Dacryoadenitis associated with linear scleroderma en coup de sabre: A case report and review of literature.

Dacryoadenitis in the setting of linear scleroderma en coup de sabre (LScs) is an association that has not previously been described in the scientific literature. The purpose of this case report is to describe the co-existence of LScs and chronic dacryoadenitis and how it was managed. We report the case of a 42-year-old woman who presented with a 4-month history of left upper eyelid swelling with radiological enlargement of the left lacrimal gland on orbital CT and MRI imaging. Clinical examination revealed a left erythematous, swollen upper eyelid with lateral conjunctival injection and a palpable left lacrimal gland. An ipsilateral band-like cutaneous depression in the fronto-temporal region was also noted, which extended to the ipsilateral upper eyelid and brow. Serology revealed nucleolar antinuclear antibodies. A further incisional biopsy of the lacrimal gland confirmed chronic inflammatory changes and fibrous tissue. Based on both the histological and clinical findings, a diagnosis of dacryoadenitis in association with LScs was made. Oral methotrexate was commenced. The patient responded well with less frequent episodes of eyelid swelling and reduced periocular pain. This case describes for the first time, the ophthalmological manifestation of chronic dacryoadenitis in association with linear scleroderma en coup de sabre.

Silent sinus syndrome: potentially misleading features that should be recognized.

OBJECTIVE: Silent sinus syndrome (SSS) usually manifests clinically as hypoglobus and enophthalmos. Patients may experience different symptoms and may present to an assortment of specialties and delay diagnosis and management. The objective of this article was to describe the different and sometimes misleading signs and symptoms of SSS to improve the level of suspicion and reduce time to diagnosis. METHODS: A retrospective consecutive audit of the records of all patients diagnosed with SSS between 2015 and 2019 in the Oxford Eye Hospital, Oxford University Hospitals NHS Foundation Trust. Demographic and clinical data including presentation, diagnosis, and symptoms were obtained from the patients' medical files. RESULTS: Ten patients were included; mean age was 42.5 ± 11.5 years (range, 16-56 years). Four patients were initially referred to an ophthalmologist with globe asymmetry, diplopia, eyelid asymmetry, or retraction. Three patients were initially referred to an ear, nose, and throat specialist with facial asymmetry or infraorbital paraesthesia. Two patients were referred from the maxillofacial department with an incidental finding, and the last patient was seen initially by the neurology team with headaches. CONCLUSION: SSS has a variable presentation. Patients may have common or misleading signs. Patients may attend different clinics and subspecialties, and physicians should be aware of the broad range of presenting signs in this condition to prevent delay in diagnosis and further morbidity.

Paediatric orbital lymphoma; a case series and review of the literature.

OBJECTIVE: To describe a series of paediatric orbital lymphoma patients in a single tertiary referral centre. METHODS: A retrospective case-note search in the Oxford Eye Hospital of all patients under the age of 18 years with orbital lymphoma between 2010 and 2020. Demographic and clinical data were obtained, and a literature review was conducted. RESULTS: Five patients were identified with orbital lymphoma, mean age 48.2 ± 36 months (1-109 months), three were males. Clinical presentation included: ptosis, proptosis, lethargy, visual loss, and strabismus. Two patients had bilateral orbital disease and one patient was diagnosed within the first month of life. The tissue diagnosis revealed four cases of Burkitt's lymphoma and one case of T- lymphoblastic lymphoma. Central nervous system (CNS) sampling was also positive in the four cases of Burkitt's lymphoma. All patients were treated systemically for the lymphoma with chemotherapy. Complete remission was achieved in all cases post chemotherapy. Follow-up of 36.4 ± 18.9 months (10-61 months). CONCLUSION: This is the largest published case series of paediatric orbital lymphoma. We described a patient diagnosed within the first month of life and we believe this to have developed intra-uterine. In this series, patients were younger, had more bilateral disease and had better outcome than previously described. This rare condition should be considered in any child with an orbital mass, at any age. When managed appropriately, good outcomes can be achieved.

Psychometric Validation of the FACE-Q Craniofacial Module for Facial Nerve Paralysis.

Background: Systematic reviews have identified the need for a patient-reported outcome measure for facial nerve paralysis (FNP). The aim of this study was to determine the psychometric properties of FACE-Q Craniofacial module scales when used in a combined sample of children and older adults with FNP. Methods: Data were collected between December 2016 and December 2019. We conducted qualitative interviews with children and adults with FNP. FACE-Q data were collected from patients aged 8 years and older with FNP. Rasch measurement theory analysis was used to examine the reliability and validity of the relevant scales in the FNP sample. Results: Twenty-five patients provided 2052 qualitative codes related to appearance, physical, psychological, and social function. Many patient concerns were common across age. The field-test sample included 235 patients aged 8-81 years. Of the 13 scales examined, all 122 items had ordered thresholds and good item fit to the Rasch model. For 12 scales, person separation index values were ≥0.79 and Cronbach's alpha values were ≥0.82. The 13th scale's reliability values were ≥0.71. Conclusion: The FACE-Q Craniofacial module scales described in this study can be used to collect and compare evidence-based outcome data from children and adults with FNP.

Investigating the presence of peer bias against ptosis in young children.

PURPOSE: To investigate the presence of peer bias directed at young children aged 3-6 years of age and the age at which this bias emerges. METHODS: Children (3-6 years) completed an individual testing session involving two tasks: (1) a paired photograph task examined the effect of the appearance of ptosis on children's preferences in two different social situations (toy sharing and birthday party); (2) a ranking task investigated whether different grades of ptosis are evaluated differently by children of different ages. RESULTS: A total of 217 children were included. The paired photograph task showed that with increasing age, subjects were less likely to invite children with ptosis to share a toy or attend their birthday party. Three-year-olds did not show a bias against peers with ptosis; however, 4- to 6-year-olds did chose significantly fewer children with an apparent ptosis across both social situations. The ranking task highlighted that 6-year-olds viewed a grade 3 ptosis significantly more negatively than 3- and 4-year-olds; however, there was no effect of age on the rankings given to other grades of ptosis. CONCLUSIONS: Bias against ptosis emerges at about 4 years of age and is still evident at 6 years.

Diplopia Following the Insertion of a Canalicular Bypass Tube: Etiology, Risk Factors, Management, and Outcomes.

PURPOSE: To report the etiology, management, and possible risk factors for diplopia after canalicular bypass surgery. METHODS: A multicenter retrospective, noncomparative case series of patients who developed diplopia following canalicular bypass surgery were assessed. RESULTS: Twenty-four cases of diplopia were identified across 12 institutions. Tubes were inserted as a primary procedure with external dacryocystorhinostomy (DCR) (1; 4%) or without DCR (10; 42%) or as a secondary procedure after external (8; 33%) or endonasal (5; 21%) DCR. Factors predisposing to local damage were noted in 17 (71%): these factors included preexisting autoimmune/inflammatory condition (7 cases), medial canthal tumor resection (5 cases), preoperative radiotherapy (2 cases), 2 drug treatments (topical and systemic), and 1 local surgery. Horizontal diplopia was due to restriction of abduction and first noted at a median of 3.5 months (mean: 17.8 months, range: 1 day to 112 months) and persisted in 23 (96%) cases with a mean restriction of -2, affecting primary gaze in 4 patients and activities of daily living in 13 (42%). Seventeen patients received various treatments: 10 were operated on resulting in cure in 1 and improvement in 9. A stable degree of diplopia persisted in all but one patient. CONCLUSIONS: Restriction of abduction causing horizontal diplopia is a rare complication with canalicular bypass surgery and a notably high proportion occurred after tube placement without DCR; carunculectomy was not ubiquitous. Although in some the diplopia may be improved with intervention, the chance of cure is low. This complication should probably be included during informed consent for canalicular bypass tubes.

The management of periorbital nodular fasciitis using intra-lesional triamcinolone: a case report and review of the literature.

Nodular fasciitis (NF) is a subcutaneous, nodular, pseudo-sarcomatous, fibroblastic proliferation. It is rarely reported in the periorbital region and the management approach is variable.Presented is an eight-year-old female with a three month history of a periorbital mass. Incisional biopsy histologically confirmed nodular fasciitis with a unique gene translocation. The lesion was treated primarily with one intra-lesional injection of triamcinolone acetonide. Four months post-injection, the lesion resolved completely. No recurrence was seen at 12-months follow-up post-injection. No side effects were noted.To our knowledge. this is the first reported use of intra-lesional triamcinolone acetonide as a first-line treatment in periorbital NF. We found this to be a safe and effective treatment, which can obviate the need for surgical excision in a cosmetically sensitive region.

Non-Traumatic Carotid Artery Dissection Following an Episode of Orbital Cellulitis: A Case Report.

Carotid artery dissection (CAD) is a haemorrhage into the arterial wall disrupting the intimal layers of the vessel. We present a case of a 16-year-old male with a non-traumatic spontaneous CAD. The patient presented with Horner's syndrome following an episode of orbital cellulitis secondary to sinusitis requiring sinus drainage surgery. Subsequent magnetic resonance imaging (MRI) revealed a CAD. The patient was treated with antiplatelet medication.

A Systematic Review of the Psychosocial Adjustment of Children and Adolescents with Facial Palsy: The Impact of Moebius Syndrome.

INTRODUCTION: Facial palsy is often associated with impaired facial function and altered appearance. However, the literature with regards to the psychological adjustment of children and adolescents with facial palsy has not been systematically reviewed to date. This paper aimed to review all published research with regards to psychosocial adjustment for children and adolescents with facial palsy. METHODS: MEDLINE, CINAHL, Embase, PsychInfo and AMED databases were searched and data was extracted with regards to participant characteristics, study methodology, outcome measures used, psychosocial adjustment and study quality. RESULTS: Five studies were eligible for inclusion, all of which investigated psychosocial adjustment in participants with Moebius syndrome, a form of congenital facial palsy. Many parents reported their children to have greater social difficulties than general population norms, with difficulties potentially increasing with age. Other areas of psychosocial adjustment, including behaviour, anxiety and depression, were found to be more comparable to the general population. DISCUSSION: Children and adolescents with Moebius syndrome may experience social difficulties. However, they also demonstrate areas of resilience. Further research including individuals with facial palsy of other aetiologies is required in order to determine the psychosocial adjustment of children and adolescents with facial palsy.

The psychosocial impact of facial palsy: A systematic review.

PURPOSE: Facial palsy is a condition which can lead to significant changes in facial function and appearance. People with facial palsy often report psychosocial difficulties, including withdrawal from social activities, anxiety, negative body image, and low mood. This paper aimed to review all published research investigating the psychosocial impact of facial palsy on adults. METHODS: A systematic search of MEDLINE, CINAHL, EMBASE, PsycINFO, and AMED databases was performed. The quality of included studies was assessed, and data were extracted with regard to characteristics of participants; study methodology and design; outcome measures used; and psychosocial outcomes. RESULTS: Twenty-seven studies met inclusion criteria. A high proportion of people with facial palsy reported clinically significant levels of anxiety and depression, with greater difficulties typically reported by females, compared to males. Other difficulties consistently reported include low quality of life, poor social function, and high levels of appearance-related distress. Objective severity of facial palsy was consistently shown to not be associated with anxiety or depression, with psychological factors instead likely mediating the relationship between the severity of facial palsy and psychosocial well-being. CONCLUSIONS: Irrespective of objective symptom severity, facial palsy has the potential to have a significant impact on psychosocial well-being and quality of life. The various methodological limitations of the included studies are discussed, along with clinical implications, including the need for greater access to psychological screening and interventions for people with facial palsy.

Orbital Compartment Syndrome: An Update With Review Of The Literature.

Orbital compartment syndrome (OCS) is a potentially blinding condition characterized by a rapid increase in intra-orbital pressure. OCS is most commonly seen in the context of intra-orbital hemorrhage secondary to either trauma or surgery. A review of the literature indicates that better visual outcomes are achieved when interventions occur within the first 2 hrs. There are reports of visual recovery after a delay in management and consideration should be given to intervention even when presentation is delayed. Reported interventions include: lateral canthotomy with cantholysis, bony orbital decompression and treatment of the underlying cause.

Early low-dose rituximab for active thyroid eye disease: An effective and well-tolerated treatment.

BACKGROUND: Thyroid eye disease (TED) is an autoimmune inflammatory disease that can be disfiguring and potentially sight threatening. Suppression of inflammation in active disease can reduce the risk of visual loss and limit long-term sequelae. Current management involves inflammation suppression using glucocorticoids. The aim of this study was to evaluate the efficacy of early disease intervention with targeted immunomodulatory therapy to alter disease course. This paper reports the efficacy of low-dose rituximab in reducing clinical activity in TED in a small population. METHODS: A retrospective audit of consecutive patients with active TED managed primarily with a 100 mg rituximab infusion. Further glucocorticoid or steroid-sparing agents were prescribed if clinically indicated. Clinical activity score, VISA overall severity score and Oxford Quality of Life score were recorded at each visit as well as TSH receptor antibody levels (TRAb), B cell subsets and adverse reactions. RESULTS: Twelve patients had mean follow-up of 6.3 months. Clinical activity scores significantly decreased (mean score 5.08 to 1.58; P < 0.001), VISA overall severity scores reduced by 50% from 12 to 6, P < 0.001 and the mean cumulative dose of IV methylprednisolone was 2.3 g. 100 mg rituximab induced significant CD19+ B cell depletion (n = 8, P < 0.001). There was no significant reduction in serum TRAb (n = 8, P = 0.06). A transient infusion-related rash was the only adverse effect, n = 4. QoL scores did not differ markedly before and after treatment. CONCLUSION: Low-dose rituximab is an efficacious, well-tolerated and safe treatment for active TED; reducing disease activity and allowing reduced administration of systemic steroid.

Exploring Patient Experience of Facial Nerve Palsy to Inform the Development of a PROM.

BACKGROUND: There is currently a mandate globally to incorporate patient's perceptions of their illness into outcome measures, in order to provide a deeper insight into medical practice. Facial nerve palsy (FNP) is a devastating condition that can significantly impact quality of life. However, no measure currently exists that comprehensively assesses outcome in FNP using patient perception. The aim of this study is to explore patients' experiences of FNP with the aim of informing the development of a patient-reported outcome measure. METHODS: Presented is a qualitative study, using in-depth semi-structured interviews with FNP patients. An interview guide was developed using expert opinion and a literature review. Interpretative description was used as the qualitative approach. Interviews were audio-recorded, transcribed, and coded line-by-line. Codes were refined using the constant comparison approach. Interviews continued until data saturation was reached. The data were used to develop a conceptual framework of patient perceived issues relating to FNP. RESULTS: The sample included 5 men and 9 women aged 57.7 years (range, 36-78) with a range of causes of FNP, including Bell's palsy (n = 5), acoustic neuroma (n = 3), trauma (n = 2), meningioma (n = 1), muscular dystrophy (n = 1), congenital (n = 1), and Ramsay Hunt syndrome (n = 1). Analysis of the 14 participant interviews led to identification of 5 major domains including "facial function concerns," "appearance concerns," "psychological function," "social function," and "experience of care." CONCLUSION: This study provides a conceptual framework covering outcomes that matter to patients with FNP, which can be used to inform the development of a new comprehensive FNP-specific patient-reported outcome measure.

The kissing puncta: an under-reported and stubborn cause of epiphora.

BACKGROUND: 'Kissing puncta' (KP) or punctal apposition is an anatomical phenomenon sparsely reported in the English literature. We describe our experience of managing chronic epiphora in patients with punctal apposition. METHODS: A retrospective audit of five patients (nine eyes) with KP associated with epiphora. Data including: presenting symptoms, physical signs and surgical outcomes were collected. RESULTS: Five patients aged between 66 and 77 years were reviewed. Common clinical features were: chronic epiphora, involutional eyelid laxity, kissing puncta (present at all phases of the blink) and reduced upper and lower margin-reflex distances. Medial upper eyelid ptosis with orbital fat prolapse was a prominent feature. Four patients (nine eyes underwent eyelid-tightening surgery to restore normal anatomical position of the puncta. Only one of the four patients achieved improvement in epiphora at 3 months. One patient with continued epiphora underwent subsequent dacrocystorhinostomy with improvement in symptoms. The fifth patient had mild laxity and underwent dacrocystorhinostomy at first instance, with no improvement in symptoms, despite surgical success. CONCLUSIONS: The KP sign is commonly found in those with involutional eyelid changes. Epiphora is present in variable degrees in the presence of punctal apposition. Restoration of normal punctal position with eyelid-tightening surgery does not always confer an improvement in epiphora. Surgical management in the setting of KP is therefore challenging with a guarded prognosis. Symptomatic patients with KP should be counselled accordingly.

Periocular Manifestations of Afatinib Therapy.

Epidermal growth factor receptor tyrosine kinase inhibitor therapy has been increasingly employed in the treatment of a variety of tumors. The authors report the rarely documented side effect of trichiasis with the use of Afatinib in a patient with spinal chordoma and review-related literature. A 67-year-old lady was referred to the oculoplastic service with a 3-month history of ocular irritation and pain associated with blurred vision. She has a 4-year history of spinal chordoma treated with daily Afatinib 50 mg over the past 6 months. Clinical examination revealed trichomegaly and trichiasis affecting all 4 eyelids associated with blepharitis, conjunctival, and corneal abrasion on fluorescein staining. Hypertrichosis of the eyebrow bilaterally was also present. Afatinib and other epidermal growth factor receptor tyrosine kinase inhibitor-associated changes to eyelash and eyebrow hair is a result of epidermal growth factor receptor pathway activation in keratinocytes results in remodeling of the hair follicle. This results in the variation in the severity of clinical presentation of trichiasis.

Early use of steroid-sparing agents in the inactivation of moderate-to-severe active thyroid eye disease: a step-down approach.

OBJECTIVES: The current first-line treatment for management of active thyroid eye disease (TED) is high-dose intravenous corticosteroids, which have the potential for serious adverse effects. Our aim was to evaluate the effect of steroid-sparing agents (SSAs) in patients with moderate-to-severe active TED, using methotrexate as first-line. METHODS: Presented is a retrospective, four-year, single-centre, consecutive case series of patients with moderate-to-severe TED treated using the Oxford protocol. Treatment modality, disease activity, and adverse effects are reported at presentation, 6- and 12-month follow-up. RESULTS: 104 consecutive TED patients treated by the Oxford TED team were reviewed. 24 patients with moderate-to-severe active disease were identified (mean age 46.8 years;12 female) with a mean pretreatment VISA inflammatory index score of 5.5/10 (SD = 1.98; range 1-9). Intravenous methyl-prednisolone (IVMP) and an SSA was commenced in all patients. Mean total steroid dose was 2.72 g (SD = 1.4;1.0-6.9). 38% of patients (n = 9) received ≤1.5 g of IVMP. Only two patients required >4.5 g of IVMP equating to the EUGOGO treatment protocol dose for this patient group. There was significant improvement in inflammatory index score both at the intermediate review (mean score 2.7; SD = 2.8; P < 0.001; mean follow up 25.2 weeks) and at one year or last follow-up (mean score 1.4; SD = 1.5; P < 0.001; mean follow up 48.0 weeks). No serious or long-term adverse effects were reported. CONCLUSION: This study suggests that the initiation of an SSA, using methotrexate as first-line, with limited adjuvant IVMP is an effective and safe treatment for moderate-to-severely active TED, resulting in a significant reduction in both disease activity and total steroid load.

The Nonsurgical Management of Orbital Dystopia Using Refractive Lenses and Prosthetic Shells.

Surgical rehabilitation of orbital dystopia can be challenging. The authors demonstrate the effective use of spectacle lenses to visually correct misalignments of the globe and the orbit. Presented is a retrospective review of 4 patients undergoing aesthetic rehabilitation through use of spectacle lenses and in a number patients a cosmetic shell.Two patients with neurofibromatosis presented with inferior dystopia of the globe and orbit. A base-down prismatic lens applied to the spectacles in conjunction with a prosthetic shell successfully visually corrected the facial asymmetry and improved patients' aesthetic appearance. One patient with a history of traumatic retinal detachment, who did not want any surgical intervention, a "plus" (hypermetropic) lens was used to magnify the perceived image of an enophthalmic and phthisical globe, to enhance appearance and improve symmetry. In the fourth patient, with Goldenhar syndrome, the appearance of a hypotropia and concurrent esotropia was successfully treated with a Fresnel prism and a prosthetic shell.This case series illustrates the successful role of various refractive lenses often in conjunction with prosthetic shells in patients with reduced vision and orbital dystopia to improve facial symmetry. This conservative treatment is especially useful when surgery is not a desired or not considered a suitable option for the patient.

A quality of life survey in patients with long-term silicone oil or phthisis bulbi.

AIM: The aim of this study is to determine whether there is any difference in the quality of life of patients with a blind eye with long-term silicone oil compared to without. METHOD: Patients with either long-term silicone oil in situ (N = 17), defined as a period greater than 6 months duration with no plan for future removal, or those with a phthisical, non oil-filled eye were identified (N = 13). Two validated questionnaires (NEI VFQ-25 and the FACE-Q) that cover indicators for visual function, pain and cosmesis were sent to all patients in the two cohorts. RESULTS: There was no significant difference found in quality of life outcomes between the two groups in terms of visual function, pain or cosmesis. CONCLUSION: The results of this study support a holistic approach to the consent process before vitreoretinal surgery. Patients that may need to undergo multiple vitreoretinal procedures, where the endstage result is a long-term silicone oil fill, should be informed that their functional outcome may be similar to having no surgical intervention.