Cutaneous pleomorphic adenoma of the periocular region - a case series.

Mixed tumour of the skin is a rare entity also known as chondroid syringoma and pleomorphic adenoma. These usually present as slow-growing skin nodules with a smooth surface, clear boundaries, and no ulceration. Case series exist describing pleomorphic adenomas in the periocular region including the lids and orbit, separate to the more familiar lacrimal gland pleomorphic adenoma. These may arise from accessory or ectopic lacrimal gland tissue but in the eyelids are more likely to arise from sweat glands in the skin. Histopathological analysis of these lesions is important to identify complete excision, minimising recurrences and in identifying rare but potential malignant transformation. We describe the clinical features and outcomes in three cases of pleomorphic adenoma with two at the medial canthus (including one recurrence) and one in the brow region.

Current practice trends for lacrimal gland neurotoxin in the management of epiphora-a BOPSS survey.

PURPOSE: Injection of botulinum neurotoxin A (BoNTA) to the lacrimal gland (LG) offers a simple and effective treatment in the management of epiphora. However, there is little data on current practice trends or uptake as an alternative to surgery. This study assesses current practice trends of such treatment amongst BOPSS (British Oculoplastic Surgery Society) members. METHODS: All consultant BOPSS members were invited to participate in a web-based survey which consisted of 5 questions, with a reminder invitation to participate. The role, dose, potential side effects, use as an alternative to surgical intervention, and impact on service delivery were assessed. RESULTS: Fifty-one BOPSS consultants (43% uptake) completed the survey. Ninety percent of respondents were regularly using LG BoNTA in their management of epiphora. The main indicators for considering BoNTA use were medical comorbidities and elderly patients. The mean first treatment dose of Botox® was 3.6 units (SD 1.5). Diplopia and ptosis complications were always discussed in the consent for treatment in addition to dry eye. Twenty-five percent of surgeons reported doing less conjunctivo-dacryocystorhinostomies (cDCR) due to the availability of LG BoNTA. No respondents felt that the requirement for repeated BoNTA treatments was impacting on their service delivery. CONCLUSION: Uptake of LG BoNTA in the management of epiphora is at a similar rate to all other available treatments. As a result, respondents are performing less surgical procedures, particularly cDCR in patients at higher surgical morbidity.

Prostaglandin analogues: A double-edged sword in orbital glaucoma disease management.

INTRODUCTION: The authors describe benefits of the recognised adverse effects of prostaglandin analogues on periocular structures in patients with unilateral proptosis and intraocular pressure rise. This case points to intentional consideration of prostaglandin analogue therapy in this selected cohort of patients with secondary ocular hypertension and proptosis. CASE DESCRIPTION: A 70-year-old gentleman who presented with a 1-week history of a red and painful right eye associated with tortuous and dilated episcleral blood vessels. Visual acuity was unaffected. A diagnosis of idiopathic orbital inflammatory disease was made by extraocular muscle biopsy. Two weeks later, the patient presented with worsening pain, reduced vision and raised intraocular pressure. The secondary ocular hypertension was successfully treated with topical preserved eye drops, including latanoprost, a prostaglandin analogue. Over 6 months, the patient developed drop intolerance and punctate keratopathy leading to therapy non-adherence. Interestingly, the patient reported improvement in periocular appearance related to prostaglandin-associated periorbitopathy. Ocular surface disease and intraocular pressures were subsequently managed with preservative-free eye drops. CONCLUSION: Secondary ocular hypertension is not an uncommon consequence of orbital disease. Prostaglandin analogue can act as a double-edged sword in the management of raised intraocular pressure by reducing eye pressure at the cost of developing adverse effects of prostaglandin-associated periorbitopathy. These adverse effects however can be beneficial in the aesthetic rehabilitation of proptosis and associated exposure keratopathy in patients with unilateral orbital disease and probably should be sought as first line treatment in those with proptosis and raised intraocular pressure.

Incidence of canalicular closure with endonasal dacryocystorhinostomy without intubation in primary nasolacrimal duct obstruction.

PURPOSE: To describe the incidence of canalicular closure with powered endonasal dacryocystorhinostomy (DCR) without canalicular intubation in primary acquired nasolacrimal duct obstruction (PANDO). DESIGN: A single-surgeon, prospective, nonrandomized, noncomparative, interventional case series. PARTICIPANTS: Consecutive patients attending a specialist clinic of an oculoplastic surgeon (DS) with radiologically confirmed diagnosis of PANDO. Cases of canalicular disease were excluded. METHODS: Patients with radiologically confirmed PANDO without canalicular involvement underwent endonasal DCR without intubation. The operation was performed by 1 surgeon (DS) and follow-up was at 4 weeks and 12 months. MAIN OUTCOME MEASURES: Outcomes were recorded as subjective symptomatic relief at 12 months and endoscopic evidence of ostium patency and canalicular patency. RESULTS: There were 132 cases that fulfilled the inclusion criteria. Three cases were lost to follow-up. Preoperatively, 96.3% of cases had Munk scores of >2. Of the 129 cases, 127 (98.5%) had endoscopic evidence of a patent ostium with a positive endoscopic dye test at the 12-month follow-up. All cases had a patent canalicular system as demonstrated by syringing and probing. Of the 129 cases, 117 (90.7%) had subjective improvement of epiphora at 12 months with 88.4% of cases reporting Munk scores of ≤1. CONCLUSIONS: In this prospective series of nonintubation for PANDO, there were no cases of canalicular closure or stenosis at 12 months. Anatomic and functional success was similar to reported outcomes for DCR with intubation for PANDO. We advocate that routine intubation for the purpose of maintaining canalicular patency is not necessary when performing endonasal DCR in PANDO. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Endoscopic transcaruncular repair of large medial orbital wall fractures near the orbital apex.

PURPOSE: To study the suitability of an endoscopic transcaruncular approach (ETA) for repair of large medial orbital wall fractures (MOWFs) near the orbital apex. DESIGN: A retrospective, noncomparative case series with description of the surgical technique. PARTICIPANTS: Ninety-three consecutive patients (93 orbits) with large isolated MOWFs near the orbital apex. METHODS: The isolated MOWFs were determined by computed tomography (CT) scans of the orbit in all patients. All patients underwent fracture repair by an ETA, and the vertical and horizontal dimensions of the defects were measured during surgery. Porous polyethylene sheets (1.0 mm thick) were used to repair the bony defects. Patients were followed for 6 to 15 months. MAIN OUTCOME MEASURES: Size of vertical and horizontal fracture defects, rate of complete repair of the fracture defects, correction of enophthalmos, resolution of diplopia, and complications 6 months after surgery. RESULTS: All surgeries were completed uneventfully. The mean postoperative follow-up time was 9.7 ± 3.0 months. Under direct endoscopic visualization, all entrapped and herniated orbital contents were released and reposited, the entire boundary of the fractures were exposed adequately, and the implants were placed to overlie all edges of the fracture stably in all cases. The vertical and horizontal fracture defects measured during surgeries ranged from 16 to 30 mm and from 25 to 34 mm, respectively. Six months after surgery, complete reconstruction of the bony defects was demonstrated by orbital CT scans, and symmetry of the both eyes was acquired in 92 of 93 patients (98.9%). Of 30 patients with significant preoperative enophthalmos of more than 2 mm, 29 (96.7%) were corrected, with a mean improvement of 3.37 ± 0.77 mm. Diplopia within the 30° visual field of the gaze was resolved in 40 of 43 patients (93.0%). Three patients (7.0%) had residual diplopia on medial gaze because of presumed paralysis of the medial rectus muscle. Intraorbital hemorrhage occurred in 1 patient the day after surgery and resolved with conservative treatment. CONCLUSIONS: The ETA seems to be a useful method for recovery of the normal anatomic features of the orbits for patients with large MOWFs near the orbital apex.

Management of upper eyelid cicatricial entropion.

PURPOSE: There is a paucity of published data on the management of upper eyelid cicatricial entropion. We report on our results using such techniques as lamella repositioning, recession or augmentation and terminal tarsal rotation. DESIGN: Observational retrospective case series. PARTICIPANTS: Consecutive cases of upper eyelid cicatricial entropion of two specialist oculoplastic centres (Corneoplastic Unit, East Grinstead, UK and South Australian Institute of Ophthalmology, Adelaide, Australia) were reviewed over a 7-year period. METHODS: All patients underwent anterior lamellar repositioning or terminal tarsal rotation. MAIN OUTCOME MEASURES: Success was defined by two definitions: anatomical success was defined where the lid margin was restored to its normal position. Complete success was defined where there were no eyelashes touching the globe. Gain or loss (≤ or ≥2 Snellen lines) in best corrected visual acuity using a Snellen chart and resolution of any corneal epitheliopathy at final follow-up were also recorded (as graded by experienced oculoplastic consultants). RESULTS: Fifty-two procedures were performed on 41 patients (11 bilateral). All patients underwent either an anterior lamellar repositioning or a terminal tarsal rotation. Trachoma, previous upper lid surgery, Stevens-Johnson syndrome and meibomian gland dysfunction were the commonest underlying diagnoses. Ninety-eight per cent of the group had a normal anatomical lid position at follow-up. Nine eyelids (17%) of the group had recurrence of trichiasis. CONCLUSION: This large case series demonstrates that upper eyelid cicatricial entropion is managed effectively utilizing procedures that involve recession and reposition. We recommend that excision of tissue is avoided, especially in pathology that has a progressive immunological cicatricial drive.

Endoscopic transethmoidal and transconjunctival inferior fornix approaches for repairing the combined medial wall and orbital floor blowout fractures.

PURPOSE: To assess the combination of an endoscopic transethmoidal approach (ETEA) and a transconjunctival inferior fornix approach (TIFA) for repairing combined orbital floor and medial wall blowout fractures (COF-MWBOFs). METHODS: Patients with unilateral COF-MWBOFs were randomized to 2 groups: ETEA combined with TIFA or medial canthal incision (MCI) combined with TIFA. Thin porous polyethylene sheets were implanted to cover the orbital defects. The postoperative follow-up time was 6 months. For both groups, degree of diplopia, enophthalmos, ocular motility, and complications were recorded and compared. RESULTS: Our study included 32 patients in the ETEA group and 27 patients in the MCI group. The time from trauma to surgery ranged from 10 to 48 days. The herniated orbital contents were completely reduced in 96.9% (31/32) of the ETEA group and in 77.8% (21/27) of the MCI group (P=0.040). At the 6-month review, the success rate of enophthalmos correction in the ETEA group achieved 96% (24/25), higher than that in the MCI group (66.7%, 12/18; P=0.015). The improvement of the enophthalmos was better in the ETEA group than in the MCI group (4.5±0.7 mm versus 3.2±1.2 mm) at 6 months (P=0.000). We found no difference in the resolution rate of diplopia within a 30-degree visual field or extraocular muscle function between the 2 groups. Complications in the MCI group included 1 patient with optic neuropathy after the procedure and 4 patients with medial canthal webbing. CONCLUSIONS: Endoscopic transethmoidal approach combined with TIFA is a promising technique for repairing extensive COF-MWBOFs.

A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation.

INTRODUCTION: To describe our attempt in establishing a definitive diagnosis in patients with hypertrophic pachymeningitis in combination with orbital inflammatory disease and report on the outcome. MATERIALS AND METHODS: This was a retrospective case series of all patients presenting with hypertrophic pachymeningitis in association with orbital inflammation in 4 centres. Ophthalmic and neurological examination data, laboratory data, histology data, treatment plans and clinical outcome data were recorded. Patients underwent orbital/brain computed tomography and magnetic resonance imaging. RESULTS: Six patients were identified; the median age was 46.5 years. Headache was the commonest presenting symptom, followed by diplopia and reduced visual acuity. Three patients underwent orbital biopsy, 1 patient underwent dura mater biopsy, 1 patient underwent both and 1 patient underwent nasal biopsy. Four patients were diagnosed with Wegener granulomatosis and 2 patients with tuberculosis. Corticosteroid therapy was initiated in 4 patients, with steroid-sparing drugs added later. Two patients received anti-tuberculosis treatment and 1 patient was commenced on pulsed cyclophosphamide. On follow-up, 1 patient required an exenteration for a painful blind eye and 1 patient's visual acuity remained at no perception to light. One patient had complete resolution of symptoms on treatment, 1 patient had persistent reduced visual acuity and 1 patient was lost to follow-up. CONCLUSION: We postulate that the combination of orbital inflammation and pachymeningitis is strongly suggestive of Wegener granulomatosis, although it may take a number of years to confirm. Tuberculosis should also be considered.

Visual acuity recovery in traumatic optic neuropathy following endoscopic optic nerve decompression: a case report.

A 59-year-old Chinese man presented with no light perception in the left eye 1 day following functional endoscopic sinus surgery. The patient underwent endoscopic optic nerve decompression with topical and systemic application of nerve growth factor and steroids after a failed trial of high-dose intravenous corticosteroids. Visual acuity improved to 20/25 at 3 weeks and remained stable at 9 months. Reports of full visual recovery are exceedingly rare following this functional endoscopic sinus surgery complication.

The ophthalmic side-effects of imiquimod therapy in the management of periocular skin lesions.

AIM: To describe the ophthalmic side-effects of topical imiquimod for periocular actinic keratoses, squamous cell carcinoma in situ and basal cell carcinoma. METHOD: A retrospective study was carried out in two centres of all patients who underwent topical imiquimod therapy between January 2004 and January 2009. Imiquimod was applied three times weekly for 4-6 weeks. Diagnosis of the lesions, complications, clinical resolution and long-term ophthalmic side-effects was recorded. Patients on therapy were reviewed fortnightly and then every 6 weeks following completion of treatment. RESULTS: 47 patients were identified; the mean age was 74 years. 37 patients had actinic keratoses, seven patients had Bowen disease, and three patients had BCC. The lower lid was the commonest site involved (68%). Application site erythema occurred in all patients. Conjunctivitis occurred in 15 patients, and six patients complained of ocular stinging on application of imiquimod. One patient had a staphylococcal keratitis, which responded to topical antibiotic and steroid therapy. Two patients required oral antibiotics for preseptal cellulitis. Three patients had delayed conjunctivitis at a mean of 2.3 weeks. Nine patients discontinued imiquimod due to ocular irritation and conjunctivitis, of whom four patients recommenced and finished the treatment after a rest period. At a mean follow-up of 16 weeks, 34 patients had clinical resolution of the periocular lesions and no patient had any residual ophthalmic side-effects from imiquimod. CONCLUSION: Conjunctivitis and ocular stinging were the commonest ophthalmic side-effects encountered with the application of imiquimod for periocular skin lesions. These effects were temporary and resolved on terminating the imiquimod therapy.

Composite grafts in eyelid reconstruction: the complications and outcomes.

AIMS: To describe the outcomes and complications for composite eyelid grafts in a large case series over a 9-year period. METHOD: A retrospective study of all patients who underwent a composite graft for eyelid reconstruction between January 2000 and May 2009. A composite eyelid graft involves the excision of a full thickness pentagonal wedge from the donor eyelid, which is inserted into the recipient eyelid defect. The orbicularis muscle is discarded from the composite graft. Initial diagnosis, complications, postoperative eyelid contour and colour were recorded. RESULTS: Forty-two patients were identified in the study period; the mean age was 69 years. Reconstruction following basal cell carcinoma excision was the commonest indication for surgery. Thirty-five composite grafts were performed for lower eyelid defects and seven for upper lid defects. The average size of the grafts was 9.1 (range 7-12) mm. Five patients required two grafts to reconstruct the defect. Thirty-six (86%) patients achieved a satisfactory contour and 33 (78%) patients had good matching skin colour. Partial dehiscence occurred in five patients and three patients had bulkiness of the graft postoperatively. We had no episodes of graft failure, although two patients had skin necrosis, which resolved. CONCLUSION: Composite eyelid grafts when combined with mobilisation of the recipient orbicularis muscle can result in good preservation of eyelid function with satisfactory cosmesis.

The use of soft tissue expanders in periocular reconstruction: a case series.

PURPOSE: To describe the use of soft tissue expanders for periocular reconstruction in the presence of limited local tissue availability. METHODS: A retrospective case series of 5 patients who attended the Manchester Royal Eye Hospital. They presented a reconstructive challenge due either to the presence of periocular scarring or to a marked loss or limitation of normal local skin for reconstruction. The soft tissue expander (SOE-3624 device) was inserted under the skin and subcutaneous tissue at a site selected to provide the desired additional tissue. Over periods of time varying from 6 to 16 weeks, the soft tissue expander was gradually inflated with injections of sterile saline in the outpatient clinic. As soon as the desired amount of additional skin and subcutaneous tissue had been obtained, reconstructive surgery was undertaken. Functional and cosmetic results were the main outcomes of the intervention. RESULTS: Four of the patients had the device inserted in the lower eyelid or cheek area and one patient had the device inserted above the eyebrow. No episode of tissue necrosis occurred in our patients. All the devices were tolerated by the patients and were removed without any complications. The implant migrated in one patient, limiting the reconstructive surgery, and one patient had a lateral canthal dehiscence following reconstruction. All patients thought that the surgery had improved their cosmesis. No patient required any additional surgical procedures. CONCLUSIONS: The authors conclude that the SOE-3624 tissue expander represents a good option in providing additional tissue for periocular reconstruction, particularly in younger patients. It was well tolerated and effective in this series.

Recovery of Visual Function in a Patient with an Onodi Cell Mucocele Compressive Optic Neuropathy Who Had a 5-Week Interval between Onset and Surgical Intervention: A Case Report.

Purpose. To report on a patient with compressive optic neuropathy secondary to an Onodi cell mucocele, who fully recovered visual function following surgery. Method. Case report. Results. A 28-year-old male was admitted with a right visual acuity of 20/100 following treatment for an initial diagnosis of optic neuritis. Subsequent examination suggested compressive optic neuropathy, and neuroimaging confirmed the presence of an Onodi mucocele compressing the optic nerve. The patient underwent a right endonasal sphenoethmoidectomy with decompression 5 weeks after the initial onset of symptoms. Three weeks following surgery, the visual acuity was 20/20, and there was complete resolution of the visual field defect, which has remained stable at 1 year. Conclusion. Onodi cell mucocele should be included in the differential diagnosis of a young patient with compressive optic neuropathy. Surgical decompression should be considered even when symptoms have been present for over a month.

Lesions mimicking lacrimal gland pleomorphic adenoma.

AIM: To report a series of patients with lacrimal gland lesions simulating the clinicoradiological features of lacrimal gland pleomorphic adenoma (LGPA). METHODS: Multicentre retrospective, interventional case series. Clinical records of all patients with lesions mimicking LGPA seen in five orbital units were reviewed. RESULTS: The study included 14 patients (seven men and seven women) with a mean age of 50.9 years. The diagnosis of LGPA was made in all cases by experienced orbital surgeons, based on clinicoradiological features, and lacrimal gland excision was performed. Postoperative histology revealed lymphoma (four patients), chronic dacryoadenitis (three patients), adenoid cystic carcinoma (two patients), Sjogren's syndrome (two patients), cavernous haemangioma (one patient), benign lymphoid hyperplasia (one patient) and granulomatous dacryoadenitis (one patient). Comparison with the total number of histologically confirmed LGPA cases seen during the study period revealed that 22.6% of cases of suspected LGPA were misdiagnosed based on clinicoradiological criteria. CONCLUSIONS: Many different lesions may mimic the clinicoradiological features of LGPA. The accepted clinicoradiological criteria used for the diagnosis of LGPA have a high false-positive rate, even in experienced hands. Based on this study, the authors believe that fine-needle aspiration biopsy or intraoperative biopsy and frozen section diagnosis may help reduce unnecessary lacrimal gland excision.

Extramedullary plasmacytoma arising from the lacrimal gland.

An 80-year-old man presented with an 8-week history of painless swelling in the right lacrimal gland region with infero-medial dystopia of the globe. The lesion was excised and histology confirmed an orbital plasmacytoma. Multiple myeloma screening was negative and a solitary extramedullary plasmacytoma arising from the lacrimal gland was diagnosed. The patient was subsequently treated with radiotherapy.

Hypertrophic changes of the lower eyelid margin after hughes procedure for eyelid reconstruction: the management and outcomes.

PURPOSE: To describe the management of lower eyelid margin hypertrophy as a complication of the Hughes procedure for eyelid reconstruction. METHODS: A retrospective review of all patients with lower eyelid hypertrophy after Hughes procedure. Patient demographics, management, histologic findings, and outcomes were recorded. The patients underwent wedge excision of the hypertrophic segment of the eyelid with direct closure achieved in the majority of cases. RESULTS: Five patients were identified. The mean age at presentation was 66 years. The mean onset for the hypertrophic margin was 8.4 weeks after the Hughes flap. Four patients complained of ocular irritation and 1 patient had a constant ocular discharge. All patients were concerned by the appearance of the eyelid margin. Four patients received topical steroid treatment and 4 patients underwent triamcinolone subcutaneous injections with no improvement. The wedge excision was successful in 4 patients at the mean follow-up of 10 months, with no recurrence of the hypertrophic margin. One patient was reviewed in another center. CONCLUSION: The authors found no beneficial effect with topical or subcutaneous courses of steroids on eyelid margin hypertrophy. In situations of horizontal eyelid laxity, a full-thickness wedge excision offers a good means of removing the offending eyelid segment.

Difference in response to botulinum toxin type A treatment between patients with benign essential blepharospasm and hemifacial spasm.

BACKGROUND: Botulinum toxin (BTX) is the first-line treatment in managing benign essential blepharospasm (BEB) and hemifacial spasm (HFS). We wished to assess the difference in duration of effect and the number of BTX treatments required to treat patients with BEB and HFS. METHODS: A prospective study of patients attending the BTX clinic in Manchester Royal Eye Hospital over 6 months. All treatments were administered by a single experienced ophthalmologist. A questionnaire was completed for each patient. In patients with BEB where the BTX was injected bilaterally, one side was randomized to compare with HFS patients. Patient demographics, cumulative dose of BTX, duration of BTX effect with patient satisfaction and the number of previous BTX injections were recorded. RESULTS: Sixty-four patients were included in the study. The mean age was 60.8 years. Among them, 30 patients had BEB and 34 had HFS. Patients with HFS received a lower mean dose of BTX than patients with BEB (12.23 units vs. 16.2 units). The patients with HFS had a longer duration of effect than patients with BEB, with fewer BTX treatments. Of all patients, 90% with HFS and BEB were satisfied with the effect of their last BTX injection. Three unsatisfied patients in the BEB group were referred on for surgical management of their disorder. CONCLUSIONS: We have shown that patients with BEB have a shorter duration of effect with BTX and require more frequent BTX treatments than patients with HFS, highlighting that facial dystonias in patients with BEB is more challenging to manage.