Orbital Signs Research Articles

Commentary

Vascular malformations in and around the brain and skull base are not rare, and many cause neurological and neuro-ophthalmic signs and symptoms. When the orbit is involved, arteriovenous malformations (AVMs) may manifest with primary orbital signs, including proptosis, chemosis, and dystopia. Secondary signs may include problems associated with optic nerve function and morbidity related to orbital venous congestion. Digital subtraction angiography remains the gold standard of diagnosis, but the past 15 years have provided a revolution in neurointerventional therapeutic techniques. Small tracker catheters and more experienced neuroradiologists can maneuver into small feeders, embolizing AVMs transarterially or even transvenously.

Clinical signs of orbital wall fractures as a function of anatomic location.

The objective of this study was to see whether clinical signs of medial orbital wall fractures distinguished these fractures from fractures of the lateral orbital wall and the orbital floor. The orbital fractures of 424 patients were analyzed. The patients were divided into 2 groups: (1) patients with orbital fractures with a medial orbital wall component and (2) patients with orbital fractures without a medial orbital wall component. Orbital fractures with involvement of the medial orbital wall showed a significantly higher incidence (P =.001) of diplopia and exophthalmos (P =.039) than fractures without involvement of the medial wall. Posttraumatic orbital clinical signs are associated with a higher incidence of medial orbital wall component fracture. Apparent lack of involvement of the medial orbital wall should not be an exclusion criterion for a surgical intervention when clinical orbital signs exist.

Orbital presentation of posttransplantation lymphoproliferative disorder: A small case series

Objective To describe a small series of patients with orbital presentation of posttransplantation lymphoproliferative disorder (PTLD). Design Retrospective, interventional case series. Participants Three patients with orbital presentation of histologically diagnosed PTLD. Methods Review of medical records. Main outcome measures Measured parameters included vision, proptosis, and tumor extent. Results Three cases of orbital PTLD are described. In two of the cases, the tumor initially demonstrated orbital signs and symptoms, whereas in the third case, orbital and systemic signs were synchronous. Two of three patients had disseminated disease discovered at the time of presentation. One adult patient had synchronous presentation of PTLD in the orbit and prostate. One pediatric patient had tumor dissemination to the liver at the time of presentation. The PTLD tumors were classified histologically as diffuse large cell lymphoma of monomorphic or immunoblastic type in all three cases. Treatment included local irradiation, decreased immunosuppression, and antilymphocyte monoclonal antibodies. Conclusions Orbital presentation is a rare manifestation of PTLD. However, ophthalmologists must consider this diagnosis carefully in organ transplant recipients with subtle orbital signs and symptoms at presentation. Early detection may alter prognosis. In each case presented, the diagnosis was established via lesion biopsy and subsequent histologic or flow cytometric evaluation, or both.

Bilateral orbital signs predict cortical venous drainage in cavernous sinus dural AVMs. Steibel-Kalish H, Setton A, Berenstein A, Kalish Y, Nimii Y, Kupersmith MJ. Neurology 2002;58:1521-1524.

Cavernous sinus dural arteriovenous malformations (CSdAVM) cause a spectrum of ophthalmic signs and symptoms ranging from a purely ocular or orbital syndrome to less common aggressive neurologic dysfunction due to intracerebral hemorrhages or cerebral venous infarction. Most CSdAVMs have a benign clinical course, but those that drain into cortical veins have an increased risk for neurologic complications. The objective of this study was to determine whether a specific clinical sign predicts cortical venous drainage in CSdAVMs. The records of 118 patients with CSdAVMs were evaluated for the clinical features of the disorder and tested for predictive value of cortical venous drainage. Clinical signs that predicted the presence of cortical venous drainage included bilateral orbital signs (P = 0.004 OR = 23.84) and presence of a postauricular bruit (P = 0.035 odds ratio (OR) = 23.8). No other clinical sign predicted the presence of cortical venous drainage, including extraocular muscle dysfunction, abducens or oculomotor nerve dysfunction, increased intraocular pressure, venous stasis retinopathy, chroroidal effusion, optic neuropathy, subjective bruit, and objective orbital bruit. The authors concluded that patients who present with or develop bilateral orbital congestion from CSdAVMs should be recognized as being at increased risk for cortical venous drainage and venous infarction. These patients should have a contrast-enhanced MRI scan and be considered strongly for timely cerebral angiography and endovascular therapy if cortical venous drainage is found.—Nancy J. Newman

Bilateral orbital signs predict cortical venous drainage in cavernous sinus dural AVMs.

Most cavernous sinus dural arteriovenous malformations (CSdAVM) have a benign clinical course. Those CSdAVM that drain into cortical veins have an increased risk for neurologic complications. To find whether a specific clinical sign predicts cortical venous drainage (CVD) in CSdAVM. The records of 118 patients with CSdAVM were evaluated for the clinical features of the disorder and tested for predictive value of CVD demonstrated angiographically or suggested by MRI using logistic regression and odds ratio (OR) analysis. Clinical signs that predicted the presence of CVD included bilateral orbital signs (p = 0.004, OR = 23.84) and presence of a postauricular bruit (p = 0.035, OR = 23.8). No other clinical sign predicted the presence of CVD, including extraocular muscle dysfunction, abducens or oculomotor dysfunction, increased intraocular pressure, venous stasis retinopathy, choroidal effusion, optic neuropathy, subjective bruit, and objective orbital bruit. Patients who present with or develop bilateral orbital congestion should be recognized as being at increased risk for CVD.

Orbital extension of a frontal sinus osteoma in a thirteen-year-old girl

Osteomas are uncommon, slow-growing, benign osteogenic neoplasms that arise most frequently in the craniofacial skeleton. 1,2 Osteoma is the most common benign tumor of the nose and paranasal sinuses and the most common neoplasm of the frontal sinus. 3–5 Paranasal sinus osteomas originate in the sinus wall, fill the lumen with well-defined mature osseous tissue, and occasionally extend into the orbit where they give rise to orbital signs and symptoms. Osteomas most commonly become symptomatic in the second to fifth decade in life, but orbital involvement has rarely been reported in patients aged 18 years and younger. 2,6–10 We report a case of a frontal sinus osteoma with orbital extension in a 13-year-old girl.

Evolution of spin and orbital magnetic moments anisotropies with chemical order in Fe 0.50Pd 0.50 alloy films

Large spin and orbital anisotropies have been obtained in MBE-grown Fe 0.50Pd 0.50 alloys of various chemical orders, using magnetic circular X-ray dichroism (MCXD) at the Pd L 2,3 edges. Both anisotropies are shown to vary smoothly as a function of chemical order. The Pd orbital anisotropy changes sign for the transition between in-plane and perpendicular magnetic anisotropy (PMA). Some discussion on the microscopic understanding of the magnetocrystalline energy (MAE) is given.

Myosite orbitaire aiguë et pseudotumeur inflammatoire idiopathique de l'enfant : Trois cas

Orbital pseudo-tumors account for approximately 8% of the orbital tumors. They are scarcely described in children and raise diagnostic dilemas, especially when they present as an isolated extra-ocular muscle swelling called idiopathic orbital myositis (IOM). A diagnosis of inflammatory orbital pseudotumors (IOPT) was made in three children aged 7, 13 and 14 years from clinical and CT scan and/or MRI findings associated with histological data in two of them. Two of these IOPT presented as IOM. Analysis was made in a effort to eliminate intra-orbital tumors, especially rhabdomyosarcoma. The abrupt onset of the orbital signs, often related in the literature, was not a specific diagnostic criterion regarding to embryonic rhabdomyosarcoma. The inconstant presence of inflammatory signs and the absence of local osseous defect could be more discriminating. Although diagnostic procedure, including histological documentation, was difficult, even dangerous, surgical biopsy should be recommended. Eventually, considering the mandatory multidisciplinary care, steroid therapy response should be evaluated as a diagnostic test.

Three cases of isolated mucoceles and one case of an Aspergillus infection in the sphenoid sinus with orbital signs and symptoms

Four patients with an isolated sphenoid lesion: three with a mucocele and one with an Aspergillus infection of the sphenoid sinus in whom the orbital impairment had a rhinogenic cause, are described. They all presented with one or more of the following features: visual loss, motility disturbances, visual field defects, and sensitivity loss of the infraorbital nerve. In one patient, optic disc edema was seen. Diagnostic imaging technologies such as computer tomography (CT) and magnetic resonance (MRI) make an early diagnosis possible but nevertheless, very large mucoceles with major functional loss were detected at a late stage of the disease. The Aspergillus infection was detected at an early stage without delay because of its specific appearance on CT and MRI. The signs and symptoms of these lesions are more striking in the orbital and neuro-ophthalmological field than in the ear-nose-throat (ENT) field, but nevertheless, primary treatment should be marsupialization by an ENT-surgeon.

The Groote Schuur hospital classification of the orbital complications of sinusitis.

The complications of sinusitis have been well described. The most common classifications used for orbital complications have been that of Chandler et al. (1970) and Moloney et al. (1987). With the ready availability of high-resolution computed tomography (CT) scanners, limitations of these classifications have become apparent. The aims of this study were to determine the relative frequency of the various complications associated with acute sinusitis, to determine which groups of sinuses were most frequently involved and to correlate the orbital signs with a new proposed classification of orbital complications. Over a five-year period, 87 consecutive patients were admitted with acute sinusitis. Sixty-three patients (72.4 per cent) had one or more complications. When orbital complications were classified under the proposed classification, all patients with proptosis and/or decreased eye movement had post-septal infection. Visual impairment occurred only in the post-septal group. Most complications had a combination of sinus involvement with the maxillary/ethmoid/frontal combination being the most common. The authors propose a modification of Moloney's classification for orbital complications of acute sinusitis that allows a clear differentiation between pre- and post-septal infection and a radiological differentiation to be made between cellulitis/phlegmon and abscess formation. The latter is of importance when a decision is made on whether surgical intervention is appropriate or not.

Prominent Proptosis in Childhood Thyroid Eye Disease

Background: Orbital signs and symptoms occur in approximately one half of children with Graves disease, but the symptoms are usually minor and limited to the eyelids. Prominent proptosis is uncommon in children with this disorder.Methods: Review of eight children with prominent proptosis associated with thyroid eye disease. Four patients were treated at the Children's Hospital of Philadelphia, the other four at the Columbia Presbyterian Medical Center.Results: At initial presentation, children ranged in age from 3 to 16 years. There were five girls and three boys. Seven of eight children had hyperthyroidism at ophthalmic presentation. Four patients had restrictive myopathy, and all of the seven patients who underwent neuroimaging had extraocular muscle enlargement. Five patients were treated with lubrication. Two underwent orbital fat decompression. One patient had thyroid eye disease and myasthenia gravis.Conclusions: Proptosis in childhood thyroid eye disease usually is associated with a hyperthyroid state. The proptosis may be dramatic, but corneal exposure and restrictive myopathy are seen in only some of the patients. Neuroimaging shows enlarged extra-ocular muscles. Most children with this complication can be treated conservatively with topical lubrication, but orbital fat decompression may be considered in patients with more advanced conditions.

Intraocular Retinoblastoma Presenting As Ocular and Orbital Inflammation

In the pediatric population, retinoblastoma must be included in the differential diagnosis of intraocular or orbital inflammation, especially if the case is atypical of unresponsive to medical therapy. Diffuse infiltrating retinoblastoma, a rare subtype of retinoblastoma, often presents as unilateral intraocular inflammation, usually occurs in older children, and may contain insufficient calcification for detection by CT scan. Orbital inflammatory signs may also be associated with intraocular retinoblastoma. The clinician must perform a thorough ocular examination in these cases with the suspicion of retinoblastoma. He or she must also understand the subtleties of noninvasive imaging in such cases. The goal is to diagnose this tumor more accurately and expediently so that treatment and counseling can be provided without delay.

Hydraulic Orbital Injection Injuries

A high-pressure stream from mechanical equipment may inject gas or liquids deep into the orbit with few initial clinical signs. Aggressive surgical debridement as used in the extremities for the treatment of injection injuries is not possible in the orbit. Four patients with orbital injection injuries from farm or industrial equipment are presented. Previously reported cases of high-pressure injection injury are reviewed. Two patients suffered localized anterior orbital inflammation partially responsive to steroidal and nonsteroidal anti-inflammatory agents. Late debridement was required in one patient for a persistent lipogranuloma. Two patients suffered more dramatic and diffuse injections of hydrocarbon mixtures, requiring emergent early surgical debridement and decompression for compressive orbital signs. All patients attained an adequate functional outcome, with one patient's vision limited by a coexisting ocular injury. High-pressure orbital injection injuries manifest a spectrum of signs ranging from acute inflammation with tissue necrosis and compressive visual loss to late chronic inflammation with a pseudotumor-like course. The authors recommend the initial treatment of orbital injection injuries with systemic antibiotics followed by prompt neuroradiologic imaging. Systemic corticosteroids should be added for confirmed injection injuries with surgical debridement of discrete masses and orbital decompression when indicated. Continued therapy with anti-inflammatory medication may be required to suppress chronic inflammation with selective late surgical debridement of lipogranulomas.

Orbital and periorbital pain caused by cavernous sinus and superior orbital fissure lesions

The causes of superior orbital fissure/cavernous sinus syndrome, especially in relation to orbital pain, are discussed. The type of orbital pain, associated with other orbital and neuroophthalmological signs and symptoms, is a guideline for establishing the diagnosis and choosing the appropriate treatment. The signs and symptoms are so characteristic that the diagnosis is obvious only from taking an accurate history, and simple tests such as erythrocyte sedimentation rate, inspection and auscultation, perimetry and CT-scan.The Tolosa-Hunt syndrome, giant aneurysm, parasellar tumors, cavernous sinus thrombosis and carotid cavernous sinus fistula are discussed

Orbital Phlebography and Signs of Inflammation in Episodic and Chronic Cluster Headache

One of our 7 patients (14%) with chronic cluster headache had an abnormal orbital phlebogram; this was significantly less than the 61% encountered in our 13 patients with active episodic cluster headache who had this test done. There were no pathologically increased values for serum haptoglobin or orosomucoid in our 9 patients with chronic cluster headache, again significantly less than in our 43 patients with active episodic cluster headache, 51 percent of whom had pathologically increased values of haptoglobin or orosomucoid. These inflammatory signs decreased after the episodic cluster headache was over. Episodic cluster headache we suggest to be due to temporary sympathicoplegia caused by venous vasculitis in the cavernous sinus region; chronic cluster headache we attribute to permanent post-inflammatory sympathicoplegia in the middle fossa.

Carotid and Vertebral Artery Trauma: Clinical and Angiographic Features

Injury to the carotid or vertebral artery is an important clinical entity that requires angiography for definitive diagnosis and evaluation. The common carotid artery may be injured by penetrating trauma while the internal carotid artery is usually damaged by either trivial or blunt trauma. With trivial trauma extracranial internal carotid artery dissection should be considered if there is unilateral headache, Horner's syndrome or delayed transient ischaemic attack, and intracranial dissection if a profound neurological defect occurs immediately following trauma. Injury to the internal carotid artery following blunt trauma includes dissection of the extracranial internal carotid artery, carotid-cavernous fistula and pseudoaneurysm formation. These should be considered in a patient with delayed neurological deficit, mandibular or skull fracture, a constellation of orbital signs or diffuse subarachnoid haemorrhage, respectively. Vertebral artery injury is less frequent. Dissection typically follows abrupt cervical rotation and occurs at C1-2, whereas penetrating trauma may involve either the proximal or distal vertebral artery and occlusion, arteriovenous fistula or pseudoaneurysm may be found. Endovascular techniques may be used in either the carotid or vertebral artery to close fistulae or occlude an extensively damaged vessel.

Metastatic thyroid carcinoma masquerading as lacrimal gland tumor

A 56-year-old woman presented with orbital signs and symptoms suggestive of lacrimal gland tumor. An excised biopsy specimen was obtained and showed glandular tissue, which could be confused with lacrimal gland acini. Closer microscopic examination and immunohistochemical studies revealed a metastatic tumor of thyroid gland origin. Initially, there was no identifiable nodule in the thyroid, but 3 months later a thyroid nodule was found by ultrasound and radioisotope scan. The histopathologic appearance of the thyroid nodule was similar to that found in the orbit. This case demonstrates the usefulness of immunohistochemistry in establishing a diagnosis when the microscopic appearance is inconclusive.

Post-operative cyst and orbital walls: a CT image analysis

A set of coronal CT images obtained by 118 side (109 cases) of post-operative cysts of maxilla, seen from 1982 to 1988, was analyzed as to the orbital wall affections. Coronal images were grouped into five consecutive planes, i.e. the first plane being the one which is 5mm posterior to the nasion, and the other planes in successive orders each being 5mm apart. The orbital walls were classified into inferior, medial, and transition of these two. The orbital wall affections were classified into dehiscence, thinned-out (less than 1mm), and normal. The orbital signs and symptoms (epiphora, pain, asthenopia, visual loss, diplopia, and exophthalmos) were analyzed based upon the questionnaires by patients. The inferior wall affections (dehiscence and thinned-out) were seen most often (57%) at the third plane (2cm posterior to the nasion). The orbital signs and symptoms in general were seen in half of the cases of which 20% were of grave (visual loss, diplopia and exophthalmos). These signs and symptoms were the more often, the more anterior the site of affections located. Grave signs and symptoms were seen at 2 to 2.5cm posterior to the nasion when the orbital floor was affected and the inferior rectus were either pushed up or in direct contact with the cysts.

Optic nerve sheath meningioma: diagnostic features and therapeutic alternatives.

Twenty-two cases of optic nerve sheath meningioma were reviewed. The clinical features included slowly progressive visual loss in every case. Orbital signs of limited ocular movements and mild proptosis were present in only a third of cases; gaze-evoked amaurosis occurred in 3 cases. The disc was abnormal in every case, usually swollen if vision was 6/12 or better, atrophic if the vision worse than 6/12. Optociliary shunt vessels occurred in 5 patients and were of diagnostic significance. The diagnosis was made by a high-resolution CT scan of the orbits showing tubular expansion of the optic nerve sheath. Calcification of the optic nerve was present in 12 cases. Tubular expansion of the optic nerve sheath may occur in raised intracranial pressure, optic nerve glioma, granuloma, lymphoma or metastatic disease. In the absence of calcification these alternative diagnoses must be considered. Surgery, undertaken for the intracranial component of the tumour, did not halt progressive visual loss.

Ophthalmic Manifestations of Esthesioneuroblastoma

Esthesioneuroblastoma, a tumor that arises from the olfactory sensory epithelium, often manifests with ocular and orbital signs and symptoms. A review of 38 cases of esthesioneuroblastoma at the Mayo Clinic revealed that ophthalmic signs or symptoms occurred in 28. Twenty patients had ophthalmic signs or symptoms at the time of presentation, and five of these had ocular symptoms as the primary complaint. Five patients presented to an ophthalmologist, but a nasal or paranasal sinus tumor was not suspected. Periorbital pain and excessive tearing were the most commonly reported symptoms. The most common ophthalmic sign was eyelid edema followed by proptosis, globe injection, and ptosis. Recognition of the signs and symptoms of neoplasms of the nose and paranasal sinuses and consideration of esthesioneuroblastoma in the differential diagnosis by clinicians and pathologists allow for prompt management of this tumor.