. The presentation of three pediatric patients and one adult patient to the same practice with OIS and secondary uveitis within a 2-year period may indicate that the association is underreported.     

Non-infectious orbital vasculitides

Non-infectious vasculitides comprise a large number of diseases. Many of these diseases can cause inflammation within the orbit and a clinical presentation, which mimics numerous other processes. Orbital disease can often be the initial presentation of a systemic process and early diagnosis can help prevent long-term, potentially fatal consequences. The evaluation and treatment of non-infectious orbital vasculitides are often complicated and require a thorough understanding of the disease and underlying systemic associations. The long-term prognosis visually and systemically must be weighed against the risks and benefits of the treatment regimen. A large variety of corticosteroid formulations currently exist and are the mainstay of initial treatment. Traditional steroid-sparing immunosuppressive agents are also an important arsenal against these vasculitides. Recently, a new class of drugs called biologics, which target the various mediators of the inflammation cascade, may potentially provide more effective and less toxic treatment. This review aims to synthesize the current literature on non-infectious orbital vasculitides.     

Pituitary apoplexy presenting with an orbital bruit

Pituitary apoplexy is a sight-threatening syndrome in which a pituitary adenoma undergoes sudden enlargement as a result of haemorrhage, infarction or both. Classic features of the syndrome include sudden severe headaches, reduced consciousness, visual impairment, ophthalmoplegia and/or endocrinological disturbance. Pituitary apoplexy has been reported following cardiac bypass surgery. The case is reported of a 68-year-old man who presented with left external and internal ophthalmoplegia, complete ptosis, mild chemosis, reduced vision, and an orbital bruit following coronary artery bypass grafting. Carotid angiography showed the left internal carotid artery to be bowed anteriorly and narrowed. Magnetic resonance imaging demonstrated features consistent with pituitary apoplexy. It is believed that an orbital bruit has not previously been reported in pituitary apoplexy.     

Orbital colour Doppler imaging in chronic ocular ischaemic syndrome

Background: The clinical syndrome of chronic ocular ischaemia may be difficult to diagnose due to its wide range of non-specific presentations, and its diagnosis often implies the presence of underlying severe carotid occlusive disease. Colour Doppler imaging (CDI), a recent advance in ultrasonography that allows for colour-encoded blood flow data of a vascular structure to be displayed simultaneously on a conventional real-time gray-scale B mode image, has been used in the assessment of ocular and orbital vasculature. Method: Four patients with chronic ocular ischaemic syndrome whose diagnoses were aided by CDI are presented. Results: Orbital CDI was successful in confirming the diagnosis in all four cases. Reversal of blood flow in the ophthalmic arteries was demonstrated. Central retinal vessels were not detected with CDI in all four patients with complete occlusion of the ipsilateral internal carotid artery. One of the four patients had intermittent reversal of ophthalmic arterial flow ipsilateral to the 60% stenosed internal carotid artery. Conclusion: This non-invasive technique is an excellent alternative to invasive vascular studies such as angiography for the diagnosis and evaluation of the chronic ocular ischaemic syndrome.     

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.     

Diagnosis and management of enophthalmos

Enophthalmos is a relatively frequent and misdiagnosed clinical sign in orbital diseases. The knowledge of the different etiologies of enophthalmos and its adequate management are important, because in some cases, it could be the first sign revealing a life-threatening disease. This article provides a comprehensive review of the pathophysiology, evaluation, and management of enophthalmos. The main etiologies, such as trauma, chronic maxillary atelectasis (silent sinus syndrome), breast cancer metastasis, and orbital varix, will be discussed. Its objective is to enable the reader to recognize, assess, and treat the spectrum of disorders causing enophthalmos.