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Biomedical subjects

J Rootman

Publications and source records attributed to J Rootman.

At least 37 records · Page 2Linked to original sources

Enchondroma of the orbit.

PURPOSE: To report a documented case of orbital enchondroma. METHODS: A 25-year-old woman had an asymptomatic orbital mass. Computed tomographic scan was performed. The mass was surgically removed from the medial wall of the orbit, and the specimen was submitted for histopathologic examination. RESULTS: The tumor showed typical radiologic and histopathologic appearance of an enchondroma with typical hypocellular lobules of cartilage enclosed by lamellar bone. CONCLUSION: Cartilaginous neoplasms in the orbit are extremely rare.

Adult↗

Tumors arising in the palpebral lobe of the lacrimal gland.

PURPOSE: Tumors arising in the palpebral lobe of the lacrimal gland are uncommon. There have been only eight reported patients, all of whom had pleomorphic adenomas. The authors present another six patients with primary palpebral lobe tumors. METHODS: The clinical presentation, computed tomographic, and pathologic findings of these six patients were reviewed. RESULTS: Tumor types include three pleomorphic adenomas, one well-differentiated adenoid cystic carcinoma, one carcinoma in pleomorphic adenoma, and one carcinoma ex pleomorphic adenoma. All six patients presented with a painless mass in the upper outer eyelid without significant displacement of the globe, but the superficial location of these lesions led to early detection. The tumors were localized to the lacrimal gland, predominantly anterior to the orbital rim. In all six patients, the involved palpebral lobe was removed, five via a direct anterior subcutaneous approach and one by superolateral orbitotomy. In two patients (adenoid cystic carcinoma and carcinoma ex pleomorphic adenoma), repeat surgery via superolateral orbitotomy was necessary to achieve complete removal of the lacrimal gland and surrounding tissue. No recurrences were detected during follow-up (range, 6 months to 7 years; mean, 26 months). CONCLUSION: Palpebral lobe tumors of the lacrimal gland are more common and consist of a greater variety of histologic types than previously described. In our series, these tumors comprise 17% of all epithelial lacrimal gland lesions. These are the first reported cases of malignancy in this location. A longer follow-up period is necessary to determine the true recurrence rate of these tumors.

Adenocarcinoma↗

Primary ductal adenocarcinoma of the lacrimal gland.

PURPOSE: To their knowledge, the authors report the first recognized case of ductal adenocarcinoma of the lacrimal gland (histologic equivalent of salivary duct carcinoma). Primary adenocarcinoma of the lacrimal gland is rare and has been described generically. In contrast, primary adenocarcinomas of the major and minor salivary glands are much more common and have been classified into histopathologic subtypes that have different clinical characteristics and outcomes. METHODS: A 68-year-old man presented with a 6-month history of a painless mass in the right upper outer eyelid. The authors describe the clinical, radiologic, and histopathologic features of this case and review the lacrimal gland literature. RESULTS: A modified en bloc orbitectomy was performed, and postoperative radiotherapy was administered. The patient was alive and well without evidence of tumor recurrence 10 months after surgery. CONCLUSION: The World Health Organization classification of salivary adenocarcinomas (1991) provides a framework for further insight into the presentation and biologic behavior of the less common lacrimal carcinomas.

Aged↗

Adverse effects of bone wax in surgery of the orbit.

The goal of this article is to establish the incidence and scope of adverse reactions to bone wax in a large orbital surgical series. We report two patients with bone wax granulomas of the orbit as a remote surgical complication. These are the first reported cases of adverse reactions to bone wax in the ophthalmic literature. A chart review was conducted on all patients from the University of British Columbia Orbit Clinic that had surgery with temporary or permanent removal of orbital bone. Two patients with bone wax granulomas were identified. In one case, intraoperative cultures grew Staphylococcus aureus, confirming that the wax may indeed act as a nidus for infection. No cases of pseudoarthrosis have occurred. This syndrome of chronic granulomatous giant cell foreign body inflammation has characteristic clinical, radiologic, and histopathologic features. The literature regarding adverse reactions to bone wax is reviewed, and specific implications for orbital surgery are discussed.

Child, Preschool↗

Monoclonal origin of localised orbital amyloidosis detected by molecular analysis.

AIMS: Primary localised orbital amyloidosis is a rare disease. The purpose of this study was to describe two cases of primary orbital amyloidosis and emphasise the value of molecular analysis of immunoglobulin gene rearrangement in identifying a monoclonal population of cells responsible for the amyloid production. METHODS: Charts and biopsy specimens of each case were reviewed. Conventional light microscopy, immunohistochemistry, and polymerase chain reaction (PCR) analysis for immunoglobulin gene rearrangement were performed in both cases. RESULTS: An unusual presentation of localised primary amyloidosis with bilateral and extensive enlargement of multiple extraocular muscles was seen in case 1. The presence of amyloid deposits was confirmed by biopsy in both cases. Evidence of a monoclonal population of plasma cells was shown by immunohistochemical analysis in case 2 only. The monoclonal origin of the cells responsible for the amyloid deposition was determined by PCR analysis demonstrating immunoglobulin heavy chain gene rearrangement in both cases. CONCLUSIONS: A monoclonal population of plasma cells responsible for the amyloid deposition was present in these two cases. PCR analysis is extremely helpful in determining monoclonality, a finding that may have important therapeutic and prognostic implications.

Adult↗

Histopathologic findings and frequency of clonality detected by the polymerase chain reaction in ocular adnexal lymphoproliferative lesions.

We report the reclassification according to recently described histologic categories of 48 patients with ocular adnexal lymphoproliferative lesions with long-term follow-up (mean, 8.1 yr). We used available formalin-fixed, paraffin-embedded, and frozen tissues to assess the frequency of immunoglobulin heavy chain gene rearrangement detectable by polymerase chain reaction in these lesions. We reviewed patient records, obtained follow-up data, and examined hematoxylin- and eosin-stained slides. DNA extracted from tissues was amplified with consensus V- and J-region primers to detect immunoglobulin heavy chain gene rearrangement. We examined 28 orbital, 10 lacrimal, and 10 conjunctival lesions, of which 2 lesions were lymphoid hyperplasias, 3 were indeterminate, and 43 were lymphomas. Of the 44 patients with follow-up, systemic lymphoma developed in 24 (55%), of whom 11 died of the disease, and 6 are alive with disease. Thirty-one patients had sufficient DNA for polymerase chain reaction analysis; 9 specimens were nonclonal, 21 were clonal, and 1 failed to amplify. The nonclonal lesions included one hyperplasia, one indeterminate lesion, and seven lymphomas; two of these patients died of the disease, and one is alive with disease. The clonal lesions included 1 indeterminate lesion and 20 lymphomas. Systemic lymphomas developed in 16 patients; 8 died of the disease, and 4 are alive with disease. Of the lesions histologically classified as lymphoma, 74% were clonal. We conclude that most ocular adnexal lymphoproliferative lesions can be histologically classified as lymphomas, that systemic lymphoma will develop in at least 50% of these patients if they are followed for sufficient time, and that most lesions classified as lymphomas will be clonal using polymerase chain reaction techniques. Lack of amplification using a consensus primer strategy may account for the inability to detect clonality by polymerase chain reaction in some histologically identified lymphomas.

Adolescent↗

Subperiosteal, subperiorbital dissection and division of the anterior and posterior ethmoid arteries for meningiomas of the cribriform plate and planum sphenoidale: technical note.

Removal of meningiomas from the region of the cribriform plate and the planum sphenoidale may entail a bifrontal craniotomy and an interruption of the tumor's blood supply along the floor of the anterior cranial base. However, with this approach, the presence of bulky tumor above makes it difficult to control bleeding from multiple bony foramina in the anterior cranial base and to expose these foramina. The blood supply to the dura in this region, and, therefore, to these tumors, is predominantly from the anterior and posterior ethmoid arteries. Preoperative embolization of ethmoid arteries is not without a significant and prohibitive risk of blindness. A frontoethmoidal approach to the arteries on both sides requires two separate skin incisions. Therefore, a subperiosteal, subperiorbital dissection and division of these arteries via a bicoronal skin incision is a practical alternative.

Arteries↗

Prognostic value of DNA ploidy as assessed with flow cytometry in uveal melanoma.

OBJECTIVE: To investigate the prognostic value of tumour cell DNA content, as determined with flow cytometry, in formalin-fixed paraffin-embedded tissue from patients with uveal melanoma. DESIGN: Case series. SETTING: Pathology Laboratory, Vancouver General Hospital. PATIENTS: Ninety patients with primary uveal (choroid) malignant melanoma diagnosed between 1975 and 1984 followed for at least 60 months. OUTCOME MEASURES: Tumour cell DNA content, 5-year and 10-year disease-specific survival rates. RESULTS: The DNA histograms were classifiable as diploid (67 cases) or aneuploid (18 cases) in 85 cases. On univariate analysis DNA content, age at diagnosis, histologic type of tumour, largest single tumour dimension and tumour volume were predictors of 10-year survival. Multivariate analysis with the stepwise Cox proportional hazards regression model did not confirm cellular DNA content as an independent prognostic factor. However, age at diagnosis, largest tumour dimension and presence of spindle vs. mixed or epithelioid cell pattern were significant predictors of death from uveal melanoma. CONCLUSIONS: DNA ploidy of uveal melanoma is not a statistically significant predictor of survival.

Adult↗

Epithelioid sarcoma of the orbit.

BACKGROUND: Epithelioid sarcoma is a rare and aggressive malignancy commonly occurring in the tendon sheaths of the distal extremities of young adults. This article presents the clinical and pathologic details of two patients with epithelioid sarcoma in the orbit, the first to be reported as occurring in this location. METHODS: The clinical history, results of physical and radiologic examination, surgical procedures, pathologic specimens, and clinical course of each patient were reviewed and recorded. RESULTS: Biopsies were performed of the masses that were found in the superotemporal orbit of both patients. Results of pathologic examination in each patient caused considerable difficulty, and the correct diagnosis was reached only after extensive consultation. The first patient underwent wide soft tissue and bone exenteration and is alive without recurrence at 3 years. The second patient had multiple recurrences and died of recurrent and metastatic disease at 29 months. CONCLUSIONS: Epithelioid sarcoma must be added to the differential diagnosis of orbital sarcomas. The histology is nodular with a pseudogranulomatous appearance. Based on our limited experience, epithelioid sarcoma of the orbit may have a course similar to that which occurs elsewhere in the body, requiring aggressive surgical treatment at the outset.

Adolescent↗

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.

Adolescent↗

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 infra-orbital 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.

Adult↗

Muscarinic receptor M1 and M2 subtypes in the human eye: QNB, pirenzipine, oxotremorine, and AFDX-116 in vitro autoradiography.

Muscarinic cholinergic agents are used to lower intraocular pressure in the medical management of glaucoma and subtypes of muscarinic receptors have now been recognised in many tissues including the eye. To localise muscarinic receptors and their M1 and M2 subtypes in the human eye, in vitro ligand binding and autoradiographic techniques with densitometric quantitation on postmortem eye sections were used. As ligands, [3H] quinuclydinyl benzylate (QNB) (non-subtype specific muscarinic antagonist), [3H]pirenzipine (M1 antagonist), [3H]oxotremorine (M2 muscarinic agonist), [3H]AFDX-116(11[(2[diethylaminomethyl]1-piperidinyl)acetyl]5 , 11dihydro-6H-pyrido [2,3b][1,4]benzodiazepine-6-one) (M2 antagonist) were studied. Specific binding sites for QNB, pirenzipine, and AFDX-116 were localised in the entire ciliary muscle, the iris, and ciliary epithelium. [3H]oxotremorine localised only in the longitudinal portion of the ciliary muscle, and additionally, was not localised in the iris or ciliary epithelium. These results suggest that oxotremorine, by binding selectively to receptors on the longitudinal ciliary muscle and inducing its contraction, may modulate outflow facility independently from accommodation and miosis.

Adult↗

Localization of M3 muscarinic receptor subtype and mRNA in the human eye.

Cholinergic agents used to lower intraocular pressure in the medical management of glaucoma act on muscarinic receptors in the eye. Recently, subtypes of muscarinic receptors have been recognized in many tissues including the eye. To localize the M3 muscarinic receptor subtype and m3 messenger RNA (mRNA) in the human eye, we used in vitro ligand binding and in situ hybridization techniques on post-mortem sections. We used an M3 antagonist, [3H]-4-DAMP ([3H]-4-diphenylacetoxy-N-methylpiperidine methiodide) to identify M3-binding sites and a [35S]-labelled oligonucleotide probe to detect and localize m3 mRNA. M3 and m3 mRNA were both localized in the ciliary muscle, ciliary epithelium, iris, corneal epithelium and anterior lens epithelium. The m3 transcript was also detected in the trabecular meshwork and corneal endothelium.

Acetylcholine↗