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Teleophthalmology: the use of real-time telementoring to remove an orbital tumor.

OBJECTIVE: To determine whether current telemedicine technology can support real-time surgical telementoring to remove an orbital tumor. DESIGN: Interventional case report METHODS: An orbital specialist guided a general ophthalmologist in the removal of a lateral orbital tumor from a site 210 miles away on another island. A 3-mm endoscope connected to a three-chip digital video camera attached to a Concorde 4500 Picture-Tel videoconferencing system was used to transmit images at a rate of 384 kb per second over integrated service digital network lines. MAIN OUTCOME MEASURES: Successful performance and outcome of the telementored procedure comparable to hands-on orbital surgery. RESULTS: The surgical procedure was successfully telementored with uncomplicated removal of the orbital tumor. CONCLUSIONS: Current telemedicine technology can support real-time telementoring to remove an orbital tumor.

Adolescent↗

Graded orbital decompression based on severity of proptosis.

OBJECTIVE: To study the results of orbital decompression based on the severity of preoperative proptosis. DESIGN: A retrospective noncomparative interventional case series. PARTICIPANTS: Thirty-nine orbits in 23 patients with thyroid-related orbitopathy at a university-based referral center. INTERVENTION: Graded orbital decompression was performed in all patients based on the severity of preoperative exophthalmometry. MAIN OUTCOME MEASURES: Exophthalmometry, visual acuity, margin-to-reflex distance, prism cover testing, and intraocular pressure. RESULTS: Mean proptosis reduction in all orbits was 6.4 +/- 2.7 mm (P < 0.01). In group 1 (preoperative exophthalmometry <22 mm), proptosis decreased with a mean of 4.8 +/- 1.3 mm (P < 0.01); mean proptosis reduction was 6.0 +/- 2.3 mm (P < 0.01) and 8.9 +/- 3.4 mm (P < 0.01) in group 2 (exophthalmometry between 22-25 mm) and group 3 (exophthalmometry >25 mm), respectively. In four of five eyes with compressive optic neuropathy there was an improvement of best-corrected visual acuity of 2 lines or more. Margin-to-reflex distance of the upper and lower lids and intraocular pressure were reduced in all groups. New-onset diplopia developed in two patients (8.7%); 13 of 15 patients (86.7%) who had diplopia preoperatively had persistent diplopia postoperatively. Two patients (13.3%) had relief of diplopia postoperatively. CONCLUSIONS: Graded orbital decompression based on the severity of preoperative exophthalmometry is useful to determine the type and amount of orbital surgery to be performed.

Adult↗

The aftermath of orbital radiotherapy for graves' ophthalmopathy.

OBJECTIVE: To determine whether long-term improvement could be observed after orbital radiotherapy for Graves' disease; in addition, to evaluate ancillary treatments needed for those who have received radiotherapy, to search for late-emerging adverse consequences of radiotherapy, and to relate orbital changes to serum levels of thyroid-stimulating immunoglobulin (TSI). DESIGN: Three-year follow-up of noncomparative interventional case series. PARTICIPANTS: Forty-two patients. INTERVENTION: All patients had received orbital radiotherapy within 6 months of study entry. Twelve months after study entry, patients were free to select any additional treatment for their ophthalmopathy. MAIN OUTCOME MEASURES: Need for surgery, steroid therapy, volume of extraocular muscles and fat, proptosis, area of diplopia fields and range of extraocular muscle motion, volume changes after decompression and correlations of eye findings with serum TSI levels, retinal status. RESULTS: Half of the patients elected to have a surgical procedure on their eyes or orbits. Among patients who were not decompressed, we found only slight improvement in some of the main outcome measures. TSI did not positively correlate with baseline status or with any observed change in major outcome measures. After orbital decompression, the volumes of both muscle and fat increase, but bony orbital volume increases more and proptosis diminishes. Retinal microvascular abnormalities consistent with radiation retinopathy developed de novo in five eyes of three patients within 3 years of radiation therapy. CONCLUSIONS: In this 3-year uncontrolled follow-up phase, limited evidence for a clinically significant improvement was observed, which may be the result of treatment or of natural remission. In either case, the changes are of little clinical significance. Because it is neither effective nor innocuous, radiotherapy does not seem to be indicated for treatment of mild to moderate ophthalmopathy.

Adult↗

Giant cell reparative granuloma of the orbit associated with cherubism.

PURPOSE: To report a case of cherubism with extensive, bilateral orbital involvement occurring in a 27-year-old woman who had the diagnosis established at the age of 4 years. DESIGN: Single interventional case report. INTERVENTION: Ophthalmologic examination and computed tomography were performed. The patient underwent multiple surgical excisions using a bicoronal and transorbital approach. The excised orbital tissues were studied histopathologically. RESULTS: Computed tomography showed bilateral inferior lateral masses involving the orbital floors and producing marked superior displacement of the orbital contents. The intrinsic expansile bone lesions involved the inferior and lateral orbital walls with apical compression of the optic nerves. Histopathologic examination of the masses revealed scattered giant cells in a fibroblastic stroma containing small vascular channels. The lesion was interpreted as giant cell reparative granuloma. CONCLUSIONS: Giant cell reparative granuloma is an uncommon bone lesion that might involve the orbit. Cherubism should be included in the differential diagnosis of lesions that show the histopathologic features of giant cell reparative granuloma.

Adult↗

Invasive squamous cell carcinoma arising from asymptomatic choristomatous cysts of the orbit. Two cases and a review of the literature.

BACKGROUND: Epithelial choristomatous cysts are common orbital lesions, the most frequent of which are dermoid or epidermoid tumors. Massive enlargement or extraorbital extension of these benign lesions may occur. Malignant transformation of the epithelial lining of epidermoid cysts is rarely reported. METHODS: Two patients are presented in whom the epithelial lining of a previously asymptomatic choristomatous cyst of the orbit underwent malignant transformation to produce invasive squamous cell carcinoma. The unusual origin and clinical presentation of the lesions caused a delay in the diagnosis and therapy in both patients. Metastatic workup was negative in both patients, who underwent resection of their tumors via orbital exenteration with craniofacial resection. Reported cases of malignant transformation in analogous choristomatous cysts elsewhere in the cranium are reviewed. RESULTS: One patient is alive and well without recurrent disease 40 months postoperatively. The second patient died of a pulmonary embolus 2 months postoperatively. Autopsy showed no residual tumor. Overall, only 3 of 18 reported patients with epidermoid choristoma of the head and orbit with malignant transformation were alive when reported. CONCLUSIONS: Malignant squamous metaplasia is believed to be a rare complication of orbital dermoid or epidermoid cysts, with only two previously reported cases. However, malignant transformation is relatively frequent in analogous epidermoid cysts found elsewhere in the cranial vault, especially after incomplete excision. These reports encourage the complete removal of epithelial choristomas of the orbit.

Aged↗

Melanoma metastatic to the orbit.

Cutaneous malignant melanoma often metastasizes to the lung, bone, liver, subcutaneous tissue, and lymph nodes. Six cases of malignant melanoma metastatic to the orbit are presented, five from cutaneous melanomas and one from a contralateral choroidal melanoma. Melanoma rarely metastasizes to the orbit, but when orbital involvement occurs, widespread metastases are already present. Twenty-two cases were reviewed from the literature; 13 of 14 patients, with sufficient follow-up data, had widespread metastases when they presented with orbital disease. Extraocular muscles appear to be a favored site of metastases. The mean time from orbital presentation to death was 4 months. Orbital metastases from a distant melanoma is, therefore, part of a widespread metastatic process. Treatment should be directed toward palliation.

Aged↗

A two-layer closure of sino-orbital fistula.

A two-layer closure of sino-orbital fistulae using tension-free local flaps is described. For small fistulae, the opening is first closed with a mucosal turnover flap; this is combined on the orbital surface with a local rotational advancement flap of the orbital skin. For larger fistulae where a mucosal turnover flap cannot be fashioned, a two-stage, delayed, composite mucocutaneous rotational flap is used. The advantages of these techniques are: (1) a mucous membrane to provide nasal or sinus lining, insulating the raw ventral surface of the skin flap; (2) each flap has an independent blood supply; (3) local flaps are used, avoiding the use of a pedicle flap from forehead; (4) excellent cosmesis since tissues outside the orbital rim are not disturbed; and (5) does not require refitting of exenteration prosthesis since the integrity of the orbital rim is maintained. The authors have used these techniques in six patients with chronic sino-orbital fistulae of the exenterated socket. In one patient, a wound dehiscence occurred and a small fistula measuring 3 to 4 mm in diameter persisted. The fistulae of the remaining five patients were successfully closed.

Adult↗

Coralline hydroxyapatite spheres as secondary orbital implants in anophthalmos.

BACKGROUND: Secondary reconstruction of the anophthalmic socket is frequently challenging. A number of surgical techniques are described to improve the cosmetic and functional outcome. Recently, the use of hydroxyapatite as a primary orbital implant after enucleation has gained popularity and yielded encouraging surgical results. There are no published reports concerning the use of hydroxyapatite as a secondary orbital implant in anophthalmos. METHODS: The authors reviewed the charts of 17 patients who received a secondary hydroxyapatite orbital implant to rehabilitate the anophthalmic socket. Each patients' preoperative and postoperative photographs, socket appearance, implant and prosthetic motility, and overall impressions of the surgical outcome were analyzed. Complications of surgery and secondary procedures were reviewed. RESULTS: All except one patient attained an improved prosthetic appearance postoperatively and 76% achieved improved implant and prosthetic motility. Four patients undergoing surgery early in the series had worsening of pre-existing ptosis. In two patients, implant exposure developed; a socket infection with subsequent loss of the implant developed in one of these patients. Eight patients underwent additional eyelid and orbital surgery at the time of secondary implant surgery, whereas nine patients required additional staged procedures. CONCLUSION: Secondary orbital implantation with the coralline hydroxyapatite sphere is an encouraging alternative to traditional secondary orbital implant surgery. Surgical outcomes in this study were excellent with few complications and high patient satisfaction.

Adolescent↗

Multidisciplinary management of refractory orbital rhabdomyosarcoma.

PURPOSE: Combined chemotherapy and radiation therapy have improved the survival of children with primary orbital rhabdomyosarcoma, but recurrence or persistence of the local orbital tumor still occurs. There are no established guidelines for dealing with these uncommon patients, and the authors present a review of the combined method treatment and outcome of children with refractory primary orbital rhabdomyosarcoma. METHODS: From clinical databases, 67 children with orbital rhabdomyosarcoma were identified. Seven (10%) of the 67 children had tumors refractory to combined chemotherapy and radiation therapy and underwent exenteration or eye-sparing tumor excision. Their clinical course and outcome were reviewed retrospectively. RESULTS: No patient was lost to follow-up, which ranged from 3.2 to 11 years. Five (71%) of the seven children with refractory tumor are still alive at more than 3 years after surgery (3.2-11 years; mean, 6.9). In one of the two children who died, tumor extended beyond the operative margins at exenteration, and the other child died with regional metastasis within a month of exenteration. CONCLUSIONS: Although more than 90% of children with orbital rhabdomyosarcoma respond to combined therapy by pediatric oncologists and radiotherapists, local orbital (salvage) surgery by ophthalmologists may be of value in the minority of children with refractory tumors. All of the five surviving children appear to be disease free.

Adolescent↗

High resolution magnetic resonance imaging of neurovascular orbital anatomy.

PURPOSE: This study describes the magnetic resonance imaging (MRI) anatomy of the blood vessels and nerves of the orbit to provide a morphological basis for the interpretation of clinical findings. METHODS: Seven volunteers aged 29 to 54 years underwent high-resolution MRI of the orbit on a 1 Tesla unit (Impact, Siemens, Germany). T1-weighted oblique-sagittal, coronal, and axial images were obtained using a surface coil. Anatomic structures on the magnetic resonance images were identified by comparison with corresponding histologic sections of the orbit. RESULTS: The ophthalmic artery and most of its branches (central retinal artery, posterior ciliary arteries, lacrimal artery, anterior and posterior ethmoidal arteries, supratrochlear artery, supraorbital artery, dorsal nasal artery) are visualized. The superior ophthalmic vein, the lacrimal vein, the medial ophthalmic vein, the inferior ophthalmic vein, the medial and lateral collateral veins, and the vorticose veins are also delineated. Furthermore, branches of the oculomotor nerve, the abducens nerve, the frontal nerve, the nasociliary nerve, the lacrimal nerve and the infraorbital nerve are identified in the magnetic resonance images. CONCLUSION: High-resolution MRI is capable of delineating the orbital arteries, veins, and nerves. This is mainly based on two principles. First, blood vessels appear mostly dark on magnetic resonance images because of the signal void of flowing blood. Second, the bright background of the orbital fat on T1-weighted magnetic resonance images accounts for the good soft-tissue contrast in the orbit. With improved MRI technology and reduced imaging time, high-resolution-MRI may be applied routinely for diagnostic purposes.

Abducens Nerve↗

Outcome of orbital myositis. Clinical features associated with recurrence.

PURPOSE: Although orbital myositis usually responds to prompt systemic therapy, recurrent or persistent episodes often require prolonged therapy. This study presents the clinical and radiologic features of 26 patients with orbital myositis and suggests a treatment regimen with special emphasis on recurrent or persistent orbital myositis. METHODS: Retrospective analysis and comparison of clinical and radiologic parameters with clinical outcome in 26 patients with strictly defined orbital myositis was performed. A consultant radiologist reviewed computed tomography studies without knowledge of the clinical data or outcome. The frequency of certain clinical and radiologic parameters among two outcome groups was analyzed using Fisher's exact test. RESULTS: Comparison of clinical and radiologic features of 20 patients with a single acute episode and 6 patients with recurrent episodes disclosed several characteristics associated with recurrence. Male gender, lack of proptosis, eyelid retraction, horizontal extraocular muscle (EOM) involvement, multiple or bilateral EOM involvement (P = 0.05), muscle tendon sparing, and lack of response to systemic corticosteroids (P = 0.01), or nonsteroidal anti-inflammatory agents (P = 0.01) were associated with recurrent orbital myositis. CONCLUSIONS: A treatment algorithm is suggested for patients with orbital myositis. Those patients whose clinical or radiologic features are associated with recurrence may benefit from early systemic steroid therapy.

Acute Disease↗

Preseptal and orbital cellulitis in childhood. A changing microbiologic spectrum.

OBJECTIVE: The authors sought to determine whether the microbiologic spectrum of preseptal and orbital cellulitis had changed over the past decade. DESIGN: A retrospective chart review of all inpatient and outpatient children with an ICD-9 diagnosis of preseptal or orbital cellulitis seen at Vanderbilt University Medical Center since the introduction of the Haemophilus influenzae type-B (HiB) vaccine (1986-1996). MAIN OUTCOME MEASURES: Blood and abscess cultures from children with preseptal and orbital cellulitis were tabulated. RESULTS: During this period, 70 cases of preseptal cellulitis were seen. Blood cultures were obtained in 59 cases; only 6 were positive. Five cultures grew Streptococcus species. The one positive H. influenzae culture occurred in 1987 in a child who did not receive the HiB vaccine. There have been no new patients with preseptal cellulitis and H. influenzae bacteremia at Vanderbilt for 10 years. There were ten cases of orbital cellulitis, of which blood or abscess or both were cultured in eight. Six cases had positive cultures. Four cultures grew Streptococcus species. The other two grew H. influenzae and mixed H. influenzae/gram-positive cocci. CONCLUSION: The incidence of hemophilus-associated bacteremia in patients with preseptal cellulitis has decreased dramatically over the past 10 years. Streptococcus species now are the predominant cause. Orbital cellulitis due to H. influenzae may still occur, but it is much less likely. A more conservative approach to the diagnosis and management of preseptal and orbital cellulitis may be warranted.

Adolescent↗

Management of massive orbital involvement of intraocular retinoblastoma.

OBJECTIVE: This study aimed to evaluate various combinations of therapeutic approaches in an attempt to improve the poor outcome of advanced cases of orbital involvement of intraocular retinoblastoma. DESIGN: The study design was a retrospective, uncontrolled descriptive case series of consecutive patients with massive orbital retinoblastoma treated and observed at a referral center. PARTICIPANTS: Sixteen patients with unilateral orbital involvement of intraocular retinoblastoma were studied. Eight patients had orbital involvement at initial presentation and eight children presented with orbital tumor after enucleation for intraocular retinoblastoma. Patients having microscopic evidence of tumor invasion at surgical section of the optic nerve and subsequently receiving other forms of treatment were not included in the study. INTERVENTION: Six patients had orbital exenteration followed by external beam radiation therapy (4600-6000 cGy in fractionated doses) and chemotherapy. Five patients received chemotherapy only. Three patients were treated by exenteration and chemotherapy, whereas two patients underwent radiation therapy and chemotherapy. MAIN OUTCOME MEASURES: Tumor regression monitored by clinical observations and imaging studies and patient survival was studied. RESULTS: The median follow-up was 22 months (range, 4-168 months). All forms of treatment caused satisfactory local and systemic tumor regression. Two children achieved a survival of more than 10 years. However, two patients in the exenteration followed by chemotherapy and radiation therapy group and one who received chemotherapy only had central nervous system metastases develop and died. Only one patient was lost to follow-up. CONCLUSION: Highly individualized aggressive treatment by combining radical surgery, chemotherapy, and external beam radiation therapy allowed most of the authors' patients to have longer survival and disease-free intervals than expected.

Chemotherapy, Adjuvant↗

Malignant granular cell tumor metastatic to the orbit.

OBJECTIVE: Malignant granular cell tumor is a rare type of soft tissue sarcoma. To our knowledge, ocular (eyelid) involvement has been described in only two cases. Herein, we report the clinicopathologic features of an unusual case of malignant granular cell tumor metastatic to the orbit. DESIGN: Observational case report. METHODS: Retrospective review of the medical record and the histopathologic and electron microscopic findings and review of the literature. RESULTS: A 72-year-old man with biopsy-proven granular cell tumor in the cervical region was initially seen with proptosis and motility disturbance. A magnetic resonance imaging scan showed a large intraconal mass, and biopsy of the orbital mass revealed granular cell tumor. Histopathologic examination of the primary neck tumor and the orbital mass revealed increased nuclear atypia and pleomorphism in the consecutive lesions. The morphologic impression of granular cell tumor was also supported by the immunohistochemical demonstration of S-100 protein expression and ultrastructural findings typical of granular cell tumor. Six months after the orbital involvement, systemic workup revealed multiple apparent bony and lung metastases. CONCLUSIONS: We report the first malignant granular cell tumor metastatic to the orbit and suggest the inclusion of this tumor in the differential diagnosis of metastatic orbital lesions.

Aged↗

Expansion of the human microphthalmic orbit.

OBJECTIVE: To determine the effects of long-term, incremental enlargement of an orbital tissue expander on bone and eyelid growth in microphthalmia. DESIGN: A prospective, noncomparative case series. PARTICIPANTS: Five consecutive patients with microphthalmos treated with orbital expansion were evaluated. INTERVENTION: A tissue expander was placed into the orbits of five children (age, 10 months-6 years) with unilateral microphthalmos and gradually enlarged by saline injections. MAIN OUTCOME MEASURE: The midorbital width of each patient was determined from axial computed tomographic scans before insertion of the device. The length of the normal and abnormal eyelid fissures was measured at surgery. The postexpansion dimensions of both the normal and microphthalmic orbits and the eyelids were remeasured when the expanders were removed. The residual deficits between the normal and the microphthalmic sides were expressed in percentages. RESULTS: Gradual inflation of the expander to a diameter of 22 mm reduced the average preoperative orbital dimension deficit of the group from 14.6% (range, 8%-25%) to 3.8% after surgery (range, 0.5%-6.3%). The average pre-expansion eyelid length deficit for the group was 17.5% (range, 12%-26%) compared to 2.3% (range, 0.0%-5.3%) after expansion. The average expansion period was 56.8 weeks (range, 20-100 weeks). Two outpatient surgical procedures were required in each patient. CONCLUSION: Incremental inflation of a tissue expander placed within the microphthalmic orbit induced sufficient osseous and eyelid growth to ameliorate the major stigmata of this syndrome in all patients treated.

Child↗

Pediatric non-orbital pseudotumor of the head and neck.

OBJECTIVE: To evaluate the incidence, sites of presentation, and treatment outcome of non-orbital pseudotumor of the head and neck in the pediatric population. METHODS: 10-year retrospective case series at a tertiary care center. All patients 18 years of age and younger with non-orbital pseudotumor of the head and neck evaluated and treated at our institution over the study period (1990-2000) were identified through the Systematic Nomenclature of Medicine (SNOMED) database with the keyword 'pseudotumor'. RESULTS: Among 146 patients identified with head and neck pseudotumor at our institution, 15 (10%) were pediatric patients, only three (20%) of whom had pseudotumor in non-orbital sites (the infratemporal and pterygopalatine fossae, submandibular gland, and masseter space). Four (27%) of the 15 had pseudotumor limited to the lacrimal gland, with the remaining eight (53%) being orbital pseudotumor cases. CONCLUSIONS: Similar to cases of orbital pseudotumor, long term systemic steroid therapy proved effective in the management of non-orbital pseudotumor in all three children with eventual disease resolution.

Child↗

[Three-dimensional computer-assisted modeling of the orbital cavity and the eyelids].

PURPOSE: To develop a virtual model of the orbital cavity and the eyelids using a three-dimensional model. METHODS: Amapi Designer 7 and Carrara 3 Studio (Eovia, Mountain View, CA, USA) software were used to obtain 3D models of the orbital cavity and the eyelids. Three-dimensional creation is divided into two main processes. In the modeling process, a 3D model of the orbit, the arterial supply, and the eyelids are created using polygonal meshes and surgical tools with parametric surfaces. In the rendering process, colors are arbitrarily chosen to make elements in the model easier to read. A virtual camera is adjusted and light sources are created to produce virtual simulations of the orbit and the eyelids. RESULTS: Surgical techniques were illustrated with 3D models. Ptosis surgery (resection of the levator muscle and Müller's muscle conjunctival resection) and the transconjunctival approach to lower eyelid blepharoplasty were described step by step. CONCLUSION: Three-dimensional models have applications in many medical fields, providing representation of anatomical structures. They can be used to depict the anatomy of the orbital region in a realistic way. Three-dimensional computer-generated images are an aid in teaching residents in orbital anatomy and surgery.

Blepharoplasty↗

Bilateral simultaneous orbital decompression for Graves' orbitopathy with a combined endoscopic and Caldwell-Luc approach.

Graves' orbitopathy can lead to cosmetic deformity, orbital pain, and visual impairment. Surgical intervention can improve proptosis, cosmetic appearance of the eyelids, vision, and orbital pain with minimal morbidity. Ten patients with dysthyroid orbitopathy underwent concurrent bilateral orbital decompressions. Of these, 9 underwent simultaneous bilateral endoscopic and transantral decompressions, and 1 had only bilateral endoscopic decompressions. Medical management of keratopathy was attempted before surgery. All patients were previously treated with radioiodine and high-dose corticosteroids, and 2 patients had prior low-dose orbital irradiation. Preoperative and postoperative visual acuity, color vision testing, and measurement of proptosis were recorded for all patients. In addition, photographs and CT scans of the orbit and sinuses were done. After surgery, visual acuity improved in 8 patients and remained unchanged in 2 patients. Diplopia in the primary and downward gaze improved in 1 patient and remained unchanged in the 3 others who had it before surgery. Color vision deficits in the blue/yellow range were present in 8 patients before surgery and all reversed within 2 weeks after surgery. Proptosis decreased by an average of 4.83 mm (range 4-7 mm) in patients undergoing the combined decompression and decreased by 4 mm in the 1 patient who had the endoscopic decompression only. Ethmoid sinusitis developed in 1 patient but resolved with oral antibiotics, and another patient had an asymmetric result requiring additional unilateral surgery, which corrected the asymmetry. Overall, this approach avoids external incisions and allows excellent visualization in the regions of the orbital apex and ethmoid roof, facilitating maximal decompression without the increased risk of bleeding or visual disturbances.

Adult↗