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Orbital inflammatory pseudotumor due to hypersensitivity vasculitis and mononeuritis multiplex in a patient with atypical, cANCA-positive Wegener's granulomatosis.

OBJECTIVE: We report on a 60-year-old woman with a retro-orbital pseudotumor and polyneuropathy. The retro-orbital inflammation was histologically diagnosed as hypersensitivity vasculitis (HV). As cytoplasmatic antineutrophilic cytoplasmatic antibody (cANCA) and anti-proteinase-3 antibody were detected, the differential diagnosis also included atypical Wegener's granulomatosis. Hypersensitivity vasculitis is defined as small-vessel vasculitis mediated by the deposition of immune complexes (Arthus reaction) after exposure to various agents such as drugs, toxins, and infections. Since an inflammatory retro-orbital pseudotumor due to HV has not previously been reported, the following case is presented. METHODS AND MAIN OUTCOME MEASURES: Magnetic resonance imaging (MRI) revealed retro-orbital infiltrate without granuloma. Histology from an orbital biopsy confirmed HV. Electromyography was used for the diagnosis of polyneuropathy. Serum investigation indicated erythrocyte sedimentation rate (ESR) >100 mm/h, C-reactive protein (CRP) 223 mg/l, antinuclear antibodies 1:80, and cANCA 100 U/ml. RESULTS: The bilateral orbital pseudotumor, polyneuropathy, and serum levels of inflammation reactants (ESR and CRP) improved from therapy with corticosteroids (1 g of methylprednisolone initially) and azathioprine (150 mg/day). CONCLUSIONS: Because of cANCA and anti-proteinase-3 antibody positivity, this case can be viewed more as an atypical Wegener's granulomatosis than a systemic HV. The causal variety of inflammatory orbital pseudotumor, including HV and different therapeutic consequences, requires histological differentiation from usual orbital pseudotumors.

Antibodies, Antineutrophil Cytoplasmic↗

Cavernous malformations of the orbit: a distinct entity? A review of own experiences.

We collected data to provide evidence that orbital cavernous malformations (CMs) are histopathologically, neuroradiologically, and clinically different from cerebral CMs and may represent a distinct entity. In this study, the main clinical, histopathological and radiological characteristics of 19 patients (11 females and eight males, mean age 49.1 years) with orbital CMs out of a series of 376 orbital tumors are analyzed and compared with 107 cases with cerebral CMs treated in the same period. Decrease of visual acuity and painless progressive proptosis were the main clinical signs observed in 17 patients (89%). Complete microsurgical excision of lesions via individualized approaches was obtained in all cases. Follow-up examinations were obtained after a mean of 3.1 years (11 months to 7 years) and yielded complete recovery in 14 patients, while five remained clinically unchanged. Based on clinicopathological and neuroradiological studies of these 19 patients with orbital and 107 patients with cerebral CMs treated in the same period, we found evidence that orbital CMs have specific features to distinguish them from cerebral CMs. Orbital CMs, in contrast to the cerebral CMs, showed non-degenerated well-developed vessel walls and were covered by a hard and compact capsule. Clinical symptoms are characterized by the growth of orbital CMs due to intraluminal thrombosis and subsequent recanalization of cavernous vessels; there were no signs of hemorrhage. We found evidence to suggest that orbital CMs represent a distinct clinicopathologic and neuroradiologic entity.

Adult↗

Orbital exenteration: one size does not fit all.

PURPOSE: To evaluate the clinical indications for orbital exenteration in a tertiary referral center and to compare clinicopathologic correlation and cosmetic outcome with previously reported data. DESIGN: Retrospective, nonrandomized, consecutive case series. METHODS: Review of Electronic Medical Record system, Orbital Clinic, Jules Stein Eye Institute, between January 1999 and December 2003. main outcome measures: Surgery type, clear margins histologically, survival, and wearing an eye patch. RESULTS: Thirty-four patients (mean age 67 years) underwent orbital exenteration; mean follow-up 1.2 +/- 1.5 years (6 months to 6 years). Diagnosis included orbital, ocular, and adnexal malignancies, with squamous and basal cell carcinoma being the most common. Twenty-one patients (62%) underwent total or extended orbital exenteration, and 13 patients (38%) underwent subtotal exenteration including tissue reconstruction. Clear surgical margins were obtained in 23 cases (68%), whereas positive margins were left in 11 cases (32%). Many of the patients preferred an eye patch to cover the surgical region regardless of surgical reconstruction. Only 4 patients (11.8%) who underwent subtotal exenteration with orbital prosthesis did not use a patch. During follow-up period 3 patients expired, only 1 of which was tumor-related. CONCLUSIONS: Clinical indications for orbital exenteration remain similar over the last four decades with a higher prevalence of squamous cell carcinoma in our institute. Orbital exenteration is considered curative in cases of basal or squamous cell carcinoma but not in cases of malignant infiltrative processes such as adenoid cystic carcinoma of the lacrimal gland. Patients are likely to wear an eye patch regardless of any attempt at surgical reconstruction.

Adult↗

Assessment of biomechanics of orbital fracture: a study in goats and implications for oculoplastic surgery in humans.

PURPOSE: To determine the biophysical characteristics of energy absorption of the various orbital structures and the amount of force/energy required to produce an orbital fracture in goats, and to interpret results in terms of human clinical applicability. DESIGN: Experimental animal study. METHODS: The energy required to fracture the orbit on direct globe impact was measured. We used a variety of other measurements, including high-speed videography, intraocular pressure, and intraorbital pressure measurements to describe the biophysics of floor fracture. Twenty-one goats and 42 orbits were studied in this single institutional site study. RESULTS: Our measurements determined the fracture threshold for the caprine orbit averaged 26.5 N (SE 3.1 N) with a total energy threshold for fracture of 2600 mJ (SE 280 mJ). Eye deformation measured 6 mm with deformation energy dissipation measuring 100 to 180 mJ (SE 10 mJ). Eye displacement measured 10 mm with dissipation measuring 50 to 75 mJ (SE 4.0 mJ). The deconvoluted bone fracture energy averaged 2350 mJ (SE 260 mJ). CONCLUSIONS: Impact energy applied to the orbit through the eyeball is absorbed and dissipated by three primary mechanisms: (1) globe deformation, (2) globe displacement, and (3) orbital fracture. Excess energy (above the energy required for deformation and displacement) >or=2350 mJ will result in an orbital fracture.

Animals↗

Individually preformed titanium mesh implants for a true-to-original repair of orbital fractures.

The purpose of this investigation is to present the results using preoperatively-formed titanium mesh implants for a true-to-original primary repair of extensive orbital floor and medial wall fractures. Individually preformed implants were used to repair extensive orbital floor injuries in 19 patients at the University Hospital, Freiburg. The form of the orbital floor and walls was analysed by preoperative diagnostic CT scan data. The form of the virtual reconstructed orbit was transformed into a model of the orbital cavity by a template machine. Postoperative imaging by or CT scan verified the exact 3D reconstruction of the orbital cavity 'true to original'. None of the patients demonstrated diplopia or enophthalmos postoperatively. Using individually preformed titanium mesh implants, the accuracy of the 3D orbital reconstruction was within a range of 1mm. The reconstruction using preformed implants proved to be less time consuming, more precise and less invasive, compared to 'free hand' efforts, for the repair of orbital injuries using titanium mesh and calvarial grafts.

Biocompatible Materials↗

Topographical CT-data analysis of the human orbital floor.

The aim of this study was to evaluate the topographical anatomy of the human orbital floor for the production of prefabricated implants on the basis of computer tomography data. A database of 279 CT scans of Caucasian patients without traumatic deformation of the midface was analysed. 3D-image segmentation of the midfacial skeleton was performed using a computer-assisted protocol. A virtual plane (50 x 50 mm (2)) was constructed using defined landmarks above the orbital floor. An automated procedure was used to measure the distance between the orbital floor and the constructed plane at 400 distinct points. A mathematical algorithm was used to analyse the data, and to calculate a map of the orbital floor. Statistical analysis of the data revealed that orbital floor topography could be classified as distinct clusters. There were 12 variations of orbital floor anatomy: three unique patterns of the orbital floor for the right orbit and three corresponding patterns for the left side, all of which varied between the sexes. The 12 patterns were constructed with a statistical confidence interval of 1.36+/-0.6mm.

Adult↗

Atypical mycobacterial infection of the orbit.

PURPOSE: To describe the clinical presentation and successful management of an orbital infection caused by Mycobacterium abscessus, a formerly unrecognized cause of orbital disease after penetrating trauma. METHODS: An orbital infection due to M. abscessus is described, and previously reported ocular and extraophthalmic infections caused by M. abscessus are reviewed. RESULTS: A 5-year-old boy had acute, painless visual loss shortly after being struck in his left lower eyelid with a fishing rod. Radiologic evaluation established a mass in the orbital apex. Initial biopsy and cultures of the apex mass were negative; however, additional orbital exploration and cultures demonstrated M. abscessus to be the causative organism. The orbital infection was treated successfully with long-term oral clarithromycin. Review of the literature on ocular/adnexal and soft tissue infections caused by atypical mycobacteria shows characteristic clinical and histopathologic features. CONCLUSIONS: To the authors' knowledge, only seven patients with atypical mycobacterial infections of the ocular adnexa have been reported. The patient reported in the current study illustrates the difficulty in establishing the preoperative diagnosis of atypical mycobacterial infections of the orbit. A chronic draining wound or a localized orbital abscess, after penetrating trauma, should alert the physician to the possibility of an M. abscessus infection. Clarithromycin, an oral macrolide antibiotic, appears to be the most effective medical therapy for these patients.

Administration, Oral↗

Rhabdomyosarcoma presumed metastatic to the orbit.

BACKGROUND: Orbital metastasis from rhabdomyosarcoma is a rare disorder with a poor prognosis for long-term survival. Only one other detailed account of this disorder has appeared in the ophthalmic literature. METHODS: The authors report the clinical features of four patients with presumed orbital metastasis from alveolar and embryonal rhabdomyosarcoma. RESULTS: The most common ophthalmic manifestations of orbital metastasis from rhabdomyosarcoma in these patients included proptosis, reduced visual acuity, orbital pain, and motility disorders. Computed tomography documented orbital masses in all of the patients. In those patients with a primary tumor of the maxillary sinus, there was no evidence of direct extension into the orbit. Despite combination chemotherapy and radiation, all four patients died of their illness within 6 months of orbital metastasis. CONCLUSIONS: Although uncommon, rhabdomyosarcoma should be considered in the differential diagnosis of metastatic tumors to the orbit in children and adults. Despite the poor prognosis, prompt diagnosis and palliative radiotherapy may improve the quality of life for these patients with terminal disease.

Adolescent↗

Surgery of the orbit.

Orbital surgery is performed infrequently but when necessary, requires detailed understanding of orbital anatomy and the probable biologic behavior and extent of the pathologic process affecting the orbit. Thorough preoperative characterization of an orbital disease allows the surgeon to develop a surgical strategy. Inaccurate or hasty preoperative localization, determination of extension, and diagnosis may result in selection of an inappropriate surgical approach or discretionary surgery when medical treatment is indicated. In most instances, diagnostic images (MR, CT, echography) should always be made and fine-needle aspiration be done before orbital surgery is performed. The choice of surgical approach or combination of approaches is determined primarily by the type, location, size, and extent of disease present. Extensive surgical exposure of the orbit is limited to centimeters or fractions of a centimeter because of the compact anatomy and tight confines of the orbital region. Careful tissue manipulation, surgical dissection, and postoperative assessment are necessary to preserve the globe and functional vision when orbital disease endangers function.

Animals↗

The use of maxillary sinus endoscopy in the diagnosis of orbital floor fractures.

PURPOSE: The goal of the current study was to evaluate the ability to diagnose the presence of an inferior orbital wall fracture through the use of a transantral endoscopy technique at bedside. PATIENTS AND METHODS: Seven trauma patients with initial axial computed tomography (CT) scan findings consistent with an orbital floor fracture were studied. Before endoscopy, the patients underwent a coronal CT scan with 3-mm cuts for later comparison with the endoscopic findings. The surgeon performing the endoscopy procedure was blinded to the results of the coronal CT scan. Visual acuity, intraocular pressure, and measurement for enophthalmos were performed before endoscopy. The endoscopic procedure was performed at the bedside using local anesthesia. A trocar was used in the canine fossa to gain access to the maxillary sinus. A 30 degrees and then a 70 degrees endoscope were introduced through the trocar to evaluate the integrity of the orbital floor (ie, maxillary sinus roof). The degree of mucosal injury of the orbital floor and the presence of blood or orbital contents in the sinus were recorded. The ophthalmologic examination was repeated after completion of endoscopy. RESULTS: The endoscopic procedure was able to be completed in all patients. There was no change in the ophthalmologic examination in any patient as a result of endoscopy. In six of the seven patients studied, the endoscopic findings correlated with the need for surgical intervention to repair the orbital floor predicted on the basis of coronal CT scan. This was determined by the degree of injury to the orbital floor and the presence of hematoma, exposed bone, or fat. In the remaining case, endoscopy was not diagnostic for the presence of a fracture because only ecchymosis of the orbital floor was noted. CONCLUSIONS: The ability to perform endoscopy under local anesthesia at the bedside is useful in those trauma patients whose concomitant injuries may prohibit other diagnostic modalities.

Adolescent↗

Synthetic hydroxyapatite orbital implants: a clinical and MRI evaluation.

PURPOSE: Coralline hydroxypatite orbital implants have been used since the 1980s. More recently, synthetic hydroxyapatite orbital implants have been used, in both primary and secondary orbital implantation surgery. The implant may be drilled and pegged, if required, after adequate vascularisation of the implant has occurred. In this study we evaluated the clinical results and vascularisation of synthetic hydroxyapatite orbital implants. METHODS: Twelve consecutive patients who had synthetic hydroxyapaptite orbital implants were evaluated clinically and 8 of these patients had orbital magnetic resonance imaging (MRI) scans with intravenous gadolinium performed at least 9 months post-operatively to assess vascularisation of the implant. Six patients had primary orbital implants at the time of enucleation and 6 patients had secondary implants. RESULTS: No significant complications occurred following insertion of the synthetic hydroxyapatite orbital implants. All patients reported cosmetic satisfaction with the results of surgery. MRI scans revealed inhomogeneous enhancement in 3 of the 4 patients receiving primary implants. All patients with secondary implants and 1 patients who had a primary implant had moderate to large areas of poor enhancement in the implant. CONCLUSION: Synthetic hydroxyapatite implants gave good clinical results but variable vascularisation occurs, especially with secondary implants.

Adolescent↗

Update on orbital anatomy.

The orbit is a confined space bounded by four bony walls. Apart from the globe, orbital fat, and extraocular muscles it contains many important neurovascular structures. It is intimately related to the paranasal sinuses as well as the anterior and middle cranial fossae. Consequently, sinus pathology and intracranial disease may spread to involve the orbit and the converse applies. A thorough understanding of orbital anatomy is essential to fully appreciate the effects of disease on the orbit and is of paramount importance for performing safe orbital surgery. The anatomy of the orbit is discussed with reference to relevant orbital disease.

Cranial Nerves↗

Sino-orbital fistula: two case reports.

A fistula between the paranasal sinuses and the orbit as a late complication of orbital fractures is rare and may present with intermittent symptoms due to air passing into the orbit. A case note review of two patients with sino-orbital fistula is presented. Two patients, 23- and 30-year-old males, presented with intermittent symptoms of globe displacement, diplopia or discomfort months after repair of an orbital floor fracture with a synthetic orbital floor implant. The symptoms occurred after nose blowing. They were both cured by removal of the implant and partial removal of the tissue surrounding the implant. A sino-orbital fistula may complicate the otherwise routine repair of an orbital floor fracture, but may be cured by removal of the implant and part of the surrounding pseudocapsule.

Adult↗

Orbital floor reconstruction with an alloplastic resorbable polydioxanone sheet.

The orbital floor is frequently reconstructed after blow-out fractures or midface fractures to avoid a relapse of the repositioned orbital tissue and to prevent enophthalmos. A total of 31 patients underwent reconstruction of internal orbital wall fractures with a resorbable 0.25 mm or 0.5 mm-thick polydioxanone implant (PDS). Skeletal and functional outcome was evaluated retrospectively with regard to fracture size. Fracture size was graded as small, moderate or large by CT scans and operating records. Two of the 25 patients with small or moderate defects showed an enophthalmos of 2-3 mm. Five of the six patients with large defects or two orbital wall fractures had enophthalmos. The scar that formed after implant resorption was to weak to provide adequate support of the globe or to compensate the enlarged orbital volume. Endoscopic follow-up examination of 12 patients showed yielding of the scar in the orbital floor already in moderate defects. Eight patients had diplopia in extreme gaze and two had significant diplopia. Blow-out and midfacial fractures with small to moderate defects in the orbital floor (up to a size of 2.5 cm2) can be reconstructed by polydioxanone sheet to avoid enophthalmos. Polydioxanone implants should only be used in cases without massive orbital fat herniation. The scar formed after implant resorption may influence functional outcome.

Absorbable Implants↗

[Orbital diseases in childhood].

BACKGROUND: Orbital diseases in childhood are rare and differ from orbital lesions occurring in adults. Most paediatric orbital tumours are benign, but rapid tumour growth can nevertheless threaten vision or lives. PATIENTS: Retrospectively, we analyzed data of 41 consecutive patients with orbital disease, treated in our hospital between 2000 and 2004. The data was evaluated using descriptive statistics and case reports. RESULTS: All children (from neonatals up to 16 years of age) with orbital disease were included in the study. The most common benign disease of the entire collective was an epidermal cyst (n = 18), followed by orbital capillary haemangioma (n = 4) in children under 3 years, and preseptal cellulitis in children older than 3 years. 5 patients were treated for malignant tumours, of which one patient under 3 years had an orbital metastasis of an abdominal neuroblastoma. CONCLUSION: Awareness of orbital diseases in childhood, as a rare entity, is crucial for timely diagnosis and appropriate treatment, to save the vision and lives of affected children.

Adolescent↗

Single stage management of complex fronto-orbital mucoceles.

Mucoceles arising in the frontal and/or ethmoid sinuses are clinically silent and often involve both the orbit and the anterior cranial fossa extensively. Five patients with advanced mucoceles of the frontal sinus involving the orbitocranial skeleton (OCM), as a result of recurrent sinusitis or trauma, were identified during a 36-month period in a major academic US institution. The range of pathology encountered involved brain herniations, CSF leaks, and extensive orbital involvement. These patients required major reconstructive efforts to restore proper orbital skeletal anatomy and function with the use of multiple calvarial bone grafts. The secondary effects of the pathologic process, such as orbital dystopia, ocular dysfunction, and CSF leak among others, resolved in all patients without further problems. Follow-up by repeat computed tomography and examination scan ranged from 6 weeks to 29 months. Adequate reconstruction of extensive OCMs involves four principles: 1) cranialization of the frontal sinus for adequate intracranial and orbital roof access; 2) complete removal of all mucosal elements from the frontal sinus, orbit, and dura; 3) precise restoration of orbital volume with calvarial bone grafts; and 4) reattachment of the medial canthal ligament to the bone grafts to avoid orbital dystopia. The results of the surgery are gratifying, but long-term follow-up is essential due to the risk of recurrence.

Adult↗

Orbital reconstruction with proplast.

Proplast was implanted in 15 cases of orbital reconstruction. Seven cases were orbital floor fractures, four cases were phthisical enophthalmos, and four cases were anophthalmic enophthalmos. In orbital floor fracture cases, thin (2-mm) Proplast was placed onto the fractured orbital floor after the incarcerated orbital tissue was released. To correct the enophthalmic socket, Proplast was shaped into small pieces (10 x 5 x 2 mm), then inserted into the subperiosteal space from all directions to fill up the orbital cavity. The results of these 15 cases were satisfactory. The average follow-up period is 13 months. The increased orbital volume seems less than the volume of the implanted Proplast; therefore, we suggest an 80-90% overcorrection rate in accordance with the computer measurements. In sum, Proplast serves as a very good material for orbital floor reconstruction and volume replacement.

Adolescent↗

Orbital exenteration. The reconstructive ladder.

Following orbital exenteration, there is a spectrum of immediate and delayed options for orbital reconstruction. Goals of reconstruction after exenteration include detection of recurrent disease, restoration of boundaries between the orbit and surrounding structures, and optimal aesthetics. Local solutions to problems of the exenterated orbit, such as healing by granulation or application of split-thickness skin grafts, are advantageous for detecting recurrent disease. Regional solutions, involving transfer of periorbital tissue into the orbit, may mask recurrent disease and create adjacent deformity; however, these solutions can be used to restore orbital boundaries and shallow the orbital cavity. Distant solutions, such as skin-muscle flaps and free tissue grafts, allow for facial reconstruction in patients with extensive orbital and periorbital defects.

Adipose Tissue↗