Orbital syndromes

The orbit is a complex anatomical structure with unique properties not observed in other regions of the body. Composed of seven bones, the orbit is filled by the eye, optic nerve, lacrimal gland, extraocular muscles, peripheral motor and sensory nerves, fat, arteries, and veins. All these structures are intimately related to one another within an intricate framework of connective tissue. A variety of traumatic, vascular, inflammatory, infectious, and neoplastic processes can affect the orbit and its structures. Aside from the many primary orbital diseases, systemic disorders and pathological processes from neighboring structures (eye, ocular adnexa, oral cavity, paranasal sinuses, and intracranial cavity) can involve the orbit. A careful history and complete physical examination, with special attention to the orbit and ocular adnexa, are required to identify subtle orbital abnormalities that otherwise could be overlooked or mistakenly contributed to a nonorbital process. This article reviews the pertinent orbital anatomy, discusses the clinical evaluation and manifestations of orbital syndromes, and highlights several important orbital syndromes germane to the neurologist, including thyroid-associated orbitopathy, nonspecific orbital inflammation (also known as inflammatory orbital pseudotumor), perineural orbital invasion of cutaneous squamous cell carcinoma, rhino-orbital-cerebral mucormycosis, and carotid-cavernous sinus fistula.     

Congenital orbital teratoma

Congenital orbital teratoma is a rare tumor that usually presents with unilateral proptosis in a normal full term infant. These tumors are usually confined to the orbit without extension into the intracranial cavity. A few cases with intracranial extension have been reported. We have developed a combined ophthalmological, otolaryngological and neurosurgical approach to deal with this rare tumor when it extends intracranially.     

Orbital tumor presented systemic sarcoidosis

Sarcoidosis is a granulomatous, multisystemic disorder of unknown origin usually affecting young Black-American adults. Bilateral hilar lymphadenopathy and skin or eyelid lesions are the most common symptoms noted. Except for lacrimal gland enlargement, orbital involvement with sarcoidosis is rare and is usually unilateral when it occurs. The aim of this article is to report an isolated case of sarcoidosis that initially presented as an orbital tumor, and to document the CT and MR appearance of the lesions.     

伽玛刀治疗泪腺上皮性肿瘤的长期疗效观察

目的研究伽玛刀治疗泪腺上皮性肿瘤的长期效果、影响因素及并发症。方法回顾性分析59例手术切除后复发的泪腺上皮性肿瘤病人的临床资料,均采用伽玛刀治疗。采用Kaplan-Meier法评估局部控制率和生存率。Cox回归模型用于分析肿瘤复发的危险因素。结果随访59例,时间15~96个月,平均55.6个月。6个月实际控制率是96.6%,1年和5年实际控制率分别是72.9%和15.2%。肿瘤侵犯颅内（P=0.002）和肿瘤直径≥3.5 cm（P〈0.001）是降低治疗效果的危险因素。是否曾行放射治疗对预后影响无统计学意义（P=0.155）。5年远处转移率是32.2%。5年生存率是50.8%。结论伽玛刀作为一种安全有效的无创性肿瘤治疗方法,治疗复发泪腺上皮性肿瘤短期效果良好,但仍需要定期复查。     

眼眶肿瘤的伽玛刀放射外科治疗

目的 总结评价Leksell伽玛刀治疗眼眶肿瘤的中长期疗效.方法 223例患者年龄4～85岁,平均(37.5±15.6)岁.男91例,女132例;125例为手术后残留或复发者,98例根据典型临床及影像表现进行诊断;肿瘤容积0.03～35.60 cm3,平均(5.4±1.9)cm3;肿瘤边缘剂量10～40 Gy.结果 随访18 - 114个月,平均(35.7±16.2)个月,肿瘤缩小129例(57.8％),无变化80例(35.9％),14例(6.3％)肿瘤增大,肿瘤控制率为93.7％;142例患者治疗后视力得到保留,其中视力提升79例,减退19例;21例曾出现一过性球结膜水肿.结论 伽玛刀治疗眼眶肿瘤可取得良好肿瘤控制率,多数患者可保留视力,并发症少,可成为眼眶肿瘤的主要治疗方法之一.     

Lacrimal preganglionic neurons form a subdivision of the superior salivatory nucleus of rat: transneuronal labelling by pseudorabies virus.

Transneuronal viral tracing was applied to localize preganglionic parasympathetic neurons in the brainstem which innervate the extraorbital lacrimal gland in the rat. The Bartha strain of pseudorabies virus was injected into the lacrimal gland, and after different survival times, the superior cervical and Gasserian ganglia, the upper thoracic spinal cords and the brainstems were immunostained by antiviral antiserum. Virus-labelled neurons appeared in the ganglia and in the ventrolateral part of the ipsilateral brainstem at the pontomedullary junction 45 h after inoculation. The virus-labelled brainstem neurons comprised a subgroup of the superior salivatory nucleus (SSN) located between the root fibers of the facial nerve and the nuclei of the superior olive, and were clearly distinguished from the tyrosine hydroxylase (TH)-immunopositive, A5 catecholaminergic neurons by double immunostaining. The number of infected cells in the ipsilateral SSN was increased by 72 h, and labelled neurons appeared in the intermediolateral cell column (IML) of the ipsilateral thoracic spinal cord. In rats with cervical ganglionectomy prior to the virus injection in the lacrimal gland, virus-infected cells appeared in the SSN, but not in the thoracic spinal cord, indicating that preganglionic SSN cells were infected via parasympathetic axons of the facial nerve. A double-virus tracer labelling technique was applied to determine the topographical relationship between the preganglionic parasympathetic neurons of the lacrimal gland and those of the submandibular gland within the SSN. Simultaneous injection of Bartha strain of pseudorabies virus into the submandibular gland, and a lacZ gene-containing Bartha-derived virus strain into the lacrimal gland (and vice versa) demarcated a ventral lacrimal and a dorsal submandibular subgroup in the SSN.     

Gamma knife radiosurgery for orbital tumors

Objective

This study was performed to investigate the radiological and functional outcomes of patients with orbital tumors treated by gamma knife radiosurgery (GKS).

Patients and methods

Fifteen patients with orbital tumors (7 meningiomas, 3 cavernous hemangiomas, 2 schwannomas, 2 metastatic tumors and 1 adenoid cystic carcinoma of the lacrimal gland) were treated. Seven patients with preserved vision and tumors located near the optic nerve were treated with multisession (3 or 4 fractions) radiosurgery. The mean tumor volume was 3695 mm³ (737–13,300). The median marginal dose was 14 Gy (13–20) in single-session radiosurgery, and the median cumulative marginal dose was 20 Gy (15–20) in multisession radiosurgery.

Results

After a mean follow-up of 20.9 months (6–50), tumor control was confirmed in 12 of 15 patients. Three patients with malignant lesions had to undergo another operation due to tumor progression. Of the 13 patients whose preoperative vision was preserved, 6 patients showed improvement in visual acuity and/or visual field, 4 patients showed no change in vision, and 3 patients showed deterioration (2 related to tumor progression).

Conclusions

As with intracranial tumors with similar pathologies, GKS may be an effective treatment option for orbital tumors. Multisession radiosurgery may be a good strategy for increasing the possibility of visual function preservation in selected cases in which the lesion is adjacent to the optic apparatus.     

酪氨酸激酶C与涎腺腺样囊性癌嗜颅神经侵袭的关系

目的观察酪氨酸激酶C(TrkC)在涎腺腺样囊性癌(ACC)的表达情况,探讨TrkC与ACC嗜颅神经侵袭的关系.方法采用免疫组化方法对32例ACC、7例正常腮腺及3例腺泡细胞癌标本中的TrkC进行检测.结果 TrkC在ACC肿瘤细胞中的阳性率为90.6%(29/32),且存在嗜神经现象组的TrkC表达水平明显高于未见嗜神经现象组(P<0.05).TrkC的表达在正常腮腺导管细胞为阳性,而在腺泡细胞癌均为阴性.结论 TrkC在ACC中的过表达可能是ACC嗜神经侵袭的机制之一,TrkC可能作为ACC嗜神经侵袭的生物学标志.     

Pattern of distribution of neuropeptides in the camel lacrimal gland

The pattern of distribution of neuropeptides, including neuropeptide-Y (NPY), vasoactive intestinal polypeptide (VIP), neurotensin (NT), serotonin (5-HT), galanin (GAL), leucine-enkephalin (LEU-ENK) and calcitonin-gene-related-peptide (CGRP), in the nerves of the camel lacrimal gland was investigated using immunohistochemical techniques. Fresh lacrimal gland segments, obtained from adult camels slaughtered in the local abattoir, were used for the immunohistochemical techniques. NPY and LEU-ENK immunoreactivity was observed in the nerve cell bodies and nerve fibers of the camel lacrimal gland. VIP, GAL and CGRP were demonstrated predominantly in fine varicose nerve fibers lying on the basolateral surfaces of the lacrimal acinar cells. NT and 5-HT were identified mainly in neurons situated in the periacinar regions, close to the basal surfaces of the acinar cells. It is concluded that the camel lacrimal nerves contain several neuropeptides including NPY, VIP, NT, 5-HT, GAL, LEU-ENK and which may modulate lacrimal fluid and protein secretion.     

Pleomorphic Adenoma with Sarcomatous Change in a Lacrimal Gland

A 69-year-old man was referred with left exophthalmos. Computed tomographic (CT) findings detected a well-circumscribed mass in the left side of the intraorbital cavity. At that time, he refused the further evaluation and treatment. About three years later, the size of the mass had enlarged, and the patient''s symptoms were getting worse. The mass was completely removed with frontotemporal craniotomy and superolateral orbitotomy. In operative findings, the mass had originated in the lacrimal gland and was well-encapsulated without invasion to the surrounding tissue. In the pathologic findings, the tumor consisted of pleomorphic adenoma with osteosarcomatous change of stromal components. Postoperatively, the adjuvant radiotherapy was done four weeks later. The patient''s symptoms were improved. The pleomorphic adenoma with osteosarcomatous change is extremely rare and appropriate treatment is not clearly established. We would like to report this rare case with a review of the literature.     

Metastasis of solid tumors in extraocular muscles

Summary Three autopsy cases with discrete metastatic involvement of one or several extraocular orbital muscles by disseminated amelanotic melanoma (one case) and lobular mammary adenocarcinoma (two cases) associated with extensive meningeal involvement are reported. Clinical ocular symptoms including pain, exophthalamus, and diplopia occurred 6 months to almost 5 years after resection of the primary tumor; in two cases CT scan showed spindle-like enlargement of orbital mucles. Pathologic examination disclosed solid localized metastatic deposits in several extraocular muscles of one (breast carcinomas) or both orbits (melanoma), with diffuse invasion of striated muscle, but without necrosis, inflammation, or involvement of other orbital adnexa, eye ball, optic nerves, or orbital bone. Since no continous invasion of orbital or intraocular structures by diffuse meningeal blastomatosis was histologically observed, rare metastatic involvement of extraocular muscles via hematogenic route is suggested.     

Magnetic Resonance Evidence of Perineural Metastasis

Contiguous spread along perineural and endoneural spaces, that is, perineural tumor extension, in cutaneous squamous cell carcinoma is fairly common. Infrequently, these tumors spread and involve intracranial structures. One consequence of th1s complication is meningeal carcinomatosis which is underrecognized. Herein described is a patient with recurrent cutaneous squamous cell carcinoma with perineural invasion along the maxillary nerve that was subsequently shown by magnetic resonance imaging to the trigeminal root The patient Initially presented with a cavernous sinus syndrome but despite aggressive treatment, extensive meningeal carcinomatosis and cauda equina dysfunction developed. Awareness of perineural invasion and proper evaluation are crucial. Penneural spread intracranially worsens the prognosis and limits treatment options to palliation.     

Reorganization of cranial sympathetic pathways following neonatal ganglionectomy in the rat

Postganglionic sympathetic innervation normally is distributed ipsilaterally to lateral cranial targets. However, contralateral outgrowth occurs following unilateral ganglionectomy in neonatal rats. This study was conducted to determine the prevalence, morphological features, ganglionic derivations, and temporal sequence of sympathetic reinnervation of denervated cranial targets. Unilateral superior cervical ganglionectomy of mature rats revealed exclusively ipsilateral distributions of catecholaminergic histofluorescent fibers to orbital targets (Meibomian gland, tarsal muscle, orbital muscle, iris, ciliary body, vasculature) and the circle of Willis, with the exception of the anterior cerebral artery. In mature rats following neonatal unilateral ganglionectomy, all targets were reinnervated by fibers displaying morphologies and target relationships similar to normal innervation, but with lower densities. Acute excision of the remaining superior cervical ganglion eliminated all fibers in 7 of 8 preparations. In adult rats receiving neonatal bilateral superior cervical ganglionectomies, cerebral vasculature was reinnervated consistently, and orbital targets contained fluorescent fibers in 6 of 16 cases, indicating that reinnervation can derive from other sources when superior ganglion outgrowth is prevented. Observations in developing rats revealed fibers along the cranial portion of the contralateral optic nerve sheath at 2-3 days postganglionectomy, and within the orbit at later ages, reaching the most distal targets by 14 days. It is concluded that widespread sympathetic reinnervation of orbital and cerebrovascular targets derives primarily from the contralateral superior ganglion. Orbital ingrowth apparently originates intracranially and enters the orbit by an atypical pathway within the optic foramen.     

Bilateral "crocodile tears syndrome" associated with Melkersson-Rosenthal syndrome--case report

We present a rare case of bilateral crocodile tears syndrome (CTS) in the course of Melkersson-Rosenthal syndrome. Melkersson-Rosenthal syndrome is characterised by a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissured tongue. The classic triad is infrequent and oligosymptomatic variants are seen more frequently. CTS is a rare complication of facial nerve paralysis characterised by inappropriate lacrimation on the side of the palsy in response to salivary stimuli. It results from aberrant reinnervation of the lacrimal gland by salivary parasympathetic fibres. The therapeutic approach for an acute bout of Melkersson-Rosenthal syndrome consists mainly of steroid administration. CTS management is composed of anticholinergic drugs and surgical procedures. Botulin toxin injection into the lacrimal gland is the most modern therapeutic option. In the case presented CTS developed in a 50-year-old man after 5 incidents of facial palsy due to Melkersson-Rosenthal syndrome. The case deserves attention due to the rarity of the observed symptoms and signs.     

经颅眶入路手术治疗眼眶血管平滑肌瘤（附文献复习）

目的:探讨眼眶肿瘤手术入路的选择,报道2例眼眶血管平滑肌瘤,并对该疾病进行了文献复习。方法:2例眼眶血管平滑肌瘤分别采用额眶入路和额颞-眶颧入路手术切除。结果:2例眼眶血管平滑肌瘤均完整切除,术后半年患者视力均有改善,无永久并发症。结论:眼眶血管平滑肌瘤是一种良性肿瘤,可采用经颅眶入路手术安全切除,手术入路的选择取决于肿瘤位于视神经的上方还是下方。     

儿童眶内视神经胶质瘤的手术治疗和随访

目的 探求儿童眶内视神经胶质瘤的手术方法.方法 总结3例15岁以下(分别是1.5岁、3岁和10岁)眼眶内视神经胶质瘤临床症状和体征,肿瘤影像学发现,采用经额底入路打开前颅底(眶顶),切除视神经肿瘤.根据肿瘤切除程度和患儿年龄采用术后放疗.结果 3例患儿视力下降,2例有下凸眼,患侧瞳孔扩大且无光反射.2例肿瘤向颅内生长其视乳头呈苍白色,1例眶内局限生长视乳头呈水肿改变.MRI和CT显示3例均为右侧眶内肿瘤,呈梭状外观,其中2例肿瘤向颅内生长,但没有超过视交叉.经冠状切口额底入路,去除眶顶板,眶内段肿瘤全部切除3例,其中2例向颅内生长部分没有全切除.术后1例10岁患儿行放疗,另2例为3岁以下患儿,未行放疗.3例患儿术后连续随访,最长8年,均没有肿瘤复发.结论 对于局限存眶内段的视神经胶质瘤应予全切除,对4岁以上患儿,如有肿瘤颅内生长,应行术后放疗,患儿可获得长期生存.     

蝶眶脑膜瘤18例手术治疗临床分析

目的 探讨经颅入路显微切除蝶眶脑膜瘤的手术方法、疗效及随访结果.方法 对18例蝶眶脑膜瘤患者选用额颞入路、眶颧入路或眶颧结合扩大前颅窝底入路硬膜外结合硬膜内切除肿瘤,在硬膜外磨除增生的蝶骨大翼骨质、眶上裂、视神经管开放减压,其中经额颞入路15例,眶颧入路2例,眶额结合扩大前颅窝底入路1例,术前、术后均有视觉功能评价、手术录像及术后影像评估肿瘤切除程度.结果 肿瘤切除程度:Simpson Ⅰ级2例,SimpsonⅡ级6例,SimpsonⅢ级8例,SimpsonⅣ级2例.18例患者术后突眼情况均有好转,视觉功能改善13例.随访6 -56个月(平均25.3个月),2例患者肿瘤复发.结论 选择合适的手术入路显微切除蝶眶脑膜瘤,可获得充分显露和眶尖、球后减压,可有效缓解突眼、视觉障碍及眼肌麻痹.因肿瘤多累及眶尖、眶上裂、海绵窦等重要结构,肿瘤难以全切,术后容易复发.     

Management of orbital metastases

True metastatic disease to the orbit is rare. We present an overview of the treatment and clinical outcome of 11 orbital metastases, carried out in our center from 1995 to 2002. The surgical approach was determined by the location and type of the lesion. The most common primary cancers that metastasized to the orbit were lung cancer, and breast cancer. In 6 patients, there was no history of cancer and in 1 patient the primary site remained obscure despite systemic evaluation. Three patients showed recurrent tumor growth despite chemo- and/or radiotherapy. The mean survival time was 15 months after diagnosis of orbital metastases. The systemic prognosis is generally poor. A multidisciplinary treatment is required. Therapeutic options include surgical biopsy, debulking or excision, hormonal therapy, chemotherapy, and radiation therapy.     

Wegener's granulomatosis of the paranasal sinuses with orbital and central nervous system involvement-diagnostic imaging

Wegener's granulomatosis (WG) is a systemic vasculitis that can affect any organic system, but primarily involves the upper and lower respiratory tracts and the kidneys. WG relatively frequently affects the nervous system (in 30-50%), usually in the form of peripheral or cranial neuropathy. Involvement of the brain is reported in a very small percentage of patients (2%-8%). Three major mechanisms have been described as the cause of central nervous system (CNS) disease in WG: contiguous invasion of granuloma from extracranial sites, remote intracranial granuloma and CNS vasculitis. CNS involvement caused by contiguous invasion of granuloma from extracranial sites is the rarest. We report the case of a 37-year-old man with WG, manifested as a pulmonary and paranasal sinuses disease, with orbital and CNS involvement, caused by contiguous invasion from the paranasal sinuses. In this report, the rich spectrum of findings achieved by computed tomography and magnetic resonance are demonstrated. The importance of computed tomography in bony destruction PNS findings, and the importance of MR imaging in evaluation of the direct intracranial spread from nasal, paranasal and orbital disease are also emphasized.