A 10-year review of orbital biopsy: the Newcastle Eye Centre Study.

PURPOSE: To review the histopathological diagnoses, visual outcome, and complication rate of orbital biopsy in a UK tertiary referral centre. METHODS: This was a retrospective, clinical-pathological, interventional, consecutive case series. All orbital biopsies performed between July 2004 and June 2014 in Newcastle Eye Centre (Newcastle upon Tyne, UK) were included in this study. All relevant data collected from the local electronic database and medical records were analysed. RESULTS: A total of 166 orbital biopsies were identified during the study period: 86 patients (53.1%) were female and the mean age was 53.7 ± 19.7 years. Of all the cases, orbital biopsies were performed unilaterally in 158 (97.5%) patients and bilaterally in 4 (2.5%) patients. The mean follow-up period was 2.2 ± 2.3 years. The two most common histopathological diagnoses were non-specific inflammatory disease (62, 38.3%) and lymphoproliferative disease (40, 24.7%). None of the patients experienced ≥ 2-Snellen line visual loss. There were 7 (4.2%) postoperative complications noted: 1 (0.6%) orbital haemorrhage with no loss of vision, 4 (2.4%) diplopia, 1 (0.6%) short-term symblepharon, and 1 (0.6%) conjunctival granuloma. Postoperative diplopia was associated with lateral orbitotomy (P = 0.044) and excisional biopsy (P = 0.015). CONCLUSIONS: Orbital biopsy serves as a safe diagnostic tool in managing orbital diseases. Patient should be made aware of the risk of postoperative diplopia. Our data provides useful guidance to clinicians when counselling patients for orbital biopsy.

Mesenchymal chondrosarcoma of the orbit: an unusual site for a rare tumour.

Mesenchymal chondrosarcoma is a rare tumour with orbital involvement being an exceptional occurrence. We present a case of a 22-year old man with such disease, together with details of his management. A brief literature review of this uncommon tumour was also enclosed.

Characterization of the inflammatory cell infiltrate in herald patches and fully developed eruptions of pityriasis rosea.

BACKGROUND: Pityriasis rosea (PR) is a common cutaneous papulosquamous disorder affecting young adults. Previous studies have suggested possibilities of a viral aetiology and the involvement of cell-mediated immunity, but these remain unproven to date. AIM: To elucidate the possible pathomechanisms in PR by characterizing the inflammatory cellular infiltrate in herald patches and fully developed PR eruptions. METHODS: In total, 12 biopsy specimens from 6 patients diagnosed with PR were examined. For each patient, biopsies were taken from both a herald patch and a secondary patch. Specimens were processed for histopathological examination and immunohistochemical staining with a large panel of monoclonal antibodies. RESULTS: Histopathologically, all specimens showed epidermal changes such as parakeratosis, orthokeratosis, epidermal hyperplasia and spongiosis. Less common results included epidermal exocytosis and focal parakeratosis. In all biopsies, the dermal infiltrate of lymphocytes stained positively for monoclonal antibodies specific for T cells. The ratio of the CD4+ (helper) vs. CD8+ (cytotoxic) T cells in the dermal infiltrate was increased in most specimens. Increased staining for Langerhans cells was seen within the dermis of lesional skin. There were no marked differences found in histopathology and immunohistochemistry between the herald patch and secondary lesions. Overall, there was a lack of natural killer cell and B-cell activities in PR lesions. CONCLUSIONS: Our results support a predominantly T-cell mediated immunity in the development of PR.

Outcome of orbital decompression for disfiguring proptosis in patients with Graves' orbitopathy using various surgical procedures.

AIM: To compare the outcome of various surgical approaches of orbital decompression in patients with Graves' orbitopathy (GO) receiving surgery for disfiguring proptosis. METHOD: Data forms and questionnaires from consecutive, euthyroid patients with inactive GO who had undergone orbital decompression for disfiguring proptosis in 11 European centres were analysed. RESULTS: Eighteen different (combinations of) approaches were used, the swinging eyelid approach being the most popular followed by the coronal and transconjunctival approaches. The average proptosis reduction for all decompressions was 5.0 (SD 2.1) mm. After three-wall decompression the proptosis reduction was significantly greater than after two-wall decompression. Additional fat removal resulted in greater proptosis reduction. Complications were rare, the most frequent being worsening of motility, occurring more frequently after coronal decompression. The average change in quality of life (QOL) in the appearance arm of the GO-QOL questionnaire was 20.5 (SD 24.8) points. CONCLUSIONS: In Europe, a wide range of surgical approaches is used to reduce disfiguring proptosis in patients with GO. The extent of proptosis reduction depends on the number of walls removed and whether or not fat is removed. Serious complications are infrequent. Worsening of ocular motility is still a major complication, but was rare in this series after the swinging eyelid approach.

Cutaneous phaeohyphomycosis due to Cladophialophora bantiana in an immunocompetent patient.

We report the case of a 56-year-old Chinese woman with phaeohyphomycosis. She presented with a 4-year history of a recurring erythematous plague initially diagnosed as chromoblastomycosis on histopathological examination. Surgical excision was performed when the lesion recurred despite intial treatment response to itraconazole. Tissue cultures of the surgical specimen grew Cladophialophora bantiana. Treatment with terbinaffine post surgery was instituted with no recurrence of the lesion to date. Cutaneous phaeohyphomycosis caused by Cladophialophora bantiana is rare and this case highlights the clinical presentation, laboratory findings and treatment of this infection.

Sweet's syndrome: a spectrum of unusual clinical presentations and associations.

BACKGROUND: Sweet's syndrome (SS) is the prototypic neutrophilic dermatosis. First described in 1964, the characterization of new clinical associations, unique histopathological findings and clinical variants have stimulated much interest and discussion recently. However, the prevalence of these unusual variants and clinical associations within a single cohort of patients, has not been described. OBJECTIVES: To describe and evaluate the prevalence of unusual clinical and histopathological features, as well as the clinical associations of SS seen in patients from the National Skin Centre, Singapore. METHODS: This is a retrospective study of all consecutive cases of SS seen at our centre over a 5.5-year period (June 1999-December 2004). Data on associated systemic diseases was obtained from the medical records and matched with information from the National Cancer Registry, Singapore. Patients not actively followed up for more than 3 months were contacted for their updated health status. RESULTS: Thirty-seven patients were identified. Ten (27%) had non-idiopathic SS. These were associated with haematological disorders, connective tissue disorders, infections or a drug. Twenty-nine patients (78%) had at least one atypical clinical or histopathological feature. Atypical clinical features included bullous lesions, SS with hand involvement or neutrophilic dermatoses of the hands and the concomitant existence of subcutaneous SS with pyoderma gangrenosum. SS was the presenting feature in three patients with infections caused by atypical organisms, including Mycobacterium chelonae, Penicillium species and Salmonella type D. Unique histopathological variants included subcutaneous SS and lesions containing an admixture of mature and immature neutrophils. Subcutaneous neutrophilic inflammation seemed to be more common in patients with an underlying haematological disorder. This group of patients also had a lower mean haemoglobin level. CONCLUSIONS: Unusual clinical and histopathological variants of SS described in the literature are similarly encountered in our cohort of patients, with some features being more common than others. We highlight and discuss some unique clinical and histopathological observations seen in our patients with SS.

Trigeminal trophic syndrome: an unusual cause of a non-healing cheek ulcer.

Trigeminal trophic syndrome (TTS) is an uncommon condition characterised by anaesthesia, paraesthesias and ala nasi ulceration, following peripheral or central damage to the trigeminal nerve. Only about 100 cases have been described in the literature to date. We report a 74-year-old woman who presented with a right cheek ulcer accompanied by pruritus and paraesthesia for three months. An old right cerebellar infarct was demonstrated on magnetic resonance imaging of the brain. Vertebrobasilar insufficiency leading to the cerebellar infarct is likely to have predisposed her to developing TTS. An underlying infectious, malignant and vasculitic cause for the ulcer was excluded by a skin biopsy. An increased awareness of the predisposing factors and clinical presentations of this important disfiguring condition is necessary to ensure prompt diagnosis and treatment.

Quality of life assessment after non-laser endonasal dacryocystorhinostomy.

OBJECTIVE: There has been a lack of patient-centred evidence in the Otolaryngology literature, that non-laser endonasal dacryocystorhinostomy improves the quality of life of patients. Many studies demonstrate successful outcomes based on non-validated subjective patient reporting. The aim of this survey was to evaluate the impact of non-laser endonasal dacryocystorhinostomy on the quality of life of patients using a validated questionnaire, the Glasgow Benefit Inventory (GBI). DESIGN: Prospective non-randomised case series. SETTING: Secondary otorhinolaryngology-ophthalmology centre, single centre. PARTICIPANTS: Sixty-five consecutive patients undergoing non-laser endonasal dacryocystorhinostomy were asked to complete a questionnaire at their follow-up clinic appointment. All patients had a minimum of 9 months follow-up. MAIN OUTCOME MEASURES: A consultant ophthalmologist reviewed each patient six months after surgery and recorded the outcomes as 'cure', 'better', 'no change' or 'worse'. We defined 'success' as cured or better. 'Failure' suggests no improvement or worsening epiphora. The validated 18-item GBI was used. RESULTS: Fully completed questionnaires were received from 55 patients. Mean total GBI scores were +34 for successful non-laser endonasal dacryocystorhinostomy and -19 for failed non-laser endonasal dacryocystorhinostomy (Mann-Whitney z = 3.8, P < 0.001). CONCLUSION: Successful non-laser endonasal dacryocystorhinostomy does confer significant quality of life improvement.

The use of porous polyethylene (Medpor) lower eyelid spacers in lid heightening and stabilisation.

BACKGROUND/AIMS: The management of lower eyelid retraction can be challenging, and established techniques to correct it are not always successful. Previous reports have suggested a role for the ultrathin high density porous polyethylene lower eyelid spacer (Medpor LES) in such patients. The authors report the experience of three surgeons implanting Medpor LES over 1 year, and ascertain whether such implants are a safe and effective alternative to autogenous spacers. METHODS: A prospective, interventional, non-comparative case series of consecutive patients. Surgical indications for Medpor LES were noted. Preoperative and postoperative lower marginal reflex distance (L-MRD), vertical palpebral aperture (PA), lagophthalmos, and scleral show inferior to the limbus (LSS) were recorded, together with major and minor complications. RESULTS: 32 patients (35 eyelids) had a Medpor LES inserted, 22/32 under local anaesthetic, and nine with adjunctive procedures. Mean follow up was 22 months (range 15-28 months). The Medpor LES was effective in reducing the palpebral aperture (p<0.001) and lagophthalmos (p = 0.04) and raising the lower eyelid height by reducing both L-MRD (p = 0.006) and LSS (p<0.001). However there were major complications in 7/32 patients and minor complications in 8/32, most requiring further surgery. Final outcome was good in 24/35 eyelids and satisfactory in 5/35. CONCLUSIONS: Despite a good or satisfactory final outcome in the majority of patients, the value of this technique is limited by complications, and should be reserved for those unsuitable for safer techniques.

Vogt-Koyanagi-Harada syndrome complicating allogeneic bone marrow transplantation.

Vogt-Koyanagi-Harada (VKH) syndrome is an uncommon acute ophthalmological disorder, characterised by bilateral serous retinal detachment with diffuse choroiditis, in association with specific extra-ocular manifestations. We describe a patient with unequivocal VKH syndrome arising 49 days after matched unrelated donor bone marrow transplantation (BMT) performed as treatment for severe aplastic anaemia. The visual symptoms and retinal changes responded well to corticosteroids. The haematological relevance of VKH syndrome is to distinguish it from retinitis due to cytomegalovirus, which requires different therapy and has a far worse visual prognosis.