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.

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.

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.

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.

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.

Reconstruction of extensive medial canthal defects using a single V-Y, island pedicle flap.

The medial canthus represents one of the most challenging regions of the face to reconstruct due to the anatomical structures present, the concavity of the area, and the differences in skin texture. We present a case series of 11 patients whose defects were reconstructed with a single V-Y island pedicle flap running along the nasofacial sulcus. Our single-stage flap which modified and simplified a previously described technique achieves similar cosmetic and postoperative outcomes along with a high level of patient satisfaction in an area which can be reconstructed in a variety of ways, often with suboptimal results.

The "reading man" flap for reconstruction of large periorbital defects.

PURPOSE: The "reading man" flap (RMF), a double advancement transposition cutaneous flap named for its appearance, has been described in the reconstruction of various circular skin defects, particularly in the malar region. We describe two cases where this flap was used to reconstruct the lower eyelid/periorbital region. METHODS: Two oculoplastic patients with lower eyelid basal cell carcinomas underwent Mohs micrographic excision resulting in a large skin defects. In both cases, reconstruction was performed using the RMF. RESULTS: At 6 months, both patients achieved good cosmetic results with no case of secondary eyelid malposition. CONCLUSION: The RMF is a useful adjunct to the armamentarium of the oculoplastic surgeon for the reconstruction of large periorbital defects.

Acute Sickle Cell Orbitopathy Masquerading as Orbital Cellulitis.

Sickle cell orbitopathy is a rare manifestation of a sickle cell crisis and can closely mimic orbital cellulitis, both clinically and radiologically. We present a case where a child presented with what was thought to be orbital cellulitis and was treated accordingly. Subtle radiologic features of sickle cell orbitopathy were initially overlooked, resulting in an incorrect diagnosis and a treatment delay for the patient. Correctly treated most cases resolve with no adverse affects, highlighting how important it is to consider acute sickle cell orbitopathy in the differential diagnosis of acute periorbital swelling in children with sickle disease.

Ectopic orbital meningioma: Fact or fiction?

Primary intraorbital ectopic meningiomas are rare and their existence remains controversial. We present a 30-year-old female with painless, non-axial proptosis and a palpable superomedial mass. The MRI demonstrated that the mass had no optic nerve sheath or sphenoid wing involvement and was initially reported to have no intracranial extension. The patient was initially thought to have an ectopic orbital meningioma. Subsequent multidisciplinary team (MDT) consultation and further specialist review of the MRI revealed a subtle dural tail connecting to an enhancing mass in the olfactory groove. Biopsy revealed a WHO Grade 1 transitional meningioma with an infiltrative pattern. We argue that some previously reported cases of ectopic meningioma may lack the requisite imaging to discover the primary disease. Our report highlights the importance of MRI in this group of patients and the role of a skull-base MDT with specialist neuroradiology input to determine the true origin and extent of these extradural orbital meningiomas.

IgG4-related disease presenting as posterior scleritis and vitritis, progressing to multifocal orbital involvement.

IgG4-related disease (IgG4-RD) is a rare, chronic inflammatory condition that may involve nearly every organ system. Originally identified as a cause of autoimmune pancreatitis, its characteristic histological and clinical features have been found in a wide variety of inflammatory presentations, including the eye and orbit. Here we describe an example of a case of IgG4-RD initially presenting as scleritis and vitritis, with further progression to multifocal bilateral orbital involvement. Tissue biopsy of an orbital mass was highly characteristic of IgG4-RD histology and a rapid clinical response to corticosteroids was observed. This case highlights IgG4-RD as a rare cause of intraocular inflammation that may progress to involve the orbit.

Review on surgical management of ptosis and the use of phenylephrine: A national survey of British Oculoplastic Surgery Society (BOPSS) UK Consultants.

We assess current practice using topical phenylephrine by British Oculoplastic Surgery Society (BOPSS) consultants in the surgical management of ptosis. All UK consultant BOPSS members were invited to participate in a web-based survey, consisting of 8 questions relating to the surgical management of adult primary involutional ptosis with normal levator function and the use of phenylephrine in the management of ptosis. 53 BOPSS consultants (43%) completed the survey, of which 76% perform anterior approach levator advancement as first-line surgery. Then, 40% of consultants routinely use phenylephrine unilaterally in the ptotic eye, with 90% using 2.5% as opposed to 10%. Also, 77% of consultants use topical phenylephrine to illustrate the predicted outcome of surgery for the patient's benefit and 65% modify their approach on the basis of the test. If phenylephrine raises the ptotic eyelid >2 mm, those using an anterior approach reduces to 13.6%, with majority using a posterior approach (86.4%). If phenylephrine induces no improvement, then 76% use an anterior approach. If phenylephrine induces a contralateral ptosis 79% of consultants will perform simultaneous bilateral surgery. A number of interesting trends were observed amongst BOPSS consultants in their surgical approach to ptosis based on the phenylephrine test. The majority of consultants will switch from anterior to posterior approach surgery when the phenylephrine test is strongly positive and will also perform bilateral surgery when a contralateral ptosis is induced with phenylephrine.

Key Issues When Reconstructing Extensive Upper Eyelid Defects With Description of a Dynamic, Frontalis Turnover Flap.

Reconstruction of full thickness, total upper eyelid defects represents a significant challenge in terms of recreating an upper eyelid which has acceptable cosmesis and a degree of dynamic function. Options include bridging, eyelid-sharing techniques (e.g., Cutler-Beard), or nonbridging techniques such as an anterior lamella-based flap combined with a posterior lamella free graft or a "sandwich flap." The success of these techniques depends on the size of the defect, postoperative cosmesis and whether or not the upper eyelid still has a degree of dynamic function to avoid ptosis and exposure keratopathy. The authors present an innervated frontalis turnover flap supporting anterior and posterior lamella grafts as a reconstructive solution for an extensive upper eyelid defect. This technique is cosmetically acceptable, preserves local tissues, and maintains a degree of dynamic function, which keeps the patient's eye comfortable and does not adversely affect visual acuity.