I F Moseley

Histological features of ocular adnexal lymphoma (REAL classification) and their association with patient morbidity and survival

BACKGROUND The histological characteristics of ocular adnexal lymphomas have previously provided only a limited guide to clinical outcome for affected patients. This clinicopathological relation was re-examined using the Revised European American Lymphoma (REAL) system to classify the tumours in a large cohort of patients. METHODS The biopsies and clinical follow up data for 192 patients with ocular adnexal lymphoma were reviewed, the biopsies being regraded in accordance with the REAL classification. For each of five histological groups, logistic regression analysis was used to determine the odds ratios (OR) for the presence of systemic disease at the time of orbital diagnosis and Cox regression analysis was used to assess the hazard ratios (HR) for disseminated disease and lymphoma related death. For 108 patients in whom extraorbital spread occurred, the histological category of lymphoma was compared with the sites of dissemination. RESULTS At presentation, the frequency of previous or concurrent extraorbital disease increased from marginal zone lymphoma (OR 1.0), diffuse lymphoplasmacytic/lymphoplasmacytoid lymphoma (OR 2.3), follicle centre lymphoma (OR 3.8), diffuse large B cell lymphoma (OR 4.0) to other histological lymphoma variants (OR 26.8). For all histological types, the estimated risk of extraorbital disease and lymphoma related death continued for many years and the proportion of patients with at least one extraorbital recurrence after 5 years was 47% for MZL, 48% for LPL, 64% for FCL, 81% for DLCL, and 95% for other lymphoma variants. The corresponding estimated rates for 5 year lymphoma related mortality were 12%, 19%, 22%, 48%, and 53% respectively. CONCLUSIONS Patients with ocular adnexal lymphoma can be classified by REAL into five distinct groups, which show a progressive increase in the risks of extraorbital disease at diagnosis, of disease dissemination with time, and of tumour related death.

Outcome of Orbital Myositis: Clinical Features Associated with Recurrence

PURPOSE Although orbital myositis usually responds to prompt systemic therapy, recurrent or persistent episodes often require prolonged therapy. This study presents the clinical and radiologic features of 26 patients with orbital myositis and suggests a treatment regimen with special emphasis on recurrent or persistent orbital myositis. METHODS Retrospective analysis and comparison of clinical and radiologic parameters with clinical outcome in 26 patients with strictly defined orbital myositis was performed. A consultant radiologist reviewed computed tomography studies without knowledge of the clinical data or outcome. The frequency of certain clinical and radiologic parameters among two outcome groups was analyzed using Fishers exact test. RESULTS Comparison of clinical and radiologic features of 20 patients with a single acute episode and 6 patients with recurrent episodes disclosed several characteristics associated with recurrence. Male gender, lack of proptosis, eyelid retraction, horizontal extraocular muscle (EOM) involvement, multiple or bilateral EOM involvement (P = 0.05), muscle tendon sparing, and lack of response to systemic corticosteroids (P = 0.01), or nonsteroidal anti-inflammatory agents (P = 0.01) were associated with recurrent orbital myositis. CONCLUSIONS A treatment algorithm is suggested for patients with orbital myositis. Those patients whose clinical or radiologic features are associated with recurrence may benefit from early systemic steroid therapy.

Orbital involvement in multifocal fibrosclerosis

Multifocal fibrosclerosis is a condition of unknown aetiology, characterised by fibrous lesions occurring at a variety of sites. Clinical variants include retroperitoneal fibrosis, Riedels thyroiditis, sclerosing cholangitis, and mediastinal fibrosis. Orbital pseudotumour has been reported as a manifestation of this condition. Three patients with multifocal fibrosclerosis in whom orbital involvement was the dominant feature are described.

Primary localised amyloidosis of the orbit.

AIMS/BACKGROUND: Primary localised amyloidosis is rarely encountered in the orbit. The typical clinical and radiological appearances have not been clearly established, in particular the single site deposition of amyloid has never been proved. METHODS: Six cases were reviewed in detail and their clinical and radiological characteristics are described here. Four patients had scintigraphy with 123I serum amyloid P component and one patient had typing of the amyloid fibrils. RESULTS: All the patients had a firm mass in the upper orbit with a predilection for the region of the lacrimal gland. Computed tomography showed a homogeneous mass with thickening and irregularity of the adjacent bone and/or calcification within the mass. None was associated with systemic disease. Scintigraphy with 123I serum amyloid P component demonstrated that the amyloid was confined to the orbit. In one patient the amyloid fibrils were derived from an IgG4 heavy chain constant domain. The lesions were partially excised with subsequent clinical stability over 6 months to 18 years in all but one patient who had continued enlargement of the lesion. CONCLUSION: There is a characteristic clinical and radiological pattern for primary localised amyloidosis of the orbit. The disease process is truly local and not part of a systemic process. A majority respond to simple debulking with subsequent observation.

Orbital lymphoma versus reactive lymphoid hyperplasia: an analysis of the use of computed tomography in differential diagnosis.

Computed x ray tomography (CT) studies of 40 patients with proptosis or periorbital swelling, in whom biopsy showed lymphoma in 23 and reactive lymphoid hyperplasia in 17, were analysed in an attempt to identify radiological differences between the two conditions. The results indicate that homogeneity of an orbital mass is a sensitive but non-specific indication of lymphoma, 75% of lymphomatous masses and only 23% of reactive lesions being homogeneous. Bone destruction was seen only in cases of lymphoma, but was rare. Other radiological features of the mass or the affected orbital structures did not allow discrimination of tumour from a reactive lesion.

Radiological and clinicopathological features of orbital xanthogranuloma

BACKGROUND Orbital xanthogranuloma, a diagnosis confirmed histologically, occurs rarely in adults and children. With its characteristic macroscopic appearance the adult form may be associated with a spectrum of biochemical and haematological abnormalities including lymphoproliferative malignancies. METHOD The clinicopathological features and imaging appearances on computed tomography and magnetic resonance imaging of this condition are described in eight adults and a child. RESULTS Radiological evidence of proptosis was present in seven patients. In all nine patients an abnormal infiltrative soft tissue mass was seen, with increased fat in six cases. All patients had associated enlargement of extraocular muscles suggestive of infiltration and five had lacrimal gland involvement. Encasement of the optic nerve, bone destruction, and intracranial extension was present only in the child with juvenile xanthogranuloma. Haematological and/or biochemical abnormalities were detected in seven patients and seven patients had other systemic diseases which were considered to have an immune basis. One patient subsequently developed non-Hodgkins lymphoma. CONCLUSION The investigation and management of orbital xanthogranulomas requires a multidisciplinary approach even though the diagnosis may be suspected clinically. Imaging delineates the extent of disease and involvement of local structures and may influence the differential diagnosis. The juvenile form may be more locally aggressive, causing bone destruction with consequent intracranial extension.

The frequency and clinical significance of bone involvement in outer canthus dermoid cysts.

Periorbital dermoid cysts should be removed because they commonly leak their irritant contents into the surrounding tissues. The underlying bone may, however, be involved in patients with dermoid cysts at the outer canthus. Computed tomography studies of 70 patients (43 men and 27 women, aged 30 months to 63 years, mean 29 years) with proved dermoid cysts of this type were reviewed. The lesion was always unilateral; 34 were on the left. The bone of the lateral wall and superotemporal angle of the orbit showed the following abnormalities, often in combination: pressure erosion in 61 cases and an otherwise abnormal shape, probably developmental, in 55; the dermoid cyst entered a tunnel or canal through the lateral wall in 24; a blind pit or crater in 15; and a cleft in 20; many patients also showed abnormal bone texture. These findings are extremely important for planning adequate surgery, and indicate that bony involvement is much more frequent than previously appreciated.

Bilateral multiple cavernous haemangiomas of the orbit

Orbital cavernous haemangioma, a common orbital tumour, is usually single and unilateral. We report the first case of histologically confirmed bilateral multiple cavernous haemangiomas of the orbit.

Intubation Dacryocystography in Patients with a Clinical-Diagnosis of Chronic Canaliculitis (Streptothrix)

Magnification dacryocystography in 18 patients with chronic infective canaliculitis showed dilatation and irregularity of the affected lacrimal canaliculi, with prominent filling defects in almost all cases. Filling defects are uncommon in other diseases of the canaliculi and serve to confirm the diagnosis.

Diagnostic value of 'optic foramen views': experience from an eye hospital.

A prospective survey of 174 consecutive patients in whom plain radiographs of the optic canals were requested by ophthalmologists disclosed no cases in which these films made a positive contribution to management. It is suggested that as a general rule this radiographic examination has no useful role.