Purpose Although systemic or eyelid involvement by ocular adnexal lymphoma carries a worse prognosis, there have been few reports of the outcome in relation to clinical presentation. The outcome of malignant ocular adnexal lymphoma was, therefore, related to presenting clinical symptoms and signs. Design and Methods A retrospective, noncomparative case-note review of 326 patients treated in the Orbital Clinic at Moorfields Eye Hospital. The associations between presenting symptoms or signs and three outcome measures (v.i.) were assessed by univariate and multiple variable regression together with Kaplan–Meier analysis. Main Outcome Measures (i) Presence of extra-orbital disease at the time of presentation; (ii) development of systemic lymphoma after new presentation with solely ocular adnexal disease; and (iii) death attributable to widespread lymphoma. Results Presentation with disseminated disease was rarer with over 1 year's ophthalmic symptoms (odds ratio (OR) 0.7; 95% CI 0.5–0.9) and much more frequent with bilateral adnexal disease (OR 5.8; 95% CI 3.0–11.2). With solely adnexal disease at presentation, subsequent extra-orbital lymphoma was more frequent and earlier with lacrimal gland disease (as compared to those without; hazard ratio (HR) 1.9; 95% CI 1.2–4.5) or with eyelid disease (compared to those without; HR 2.4; 95% CI 1.2–4.5), or with bilateral disease (compared to unilateral disease; HR 2.6; 95% CI 1.4–5.2). Prior or concurrent systemic disease was the most significant predictive factor for lymphoma-related death (HR 6.8; 95% CI 4.3–10.9), but tumour-related death was also commoner and earlier with bilateral disease (HR 2.4; 95% CI 1.4–4.0) or where a relative afferent papillary defect was present (HR 2.8; 95% CI 1.6–4.9). Similarly, the rate of tumour-related death was slightly less where symptoms had been present for more than a year (HR 0.8; 95% CI 0.7–1.0) and slightly greater in the elderly (HR 1.03; 95% CI 1.01–1.05). Conjunctival lymphoma had the lowest rate of extra-orbital spread and lymphoma-related death, the rate of these two events being sequentially greater for patients with predominantly deep orbital lymphoma, lacrimal gland lymphoma, or eyelid lymphoma. Conclusion These data suggest that presenting symptoms and signs of patients with ocular adnexal lymphoma are significantly associated with the risk of systemic disease at orbital presentation, the rate of subsequent spread, and the rate of lymphoma-related death.

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.

PURPOSE:To establish the incidence of underlying orbital vascular anomalies, the presence of systemic associations and predisposing factors, the natural history and appropriate management of patients with non-traumatic orbital haemorrhage presenting in an orbital clinic.METHODS:The records of 115 patients with a diagnosis of non-traumatic orbital haemorrhage were reviewed with regard to clinical findings, investigations, management and outcome.RESULTS:Associated orbital vascular malformations were present in 104 patients (90%). Thirteen (11%) had additional or other predisposing factors (childbirth, prolonged headstands, hypertension or coagulopathies). Six patients (5%) had no predisposing factor. Acute onset painful proptosis, associated with lid swelling or a mass, was the most common presentation. Visual acuity was reduced in 37 patients (32%) at presentation. Excluding eight patients (7%) who underwent surgery for optic nerve compression, spontaneous resolution of the haemorrhage was complete in 62%, partial in 27%, while 4% had no resolution. Final visual acuity was reduced in 23 patients (20%).CONCLUSION:The majority of bleeds are associated with some form of orbital vascular anomaly. Where no such anomaly can be demonstrated a search for an underlying systemic cause should be performed. Haemorrhages in the young were usually localized whereas those in older patients were diffuse. Orbital imaging, with a combination of computed tomography and magnetic resonance imaging, was helpful in the assessment of these lesions. Most bleeds are venous and self-limiting. Surgical intervention was rarely necessary and should be confined to those with optic nerve compromise or a localized lesion which persists.

We reviewed computed tomography (CT) studies in 30 patients with histologically confirmed orbital rhabdomyosarcoma. There were 17 male and 13 female patients, mean age 8.8 years (median age 7 years, age range 1 month to 51 years). The tumour occupied the intra and extraconal compartments in 14 (47%) cases; it was solely extraconal in 11 (37%) and intraconal in five (16%), Within the orbit the upper inner quadrant was the most common site, 20 (67%) of the tumours lying in this region. More than one quadrant was involved in 16 (53%) cases. The epicentre of the tumour was in the eyelids of three (10%) patients. At presentation the mean size of the mass was 25 x 17 mm in long and short axis diameters, respectively. The tumours were of soft tissue density with moderately well defined margins and an irregular shape. They showed mild to moderate contrast enhancement, Calcification was seen in one case. Changes in the adjacent bone were seen in at least 12 (40%) patients. Intracranial extension was present in one (3%) patient and invasion of the paranasal sinuses was also seen in one (3%) patient. The intraorbital structures were displaced or encased by the tumour mass. The globe was displaced and distorted but not invaded by the tumour. Magnetic resonance imaging (MRI) studies were also reviewed in five patients, On T1-weighted images the tumour gave a similar signal to muscle but on T2-weighted images it gave a higher signal.

PURPOSE:Combined chemotherapy and radiation therapy have improved the survival of children with primary orbital rhabdomyosarcoma, but recurrence or persistence of the local orbital tumor still occurs. There are no established guidelines for dealing with these uncommon patients, and the authors present a review of the combined method treatment and outcome of children with refractory primary orbital rhabdomyosarcoma.METHODS:From clinical databases, 67 children with orbital rhabdomyosarcoma were identified. Seven (10%) of the 67 children had tumors refractory to combined chemotherapy and radiation therapy and underwent exenteration or eye-sparing tumor excision. Their clinical course and outcome were reviewed retrospectively.RESULTS:No patient was lost to follow-up, which ranged from 3.2 to 11 years. Five (71%) of the seven children with refractory tumor are still alive at more than 3 years after surgery (3.2-11 years; mean, 6.9). In one of the two children who died, tumor extended beyond the operative margins at exenteration, and the other child died with regional metastasis within a month of exenteration.CONCLUSIONS:Although more than 90% of children with orbital rhabdomyosarcoma respond to combined therapy by pediatric oncologists and radiotherapists, local orbital (salvage) surgery by ophthalmologists may be of value in the minority of children with refractory tumors. All of the five surviving children appear to be disease free.

PURPOSE:The purpose of the study is to establish the natural history, probable nature, and optimal treatment of lesions within the orbit described previously as lymphangiomas or orbital varices.METHODS:The clinical and radiologic records of 158 patients with these lesions were reviewed. Of these, 91 had surgery, and histologic specimens from 57 were re-examined.RESULTS:Most patients were infants or children with a dark blue swelling in the superomedial part of the orbit, orbital hemorrhage, or proptosis. Computed tomography showed smooth, contoured lesions denser than brain extending posteriorly. Half enlarged with the Valsalva maneuver, 31% contained phleboliths. Surgery was performed in 91 patients, mainly for cosmesis. Excised tissue included endothelium-lined channels containing blood in the orbit and clear fluid in many superficial specimens.CONCLUSIONS:The behavior of these lesions and their prevalence in infancy and childhood favor a hamartoma. The authors observed a seamless range of clinical features that they could not subdivide, particularly in relation to any connection with the orbital veins. Many bleed and enlarge permanently and need excision, but surgery is difficult and management should be as conservative as possible. The origin of these lesions cannot be determined by histopathologic analysis, although the authors have evidence of venous features in the orbit and lymphatic features more superficially. The authors' clinical findings support a venous origin. Two-thirds have either a free venous connection or phleboliths. Their distribution corresponds with that of the normal orbital veins, and at surgery they derive from or replace those veins. "Orbital venous anomaly" is the most accurate term for their description.

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.

We reviewed the CT studies of 176 patients with biopsy-proven inflammatory pseudotumour of the orbit, in an attempt to establish the incidence of bone sclerosis in this condition, We found 20 patients (11.5%) to show definite bone sclerosis and/or hyperostosis, and in a further 10 (6%) there was probable sclerosis, giving a total of 30 patients (17%) with some evidence of bony change. Bone-window films were available for a minority of patients, and the frequency of sclerosis was therefore probably underestimated, This feature, which presumably represents a low-grade, chronic sterile osteitis, has not previously been emphasised.

PURPOSE:To emphasize that solitary orbital extramedullary plasmacytoma, a rare tumor, is not invariably radiosensitive.METHOD:We examined a patient who had undergone empirical irradiation that failed to control an orbital tumor.RESULTS:After a second biopsy had secured the diagnosis of a solitary extramedullary plasmacytoma, further irradiation proved ineffective and exenteration was required. The patient was free of recurrence one year after exenteration and had satisfactory cosmesis with a prosthesis.CONCLUSION:A histologic diagnosis is essential before treatment is commenced so that the correct radiation dosage is used.

1. Primary orbital amyloidosis is a rare form of localized amyloidosis in which the precursor protein has not previously been identified. We report here the first extraction of amyloid fibrils from a tissue biopsy containing orbital amyloid, and characterization of the fibril protein. 2. The N-terminal nine residues were identical with residues 278-286 and 275-283 of the third constant (CH3) domains of IgG1 and IgG4 gamma heavy chains, respectively. The mass of the fibril subunit protein was 6125Da by time-of-flight mass spectrometry, compared with the expected masses of 6169.9Da and 6214.9Da for the CH3 domains of gamma 1 from residue 278 and gamma 4 from residue 275, respectively. The fibril protein thus appeared to consist exclusively of an immunoglobulin heavy chain constant domain. 3. Only two examples of immunoglobulin heavy chain derived amyloid have been reported previously and both of these, as well as all published cases of the usual immunoglobulin light chain derived amyloid, contained variable domain sequence. The present case therefore represents a form of local, presumably clonal, B-cell/plasma-cell disorder characterized uniquely by deposition of an amyloidogenic immunoglobulin heavy chain constant domain fragment.

Exenteration, or removal of the globe with part or all of the surrounding orbital contents, is generally reserved for malignancy. The procedure may, however, be of value in the management of some benign orbital diseases. The indications for exenteration in management of benign orbital disease are threefold. Firstly, patients in whom diffuse disease, such as idiopathic inflammation, has resulted in an irretrievable situation of visual loss and clinically uncontrollable pain or disfigurement; in many such cases we consider exenteration to be preferable to the (often severe) side effects of prolonged and inadequate medical therapy. The second group are those patients with grossly disfiguring orbital abnormalities, such as teratomas, extensive varices, or massive optic nerve tumours. The last group comprises patients with tumours that, while histologically benign, may have malignant potential or show a tendency to diffuse or persistent infiltration of orbital soft tissues. Sixteen illustrative cases of full or partial exenteration for benign disease are described.

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.

A case of anaerobic orbital cellulitis secondary to intraorbital wood and an approach to management are presented. Retained foreign bodies should be suspected in all penetrating orbital injuries involving wood. Computed tomography (CT) should be performed to delineate the location and size of any foreign body and to determine damage to adjacent structures. Magnetic resonance imaging (MRI) is useful in certain circumstances. Orbital infection should be anticipated and broad-spectrum antibiotic cover (including anaerobes) provided. Surgical intervention should be undertaken to remove any retained foreign bodies to prevent vision-threatening complications.

The clinical characteristics and outcome of 50 primary malignant neoplasms of the lacrimal gland are reviewed: 38 (76%) adenoid cystic carcinomas, six (12%) carcinomas arising in pleomorphic adenoma, and six (12%) adenocarcinomas or other types of carcinoma. Most patients presented with a short history and pain, though pain tended to occur less often and later with adenocarcinoma than with adenoid cystic carcinoma. Pain was unrelated to the duration of symptoms, invasion of bone, loss of trigeminal nerve function, or the frequency and time of tumour recurrence. The estimated disease-free survival for patients with adenoid cystic carcinoma was significantly (p less than 0.01) reduced where half or more of the biopsy specimen showed basaloid differentiation. Eleven patients underwent extended cranio-orbital resection, and the others received a combination of total dacryoadenectomy adenectomy and/or radiotherapy. Survival after adenoid cystic carcinomas appears to be significantly (p less than 0.05) greater when tumour resection is combined with radiotherapy than after radiotherapy alone. At present, however, the rate of disease-free survival after treatment of adenoid cystic carcinoma appears unaltered by cranio-orbital resection, though these latter patients form a relatively greater proportion of those surviving for more than 10 years. Further long-term follow-up is needed to see if this technique does influence survival.

Actinomycosis is a very rare cause of orbital abscess usually attributable to direct spread from adjacent structures. A case of actinomycosis of the orbit is presented, which was treated as orbital pseudotumour for 3 months before progression of signs and symptoms, despite high dose steroids, led to the diagnosis being reconsidered.

Orbital haemangiopericytomas are ideally managed by complete surgical excision in the first instance. This is frequently not achieved, because difficulty in making the diagnosis preoperatively results in incisional biopsy and the highly vascular nature of the tumour makes complete excision difficult. A series of 12 patients with orbital haemangiopericytoma seen over a 23-year period is presented. The following combination of clinical and radiological features is suggestive of haemangiopericytoma. 1. Painless non-axial proptosis with downward displacement of the globe. 2. Intermittent upper lid swelling. 3. A soft, superiorly located mass with poorly defined borders, especially with a blue hue. 4. A superiorly located, rounded or elongated extraconal mass on CT, isodense with brain, with smooth, well-defined borders and moderate to marked enhancement with the injection of intravenous contrast medium. 5. Significant blush in all three phases of carotid angiography, without prominent arteriovenous shunting. Once haemangiopericytoma is suspected, complete surgical excision is recommended.

To reduce the risk of recurrence and malignant transformation, pleomorphic adenomas of the lacrimal gland should be removed intact, without prior biopsy. Seventy one of the 78 patients in this series were referred without previous surgery, and, on clinical or radiological evidence, 63 (89%) tumours were correctly diagnosed and totally excised, with preservation of a margin of the surrounding normal tissue. Sixty three patients had tumour within the body of the gland and 55 (84%) had radiological signs or satisfied clinical criteria for pleomorphic adenoma that we suggested previously; that is, over 1 year of symptoms and absence of pain. Eight (16%) orbital lobe tumours were misdiagnosed preoperatively and biopsied; in all these patients symptoms had been present for less than 1 year, and four patients had pain. Eight patients with tumours arising in the palpebral lobe had a short history of an upper lid mass, and their tumours were excised without biopsy. With the surgical techniques described in this paper, there has been no recurrence of tumour in patients with follow-up as long as 21 years. A modification of surgical technique, with preservation of the palpebral lobe of the gland, has reduced the incidence of postoperative dry eye and distortion of the upper eyelid.

The onset of periorbital xanthogranuloma in adults is rare and may be accompanied by haematological abnormalities and malignancy. The appearance of the eyelid lesions is virtually diagnostic, producing readily recognisable diffuse, yellow plaques, and affected patients should be investigated and reviewed regularly for systemic disease. Three cases are described, in which periorbital cutaneous plaques were associated with abnormal tissues in the superior part of the orbit; these abnormal tissues caused displacement or restricted movement of the globe or upper eyelid. The possibility that two cases represent a necrobiotic type of xanthogranuloma is presented. Nine years after the onset of xanthogranuloma one patient developed non-Hodgkin's lymphoma. A multiple-drug regimen of systemic chemotherapy, given for lymphoma, caused a marked clinical reduction in the periorbital xanthogranuloma.

Dermolipomas are solid choristomas which present as a lateral canthal mass. A series of 35 cases is reported, 27 of whom were female. Six patients had features of Goldenhar's syndrome. Proximity of the dermolipoma to the lacrimal ductules, the lateral rectus and upper lid structures makes extensive surgical excision dangerous. A microsurgical technique of partial excision of the lesion is described and was used successfully in 13 cases without significant complications.