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Biomedical subjects

Jack Rootman

Publications and source records attributed to Jack Rootman.

At least 19 recordsLinked to original sources
PubMed2006

Periocular and orbital amyloidosis: clinical characteristics, management, and outcome.

OBJECTIVE: To present the clinical features and management outcome in a large series of patients with periocular and orbital amyloidosis. DESIGN: Retrospective, noncomparative, interventional case series. PATIENTS: All patients diagnosed with periocular and orbital amyloidosis in 6 oculoplastic and orbital units. METHODS: Clinical records of all patients were reviewed. MAIN OUTCOME MEASURES: Clinical presentation, radiological and histological findings, treatment modalities, and outcome. RESULTS: The study included 24 patients (15 female, 9 male) with a mean age of 57+/-17 years. Nineteen cases were unilateral, and 5 were bilateral. Clinical signs and symptoms included a visible or palpable periocular mass or tissue infiltration (95.8%), ptosis (54.2%), periocular discomfort or pain (25%), proptosis or globe displacement (21%), limitations in ocular motility (16.7%), recurrent periocular subcutaneous hemorrhages (12.5%), and diplopia (8.3%). Seven cases had orbital involvement, and 17 were periocular. Immunohistochemistry in 7 patients showed B cells or plasma cells producing monoclonal immunoglobulin chains that were deposited as amyloid light chains. Only 1 patient was diagnosed with systemic amyloid light chain amyloidosis. Treatment modalities were mainly observation and surgical debulking. During a mean follow-up period of 39 months, 21% showed significant progression after treatment, whereas 79% were stable or showed no recurrence after treatment. CONCLUSION: Periocular and orbital amyloidosis may present with a wide spectrum of clinical findings and result in significant ocular morbidity. Complete surgical excision is not feasible in many cases, and the goal of treatment is to preserve function and to prevent sight-threatening complications.

Adult↗
PubMed2006

Septic thrombosis of the cavernous sinus: two different mechanisms.

PURPOSE: Septic thrombosis of the cavernous sinus is a rare condition that may lead to significant morbidity and mortality if not diagnosed and treated urgently. We present two recent cases of bilateral septic cavernous sinus thrombosis with differing etiologies, the first of dental origin and the second a complication of sphenoid sinusitis. METHODS: Case report. RESULTS: A diagnosis of cavernous sinus thrombosis was made based on clinical findings and confirmed by imaging in both patients. The etiology in the first patient was related to poor dental hygiene, with resultant spread of infection to the cavernous sinuses. The second had bilateral sphenoid sinusitis, with intravascular gas noted in both orbits and cavernous sinuses. This is the first reported occurrence of such an event associated with septic cavernous sinus thrombosis. Bilateral sphenoidotomies with evacuation of the sinuses were performed, and Haemophilus influenzae was cultured from the sphenoidotomy fluid and blood. Broad spectrum intravenous antibiotics were given in both patients, and complete recovery achieved within weeks. CONCLUSION: In septic thrombosis of the cavernous sinus, early recognition and prompt management with broad spectrum intravenous antibiotics is crucial for full recovery. The primary source of sepsis may be a distant focus with septicemia preceding thrombosis of the cavernous sinus. Alternatively, infection may spread from facial regions via the facial venous plexus or from the sphenoid sinus directly to the adjacent cavernous sinus.

Adult↗
PubMed2006

Imaging of the natural history of an orbital capillary hemangioma.

PURPOSE: To describe the natural history of capillary hemangioma. METHODS: Case report. RESULTS AND CONCLUSION: We describe the imaging features of a capillary hemangioma of the orbit at presentation (computed tomographic scan done at 27 weeks) and after regression (magnetic resonance scan done at 9 years). The primary scans demonstrate the typical contrast enhancing lesion of the orbit, whereas the MR scan of the regressed lesion demonstrates a fibro-fatty lesion with some flow voids.

Child↗
PubMed2005

Lymphocytic infiltration and enlargement of the lacrimal glands: a new subtype of primary Sjögren's syndrome?

PURPOSE: To review the clinical, radiologic, serologic, histopathologic, immunohistochemical, and molecular genetic features of patients having Sjögren's syndrome (SS) with lacrimal gland enlargement. DESIGN: Retrospective case series review. PARTICIPANTS: Fourteen patients histopathologically diagnosed with SS with lacrimal enlargement. Twenty-three age- and gender-matched controls were used for comparison on radiologic analysis. METHODS: Clinical and serologic data were determined directly or by chart review. Computed tomography images from patients were compared with those from the control group. Histopathologic sections were reviewed and graded using the Chisholm-Mason scale, and quantitative immunohistochemical analysis was applied. MAIN OUTCOME MEASURES: Clinically, patients were assessed for age, gender, onset, symptoms and signs, systemic features, treatment, and outcome. Existing histologic specimens were reviewed according to the Chisholm-Mason scale, and then the percentages of plasma cells containing immunoglobulin (Ig) A, IgG, and IgM were determined. Imaging was assessed for lacrimal gland thickness, prolapse, density, and margin contour; extraocular muscle size; orbital tissue displacement; and proptosis. RESULTS: Clinical and histopathological data fulfilled the revised American-European criteria for primary SS in 79% of patients. Compared with other large series of primary SS patients, similarities were found with age, xerophthalmia, parotidomegaly, and articular involvement. Differences included a lower incidence of autoantibodies, xerostomia, and extraglandular features and a higher male-to-female ratio. In almost all patients (93%), the percentage of plasma cells positive for IgA was less than 70%, consistent with SS. Compared with controls, the lacrimal glands were enlarged significantly (P<0.0001) and prolapsed (P<0.001). Involved glands had blurred margins (P<0.007), caused displacement of adjacent tissues (P = 0.03), and were associated with hyperdense fat (P = 0.007). Lymphocytic infiltration of orbital fat was present in all patients for whom fat biopsy results were available. Three patients had monoclonal infiltrates, and 1 patient experienced subsequent extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type, 4 years after presentation. CONCLUSIONS: The criteria used to diagnose primary SS are controversial, but both diagnostic and quantitative immunohistochemical criteria suggest that these patients, with lacrimal gland enlargement resulting from lymphocytic infiltration, represent a new subtype of primary SS. This is clinically important in view of the increased risk of lymphoma associated with SS, compared with idiopathic nonspecific lacrimal inflammation.

Adult↗
PubMed2005

Mantle cell lymphoma in the ocular adnexal region.

PURPOSE: To study the clinicopathologic features of mantle cell lymphoma (MCL) in the ocular adnexal region. DESIGN: Retrospective review. METHODS: The slides of 23 suspect patients were reevaluated with a panel of monoclonal antibodies, including anti-CD20, cyclin-D1, CD5, CD3, and p53 immunostains. Patients confirmed to have MCL were examined retrospectively on the basis of chart review. RESULTS: Ten patients with periocular MCL were included in the study on the basis of characteristic histopathologic features and coexpression of nuclear cyclin-D1. This included 1 female and 9 male patients, with an age range of 32 to 84 years (median, 73.5 years). Median follow-up was 20 months (range, 5-172 months). Six of the 10 patients died, all of lymphoma. The orbit (90%) was most commonly involved followed by the lacrimal gland (50%) and lid (50%), with 90% of cases having lymphoma present at 2 or more periocular sites. Most had a primary periocular presentation (80%) that was associated with stage III/IV disease (80%), including atypical cells in the peripheral blood smear (60%) and bone marrow involvement (70%) at presentation. Three cases were CD5-negative, and 2 other cases showed composite histologic findings (MCL and follicular lymphoma and MCL and a plasma cell neoplasm). Fluorescent in situ hybridization performed in these 2 cases demonstrated t(11;14) in the MCL component. Actuarial survivals were median progression-free (PFS) survival, 12 months; median overall survival (OS), 57 months; 5-year PFS, 0; 5-year OS, 39%. CONCLUSIONS: Mantle cell lymphoma presenting in the ocular adnexal region has a male predominance and tends to affect an elderly age group, as is typical of MCL involving nodal sites. A higher frequency of these tumors fail to co-express CD5, and composite lymphomas were observed in 20% of patients. Mantle cell lymphoma presenting in the ocular adnexal region is associated with advanced-stage disease and short PFS but an OS similar to MCL at other sites.

Adult↗
PubMed2005

Arachnoid cysts with orbital bone remodeling--two interesting cases.

PURPOSE: To describe two cases of arachnoid cyst that altered the bony orbit. METHODS: Case report. RESULTS: Cases of unilateral arachnoid cyst are described in two men, both over the age of 60. The patients were evaluated with computed tomography (CT) scans and in each, an arachnoid cyst in the middle cranial fossa caused bone remodeling with exophthalmos in one. These patients were followed clinically and radiographically over a number of years with no change. CONCLUSION: Arachnoid cysts in the middle cranial fossa have the potential to result in clinically significant proptosis due to bone remodeling of orbital walls. Patients with unexplained proptosis should be evaluated with CT scans to establish the presence of bone remodeling and an arachnoid cyst, which can be confirmed with T2-weighted magnetic resonance imaging (MRI).

Aged↗
PubMed2004

Orbital lymphoma mimicking optic nerve meningioma.

PURPOSE: To describe two patients with orbital lymphoma mimicking optic nerve meningioma. METHODS: Retrospective case review. RESULTS: Both patients presented in their sixth decade with gradually increasing proptosis and decreasing vision. Computerized tomography revealed homogeneous masses encasing the optic nerves, which in conjunction with the clinical presentations were felt to represent optic nerve meningiomas. Biopsy, however, led to a diagnosis of lymphoma of mucosal-associated lymphoid tissue origin in one and a well-differentiated non-Hodgkin's lymphoma in the other. CONCLUSION: Orbital lymphoma surrounding the optic nerve may mimic the clinical and radiological appearance of an optic nerve meningioma.

Biopsy↗
PubMed2004

Malignant peripheral nerve sheath tumour metastatic to the orbit.

PURPOSE: To present a case of malignant peripheral nerve sheath tumour (MPNST) metastatic to the orbit. METHOD/RESULTS: The authors describe a 29-year-old woman with disseminated MPNST who presented with acute right orbital inflammation. Computerized tomography revealed a superolateral mass with a central radiolucent area. At biopsy, the mass was primarily subperiosteal and histology showed a spindle cell, S100-positive MPNST consistent with the primary tumour. CONCLUSION: This is the second reported case of MPNST metastatic to the orbit and illustrates that an inflammatory presentation is possible in this aggressive malignancy.

Adult↗
PubMed2003

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.

Adolescent↗
PubMed2003

Pathogenesis of orbital cholesterol granuloma.

Two cases are presented of orbital cholesterol granuloma associated with gradual proptosis arising in men aged 35 and 41 years. Computed tomography demonstrated osteo-lytic masses in the frontal bone at the lacrimal fossa. Curettage revealed a characteristic histology of foreign body reactions surrounding cholesterol clefts. In both cases abnormal bone, more consistent with fibrous dysplasia than reactive change, was found at the periphery. The finding of abnormal bone associated with orbital cholesterol granulomas suggests that a pre-existing bone anomaly may be present in a subset of these cases.

Adult↗
PubMed2003

Vascular malformations of the orbit: hemodynamic concepts.

Vascular malformations of the orbit can best be understood within the context of their hemodynamics, of which there are three types. Type 1 (no flow) lesions have essentially little connection to the vascular system and include lymphangiomas or combined venous lymphatic malformations. Type 2 (venous flow) lesions appear as either distensible lesions with a direct and rich communication with the venous system or nondistensible anomalies that have minimal communication with the venous system. Types 1 and 2 can be combined with both features of distensible and nondistensible hemodynamics. Arterial flow lesions, type 3, include arteriovenous malformations characterized by direct antigrade high flow through the lesion to the venous side. Cavernous hemangiomas are also malformations that demonstrate direct low flow through the lesion.

Abnormalities, Multiple↗
PubMedDate not supplied

Primary bone tumors of the orbit.

Primary tumors of orbital bone constitute 0.6% to 2% of all orbital tumors. Our experience over a 24-year period in the Orbital Clinic at the University of British Columbia yielded 62 (1.9%) cases from a total of 3,340 orbital tumors. Although a heterogeneous group, primary orbital bone tumors may be classified on a clinicopathologic basis into benign fibro-osseous or cartilaginous, reactive, neoplastic and vascular disorders. Presentation is usually a gradual mass effect, with infiltration and acute hemorrhage being features of malignant and reactive lesions respectively. The two most commonly encountered entities were fibrous dysplasia and osteoma, accounting for 22 cases. Although both these conditions rarely present a diagnostic challenge, nonspecific histologic and radiologic appearances can result in poor characterization of several of the rarer lesions. Hence, close cooperation between clinician, radiologist and pathologist is essential for accurate diagnosis.

Bone Neoplasms↗
PubMedDate not supplied

Orbital reconstruction after eyelid- and conjunctiva-sparing orbital exenteration.

PURPOSE: To describe the technique and results of eyelid- and conjunctiva-sparing orbital exenteration with temporalis transfer and dermis-fat graft. METHODS: Five patients who underwent this procedure were studied, based on retrospective chart review. Schematic drawings were produced to illustrate the surgical technique. RESULTS: The study included three female and two male patients with an age range of 7 to 76 years. Two patients required surgery for invasive optic nerve sheath meningioma, one for hemangiopericytoma, and the fourth for mesenchymal chondrosarcoma. The last patient had eyelid-sparing exenteration performed more than 20 years earlier for retinoblastoma and underwent a similar reconstruction. One case was complicated by infection and graft atrophy. The remaining four cases retained orbital volume and eyelid structures with a reasonable cosmetic outcome. Average follow-up period was 21 months, during which one patient had development of intracranial meningioma at the proximal end of the optic canal. CONCLUSIONS: Eyelid- and conjunctiva-sparing orbital exenteration with temporalis transfer and dermis-fat graft is a useful technique that minimizes the usual deformity and achieves good results. Case selection is emphasized, as this technique is mainly reserved for histopathologically benign orbital lesions that exhibit local aggressive behavior and for malignant lesions only if there is no eyelid, lacrimal gland, or orbital fissure involvement nor significant conjunctival or deep extension of an intraocular tumor.

Adult↗
PubMedDate not supplied

Infiltrating orbital astrocytic proliferation associated with congenitally malformed eyes.

This report describes the clinical and pathologic findings in two adults with orbital infiltration by astrocytic cells associated with congenitally malformed eyes. Both cases had enlarging orbital masses and underwent complete resection. Histopathology revealed proliferation of astrocytes (fibrillary acidic protein-positive) that invaded the orbital tissues. To the best of our knowledge, there are no similar cases in the literature.

Adult↗
PubMedDate not supplied

The spectrum of orbital Rosai-Dorfman disease.

PURPOSE: To describe the spectrum and treatment of orbital Rosai-Dorfman disease and to review previously documented cases. METHODS: Retrospective, interventional case series of seven patients and literature review. RESULTS: Each patient with Rosai-Dorfman disease had unique disease expression requiring aggressive therapy, such as chemotherapy, radiation, and/or surgical excision. One systemically aggressive case presented with intraocular and choroidal invasion, a previously unreported finding. Response to therapy was variable and unpredictable. CONCLUSIONS: Rosai-Dorfman disease, although historically described as benign and self-limiting, may cause significant morbidity and mortality involving multiple organ systems. Available treatment options may not control the disease. Further research and long-term clinical correlation is necessary.

Adolescent↗