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Computed tomography in planning and evaluating orbital surgery.

Computed tomography (CT) scanning of the orbit has enhanced the diagnostic and surgical armamentarium of the orbital surgeon, localizing the lesion in the retrobulbar spaces and separating those expanding masses confined to the orbit from those that invade from adjacent structures. It relates an orbital lesion to the surgical spaces, optic nerve and globe, and bony orbital walls, guiding the operator in his surgical approach. If the lesion has escaped from the orbit into the intracranial cavity, CT scanning can detect it. In this way, the surgical teams and surgical approaches are chosen. After surgery, CT scanning can detect a local recurrence of a tumor and note if it has extended intracranially. However, invasion of the optic canal may be better detected by polytomography. There is also some difficulty in distinguishing between a tumor recurrence and postoperative inflammation. Postoperative follow-up evaluation by serial CT scanning every six months is advocated to determine these changes.

Adolescent↗

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.

Aged↗

Tissue eosinophilia and eosinophil degranulation in orbital pseudotumor.

To investigate the participation of the eosinophil in orbital pseudotumor, the authors studied surgical biopsy specimens from nine patients with pseudotumor. Surgical biopsy specimens of orbital tissue from four patients with Graves' ophthalmopathy and autopsy specimens of orbital tissue from six patients without orbital diseases served as controls. Eosinophil infiltration and degranulation were assessed by immunofluorescence staining of formalin-fixed, paraffin-embedded tissues for the cytotoxic eosinophil granule major basic protein. Eosinophil infiltration and extracellular major basic protein deposition were evident in all orbital pseudotumor specimens. In contrast, no eosinophil infiltration or extracellular major basic protein deposition was present in any of the ten control specimens. These findings indicate that eosinophil degranulation is found in orbital pseudotumor, and they also identify yet another clinical entity where eosinophil infiltration and degranulation are associated with fibrosis.

Adolescent↗

Unilateral Blastomyces dermatitidis endophthalmitis and orbital cellulitis. A case report and literature review.

PURPOSE: The authors report the clinical, cytologic, and histopathologic findings of a unique presentation of concomitant unilateral endophthalmitis and orbital cellulitis secondary to Blastomyces dermatitidis. DESIGN: Case report. METHODS: A 29-year-old healthy woman with a history of pulmonary tuberculosis presented with a painful right eye and rapidly decreasing vision. Fundus examination showed a diffuse elevated choroidal lesion at the posterior pole. With an otherwise unremarkable systemic work-up, the patient was treated with systemic antibiotics and corticosteroids for a presumed diagnosis of choroidal tuberculous granuloma. After an initial response to the treatment, the patient's condition deteriorated rapidly with visual acuity decreasing from 20/25 to no light perception in 3 months. Ipsilateral proptosis developed with magnetic resonance imaging showing a poorly defined orbital mass. Surgical enucleation and an orbital biopsy were performed. RESULTS: Histopathologic examination of the orbital specimen and an intact enucleated globe showed a diffuse necrotizing granulomatous process with the presence of numerous yeasts consistent with B. dermatitidis. This subsequently was confirmed by positive culture of B. dermatitidis from the orbital specimen. CONCLUSIONS: This is a unique case of concurrent unilateral endophthalmitis and orbital cellulitis secondary to B. dermatitidis. Intraocular dissemination of blastomycosis should be suspected in the differential diagnosis of endophthalmitis in patients with previous or active pulmonary lesions of equivocal nature. Early diagnosis and prompt treatment with antifungal medications are essential.

Adult↗

Suck and spit, don't blow: orbital emphysema after decompression surgery.

PURPOSE: To describe the occurrence of vision-threatening orbital emphysema in patients awakening from orbital decompression surgery and to assess risk factors and preventive measures. DESIGN: Small noncomparative case series. PARTICIPANTS: Three patients undergoing bilateral orbital two-wall decompression experienced significant orbital emphysema associated with persistent coughing and Valsalva at the time of extubation. INTERVENTION: In two patients, symptoms resolved with simple observation, whereas one patient required sedation, topical anesthesia around the endotracheal tube, and needle decompression of trapped air. MAIN OUTCOME MEASURES: Visual acuity, pupils, visual fields, and sensorimotor examination. RESULTS: No patient experienced a permanent deficit of visual or sensorimotor function. CONCLUSIONS: Acute orbital emphysema can occur after orbital decompression surgery despite the large bony opening created. Violent coughing spells at the time of extubation are more common in patients with a history of heavy tobacco use and may be causative. Opening the periorbita may be another specific predisposing risk factor. Knowledge of this dangerous phenomenon, along with appropriate perioperative management, may prevent this complication from occurring.

Acute Disease↗

Orbital emphysema: how common, how significant?

STUDY OBJECTIVE: To describe the natural history of orbital emphysema and its management in the emergency setting. DESIGN: Retrospective medical record review. SETTING: Inner-city integrated hospital system (four divisions) including one Level I trauma center. PARTICIPANTS: All cases of orbital floor fracture and orbital emphysema from January 1, 1988, to December 31, 1993. RESULTS: The average patient was aged 33 years, and the male-to-female ratio was 7 to 1. Trauma was the underlying etiology in all patients. Seventy-four percent of patients required hospitalization, and 43% developed significant complications. Eighty-three percent were managed with antibiotics. Orbital emphysema was identified on conventional radiographs in 65% of cases. CONCLUSION: Identification of orbital emphysema in the ED should prompt a careful search for associated injury. Consultation should be sought in the presence of orbital or ocular injury. Antibiotic prophylaxis is not necessary for clean wounds.

Adolescent↗

A prospective study of orbital fracture sequelae after change of surgical routines.

PURPOSE: The present study was undertaken to investigate the circumstances surrounding the considerable increase in the number of orbital floor fracture repairs at the ORL Clinic at Sahlgrenska University Hospital at the end of the 1990s. PATIENTS AND METHODS: All the patients during a period of 1 year with a fracture involving the orbital floor were followed for 1 year using clinical assessments and questionnaires. Etiology, surgical methods, and the occurrence of long-term sequelae were investigated. RESULTS: Fifty-one patients were included. The main etiologies were assaults and falls. Surgery of the internal orbit was performed in 76%, often combined with a Gillies reduction. Porous polyethylene sheets (Medpor; Porex Surgical Inc, Newnan, GA) were used for the repair of large floor defects. Compared with previous results, the number of computed tomography investigations had considerably increased. The number of operations on the internal orbit due to fractures had doubled. The occurrence of diplopia was reduced compared with the situation when the floor was stabilized with an antral packing. Late enophthalmos developed in 11%. Despite fixation, the majority (67%) reported permanently affected sensibility. CONCLUSION: The frequency and severity of diplopia decreased parallel to the introduction of Medpor implants and the termination of the antral packing technique. Tetrapod zygomatic fractures, possibly unnecessarily submitted for surgery of the internal orbit, were in part responsible for the increase in the number of orbital explorations, in spite of preceding computed tomography scans. This indicates inadequate diagnostic techniques. Studies evaluating alternative complementary diagnostic methods are in progress.

Adolescent↗

Fractionated, three-dimensional, planning-assisted proton-radiation therapy for orbital rhabdomyosarcoma: a novel technique.

PURPOSE: Most children with orbital rhabdomyosarcoma will survive their disease. However, conventional photon-radiation treatment, as part of multimodality therapy, results in varying degrees of long-term functional and cosmetic side effects. This report introduces external beam proton radiation therapy (PRT) as a conformal, three-dimensional planned radiation technique for this disease, analyzes normal tissue dosimetry, and describes the technique's application in the first 2 patients. MATERIAL AND METHODS: Between January 1995 and February 1996, 2 patients underwent PRT following biopsy and chemotherapy for orbital rhabdomyosarcoma. Fifty and 55 Cobalt Gray Equivalent (CGE) were delivered to the gross tumor volume and 40 CGE to clinical target volumes in both patients. A relative biologic effectiveness (RBE) of 1.1 was utilized to correlate proton dose calculations with CGE. To achieve dose conformity, a "patch technique" was utilized, where target regions were divided into segments, each treated by a separate proton field. Dose-volume histograms were obtained for target and nontarget regions, including lens, bony orbit, pituitary gland, optic chiasm, optic nerves, lacrimal gland, and ipsilateral frontal and temporal lobes. RESULTS: At 3.4 and 2.5 years after PRT, both patients are clinically and radiographically free of disease. Visual acuity remains excellent, without signs of cataract formation; pituitary function is normal; cosmetically, only mild enophthalmos is noticeable. Doses to 90%, 50%, and 5% of lens volume were kept at less than 1%, less than 2%, and less than 8%, respectively. Fifty percent of lacrimal gland volume received less than 36% of the prescribed dose and 50% of the volume of the optic chiasm, pituitary gland, and hypothalamus were restricted to less than 2%. Proton conformity to orbital contents resulted in between 9% and 36% of the prescribed dose reaching the ipsilateral temporal and frontal lobes immediately adjacent to bony orbit (5% volume). CONCLUSION: PRT can offer excellent sparing of lens and selected intraorbital and ocular normal structures, while maintaining conformal target-dose coverage. The steep dose gradient beyond the orbit minimizes irradiation of normal brain parenchyma, with almost complete sparing of the pituitary gland. Reduction of integral irradiation exposure of the periorbital region will, hopefully, reduce the risk of second malignancy later in life. Reduced radiation dose to specific organs in close proximity to, but not part of the target region promises improved functional outcome and better cosmesis for childhood cancer survivors.

Child↗

Pseudotumor of the orbit in early childhood.

Orbital pseudotumor, also known as idiopathic orbital inflammation, is defined as a nonspecific, nonneoplastic inflammatory process of the orbit without identifiable local or systemic causes. The disorder, first described by Birch-Hirschfield in 1905, is more prevalent in the adult population than in the pediatric population. In our study we discuss two cases of pseudotumor of the orbit in children less than 18 months old. This report will highlight the evaluation and management of pediatric orbital pseudotumor and the importance of its inclusion in the differential diagnosis of orbital disorders in young children.

Anti-Inflammatory Agents↗

Medpor porous polyethylene implants in orbital blowout fracture repair.

PURPOSE: Various materials are used in orbital blowout fracture repair. We describe a series of patients with orbital blowout fractures that were repaired using porous polyethylene (Medpor) sheets. METHODS: A non-comparative interventional case series is described of 30 blowout fractures of 30 patients aged 7-60 years (median 29 years) who underwent orbital blowout fracture repair with Medpor sheets. The mean follow-up was 19.1 months (minimum 5 months). The indication for surgery in 6 cases was non-resolving diplopia. The remaining 24 cases had surgery for enophthalmos. Ten cases underwent primary or secondary hydroxyapatite orbital implantation at the same time as orbital floor blowout fracture repair. Data were collected on postoperative motility and diplopia, enophthalmos, cosmesis, complications and re-operations. RESULTS: In no case was diplopia worsened by blowout fracture repair. Where surgery was performed for the correction of enophthalmos, late surgery did not compromise the surgical results. There were no intraoperative complications. The one major complication was a case of recurrent implant infections leading to implant removal. There were 3 minor postoperative complications: 2 cases of postoperative infraorbital anaesthesia and one case of a palpable titanium screw. Re-operations were performed for pre-existent diplopia, lid laxity, socket abnormalities and mid-facial deformities. None of these arose from the blowout fracture repair. CONCLUSIONS: The study suggests that in orbital blowout fracture repair Medpor implants are safe and effective with few complications. Late surgery for enophthalmos is technically more difficult but is not associated with poorer functional or cosmetic results.

Adolescent↗

Follow-up study of treatment of orbital floor fractures: relation of clinical data and software-based CT-analysis.

This retrospective study quantifies isolated orbital floor fractures using software-based CT-analysis and compares the clinical outcome across surgical and non-surgical treatment groups. Depending on the surgeon's interpretation of the clinical and radiological appearance, 10 fractures were treated non-surgically and 20 fractures surgically, either with antral balloon catheter alone or in combination with an orbital implant. Ophthalmologic findings were evaluated until 12 weeks after injury. Fracture area, and volume of displaced tissue (VDT) were assessed by software-based CT-analysis. VDT was marginally significantly smaller in non-surgically than in surgically-treated patients (P=0.08). Ophthalmologic findings improved in all groups during follow-up and no statistical difference was found between the groups. Diplopia remained moderate in three patients with balloon catheter alone, and minimal in four patients in both surgical groups. In one patient with non-surgical treatment, diplopia remained minimal after 12 weeks. Although CT-analysis revealed no significant difference between both surgical groups, patients treated with balloon catheter alone presented more diplopia after 12 weeks. Using balloon catheters for fracture repair a combined approach should be performed when large fractures involve the orbital floor to achieve sufficient reduction of orbital content and placement of an orbital implant. Software-based CT-analysis is helpful for objective interpretation in managing of orbital fractures.

Adult↗

2D- and 3D-based measurements of orbital floor fractures from CT scans.

OBJECTIVE: Two methods for area and volume calculation of the orbit were evaluated following blow-out fractures of the orbital floor using computed tomography (CT) scans. MATERIAL AND METHODS: Isolated blow-out fractures of the orbital floor in human cadavers were simulated by fracturing the orbital floor and placing a defined volume of silicone within each defect. The area of fracture and the volume of silicone simulating herniated periorbital tissue were evaluated in 16 orbits by the use of a three-dimensional (3D) CT-based software package (Analyze((R)); Mayo Clinic, Rochester, MN, USA) and software based on two-dimensional (2D) coronal CT scans. Both methods were compared with direct anatomical measurements and evaluated with Lin's concordance coefficient (rho(c)). RESULTS: Between-method concordance of area and volume calculation were rho(c)=0.962, and 0.872 for the 3D-CT-based method, and 0.981 and 0.952 for the 2D-CT method, respectively. The time allocated for measurement was significantly longer for the 3D-CT than for the 2D-CT method (p<0.001). CONCLUSION: Calculations of blow-out fractures of the orbital floor by 3D-CT and 2D-CT method are accurate for assessing the area of fracture and the volume of herniated tissue. Lesser processing time and simple usage favour the 2D-CT-based calculation method.

Cadaver↗

[Orbital, osseous, meningeal and cerebral findings in oculodermal melanocytosis (nevus of Ota). Clinico-histopathologic correlation in 2 patients].

In two patients suffering from congenital oculodermal melanocytosis (nevus of Ota) orbital, osseous, meningeal, and cerebral manifestations are documented. In a 38-year-old European with a nevus of Ota on the right side an ipsilateral orbital tumor was diagnosed and surgically removed. The histological examination revealed a primary spindle shaped malignant melanoma of the orbit. In addition, many benign melanocytes were found in the optic nerve, in the extraocular muscles, in the orbital fat tissue, and in the dura of the sphenoid. In a 17-year-old Ethiopian with a nevus of Ota of the right side an increase in size of the pigmented process was observed during puberty in the orbital fat tissue, in the extraocular muscles, in the optic nerve, in the periorbital bones, in the temporal muscle, and in the cortex of the frontal brain lobe. A deep excision from the lower eye lid revealed a histologically benign oculo-orbito-dermal melanocytosis. An irradiation was performed because of growth again after segmental excision, however without success. In the ipsilateral eye a secondary open-angle glaucoma was diagnosed. In the nevus of Ota an involvement of the orbit and of the meningo-cerebral tissue must be considered.

Adolescent↗

[Differential diagnosis of Graves' disease and orbital pseudotumor: CT findings (author's transl)].

CT scans of 25 clinically typical cases of Graves' disease and 15 cases of orbital pseudotumor (idiopathic orbital inflammation) are analyzed and differential diagnostic criteria evaluated. The CT-findings overlap and are best viewed as a continuous spectrum at both ends of which characteristic patterns can be identified. Massive swelling of ocular muscles, involving of several muscles, usually without density changes of the orbital fat, are diagnostic for Graves' disease. More or less circumscribed masses of soft tissue density surrounding the globe, eye muscles or the optic nerve, severe diffuse increase of density in the retrobulbar space masking orbital structures and calcifications indicate orbital pseudotumor. Localization of the swelling within a muscle, "scleral thickening", enhanced contrast, moderate density changes of the orbital fat, and involvement of the lacrimal gland are of minor differential diagnostic value. CT pictures of clinically typical and histologically proven cases of Graves' disease indicate heterogeneity of this group, probably reflecting different immunologic features. CT shows the extent and degree of the pathologic changes and indicates the optimal approach for tissue biopsy. Evaluation of both diseases by CT requires inclusion of coronal sections.

Adolescent↗

Globe sparing orbital exenteration.

OBJECTIVE: Orbital exenteration has long been the oncologic standard for malignant craniofacial lesions that invade the periorbita/orbit from adjacent locations. Although oncologically sound, this radical surgical procedure is cosmetically disfiguring for all patients. Most of the reconstructive options available are complex, requiring further surgery and/or expense. We herein introduce an alternative surgical technique for radical orbital surgery that spares the globe, thereby allowing an early, aesthetic appearance at a nominal cost. STUDY DESIGN AND SETTING: Twenty consecutive patients requiring an oncologic orbital exenteration and meeting the indications for the new procedure underwent surgery at a tertiary care medical center. Follow-up ranged from 1 to 6 years. RESULTS: Only 1 patient recurred in the orbit due to an error in patient selection. There was no surgical morbidity resulting from the globe-sparing technique itself. CONCLUSION: Globe-sparing orbital exenteration is a one-step, oncologically sound, aesthetically superior, low-cost alternative for those patients requiring radical orbital surgery and reconstruction.

Adult↗

Diagnostic dilemmas in orbital inflammatory disease.

Orbital inflammatory disease (OID) broadly describes a variety of pathologic processes and clinical presentations. OID may be idiopathic or may be secondary to a systemic inflammatory disease, retained foreign body, or infectious disease. OID includes the spectrum of bacterial or fungal infections, diffuse inflammation of multiple tissues (e.g., sclerosing orbititis or diffuse anterior OID), and preferential involvement of specific orbital structures (e.g., orbital myositis or optic perineuritis). Mimics of OID include congenital orbital mass lesions or orbital neoplastic disease such as lymphoma or rhabdomyosarcoma. The ultimate diagnosis and treatment plan relies on a careful history and detailed clinical examination followed by the judicious use of ancillary diagnostic testing and a comprehensive treatment plan. The purpose of this review is to provide an overview of the spectrum of diseases known as OID, with emphasis on specific diagnostic challenges in the evaluation and management of patients with idiopathic OID.

Diagnosis, Differential↗

Use of enophthalmic implants in the repair of orbital floor fractures.

INTRODUCTION: High-density porous polyethylene (Medpor) enophthalmic implants are used in patients with the sunken socket syndrome to augment the orbital volume. We have used them to improve enophthalmos and hypoglobus in the repair of large long-standing orbital floor fractures. This is the first report of enophthalmic wedge implants being used in seeing eyes to improve function and cosmesis. MATERIALS AND METHODS: Four patients had large long-standing orbital floor fractures with subsequent enlargement/expansion of the orbital volume. Indications for surgical intervention were enophthalmos (> 2 mm), hypoglobus (> 2 mm) and diplopia in primary position and downgaze. A transconjunctival approach was used to access the orbital floor and a Medpor enophthalmic wedge implant was placed postero-inferiorly to the globe and periosteum. RESULTS: Patients were followed up for a minimum of 4 to 6 months post-operatively with no complications noted. All patients achieved a good cosmetic and functional result post-operatively with resolution of their enophthalmos and hypoglobus. Diplopia in primary position was corrected with only mild residual diplopia present in extreme up-gaze. CONCLUSION: We have found enophthalmic implants useful in the surgical repair of symptomatic long-standing orbital floor fractures.

Adult↗

Lymphoid infiltrates of the orbit and conjunctiva. A morphologic and immunophenotypic study of 99 cases.

Most of the earlier clinicopathologic studies of orbital and conjunctival lymphoid infiltrates were done before the development of immunohistologic methods. In this study, we have examined 99 lymphoid infiltrates of the orbit and conjunctiva immunohistologically and correlated clinical and histologic features with immunophenotype. Sixty-six infiltrates expressed monotypic immunoglobulin; one case was immunoglobulin-negative, B-lineage; and 32 infiltrates expressed polytypic immunoglobulin. Using histologic criteria, 36 cases were malignant (35 monotypic; one immunoglobulin-negative, B-lineage), 44 were indeterminate (31 monotypic, 13 polytypic), and 19 were benign (all polytypic). Combining histologic and immunophenotypic criteria, 67 cases were diagnosed as malignant lymphoma. Seventy-six percent of the lymphomas were classified as low-grade according to the Working Formulation; small lymphocytic lymphoma (45%) was the most common histologic subtype. Cytologic atypia (p less than .0001) and Dutcher bodies (p less than .05) were found only in lymphomas. Similarly, bilateral involvement (p less than .05) and dissemination at time of presentation (p less than .001) occurred only in lymphomas. No other histologic or clinical features reliably distinguished lymphomas from benign orbital and conjunctival lymphoid infiltrates. Nineteen patients had a prior history of extraorbital malignant lymphoma, and eight patients had a history of pseudolymphoma. Patients with a history of malignant lymphoma more often presented with dissemination (p less than .001) and less often had the small lymphocytic subtype (p = .07) than patients without a history of lymphoma. However, not all patients with a history of extraorbital lymphoma had monotypic orbital and conjunctival infiltrates, and the incidence of monotypic orbital and conjunctival lesions in these patients was similar to that of the patients without a history of lymphoma. Patients with a history of pseudolymphoma always presented with localized disease (p less than .01) and more often had benign lesions (p = .08) as compared with patients without a history of pseudolymphoma. We conclude that the presence of either cytologic atypia or Dutcher bodies is a reliable criterion of malignant lymphoma in the orbit and conjunctiva. However, for one-third of the cases in this study, immunohistologic studies were the only means of distinguishing malignant from benign lymphoid infiltrates.

Conjunctiva↗