Jack Rootman

Tumors metastatic to the orbit: A changing picture

Clinical characteristics of tumors metastatic to the orbit are related to primary tumor biology, and vary substantially among the various primary types. Common known primary sites include breast, lung, prostate, and melanoma. Tumor presentations can be classified into four generalized syndromes of mass, infiltrative, inflammatory, and functional effects. We found the infiltrative syndrome of presentation to be more common than for other types of orbital neoplasm. Accurate diagnosis often depends on recognition of the types of clinical syndromes and on the use of diagnostic modalities such as computed tomography, magnetic resonance imaging, fine needle aspiration biopsy, and open biopsy. Special histologic techniques are often useful in determining the origin of these often poorly differentiated tumors, and can provide a basis for specific hormonal therapy. Ophthalmologists play a vital role in the diagnosis of metastatic cancer; the orbital tumor was the presenting sign of systemic cancer in 42% of the cases reviewed. Although the overall prognosis for patients with metastatic cancer is quite poor, specific therapy is available for a growing number of cancers. Timely intervention based on accurate diagnosis can dramatically improve the duration and quality of life with selected tumors.

Optic nerve sheath meningiomas.

PURPOSE To study the natural history and growth of optic nerve sheath meningiomas and evaluate their management outcome. DESIGN Clinicopathologic retrospective noncomparative case series. METHODS A retrospective study of 88 patients who were treated between 1976 and 1999 at the University of British Columbia and the University of Amsterdam. Clinical reports, imaging studies, and histopathologic findings were reviewed. RESULTS The mean age at onset of symptoms was 40.3 years, and most were seen in middle-aged females. Patients typically presented with visual loss, frequently associated with optic atrophy or papilledema and occasionally optociliary shunt vessels. On imaging, the optic nerve demonstrated segmental or diffuse thickening of the sheath or globular growth. Calcification was seen in 31% of cases and was associated with slower tumor growth. Tumors with posterior components in the orbit had more frequent intracranial involvement. Intracranial extension was more frequent and had a greater growth rate in younger patients. Irregular margins in the orbit implied local invasion. A presenting visual acuity better than 20/50 correlated with longer preservation of vision. Patients who underwent radiotherapy showed improvement in their visual acuity, and tumor growth was halted. Optic sheath decompression did not preserve vision. En bloc tumor excision was associated with no detectable recurrence in contrast to debulked tumors that recurred. CONCLUSIONS Meningiomas show characteristic indolent growth. Management therefore should be conservative in most cases. Radiotherapy is indicated in patients with progressive visual deterioration. Surgery, when indicated, should be an en bloc excision.

Idiopathic Sclerosing Inflammation of the Orbit: A Distinct Clinicopathologic Entity

BACKGROUND Idiopathic sclerosing inflammation of the orbit is a poorly delineated, fibrosing, immune-mediated entity resulting in significant ocular disability. To characterize this process and propose more specific and effective therapy, clinical and pathologic findings in 16 cases are reviewed. METHODS The clinical records of 16 patients with biopsy-proven disease were retrospectively reviewed to determine demographic and clinical features, radiologic features, course, management, and outcome. These findings were correlated with pathologic features to describe this unique entity. Immunohistologic characteristics were compared with those of a clinically and histopathologically similar process, retroperitoneal fibrosis. RESULTS The study included 11 male and 5 female patients, ranging in age from 8 to 81 years. Disease onset was usually unilateral (14/16) and chronic (11/15), with two distinct anatomic presentations, lacrimal (11/16) and apical (3/16), characterized by infiltration (15/16), mass effect (12/16), and visual loss (3/16). The most common signs and symptoms were dull pain (13/16), proptosis (11/16), mild inflammation (11/16), restricted motility (9/16), swelling (9/16), and diplopia (8/16). Two features, a sparse, chronic inflammatory infiltrate, the immunopathologic characteristics of which suggested a cell-mediated process, and a desmoplastic stroma of early onset, dominated the pathologic picture. Treatment with corticosteroids (11/16), radiotherapy for steroid failures (8/11), and observation alone (3/16) was inadequate, resulting in blindness in 3/16 cases, restricted movement in 10/16, and complete resolution in only 2/16 patients. CONCLUSION Idiopathic sclerosing inflammation of the orbit is a unique clinicopathologic entity, similar to retroperitoneal fibrosis, that is characterized by primary, chronic, and immunologically mediated fibrosis, poor response to corticosteroid treatment or radiotherapy, and frequent visual disability. Early and aggressive immunosuppressive therapy is recommended.

The Classification and Management of Acute Orbital Pseudotumors

The clinical presentation, investigative findings, classification, and management of 17 acute pseudotumors based on patterns of orbital involvement is presented. Acute pseudotumors developed over days to weeks and were dominated by pain, neuropraxia, and inflammatory clinical features. Five patterns of acute inflammatory pseudotumor were seen. Anterior and diffuse acute pseudotumors were characterized by manifestations of inflammation of the globe and orbit including pain, lid swelling, ptosis, diplopia, uveitis, papillitis, optic neuropathy, and exudative retinal detachment. Anterior or diffuse orbital infiltration was noted on computerized tomography (CT) and ultrasound. Lacrimal involvement was characterized by local pain, tenderness, lid swelling and inflammation, with CT and ultrasound confirming an anterior inflammatory mass. Posterior or apical involvement led to an early optic neuropathy, and myositic lesions were characterized by features of muscle infiltration. Management with steroids was effective and could be followed by serial CT studies.

Orbital Decompression for Disfiguring Exophthalmos in Thyroid Orbitopathy

Background: Progressive exophthalmos from dysthyroid orbitopathy may result in marked disfigurement. Commonly affecting middle-aged women, it can be a significant social and psychologic handicap. Cosmesis is increasingly recognized as an indication for orbital decompression. Many argue, however, that decompression carries risks of operative complications which are only warranted where vision is threatened. Methods: Between 1984 and 1990, the authors performed orbital decompression for cosmetic indications on 34 patients (65 orbits) with thyroid orbitopathy. The charts of these patients were reviewed to assess the results of surgery and the nature and incidence of complications. Results: The mean retroplacement achieved was 4 mm (range, -1.0 to 10 mm). In 29 (85%) of the 34 patients, there was a difference of 1 mm or less in the proptosis of the two eyes postoperatively. Diplopia arose de novo in five (18%) previously asymptomatic patients. Postdecompression strabismus was managed successfully with adjustable surgery (mean, 1.3 operations per patient). With the exception of transient infraorbital nerve hypoesthesia, there were no surgical complications. Conclusion: Decompression surgery is effective in reducing exophthalmos in dysthyroid orbitopathy. In this series of operations, complications were rare and treatable. Decompression is often the first of a series of operations that may be necessary to correct the cosmetic sequelae of this condition. It should be considered by experienced surgeons in carefully selected and counseled patients who have disfiguring thyroid orbitopathy.

Combined Frontotemporal-Orbitozygomatic Approach for Tumors of the Sphenoid Wing and Orbit

An extension of a combined frontotemporal and orbitozygomatic exposure was developed to remove 8 hyperostosing invasive sphenoid wing meningiomas (Group 1) and 11 complicated intraorbital tumors with and without intracranial extension (Group 2). Two separate bone flaps were created: a free frontotemporal-sphenoidal (pterional) bone flap and en bloc removal of the superior and lateral orbital margins with attached zygomatic arch. Cranio-orbital reconstruction was performed using the inner table of the pterional bone flap. Complete tumor removal was achieved in 14 patients and near total removal in 5. There was no mortality and in those patients who did not require orbital exenteration excellent to good cosmetic results were achieved in all but one case. This approach affords a wide exposure of the orbit and anterior and middle skull base, so that large tumors of the orbit and tumors involving the orbital apex, sphenoid wing, and infratemporal and pterygopalatine fossae can be removed.

Clinical Characteristics of Metastatic Orbital Tumors

The authors reviewed the clinical and histopathologic records of 38 patients with metastatic orbital tumors. Diplopia, ocular motility limitation, and mass effect with displacement, proptosis, or palpable mass were common signs and symptoms. Enophthalmos occurred in 25% of cases. The authors found that the clinical presentations could be broken down into four generalized syndromes of presentation: infiltrative (20 cases, 53%); mass (14 cases, 37%); inflammatory (2 cases, 5%); and functional (1 case, 3%). An infiltrative pattern of presentation may be a clue to the metastatic nature of the orbital tumor. There may be no history of systemic cancer; in 25% of the patients in this series, the orbital tumor was the initial manifestation of systemic disease. Although the prognosis is poor for patients with metastatic cancer (average survival in this series, 10.2 months), modern treatment methods continue to improve and long-term palliation is often possible. The ophthalmologist plays a vital role in the diagnosis, histologic evaluation, and referral of these patients.

The clinical and pathologic constellation of Wegener granulomatosis of the orbit.

PURPOSE Wegener granulomatosis (WG) may present as an orbital mass without obvious upper respiratory or systemic features. The authors examined the clinical and pathologic features of a series of cases of orbital WG to define the features of presentation and progression of this disorder. METHODS Thirteen subjects with orbital presentations of WG were identified from the University of British Columbia Orbit Clinic index of diseases. Clinical features were correlated with the results of computed tomography in 12 cases and orbital biopsy in 11 cases. Antineutrophil cytoplasmic antibody (c-ANCA) testing was performed in five cases. RESULTS The main ocular symptoms were decreased vision, redness, and ocular and facial pain, whereas the main signs were proptosis, scleritis, and lid inflammation. Progression was marked by an increased incidence of bilaterality and systemic features. Ear, nose, and throat features were discovered at presentation in 11 cases and became universal during the follow-up period. Initial antineutrophil cytoplasmic antibody test results were negative in five patients but became positive later in three patients. Orbital biopsy specimens typically had features of mixed inflammation, fat disruption, and small areas of necrosis. The combination of cyclophosphamide and oral steroids was highly effective in terminating disease episodes. CONCLUSIONS Orbital WG can be recognized by a constellation of clinical and radiologic findings with evidence of an often erosive, infiltrating, and restrictive fibrotic, inflammatory mass. Concurrent ear, nose, and throat or specific ocular findings such as scleritis with typical limbal infiltrate can occur. Biopsy results show mixed inflammation with evidence of necrosis that must not be regarded as a nonspecific finding.

Localised invasive sino-orbital aspergillosis: characteristic features

Background/aim: To describe the characteristic constellation of historical, clinical, radiographic, and histopathological findings of localised invasive sino-orbital aspergillosis based on the authors’ recent experience of four consecutive cases presenting over a 6 month period. Treatment and outcome are reviewed. Methods: A case series of four patients with review of the English language literature. Results: There have been 17 reported cases of invasive sino-orbital aspergillosis in healthy individuals over the past 33 years. The authors report four patients who presented during a 6 month period with persistent and significant pain followed by progressive ophthalmic signs—clinical histories reflecting the literature. Similar imaging findings were also noted: focal hypodense areas within apical infiltrates on contrasted computed tomography correspond to abscesses seen at surgery, and sinus obliteration or involvement of the adjacent sinus lining was noted on magnetic resonance imaging. Bone erosion (often focal) was also seen. There is frequently a delay in making the correct diagnosis, and often disease progression occurs despite treatment. Conclusions: The authors encountered four cases of invasive sino-orbital aspergillosis, three of which occurred in otherwise healthy individuals. The clinician must be aware of the characteristic presentation so that earlier diagnosis, management, and improved outcomes can be achieved.

Orbital-Adnexal Lymphangiomas: A Spectrum of Hernodynarnically Isolated Vascular Harnartornas

A retrospective review of 13 cases of lymphangioma has led to a classification of these lesions into categories of superficial, deep, and combined types. The clinical manifestations, prognosis, and management directly correlate with the pathophysiology and the location of the lesions. Superficial lesions consist of isolated multicystic vascular abnormalities of cosmetic significance only. Deep orbital lymphangiomas present as spontaneous, acute proptosis due to retrobulbar hemorrhage. Combined lesions demonstrate both superficial and deep components and are also characterized by spontaneous hemorrhages. Histopathologically, lymphangiomas represent a spectrum of vascular hamartomas with a constellation of features that parallels the clinical progression and histology of similar lesions elsewhere in the head and neck. This may include the presence of diaphanous serous-filled vascular channels, a connective tissue stroma with lymphorrhages, features of old hemorrhage, dysplastic vessels, and random smooth muscle bundles. Both direct and indirect evidence suggests that these lesions are characterized by relative hemodynamic isolation.