Michael Kazim

Orbital Fat Removal: Decompression for Graves Orbitopathy

BACKGROUND Orbital decompression has been used to describe surgical procedures that remove some portion of the orbital walls to reduce pressure on the orbital contents. Substantial morbidity associated with these procedures includes infraorbital anesthesia, worsened extraocular motility, globe displacement, and blindness. The authors believe that orbital contents also may be decompressed by removing orbital fat. METHODS Eighty-one patients with nonactive Graves orbitopathy were selected for orbital fat decompression based on the presence of proptosis and associated signs and symptoms to avoid bone removal. Soft-tissue analysis by computed tomography (CT) scan showed distended pockets of fat extending into the intraconal space, which were removed through medial-upper and lateral-lower anterior orbitotomies. Decompression with bone removal was reserved for those few patients with compressive optic neuropathy unresponsive to medical treatment and those patients with residual deforming exophthalmos after fat removal. RESULTS One hundred fifty-eight fat decompressions were performed on 81 patients over 9 years. The authors measured an average reduction in proptosis of 1.8 mm (range, 0-6.0 mm). The greatest average reduction in proptosis (3.3 mm) was produced in patients with preoperative Hertel measurements of greater than 25.0 mm. Morbidity was limited to temporary motility impairment of the inferior oblique in two patients. CONCLUSION The concept of orbital decompression can include removal of orbital fat to reduce proptosis, eliminate symptoms, and improve appearance with far less morbidity than when bone decompression is used as the primary decompressive procedure.

Reversal of dysthyroid optic neuropathy following orbital fat decompression

AIMS To document the successful treatment of five patients with dysthyroid optic neuropathy by orbital fat decompression instead of orbital bone decompression after failed medical therapy. METHODS Eight orbits of five patients with dysthyroid optic neuropathy were selected for orbital fat decompression as an alternative to bone removal decompression. Treatment with systemic corticosteroids and/or orbital radiotherapy was either unsuccessful or contraindicated in each case. All patients satisfied clinical indications for orbital bone decompression to reverse the optic neuropathy. High resolution computerised tomographic (CT) scans were performed in all cases and in each case showed signs of enlargement of the orbital fat compartment. As an alternative to bone decompression, orbital fat decompression was performed on all eight orbits. RESULTS Orbital fat decompression was performed on five patients (eight orbits) with optic neuropathy. Optic neuropathy was reversed in all cases. There were no cases of postoperative diplopia, enophthalmos, globe ptosis, or anaesthesia. All patients were followed for a minimum of 1 year. CONCLUSIONS In a subset of patients with an enlarged orbital fat compartment and in whom extraocular muscle enlargement is not the solitary cause of optic neuropathy, fat decompression is a surgical alternative to bony decompression.

Treatment of acute Graves orbitopathy

The therapeutic options for the treatment of acute Graves orbitopathy include high-dose oral corticosteroids, surgical decompression, and radiotherapy. The former two treatments are associated with significant morbidity. Although there have been a number of reports of the efficacy of radiotherapy, its role in the management of this disease is still questioned. The authors reviewed 84 cases of acute Graves orbitopathy treated with either high-dose systemic corticosteroids or radiotherapy (2000 rad to each involved orbit). Radiotherapy resulted in significant improvement in subjective and objective signs of orbital congestion and was more effective than high-dose corticosteroids in relieving compressive optic neuropathy. Of the patients with compressive optic neuropathy, only 1 of 29 treated with radiotherapy required surgical decompression, whereas, 6 of 16 treated with corticosteroids required surgery. This study suggests that radiotherapy has greater efficacy and fewer complications than high-dose systemic corticosteroids in the treatment of acute Graves orbitopathy.

Management of Conjunctival Cicatrizing Diseases and Severe Ocular Surface Dysfunction

Physical or chemical injuries, infections, immunologic oculocutaneous disorders, drugs, and various systemic disorders may cause scarring of the conjunctiva and disturbances of the ocular surface. Trichiasis, lid margin malposition, and dry eye may result in persistent ocular irritation. The cornea may be primarily or secondarily involved. If severe, disturbances of the ocular surface may lead to significant visual impairment. Thorough evaluation of patients and of the underlying disease process is required for optimal management. Treatment may be challenging and should be comprehensive, combining medical measures and surgical correction of structural changes. Suppression of exogenous irritants, treatment of dry eye, antiinflammatory therapy, and immunosuppressants are paramount to control the underlying disease and allow optimal surgical results. Surgical correction of trichiasis and lid margin malposition, conjunctival grafting, mucous membrane transplantation, limbal stem cell transplantation, amniotic membrane transplantation, and penetrating keratoplasty help reestablish a physiologic ocular surface. Severe cases may require keratoprosthetics for visual rehabilitation. Corneal ulceration or perforation requires prompt attention to maintain ocular integrity. Special measures should be considered for patients who require cataract or glaucoma surgery.

Endoscopic approach to orbital apex lesions.

Purpose: To examine the role of transnasal and transantral endoscopic surgical approaches in the management of apical orbital lesions. Methods: Retrospective case series. Results: Three patients underwent combined orbital and endoscopic surgery for treatment of apical orbital lesions. In two of these patients, the orbital apex was decompressed, which resulted in visual recovery. Using endoscopic approaches to the posterior orbit and orbital apex allowed better visualization, with minimal manipulation of tissues in an area where surgical access is limited. Conclusions: Apical orbital lesion management benefits from a multidisciplinary and individualized plan. Endoscopic approaches can be used to improve visualization and access to a difficult surgical area. They also allow extra instrumentation to be used through the relatively capacious paranasal sinuses. These advantages may obviate lateral orbitotomy in some situations. Although endoscopic techniques are useful for biopsy, debulking, and removal of orbital apex lesions, they may be more difficult to apply to an intraconal lesion at the orbital apex.

Streptococcal Gangrene of the Eyelids and Orbit

PURPOSE Streptococcal gangrene, also termed streptococcal necrotizing fasciitis, is resurgent but remains exceedingly rare. Ophthalmologists and dermatologists must be aware of streptococcal gangrene, as eyelids are the most commonly affected area of the head and neck. METHODS We studied two cases of streptococcal gangrene of the orbit with clinical manifestations indistinguishable from common nonnecrotizing orbital cellulitis. RESULTS Infection progressed with dramatic rapidity to produce eyelid necrosis, respiratory failure, sepsis, and severe permanent visual loss caused by ophthalmic artery occlusions. Histopathologic analysis disclosed vascular thrombosis, necrosis, acute inflammation, and the presence of gram-positive cocci. Cultures grew heavy group A beta hemolytic Streptococcus. The first patient was infected with M type 1 carrying exotoxins A and B. The second patient was also infected with Streptococcus carrying exotoxin A. CONCLUSION Early diagnosis of this life-threatening infection is of paramount importance because survival may depend on early surgical debridement.

CT scan evidence of dysthyroid optic neuropathy.

Purpose To determine the utility of CT imaging in identifying patients with dysthyroid optic neuropathy. Methods Orbital CT scans of 12 randomly selected patients with dysthyroid optic neuropathy and 15 control subjects with Graves orbitopathy were retrospectively reviewed by two investigators blinded to the clinical data. The clinical diagnosis of optic neuropathy was made by one author before obtaining CT images and was based on clinical features. Results Optic nerve crowding (P <0.001) and intracranial fat prolapse (P <0.05) were the imaging features independently related to optic neuropathy. A muscle index greater than 50% had excellent sensitivity (100%) but did not have high specificity (47%) for dysthyroid optic neuropathy. Superior ophthalmic vein dilation and proptosis did not show significant relations with optic neuropathy. Conclusions This study suggests that patients with Graves orbitopathy who have severe optic nerve crowding, intracranial fat prolapse, and/or muscle index greater than 50% present on orbital CT scans are more likely to have coexisting optic neuropathy.

Efficacy of corticosteroids and external beam radiation in the management of moderate to severe thyroid eye disease.

Thyroid Eye Disease (TED, Graves ophthalmopathy, thyroid ophthalmopathy) is the most common cause of orbital inflammation and proptosis in adults. There is no agreement on its management although corticosteroids and external beam orbital radiation (XRT) have traditionally been believed to provide benefit in active inflammation. Our review of the published literature in English disclosed an overall corticosteroid-mediated treatment response of 66.9% in a total of 834 treated patients who had moderate or severe TED. Intravenous corticosteroids used in repeated weekly pulses were more effective (overall favorable response = 74.6%, n = 177) and had fewer side effects than daily oral corticosteroids (overall favorable response = 55.5%, n = 265). A combination of corticosteroid and radiation therapy seemed to be more effective than corticosteroids alone. Our conclusions are tempered by a notable lack of standardization within and between study designs, treatment protocols, and outcome measures. Accordingly, the North American Neuro-Ophthalmology Society (NANOS), American Society of Ophthalmic Plastic and Reconstructive Surgery (ASOPRS) and the Orbital Society, in conjunction with Neuro-Ophthalmology Research and Development Consortium (NORDIC), will investigate the design and funding of a multi-center controlled trial.

Orbital lymphangioma : correlation of magnetic resonance images and intraoperative findings

BACKGROUND Orbital lymphangiomas generally are invasive, slow-growing lesions that can produce proptosis, motility impairment, and compressive optic neuropathy. Successful surgical management requires detailed preoperative radiologic imaging with the capacity to determine the location and size of the tumors, the presence of cystic or solid components, and extent of infiltration of the tumor into normal orbital structures. METHODS The records of 12 patients with orbital lymphangioma were reviewed with attention to the clinical, radiologic, operative, and histologic findings. RESULTS Preoperative magnetic resonance imaging (MRI) produced highly detailed renderings of the orbital mass that were both diagnostic and predictive of the intraoperative findings. Magnetic resonance imaging was particularly sensitive to the presence of cysts within the tumor and was able to predict the contents of the cysts. CONCLUSION Using preoperative MRI, a more detailed and accurate surgical plan can be formulated than with any other noninvasive technique.

IgG4 Immunostaining and Its Implications in Orbital Inflammatory Disease

Objective IgG4-related disease is an emerging clinical entity which frequently involves tissue within the orbit. In order to appreciate the implications of IgG4 immunostaining, we analyzed gene expression and the prevalence of IgG4- immunostaining among subjects with orbital inflammatory diseases. Methods We organized an international consortium to collect orbital biopsies from 108 subjects including 22 with no known orbital disease, 42 with nonspecific orbital inflammatory disease (NSOI), 26 with thyroid eye disease (TED), 12 with sarcoidosis, and 6 with granulomatosis with polyangiitis (GPA). Lacrimal gland and orbital adipose tissue biopsies were immunostained for IgG4 or IgG secreting plasma cells. RNA transcripts were quantified by Affymetrix arrays. Results None of the healthy controls or subjects with TED had substantial IgG4 staining. Among the 63 others, the prevalence of significant IgG4-immunostaining ranged from 11 to 39% depending on the definition for significant. IgG4 staining was detectable in the majority of tissues from subjects with GPA and less commonly in tissue from subjects with sarcoidosis or NSOI. The detection of IgG4+ cells correlated with inflammation in the lacrimal gland based on histology. IgG4 staining tissue expressed an increase in transcripts associated with inflammation, especially B cell-related genes. Functional annotation analysis confirmed this. Conclusion IgG4+ plasma cells are common in orbital tissue from patients with sarcoidosis, GPA, or NSOI. Even using the low threshold of 10 IgG4+ cells/high powered field, IgG4 staining correlates with increased inflammation in the lacrimal gland based on histology and gene expression.