Oedipism: An unusual case of auto-enucleation including mechanism of avulsion.

INTRODUCTION: Self-inflicted enucleation, also known as auto-enucleation (AE) or Oedipism, is an uncommon and severe form of ocular injury which presents as an ophthalmic and psychiatric emergency. Usually known to occur with untreated psychosis, this case is a rare report which demonstrates AE as a result of a subsequently diagnosed drug induced psychosis. We report the clinical presentation, management and for the first time a detailed speculative account about the mechanism of AE, based on our clinicopathologic findings. CASE REPORT: A 53-year old Afro-Caribbean patient was arrested following an altercation and was incarcerated awaiting arraignment. The patient had no previous psychiatric history but tested positive for cannabis, opiates and cocaine as well as admitting to illicit drug use in the community. Whilst in custody, the patient self-enucleated his right eye. The patient declined consent to eye examination and was subsequently admitted under section 2 of the Mental Health Act. After full work-up including Goldmann visual fields and magnetic resonance imaging, he underwent right orbital exploration under anesthetic where AE was confirmed whilst the left eye showed evidence of attempted enucleation. The residual tenons and conjunctiva was subsequently repaired without placement of an orbital implant in the right orbit. The globe was sent for histology which revealed clues to the potential mechanism of auto-enucleation. CONCLUSION: This case is unique as it offers an alternative presentation to those most commonly reported in the current literature, highlights the sparsity of literature detailing the mechanism of AE and stimulates discussion around various potential systemic etiological differential diagnoses, management strategies and complications of AE.

Periocular cutaneous sarcoid: case series and review of the literature.

OBJECTIVES: To illustrate the varying clinical presentations of cutaneous sarcoidosis affecting the periocular region, which may masquerade as other clinical entities such as basal cell carcinoma or seborrheic dermatitis. Furthermore, the authors present an unusual observation of lupus pernio involving the adnexal region with the rare presence of perineural granulomas on histology following incisional biopsy. METHODS: We report a consecutive series of four cases with lesions involving the eyelids with varying clinical appearances. All four patients presented to our adnexal service undergoing incisional diagnostic biopsy. Histology following biopsy subsequently resulted in further investigation and management of both local cutaneous lesions and systemic sarcoidosis. RESULTS: Three of our four cases had evidence of pulmonary involvement on chest X-ray. Over an 18-month period, one of two patients responded to intralesional triamcinolone and subsequently to oral methotrexate (15 mg/week). Two patients were observed with their periocular lesions remaining stable without therapy. CONCLUSIONS: All four patients presented to the adnexal service with lesions of varying morphology and were diagnosed with sarcoidosis following incisional biopsy highlighting the vital role of oculoplastic surgeons in diagnosing this multisystem inflammatory disease. We describe our experience of intralesional triamcinolone, oral methotrexate and watchful observation in the management of such lesions.

Squamous cell carcinoma in the anophthalmic socket: a series of four cases with HPV-16 profiling.

PURPOSE: To present the clinical and histological features of squamous cell carcinoma (SCC) in the anophthalmic socket in four adult patients, and to determine the presence of human papillomavirus infection (HPV). METHODS: Retrospective case series of four adult patients with SCC of the anophthalmic socket. P16 immunohistochemistry and HPV testing was carried out in all cases. The authors report clinical findings, histopathology, management and outcomes for all four patients with conjunctival SCC. Previously reported cases of conjunctival SCC in anophthalmic sockets were reviewed. RESULTS: Four adult patients presented with eyelid lumps, discharge or change in prosthesis fit. Common examination findings included papillomatous changes, eyelid masses and epithelial changes. Three out of the four cases (75%) were positive for p16 by immunohistochemistry and the same cases positive for HPV-16 DNA. All patients received cryotherapy, topical or intralesional chemotherapy. Two patients proceeded to exenteration for control of invasive disease. CONCLUSIONS: To the authors' knowledge, this is the largest series of SCC in the anophthalmic socket with comprehensive annotation of HPV status. Although socket conjunctiva is protected from environmental radiation, there is still a risk of neoplastic transformation in this tissue, thus patient education and regular checking of sockets by ophthalmologists should be undertaken as a preventative measure. The potential role of HPV in these tumours warrants further investigation.

Spindle Cell Lipoma of the Conjunctiva.

A 67-year-old woman presented with progressive enlargement of a long-standing mass on the surface of her OS associated with ocular surface irritation. The mass was excised en bloc. Histopathological examination showed a well-defined encapsulated tumor composed of wiry collagen containing bland spindle cells that were strongly positive for CD34 with scattered mature adipocytes. These features confirmed a diagnosis of spindle cell lipoma.

Post-radioiodine De Novo Onset Graves' Ophthalmopathy: Case Reports and a Review of the Literature.

New-onset Graves' ophthalmopathy (GO) following radioiodine treatment (RAI) and worsening of existing GO are well-described in the endocrinology literature. These phenomena are recognized by ophthalmologists, yet poorly documented in the ophthalmology literature. Two male patients, aged 43 and 62 years, respectively, with Graves' disease without GO, received RAI. Four months later, one patient developed acute GO with unilateral reduction in visual acuity, conjunctival chemosis, lagophthalmos, bilateral severely restricted ocular motility, and lid retraction. High-dose intravenous steroids, followed by oral steroids, led to a dramatic clinical improvement. The second patient received a second dose of RAI for persistent hyperthyroidism and subsequently developed acute GO-comprising restricted ocular motility, peri-orbital swelling, and conjunctival chemosis. Symptoms gradually resolved on continued carbimazole treatment. Neither patient received pre-RAI prophylactic glucocorticoids, as currently they are only recommended for patients with pre-existing GO or multiple risk factors. We discuss the limitations of using this risk-based approach in preventing new-onset GO following RAI therapy.

Orbital presentation of a nasal midline destructive lesion in a young boy.

Midline Destructive Lesions (MDL) are well known to cause nasal problems. There is a long differential diagnosis of such lesions. However, in the pediatric population, the 2 main diseases to be aware of are Non-Hodgkin's T-cell lymphoma and granulomatosis with polyangiitis (previously known as Wegener's granulomatosis). The authors present the report of a 15-year-old boy who presented with epiphora, chemosis, and limitation of left abduction. CT scan of his orbits suggested a destructive lesion of the ethmoid sinuses. His laboratory investigations revealed a positive ANCA. The patient underwent endoscopic sinus surgery, and this was characteristic for granulomatosis with polyangiitis. He was treated with systemic steroids and then maintained on cyclophosphamide, which controlled his disease activity. This case highlights the need for ophthalmologists to have a high index of suspicion for MDL and concomitant orbital disease.

A prospective, multicentre study of malignant and premalignant lesions at the base of periocular cutaneous horns.

PURPOSE: The primary objective of the study was to determine the incidence of the various pathological conditions present at the base of the periocular cutaneous horns. The secondary objective was to study the presentation of these cutaneous horns with a view to finding any clinical indicators for premalignant, malignant and benign lesions at the base. METHODS: Prospective multicentre study of patients presenting with cutaneous horns. Informed consent followed by excision biopsy of the base lesion was performed in all the cases included for the study. The biopsy specimens were examined histologically and results analysed. RESULTS: Twenty-six patients presented with cutaneous horns in the periocular region over a period of 2 years, of these two patients presented with more than one cutaneous horn. 23 patients underwent excision biopsy. There were a total of 25 specimens. Of the base lesions 2/25 (8%) were malignant, 7/25 (28%) were pre-malignant and the remaining 16/25 (64%) were benign. Analysis of the findings on clinical presentation revealed no clinical indicators to correlate with the final histology. CONCLUSION: The incidence of malignant and pre-malignant lesions present at the base of periocular cutaneous horns was 36% with 8% of them being malignant. It is important to perform an excision biopsy with histological diagnosis of the base of the cutaneous horn as there are no definite clinical features that point towards a potential for malignancy.

Auditory-olfactory synesthesia coexisting with auditory-visual synesthesia.

Synesthesia is an unusual condition in which stimulation of one sensory modality causes an experience in another sensory modality or when a sensation in one sensory modality causes another sensation within the same modality. We describe a previously unreported association of auditory-olfactory synesthesia coexisting with auditory-visual synesthesia. Given that many types of synesthesias involve vision, it is important that the clinician provide these patients with the necessary information and support that is available.

A unique case of IgG4 sclerosing dacryocystitis.

IgG4-related ocular adnexal disease, a relatively recently described clinical entity, is important to diagnose because during the acute phase, it responds favorably to corticosteroid treatment. The diagnosis can be confirmed by simple blood tests and histology. IgG4-related dacryoadenitis and generalized orbital disease have been reported; however, this is the first report of IgG4-related disease of the lacrimal sac. We describe an 80-year-old female who presented with a painless erythematous indurated swelling in the right lacrimal sac area with complete obstruction of the right nasolacrimal system. A 9-mm lacrimal sac mass was noted on CT dacryocystogram. Blood tests revealed an elevated serum IgG4 level, and the lacrimal sac histology was characteristic of IgG4-related disease. Corticosteroid treatment was declined by the patient. She was kept under close observation for signs of progression, systemic involvement, and potential malignant transformation.

Topical diclofenac gel for the management of periocular actinic keratosis.

PURPOSE: We sought to evaluate the efficacy of topical 3% diclofenac gel in the management of periocular actinic keratosis. METHODS: Four consecutive patients with periocular actinic keratosis who were treated with topical 3% diclofenac gel twice daily for up to 4 months were included in the study. Patients were reviewed at 1 and 2 months and then as clinically required up to 13 months after treatment. RESULTS: All 4 patients had biopsy-proven actinic keratosis. Three of the 4 patients tolerated the therapy well. In 1 patient, an adverse reaction developed, with erythema, crusting, and scaling after 2 months of therapy, which resolved within a month of discontinuing topical diclofenac. In all the patients, a visible decrease in lesion severity was seen after 1 month, and complete resolution, within 4 months. Recurrences occurred in 2 patients at 4 months and 7 months after treatment. These were successfully managed, in one patient, by excision, and in the other, with further 3% diclofenac gel followed by cryotherapy. CONCLUSION: The use of 3% diclofenac gel in extraocular sites has been widely described in the dermatology literature. However, no published reports exist of its use in ophthalmology. It may be a useful therapeutic option when used alone in patients with localized disease or to reduce the size of larger, more-diffuse lesions before excision or cryotherapy, leading to improved cosmesis. Furthermore, it may also be useful in patients prone to actinic keratoses, such as organ-transplant recipients, to reduce the need for multiple excision procedures or uncomfortable ablative techniques.

Xanthogranulomatous variant of immunoglobulin G4 sclerosing disease presenting as ptosis, proptosis and eyelid skin plaques.

A 70-year-old male was referred to the oculoplastic clinic with left-sided ptosis and floppy eyelids. During follow-up, bilateral upper lid xanthelasma developed with worsening ptosis and proptosis, which was worse on the left side. A left orbital biopsy showed xanthogranulomatous inflammation of the orbit. The patient was treated with a variety of immune modulator regimes but due to a variety of side-effects, treatment was discontinued. The left orbit was surgically debulked twice and histology revealed xanthogranulomatous inflammation, with the additional features of sclerosis, lymphoid aggregates and a prominent population of plasma cells. Around 80% of the plasma cells expressed immunoglobulin G4 (IgG4). This case report reveals an association between xanthogranulomatous inflammation of the orbit and a prominent population of IgG4-positive plasma cells. We propose that the overall disease is a novel variant of IgG4 sclerosing disease of the orbit and suggest that cases of histologically proven xanthogranulomatous inflammation should be stained for IgG4 if there is an accompanying plasma cell population.

A rare case of bilateral eyelid ulceration with mycosis fungoides.

Mycosis fungoides (MF), the most common type of cutaneous T-cell lymphoma, is a low-grade lymphoma usually occurring after the age of 50 years. We present a case of a 60-year-old man with cutaneous manifestations of MF, who later developed bilateral eyelid ulceration. MF is a highly recalcitrant disease with a relentless course. Our patient highlights a delayed presentation in MF with bilateral upper lid ulcerative lesions, which responded well to systemic chemotherapy, commenced for a visceral indication.

Successful treatment of nasolacrimal duct obstruction secondary to fibrous dysplasia by external dacryocystorhinostomy with mitomycin C.

A 60-year-old woman developed polyostotic fibrous dysplasia involving the right zygomatic and maxillary bones, which led to swelling along the right side of her nose that was associated with constant epiphora. A sac washout revealed a blocked right nasolacrimal duct, which was confirmed by CT dacryocystography. CT also showed signs of fibrous dysplasia in the maxillary and zygomatic bones in the form of bony expansion and ground-glass bone density. The patient underwent external dacryocystorhinostomy with mitomycin C and O'Donoghue tube insertion. Bone punched out while fashioning the bony nasal ostium was subjected to histopathologic examination, which confirmed the diagnosis of fibrous dysplasia. The O'Donoghue tubes were removed 8 weeks later. At 15 months postoperatively, the dacryocystorhinostomy was patent and the patient was completely relieved of her symptoms.

A histopathologic study of orbital implant vascularization.

PURPOSE: To histopathologically assess the extent and pattern of vascularization of explanted porous polyethylene (PP) and hyrdoxyapatite (HA) orbital implants. To compare the vascularization in PP implants harvested after enucleation versus after evisceration. METHODS: This is a comparative case series of six orbital implants explanted between 11 months and 5 years and 4 months post implantation. The implants were subjected to histopathological examination with various stains, after complete decalcification. RESULTS: There were 2 post evisceration, 3 post enucleation and 1 secondary implant. The size of the implants varied from 18 to 23 mm diameter. The reason for explantation was exposure in all the 6 cases with additional infection in 2 cases. Histopathology revealed complete vascularization up to the core of the implant in all the cases. There was evidence of chronic inflammation within all the explanted specimens. Acute inflammation was present at the site of exposure in four of the implants. Three of these implants had additional evidence of necrosis. Two cases with clinically evident infection had Gram positive cocci within the implant. Foreign-body type giant-cells, melanophages and haemosiderin laden macrophages were also observed. Eviscerated specimens showed scleral remodelling and thickening. CONCLUSION: All the implants were vascularized up to the core. There was histologic evidence of chronic inflammation in all the explanted implants possibly indicating the foreign nature of the implant material. The sclera did not impede vascularization of the implants in eviscerated specimens. We found no difference in the degree of vascularization of the implant in enucleated versus eviscerated implants.

Soft contact lens use for upper eyelid surgery.

PURPOSE: To evaluate the usefulness of silicone hydrogel soft contact lenses in upper eyelid procedures. METHODS: Thirteen patients (14 eyes) who had full thickness upper lid reconstruction were studied. PureVision contact lenses were placed on the eyes at the commencement of the surgery. To evaluate the effectiveness of soft contact lens use, we looked at comfort, conjunctival injection, chemosis, corneal abrasion, lid oedema, evidence of infection and speed of healing. RESULTS: Nine eyes (64.29 per cent) were entirely asymptomatic. Three eyes developed corneal abrasions and changing the contact lenses led to resolution of the symptoms. One eye developed cloudy vision at one week due to accumulation of discharge behind the contact lens and another eye had foreign body sensation. Both had immediate relief with contact lenses removal. Mean duration of contact lens wear was 19.5 days (range, seven to 35 days). There were no cases of chemosis, infection, unexplained post-operative inflammation or delayed wound healing. CONCLUSIONS: Despite its limitations, our study suggests that a soft contact lens may be a useful option in upper lid surgery.

The use of AlloDerm in the reconstruction of full-thickness eyelid defects.

PURPOSE: To demonstrate the potential for the use of AlloDerm as a posterior lamellar graft in the reconstruction of full-thickness lid defects. METHOD: In our case series, we evaluated the surgical outcome of three consecutive patients, two with an upper eyelid defect and one with a lower eyelid defect who underwent lid reconstruction using AlloDerm grafts. RESULTS: AlloDerm was readily taken up into the wound defect, with complete coverage of its bulbar surface by conjunctiva. In all the cases, the cornea was not affected by its contact to the AlloDerm. A mucocutaneous junction formed over the margin of the AlloDerm graft with good cosmesis. CONCLUSION: AlloDerm has the potential to act as an effective posterior lamellar substitute in situations where there is an adequate amount of skin muscle cover available to drape over it. It is rigid enough to replace tarsus and its structure behaves as a scaffold allowing conjunctiva to readily grow over it.