G. E. Rose

Pleomorphic lipoma of the orbit: A case series and review of literature ☆

OBJECTIVE Pleomorphic lipomas are rare benign tumors that can resemble a variety of malignant soft tissue tumors on histologic examination. Six cases of patients with orbital pleomorphic lipoma, one of which was proven to be bilateral, are presented. DESIGN Retrospective, noncomparative, interventional case series with clinicopathologic correlation. METHODS Clinical and histologic review of 6 patients with pleomorphic lipomas of the orbit and histologic review of fat from 22 exenteration specimens and 20 other orbital procedures. MAIN OUTCOME MEASURES Evidence of histologic abnormalities in histologic specimens. RESULTS Pleomorphic spindle cells and multinucleated cells with nuclei arranged in a floret-like pattern were present in 7 specimens from 6 patients presenting with a clinical diagnosis of orbital fat prolapse, but there were no similar cell types present in the adipose tissue of 22 exenteration or 20 other orbital specimens. CONCLUSION Pleomorphic lipoma may arise in the orbit, presenting as what was hitherto considered to be age-related epibulbar prolapse of orbital fat.

Protocol for the combined immunosuppression & radiotherapy in thyroid eye disease (CIRTED) trial: A multi-centre, double-masked, factorial randomised controlled trial

BackgroundMedical management of thyroid eye disease remains controversial due to a paucity of high quality evidence on long-term treatment outcomes. Glucocorticoids are known to be effective initially but have significant side-effects with long-term use and recrudescence can occur on cessation. Current evidence is conflicting on the efficacy of radiotherapy and non-steroid systemic immunosuppression, and the majority of previous studies have been retrospective, uncontrolled, small or poorly designed.The Combined Immunosuppression and Radiotherapy in Thyroid Eye Disease (CIRTED) trial was designed to investigate the efficacy of radiotherapy and azathioprine in combination with a standard course of oral prednisolone in patients with active thyroid eye disease.Methods/designPatients with active thyroid eye disease will be randomised to receive (i) azathioprine or oral placebo and (ii) radiotherapy or sham-radiotherapy in this multi-centre, factorial randomised control trial. The primary outcome is improvement in disease severity (assessed using a composite binary measure) at 12 months and secondary end-points include quality of life scores and health economic measures.DiscussionThe CIRTED trial is the first study to evaluate the role of radiotherapy and azathioprine as part of a long-term, combination immunosuppressive treatment regime for Thyroid Eye Disease. It will provide evidence for the role of radiotherapy and prolonged immunosuppression in the management of this condition, as well as pilot data on their use in combination. We have paid particular attention in the trial design to establishing (a) robust placebo controls and masking protocols which are effective and safe for both radiotherapy and the systemic administration of an antiproliferative drug; (b) constructing effective inclusion and exclusion criteria to select for active disease; and (c) selecting pragmatic outcome measures.Trial registrationCurrent controlled trials ISRCTN22471573

Discoid lupus erythematosus of the periorbita: clinical dilemmas, diagnostic delays

PurposeUntreated periocular discoid lupus erythematosus (DLE), though very rare, may lead to significant morbidity with lid deformities, trichiasis, and symblepharon formation. We present the largest reported cohort of patients with biopsy-proven DLE solely affecting the periorbital region.MethodsObservational case series of patients managed over a 7-year period (2004–10).ResultsSeven patients (one male) presented to the Adnexal Service at Moorfields Eye Hospital at a median age of 47 years (range 23–71 years); median interval from symptom onset to biopsy-proven diagnosis was 38 months (range 6–86 months). Changes in peripheral skin were present in 1 patient (occurring after the initial eyelid presentation) and the presenting periocular features were dissimilar across the group, these included: chronic blepharo-conjunctivitis, madarosis, atypical chalazia, depigmentation of the eyelid margin, or marked, persistent periocular oedema with dacryoadenitis.Two cases settled spontaneously, but five required systemic hydroxychloroquine or intralesional corticosteroid injections.ConclusionPeriorbital DLE is rare and very varied in its presentation, the protean manifestations often resulting in significant diagnostic delay. All patients with unusual periocular skin disease and those with a refractory inflammatory dermopathy, should undergo biopsy of involved tissue(s), thus leading to earlier diagnosis and prevention of permanent cicatricial periocular changes.

Biopsy-confirmed spontaneous resolution of orbital langerhans cell histiocytosis.

A 17-year-old boy presented with a left upper lid swelling, headaches and diplopia. An orbital computerized tomography (CT) scan showed a mass in the left lacrimal fossa eroding bone and extending into the temporalis fossa and intracranially. An urgent biopsy without curettage was carried out and showed Langerhans cell histiocytosis. He was otherwise well and no other lesion was found. He was therefore observed and reviewed regularly. During follow-up it was noted that the mass was reducing in size. Five months after the initial biopsy a further procedure, involving curettage and an intralesional steroid, was carried out at a tertiary referral center. Histology of the material obtained showed no remaining evidence of Langerhans cell histiocytosis. Spontaneous resolution of orbital Langerhans cell histiocytosis has been described clinically and radiologically. This is the first case of spontaneous resolution to be confirmed histiologically.

Orbital decompression for Graves’ orbitopathy in England

AimsThe purpose of this study was to obtain data on orbital decompression procedures performed in England, classed by hospital and locality, to evaluate regional variation in care.MethodsData on orbital decompression taking place in England over a 2-year period between 2007 and 2009 were derived from CHKS Ltd and analysed by the hospital and primary care trust.Results and conclusionsIn all, 44% of these operations took place in hospitals with an annual workload of 10 or fewer procedures. Analysis of the same data by primary care trust suggests an almost 30-fold variance in the rates of decompression performed per unit population. Expertise available to patients with Graves’ orbitopathy and rates of referral for specialist care in England appears to vary significantly by geographic location. These data, along with other outcome measures, will provide a baseline by which progress can be judged.

Sporadic Burkitt's lymphoma presenting as solely orbital disease in a 78-year-old.

1. Suh OW, Ruttum MS, Stuckenschneider BI, Mieler WF, Kivlin JD. Ocular findings after bone marrow transplantation in a pediatric population. Ophthalmology 1999;106:1564-70. 2. Spraul CW, Lang GE, Lang GK. Corneal ulcer in chronic graft-versus-host disease: treatment with collagen shields. Klin Monatsbl Augenheilkd 1994;205:161-6. 3. Kiang E, Tesavibul N, Yee R, Kellaway I, Przepiorka D. The use of topical cyclosporin A in ocular graft-versus-host disease. Bone Marrow Transplant 1998;22:147-51. 4. Shulman HM, Sullivan KM, Weiden PL, McDonald GB, Striker GE, Sale GE, et al. Chronic graft-versus-host syndrome in man: a long-term clinicopathologic study of 20 Seattle patients. Am J Med 1980;69:204-17. 5. Calissendorff B, el Azazi M, Lonnqvist B. Dry eye syndrome in long-term follow-up of bone marrow transplanted patients. Bone Marrow Transplant 1989;4:675-8. 6. Jabs JA, Hirst LW, Green WR, et al. The eye in bone marrow transplantation. II. Histopathology. Arch Ophthalmology 1983;101:585. 7. Lawley TJ, Peck GL, Moutsopoulos HM, Gratwohl AA, Deisseroth AB. Scleroderma, Sjogren-like syndrome, and chronic graft-versus-host disease. Ann Intern Med 1977;87:707-9. 8. Jabs DA, Wingard I, Green WR, et al. The eye in bone marrow transplantation. III. Conjunctival graft-vs-host disease. Arch Ophthalmol 1988;107:1343. 9. Kasmann B, Ruprecht KW. Ophthalmologic findings in graft versus host disease. Klin Monatsbl Augenheilkd 1993;202:491-9. 10. Hirst LW, Jabs JA, Tutschka pJ, et al. The eye in bone marrow transplantation. I. Clinical study. Arch Ophthalmol 1983;101:580. 11. Franklin RM, Kenyon KR, Tutschka PI, et al. Ocular manifestations of graft versus host disease. Ophthalmology 1983;90:1. 12. Arocker-Mettinger E, Skorpik F, Grabner G, Hinterberger W, Gadner H. Manifestations of graft-versus-host disease following allogenic bone marrow transplantation. Eur J Ophthalmol 1991;1:28-32.

Cranio-orbital primary intraosseous haemangioma

PurposePrimary intraosseous haemangioma (IOH) is a rare benign neoplasm presenting in the fourth and fifth decades of life. The spine and skull are the most commonly involved, orbital involvement is extremely rare. We describe six patients with cranio-orbital IOH, the largest case series to date.Patients and methodsRetrospective review of six patients with histologically confirmed primary IOH involving the orbit. Clinical characteristics, imaging features, approach to management, and histopathological findings are described.ResultsFive patients were male with a median age of 56. Pain and diplopia were the most common presenting features. A characteristic ‘honeycomb’ pattern on CT imaging was demonstrated in three of the cases. Complete surgical excision was performed in all cases with presurgical embolisation carried out in one case. In all the cases, histological studies identified cavernous vascular spaces within the bony tissue. These channels were lined by single layer of cytologically normal endothelial cells.DiscussionIOCH of the cranio-orbital region is rare; in the absence of typical imaging features, the differential diagnosis includes chondroma, chondrosarcoma, bony metastasis, and lymphoma. Surgical excision may be necessary to exclude more sinister pathology. Intraoperative haemorrhage can be severe and may be reduced by preoperative embolisation.

Magnetic Oculomotor Prosthetics for Acquired Nystagmus

Purpose Acquired nystagmus, a highly symptomatic consequence of damage to the substrates of oculomotor control, often is resistant to pharmacotherapy. Although heterogeneous in its neural cause, its expression is unified at the effector—the eye muscles themselves—where physical damping of the oscillation offers an alternative approach. Because direct surgical fixation would immobilize the globe, action at a distance is required to damp the oscillation at the point of fixation, allowing unhindered gaze shifts at other times. Implementing this idea magnetically, herein we describe the successful implantation of a novel magnetic oculomotor prosthesis in a patient. Design Case report of a pilot, experimental intervention. Participant A 49-year-old man with longstanding, medication-resistant, upbeat nystagmus resulting from a paraneoplastic syndrome caused by stage 2A, grade I, nodular sclerosing Hodgkins lymphoma. Methods We designed a 2-part, titanium-encased, rare-earth magnet oculomotor prosthesis, powered to damp nystagmus without interfering with the larger forces involved in saccades. Its damping effects were confirmed when applied externally. We proceeded to implant the device in the patient, comparing visual functions and high-resolution oculography before and after implantation and monitoring the patient for more than 4 years after surgery. Main Outcome Measures We recorded Snellen visual acuity before and after intervention, as well as the amplitude, drift velocity, frequency, and intensity of the nystagmus in each eye. Results The patient reported a clinically significant improvement of 1 line of Snellen acuity (from 6/9 bilaterally to 6/6 on the left and 6/5–2 on the right), reflecting an objectively measured reduction in the amplitude, drift velocity, frequency, and intensity of the nystagmus. These improvements were maintained throughout a follow-up of 4 years and enabled him to return to paid employment. Conclusions This work opens a new field of implantable therapeutic devices—oculomotor prosthetics—designed to modify eye movements dynamically by physical means in cases where a purely neural approach is ineffective. Applied to acquired nystagmus refractory to all other interventions, it is shown successfully to damp pathologic eye oscillations while allowing normal saccadic shifts of gaze.

Lacrimal Canalicular Inflammation and Occlusion: Diagnosis and Management

Canalicular inflammation may lead to loss of compliance and stenosis, with lacrimal symptoms occurring despite anatomical patency. Microbial canaliculitis is frequently overlooked, leading to a delay in diagnosis and management. Failure adequately to remove canalicular stones and debris is a common cause for persistent canaliculitis. Canalicular epithelial inflammation due to primary herpes simplex infection is a common cause of canalicular, or common canalicular, occlusion. Subepithelial canalicular inflammation—as seen with lichen planus (LP)—may lead to a more severe and extensive annular fibrosis and carries a poor prognosis. Systemic chemotherapeutic agents, including radioiodine, 5-fluorouracil (5-FU), mitomycin C (MMC), and docetaxel, may injure the canalicular epithelium, the evidence suggesting active concentration of these agents by the lacrimal outflow structures. The surgical approach to canalicular occlusion depends on the extent of disease. Dacryocystorhinostomy (DCR) with retrograde canaliculostomy is the preferred surgery for proximal and midcanalicular occlusion. The indication for primary placement of a Jones canalicular bypass tube is the total absence of all distal canalicular and common canalicular structures, with this ascertained during open lacrimal surgery. The indication for secondary Jones tube placement is a functional failure after primary DCR with retrograde canaliculostomy. A canalicular bypass tube should be sutured such that the tube flange is held clear of the healing carunculectomy site; the function is not to prevent prolapse of the tube. As such, an encirclage suture is required only during primary placement of a bypass tube, when carunculectomy has just been performed.