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Orbital radiotherapy combined with high dose systemic glucocorticoids for Graves' ophthalmopathy is more effective than radiotherapy alone: results of a prospective randomized study.

We have carried out a prospective study to investigate whether orbital radiotherapy combined with high dose systemic glucocorticoids is more effective than orbital radiotherapy alone for Graves' ophthalmopathy. Thirty consecutive patients with relevant and active Graves' ophthalmopathy were randomly assigned to treatment either with orbital radiotherapy combined with systemic glucocorticoids (Group 1, n = 15) or with orbital radiotherapy alone (Group 2, n = 15). The final evaluation was made 6-9 months after beginning treatment. Two patients in each group were lost to follow-up. Ocular involvement and response to treatment were evaluated by the ophthalmopathy index and by clinical assessment. Mean ophthalmopathy index values were 5.85 in Group 1 and 5.46 in Group 2 (p = NS) before treatment, and 2.46 in Group 1 and 3.61 in Group 2 after treatment (p = 0.0001 and p = 0.003 vs initial value, respectively). The mean ophthalmopathy index decrease in Group 1 (-3.39) was significantly greater (p = 0.043) than that in Group 2 (-1.85). Favorable responses on clinical ground occurred in 9 patients (69%) in Group 1 and in 5 patients (38%) in Group 2. The difference was particularly evident on soft tissue changes and extraocular muscle involvement. Severe eye muscle restriction was substantially unaffected by either treatment. In conclusion, the association of orbital irradiation and high dose systemic glucocorticoids in the treatment of severe Graves' ophthalmopathy provides more favorable responses than orbital radiotherapy alone.

Adult↗

[MRI 3D imaging of the orbits in craniofacial abnormalities and injuries].

Craniofacial malformations and trauma often lead to changes in orbital soft tissues that require operative correction of both hard and soft tissues. CT scan and 3D reconstructions are optimal tools for the evaluation of the bony structures but there is no equivalent for the orbital soft tissues. The aim of this study was to establish a 3D-MRI technique that allows differentiated visualization of the different soft-tissue types of the orbit. A total of eight patients with different pathologic conditions of the orbit were examined. Five of these patients came for secondary correction after trauma and three showed a craniofacial malformation. 3D reconstruction was performed in volume-rendering technique after acquisition of 3 mm axial slices. It was shown that differentiated visualization of the orbital soft tissues is possible. Even though the thin bony structures have a weak signal and therefore the imaging is poor, the globe could be reconstructed reliably by different radiologists because of its circular delimitation from the bone. This technique provides additional support in the planning of orbital operations.

Craniofacial Abnormalities↗

[Primary orbital implant dislocation. A retrospective study].

BACKGROUND: To compensate the orbital volume deficit after enucleation orbital implants are used. METHOD: Between 1986 and 1996, 449 enucleations were performed. One hundred and fifty-five orbital implants were used: 96 hydroxylapatite-ceramic-silicon (HAK-silicon), 29 hydroxylapatite-ceramic (HAK), 26 Kiel bone chips, 3 Walser and 1 Bangerter implant. RESULTS: Ten orbital implants (6.45%) had to be explanted. Seven of the 96 HAK-silicon implants were explanted: four could be changed, two of them with a smaller and two with a same diameter. We observed a complication rate of 7.29% and a rate of total loss of 3.13%. One luxated HAK-implant could be refixated (3.45%), and one implant (3.85%) of the Kiel bone chip implants was explanted. CONCLUSION: Using different types of orbital implants after enucleation, we observed a complication rate of 7%. However, to avoid the post-enucleation-syndrome and to achieve better motility of the prosthesis, orbital implants should be used.

Adolescent↗

Fronto-orbital remodeling without orbito-naso-frontal bandeau.

INTRODUCTION: Fronto-orbital bilateral advance is the procedure of choice for the treatment of craniosynostosis affecting most of the anterior area of the skull and orbitomalar regions. The aim of the technique is to achieve a supra-orbital bilateral bar and a frontal bone. We have introduced a modification in order to simplify the technique. PATIENTS AND METHODS: From November 1998 to January 2002, 18 patients with craniosynostosis have been surgically treated using our technique. The mean age when the treatment was performed was 6.93 months (range 3 to 22 months). Brain computed tomography (CT) scans and three-dimensional (3-D) reconstruction of CT scans were performed before and after treatment. SURGICAL TECHNIQUE: A bifrontal craniotomy was performed taking the osteotomy up to the supraorbital rim. A new frontal bone was obtained from another region of the cranium creating new orbital edges. The osteosynthesis was conducted using absorbable materials. RESULTS: The follow-up of the patients ranged from 3 months to 3 years. All patients were studied using CT scans and 3-D reconstruction of CT after treatment, which demonstrated the persistence of the fronto-orbital advance. No secondary complications related to the new technique were found in any of the patients. CONCLUSIONS: The frontal-orbital advance obtained was stable. The technique was simplified by not creating a supraorbital bar and by reducing the bone fixation points. The manipulation of both frontal lobes and orbital globes was negligible. The aesthetic results were excellent.

Bone Remodeling↗

Catastrophic acute retinal necrosis syndrome associated with diffuse orbital cellulitis: a case report.

BACKGROUND: To report a case of severe acute retinal necrosis syndrome (ARN) associated with initial features of orbital cellulitis. METHODS: A 49-year-old immunocompetent woman presented with unilateral painful orbital inflammation associated with deep visual loss due to ARN. Polymerase chain reaction (PCR) for detection of viral DNA was applied to ocular fluids. Immunohistochemistry for detection of viral antigens and histopathologic studies were performed on orbital biopsy specimens. RESULTS: HSV-2 DNA was detected by PCR in ocular fluids. Orbital biopsy disclosed non-specific inflammation without viral replication. Orbital inflammation resolved rapidly after the initiation of antiviral drugs, whereas intraocular inflammation worsened. Final ophthalmic examination disclosed no light perception due to optic atrophy. CONCLUSION: ARN and cellulitis may be associated. Consequently, ARN should be investigated in all patients with orbital inflammation, in order to initiate immediately an appropriate antiviral strategy.

Antibodies, Viral↗

Orbital presentations of giant cell arteritis.

BACKGROUND: giant cell arteritis (GCA) is a systemic vasculitis that may affect the optic nerve and cause blindness (e.g. ischemic optic neuropathy). Orbital inflammatory disease, however, is an uncommon presentation of GCA. PURPOSE: to alert clinicians to the orbital presentations of GCA. PATIENTS AND METHODS: a retrospective case series from tertiary care academic ophthalmic referral centers of four patients with orbital manifestations of giant cell arteritis. RESULTS: presentation of cases and review of the literature. In three cases, a temporal artery biopsy was diagnostic of GCA, but in one case, an orbital biopsy was needed to confirm the diagnosis. CONCLUSION: GCA can have orbital manifestations and clinicians should be aware of this unusual presentation of GCA in cases of presumed orbital inflammatory pseudotumor in the elderly.

Aged↗

Orbital lymphangioma with positive immunohistochemistry of lymphatic endothelial markers (vascular endothelial growth factor receptor 3 and podoplanin).

BACKGROUND: Existence of true orbital lymphangiomas has been questioned in recent years. Therefore an orbital lymphangioma was analyzed with two new specific markers of lymphatic endothelium. METHODS: Case-report with clinicopathological, immunohistochemical, and ultrastructural findings. A 25-year-old man presented with recurrent lower lid "hematomas" and a pea-sized tumor palpable in the left lower lid. Magnetic resonance imaging showed an inferonasally located orbital tumor which extended to the posterior pole of the eye. The highly vascularized tumor was excised by medial orbitotomy. RESULTS: Histopathologically, the mass consisted of large, erythrocyte-filled cavernous vessels without evidence of smooth muscle cells or pericytes surrounding them. Numerous lymph follicles and small arterioles were scattered between them. Immunohistochemically, endothelial cells lining the lumina of the cavernous vessels were partly positive for podoplanin and vascular endothelial growth factor receptor 3 (flt-4), two markers of lymphatic endothelium. These markers did not react with endothelial cells lining the arterioles. Ultrastructurally, cavernous vessels displayed features characteristic of lymphatic vessels, and the smaller vessels demonstrated signs of arterioles. CONCLUSION: Ultrastructural analysis and immunohistochemistry using two new markers of lymphatic endothelium suggest a lymphatic nature of large vessels in an orbital lymphangioma. A greater series of vascular orbital tumors must be studied with these new lymph endothelial markers to confirm the existence of true orbital lymphangiomas and to analyze different profiles of lymph endothelial marker expression.

Adult↗

Ameloblastoma of the mandible metastasizing to the orbit with malignant transformation. A histopathological and immunohistochemical study.

We report here a case of ameloblastoma of the mandible with multiple local recurrences and metastasis to the orbit. The patient was a 63-year-old Japanese woman with visual disturbance of her right eye. Diagnostic imaging revealed a mass occupying the right orbital apex with partial intracranial involvement. She had been surgically treated for mandibular ameloblastoma 27 years previously, and the tumour had recurred three times in the past 5 years. The orbital tumour and recurrent ameloblastomas were investigated histopathologically and immunohistochemically. The tumour changed in morphology as it recurred, from follicular ameloblastoma without atypia to apparent malignant tumours disclosing undifferentiated or squamoid features. On immunohistochemical analysis, staining for cytokeratin was positive in the squamoid cells but not in the undifferentiated cells. Both histopathologically and immunohistochemically, the orbital tumour was almost identical to the undifferentiated recurrent tumour. The orbital tumour was distinct from the primary site or sites of recurrence of ameloblastoma, and we concluded that the mandibular ameloblastoma underwent malignant transformation with multiple recurrences and finally metastasized to the orbit.

Ameloblastoma↗

Orbital carcinoid tumor metastatic from the colon.

Acute exophthalmos and ophthalmoplegia of the left eye developed in a 58-year-old woman convalescing from a right hemicolectomy for a carcinoid tumor of the colon. Roentgenograms of the orbit and computed tomograms showed a large posterior left orbital mass. Lateral orbitotomy and biopsy confirmed the diagnosis of metastatic carcinoid tumor. Irradiation with radioactive cobalt (60Co) resulted in early relief of the ocular symptoms. To the best of our knowledge, this is the second documented case of orbital metastatic carcinoid tumor and the first patient with orbital involvement from a primary carcinoid tumor of the colon. Orbital irradiation for metastatic orbital carcinoid tumor has not been previously reported and should be used for symptomatic relief, because long-term survival is possible in patients with metastatic carcinoid tumors.

Carcinoid Tumor↗

Localized orbital neurofibromas.

Of nine patients (five men and four women, 25 to 55 years old) with localized orbital neurofibromas, only one had other systemic findings consistent with neurofibromatosis. The neurofibromas originated from sensory nerves of the orbit, producing gradual proptosis with expansion of the orbital walls in most cases. Mild orbital discomfort occurred in some patients. Preoperative and postoperative anesthesia in the distribution of the involved sensory nerves was also encountered. Five patients had multiple tumors within the same orbit. Four patients had tumors or a pedicle of the tumor extending into the superior orbital fissure. Two patients were initially thought to have fibrotic pseudotumors. Surgical excision is the treatment of choice for these tumors.

Adult↗

The role of C.T. scans in managing the orbital complications of ethmoiditis.

Thirty-three cases of ethmoidities with orbital complications were reviewed to determine the accuracy of clinical diagnosis and the benefit of CT scans in planning treatment. Patients were classified according to the Schramm et al. 1982 criteria of orbital involvement with ethmoiditis: periorbital cellulitis with chemosis (PCC)-9, and orbital cellulitis (OC)-11, subperiosteal abscess (SPA)-9, and orbital abscess (OA)-4. These patients received CT scans acutely for diagnostic purposes and demonstrated an 84% accuracy with the final clinical groupings. Of 33 patients, 9 had a shift in clinical classification based on the CT scan result interpreted by the Radiology Department or surgical findings. There were no false positives in the periorbital or orbital cellulitis patients, and no false negatives in the subperiosteal and orbital abscess patients. Thus classification changes caused no change from medical to surgical treatment in any of the cases. The conclusion is that a knowledgeable clinical exam established the correct grouping in 70% of the patients versus 82% with the CT scan. An urgent CT scan is advised for patients in clinical groups SPA, OA, or cavernous sinus thrombosis (CST) to determine the imminent need for surgery, as the cases may be underestimated. Patients with PC or PCC can be managed medically with elective CT scans not routinely indicated.

Abscess↗

Post-traumatic orbit evaluation by three-dimensional surface reconstructions.

Post-traumatic orbital osseous and soft tissue surface evaluation was performed using high resolution computerized tomography and three-dimensional surface reconstructions (3DSR) using specially developed computer software. Four patients with facial fractures involving the orbit are presented as examples of the technique. Orbital volume was measured and surface images prepared using the original CT scans as input to CT computer software developed for this purpose. The results simplify interpretation of the nature and extent of traumatic orbital disruption. The interpretation of serial high resolution CT scans of the orbit (now obtained routinely in fractures involving the orbit) has been augmented by employing three-dimensional surface reconstructions (3DSR).

Adult↗

Orbital cysticercosis-associated scleral indentation presenting with pseudo-retinal detachment.

PURPOSE: To report a clinical picture similar to retinal detachment caused by orbital myocysticercosis-associated scleral indentation. DESIGN: Interventional case series. METHODS: Of the 49 cases of orbital myocysticercosis, four eyes of four patients had pseudoretinal detachment presentations that are detailed. RESULTS: Of the four patients with pseudoretinal detachment, three were found to have orbital myocysticerci in lateral and one in inferior recti muscles, mechanically indenting the eyeball, causing scleral indentation, evidenced by ultrasonography or computed tomography. Associated signs of myocysticercosis were orbital inflammation, restricted ocular motility or proptosis. In 2 to 10 days, the cysts traveled forward and came to lie in a sub-Tenon position. Three cysts were removed surgically and one extruded spontaneously. CONCLUSIONS: Patients presenting with a clinical picture similar to retinal detachment in the presence of symptoms and signs of orbital cysticercosis, with a history of exposure to an endemic area, should be considered to have orbital myocysticerci causing scleral indentation.

Adolescent↗

Porous polyethylene orbital implant in the pediatric population.

PURPOSE: To determine the incidence of complications of primary insertion of porous polyethylene orbital implant in the pediatric population. DESIGN: Interventional case series. METHODS: Prospective nonrandomized case series of 36 eyes of 36 patients under age 15 years who underwent primary placement of an anteriorly wrapped spherical porous polyethylene orbital implant from March 1998 to August 2002, with at least 17 months of follow-up. RESULTS: The mean age at the time of surgery was 4.6 years. The histopathologic diagnoses after enucleation included intraocular tumor in 22 patients, phthisis bulbi in eight patients, microphthalmos in three patients, Coats disease in two patients, and ruptured traumatic globe in one patient. Twelve patients (33%) had prior ocular surgery. At the time of enucleation, all patients underwent primary placement of spherical porous polyethylene orbital implant anteriorly wrapped with homologous sclera in 30 patients (83%) and autologous sclera in six patients (17%). The spherical implant size was 16 mm in one patient (3%), 18 mm in 10 patients ( 28%), and 20 mm in 25 patients (69%). The prosthesis was fitted after a mean interval of 5 weeks. After a mean follow-up of 44 months (range, 17 to 68 months), there was one case of pyogenic granuloma (3%) and one case of implant exposure (3%). There were no cases of implant extrusion, superior sulcus syndrome, orbital cellulitis, or significant inflammatory response. No porous polyethylene orbital implant was drilled for peg placement. CONCLUSIONS: Anteriorly wrapped primary porous polyethylene orbital implant in the pediatric population appears to be well tolerated with few complications.

Adolescent↗

Langerhans cell histiocytosis of the orbit: a need for interdisciplinary dialogue.

PURPOSE: To explore specialty-related perceptions and treatment strategies in Langerhans cell histiocytosis (LCH) of the orbit. DESIGN: A perspective. METHODS: We reviewed the reported ophthalmic experience with unifocal LCH of the orbit, analyzed current oncologic clinical trial protocols, and provided a brief summary of contemporary knowledge and theory of LCH pathogenesis. RESULTS: Ophthalmic literature indicates that unifocal LCH of the orbit is usually responsive to local intervention. Current international oncologic protocols identify orbital LCH as a "central nervous system-risk" lesion (at risk for delayed-onset diabetes insipidus) and mandate a 6-month course of chemotherapy. Analysis suggests that the latter strategy is based on cases of orbital involvement in multifocal and multisystem disease. The pathologic Langerhans cell continues to define and unite the LCH variants, but cytokine activation of that cell may be an earlier pathogenetic determinant. Despite a common cellular mediator, LCH may be a heterogeneous process, with severity related to varied "upstream" trigger events. CONCLUSION: Treatment perspectives in LCH are influenced by dissimilar patient encounters and varied interpretations of the basic disease process. Pending documentation of linkage between unifocal orbital LCH and diabetes insipidus, we recommend local intervention, with systemic treatment reserved for incomplete response or local reactivation or the appearance of lesions elsewhere. LCH underscores the need for close interaction between specialists with intersecting clinical interests.

Histiocytosis, Langerhans-Cell↗

The role of high-resolution computed tomography and magnetic resonance imaging in the evaluation of isolated orbital neurofibromas.

PURPOSE: To analyze the imaging findings in patients diagnosed with isolated orbital neurofibromas. DESIGN: Retrospective observational case series. METHODS: Computed tomographic (CT) and magnetic resonance imaging (MRI) findings were reviewed in five patients with orbital neurofibromas in the absence of systemic neurofibromatosis in this multicenter study. The main outcome measures were bony and soft tissue abnormalities involving the orbit and surrounding anatomic spaces. RESULTS: Patient age ranged from 36 to 43 years (mean, 41 years); three patients were men and two were women. Duration of symptoms ranged from three to 20 years. Radiologic findings included intraconal and extraconal soft tissue masses in all patients, bony orbital abnormalities in three patients, and intracranial extension in two patients. In addition, MRI studies in one patient revealed the previously unreported presence of neurofibromas involving extraocular muscle. CONCLUSIONS: Isolated orbital neurofibromas may present with differing radiologic appearances. The combined use of high-resolution CT and MRI may be of benefit in the preoperative diagnosis of these uncommon orbital tumors.

Adult↗

Post-ablative reconstruction of the medial canthus and medial orbital wall using conchal cartilage graft with three illustrative cases.

When the medial third of the upper or lower eyelid has to be reconstructed after full-thickness tumour excision, we usually use Hübner tarsomarginal grafts, but when medial canthal lesions spread to the medial orbital wall without invading the orbital margin, conchal graft becomes our first surgical option. Previously reported solutions to this difficult problem are few and concern more directly medial orbital wall fractures. We found no article dealing specifically with the use of conchal graft in post-ablative reconstruction of the medial orbital wall. Nevertheless the concha presents great advantages over bone grafting or rib cartilage, because it is more flexible and malleable. And it is less prone to extrusion or infection as may be allografts implants. It is a very effective way to repair medial orbital defects, but graft reorientation must be perfect to match exactly the medial orbital wall concavity.

Aged↗

Orbital optic nerve gliomas in children with neurofibromatosis type 1.

PURPOSE: To describe the clinical course and treatment of symptomatic orbital optic nerve gliomas in children with neurofibromatosis type-1 (NF-1). METHODS: A retrospective review of the records of patients with NF-1 and symptomatic orbital optic nerve gliomas seen in a large multidisciplinary NF-1 clinic of a tertiary care children's hospital. The main outcome measures included presenting symptoms and signs, ophthalmologic examination at diagnosis, the presence of progressive disease following diagnosis, type of therapy, and the reasons therapy was instituted. RESULTS: Twelve patients with symptomatic orbital optic nerve gliomas, all of which led to proptosis (eight girls, four boys), were identified. The mean age of diagnosis of NF-1 was 20 months; the mean age of diagnosis of the orbital optic nerve glioma was 26 months. At the time of diagnosis of the tumor, 10 of 12 patients (83%) had decreased visual acuity in the affected eye. Three patients underwent optic nerve resection; eight received chemotherapy, and one was observed without therapy. Of the eight children who received chemotherapy, progressive disease prior to treatment could be documented in only three; none of these eight children had a reproducible improvement in vision following chemotherapy. There was no demonstrable improvement in vision in any treated patient with NF-1-associated orbital optic nerve gliomas. CONCLUSIONS: Although not definitively proven, our data and previous studies suggest that NF-1-associated orbital optic nerve gliomas should not be treated unless there is clear evidence of either ophthalmologic or radiographic progression. Surgical excision of tumors which have led to proptotic eyes without functional vision should be reserved for cosmetic purposes or to treat complications of exposed globes.

Child, Preschool↗