Proptosis of the eye: a potential form of Madelung's disease.

Madelung's disease is a rare disorder characterised by excessive and symmetrical deposits of adipose tissue, typically in the cervicofacial region. Alcohol is a known cause of the condition, however, there are reports that this condition is genetically inherited. Lipomatosis of the orbit has been described in the alcoholic Madelung's disease, however, in our case report, we believe this is the first reported instance of proptosis caused by the genetic form of the condition. We present a 69-year-old woman, with a medical history of genetic Madelung's disease, who presented with bilateral proptosis worse in her right eye. Her ocular examination was normal apart from exophthalmometry, showing bilateral proptosis. This was confirmed by an MRI, which further showed intraorbital fat deposition bilaterally. Due to the stability of her condition, no treatment was deemed necessary. We highlight the importance of monitoring for progressive optic nerve compromise and liposarcomatous malignant transformation.

Rectus Muscle Resection for Vertical Strabismus in Thyroid Eye Disease.

PURPOSE: Rectus muscle resection in thyroid eye disease (TED) is generally avoided due to the risk of worsening restriction or reactivating inflammation. However, for some patients with large-angle strabismus or diplopia in primary gaze despite maximum recession surgery, rectus muscle resection may be beneficial. We report our surgical experience with rectus muscle resection in the management of vertical strabismus associated with TED. METHODS: Retrospective review of eight patients with TED and vertical diplopia who underwent vertical rectus muscle resection by a single surgeon (IBM) at a tertiary referral centre in Liverpool, UK, from 2001 to 2013. The goal of surgery was elimination of diplopia in primary and reading position. Vertical deviations were measured in prism dioptres (∆) before and after surgery at one month, four months and final visit by prism alternate cover testing at ⅓ m and 6 m. RESULTS: The mean ± standard deviation vertical deviation for near and distance reduced significantly from 14.2∆ ± 8.4∆ and 15.8∆ ± 8.8∆ pre-operatively to 5.7∆ ± 4.9∆ and 6.7∆ ± 7∆ at the four-month visit, respectively (p< 0.05). At the four-month follow-up, five (62.5%) patients achieved binocular single vision in primary and reading position with either no prisms or prisms less than 5∆. Further recession surgery, Harada-Ito procedure, or lateral rectus resection were necessary in four (50%) patients with persistent diplopia. No patient developed recurrence of inflammation or increased muscle restriction. CONCLUSIONS: Vertical rectus resection could be considered as an additional surgical strategy in the management of TED patients with vertical strabismus without adverse sequelae.

Complications and outcomes of grafting of posterior orbital fat into the lower lid-cheek junction during orbital decompression.

PURPOSE: To report the complications of grafting of excised posterior orbital fat into the lower lid-cheek junction at the time of orbital decompression surgery. METHODS: Retrospective review of consecutive patients undergoing orbital decompression combined with grafting of posterior orbital fat to the pre-malar and lateral canthal area (FG). A second group of consecutive patients undergoing orbital decompression but no orbital fat grafting (NoFG) were also studied as a form of comparative control. Standard patient data, including age, sex, visual acuity, degree of proptosis, operative details, diplopia or any other complications was collected. Independent assessment of pre- and post-operative photographs graded the lower lid-cheek junction. RESULTS: Thirty-four orbits of 29 patients, of which 21 orbits underwent orbital decompression with orbital fat grafting (FG). There were no intraoperative complications, postoperative infections, or visual loss. Complications relating to fat grafting included prolonged swelling in 3 (17%) patients at 3 months, in 1 case lasting 6 months, lower lid lumps in 3 (17%), and fat seepage in 1 (6%). The FG group achieved a greater improvement in the appearance of the lower-lid-cheek junction at 12 months in comparison to NoFG. Mean grade improvement 1.24 ± 1.09 vs 0 ± 0.82 (p = 0.025). Median follow-up was 20 months (range 6-30 months). CONCLUSION: Grafting of excised orbital fat during orbital decompression can improve the appearance of the lower lid-cheek junction in patients being treated for thyroid orbitopathy. However, 24% of patients will experience swelling and/or lumpiness requiring several months to settle or further fat excision.

Clinical Outcomes of Ruptured Periorbital and Orbital Dermoid Cysts.

PURPOSE: To evaluate the clinical outcomes of ruptured dermoid cysts. METHODS: A multicenter, retrospective study of all cases of periorbital and orbital dermoid cysts with histopathological evidence of rupture, including those with clinical rupture, was performed over a 10-year period. Demographics and clinical outcomes of ruptured dermoid cysts were recorded. Persistent inflammation was defined as the presence of edema, erythema, and discomfort for at least 28 days. RESULTS: Eighty-six cases of dermoid cysts were identified. Median age was 5.5 (range, 1-63) years. Location of cyst was either periorbital (n = 60, 70%) or orbital (n = 26, 30%). There were 29 cases with clinically apparent rupture: 27 surgically ruptured (93%) and 2 spontaneous rupture (7%). Persistent inflammation was found in 1 spontaneous cyst rupture case (50%) and 1 surgically ruptured cyst (3.7%). Older age (p = 0.01) and bony attachment (p = 0.001) were significant factors for cyst rupture, while there was no influence from cyst location (p = 0.14). CONCLUSIONS: Persistent inflammation is uncommon after surgical rupture of dermoid cysts, but likely after spontaneous rupture. Older age and bony attachment are risk factors for cyst rupture.

Effect of Manuka Honey on Eyelid Wound Healing: A Randomized Controlled Trial.

PURPOSE: To report outcomes of a randomized trial on the role of "active" Manuka honey on eyelid surgical wound healing. METHOD: Prospective, randomized, single-blinded study was performed for patients undergoing bilateral upper blepharoplasty. Vaseline was applied 4 times a day to both sides for 6 weeks and in addition, one eyelid was randomized to receive Manuka honey twice daily. Postoperative wounds were graded by a masked observer at 1 week, 1 month, and 4 months using Manchester scar scale and a modified eyelid scar grading scale. Patients scored symptoms, expressed preferred side, and of any problems they experienced using honey. Standard photographs were graded by 2 independent assessors. RESULTS: Fifty-five patients were randomized. One week after surgery, 46 (29 women, 17 men, mean age 68 years, median 69, range 49-85) were available for analysis. There was a trend toward distortion of the surrounding skin being less (1.6 vs. 1.8, p = 0.07) and the scar being less palpable (1.8 vs. 2.0, p = 0.08) on the Manuka-treated side. Patients reported the scar on the Manuka side to have less stiffness (1.3 vs. 1.6, p = 0.058). At 1 month, all 3 grading scales showed no difference between the 2 sides. At 4 months, scar grading scales showed no differences; however, patients reported scar pain to be significantly less on the Manuka-treated side than control (0.48 vs. 1.9, p = 0.005). Thirty-one of 46 patients believed the scars were similar on both sides, 11 preferred the honey-treated side, and 4 preferred the control. CONCLUSION: Upper eyelid scars treated with or without Manuka honey heal well, without significant difference when assessed by validated scar grading scales; however, honey may provide subjective benefits early, postoperatively.

Bilateral optic disc edema as a presentation of an obstructing spinal plasmacytoma.

PURPOSE: To report a rare case of bilateral optic disc edema as presentation of an obstructing spinal plasmacytoma. METHODS: Case report. RESULTS: A 41-year-old healthy man presented with distortion of his peripheral vision for 9 months. He denied headaches or neurologic symptoms. Examination showed bilateral optic disc swelling, radial disc hemorrhages, and absent spontaneous venous pulsations. Brain magnetic resonance imaging (MRI) and magnetic resonance venography were unrevealing. Lumbar puncture showed a normal opening pressure of 19 cm cerebrospinal fluid (CSF). CSF protein was significantly elevated at 3.22 g/L (0.10-0.45 g/L). Spinal MRI with contrast revealed a tumor in the T9 vertebral body extending through the disc spaces into T8 and T10, compressing the spinal cord. Computed tomography-guided biopsy confirmed a spinal plasmacytoma. He received radiotherapy to the spine T7-T11 to reduce spinal cord compression followed by a 6-month course of chemotherapy with dexamethasone, cyclophosphamide, and thalidomide. Disc swelling improved on starting radiotherapy with complete resolution 8 months posttreatment. MRI spine showed reduction of tumor. CONCLUSIONS: This case highlights the importance of spinal imaging in patients with normal cranial scans and raised CSF protein levels who lack the typical idiopathic intracranial hypertension phenotype.

CADS grading scale: towards better grading of ophthalmic involvement in facial nerve paralysis.

INTRODUCTION: Ophthalmologists lack a facial nerve grading instrument (FNGI) that comprehensively encompasses the ophthalmic sequelae of facial nerve paralysis (FNP). Assessment and management of ophthalmic sequelae remains inconsistent, and outcomes of clinical studies are incomparable. We have developed and successfully adopted an FNGI based on four aspects of periorbital involvement: cornea, asymmetry, dynamic function and synkinesis. This CADS classification is specific for periorbital involvement, with objective and subjective parameters, to standardise grading. We present this classification and the results of a validation study in clinical practice. METHODS: A cross-sectional, validation study. Two clinicians independently assessed and graded each patient on the same day, blinded to each other's grading. Each grader assigned a score to each of four parameters: C (0-3, ±a), A (0-2), D (0-3), S (0-2). RESULTS: Thirty patients (19 females, mean age 60, range 30-84 years) with unilateral facial paralysis were graded. A total of 60 assessments were conducted. CADS scores ranged from C0A0D1S1 to C3aA2D3S0. In the first 30 assessments (of the first 15 patients), the two assessors disagreed over the corneal grading in four patients. The last 30 assessments of 15 patients showed complete agreement in all four parameters of the grading scale. The overall inter-observer agreement was 86.7% for cornea, 93.3% for resting asymmetry, 93.3% for dynamic function and 86.7% for synkinesis. After the first six patients, Cohen's κ reached 1 for all but synkinesis that ranged between 0.9 and 1. CONCLUSIONS: We present a validation study of an FNGI specifically designed for ophthalmic involvement of FNP. Objective and subjective parameters helped standardise grading and management planning.

Platinum segments: a new platinum chain for adjustable upper eyelid loading.

PURPOSE: Prospective study evaluating outcomes of individually sutured platinum segment chains in upper eyelid loading. METHODS: Single-centre, single-surgeon, prospective study. Upper eyelid loading with 0.4 and 0.2 g platinum segment chains for lagophthalmos. Segments were sutured to create a desired weight and attached to superior tarsus and distal levator aponeurosis following levator recession. PRIMARY OUTCOME MEASURES: improvement in lagophthalmos and complications. SECONDARY OUTCOME MEASURES: cosmesis of eyelid margin contour and implant prominence. Minimum 3-month follow-up. RESULTS: Eighteen eyelids of 17 patients received segment chains (mean weight 1.2 g, range 0.8-1.6 g) and 3 for exchange of pre-existing gold weights. Median follow-up was 10 (range 6-17) months. Mean blink lagophthalmos improved from 7 (3-10) mm to 3 (0-6) mm (p<0.0001), and gentle closure from 3.2 (0-8) mm to 1.1 (0-4.9) mm (p=0.0004). Twelve patients (71%) reported no prominence, and the remainder, mild prominence only. The chain was graded as having no prominence in 78% (14/18) eyelids. Two required segment adjustments with removal of a single 0.2 g segment at 11 months and transfer of 0.4 g segment to the contralateral eyelid at 16 months, respectively. One complication (posterior, trans-conjunctival exposure above the superior border of the tarsal plate) was seen 12 months post surgery, requiring repositioning. CONCLUSIONS: Platinum segment chains provide benefits of platinum chains with additional advantages of postoperative adjustibility. They can be used as an addition to pre-existing in situ weights or chains. TRIAL REGISTRATION NUMBER: REC reference: 13/SW/0146. IRAS project ID: 119022.

Single Injection of Bupivacaine for Correction of Strabismus.

A retrospective review of 30 patients with eso/exodeviations less than 20 prism diopters (PD) who underwent one injection of BPX into their medial/lateral rectus was carried out. Preoperative deviations were recorded in PD, using the alternate prism cover test. Postoperative angles at 1 and 3 months were measured in the same manner. 11 out of 30 patients achieved an acceptable outcome and required no further intervention. The mean angle of deviation for near in all patients reduced significantly by 3.1 PD and 2.1 PD at 1 and 3 months. The mean angle of deviation for distance in all patients also decreased significantly by 2.3 PD and 1.9 PD at 1 and 3 months. In conclusion, single injection of BPX in a heterogeneous group of patients with horizontal strabismus caused a mild improvement and a qualitative success rate of 37%.

Ophthalmic grading of facial paralysis: need for a closer look.

Very many variations of facial grading systems have been developed, but none address the ophthalmic complications of facial nerve weakness adequately. The important priorities for ophthalmic surgeon are protecting the ocular surface, maintaining sight and peripheral vision, as well as improving the cosmesis of periocular area. The absence of a universally agreed grading system on the ophthalmic plastic complications of facial nerve paralysis is an obstacle to standardised treatment. It is time for clinicians to recognise this need.

Uvulitis following general anaesthesia.

Uvular necrosis following endotracheal general anaesthesia is a rare complication. We report two cases of uvular and soft palate necrosis after atraumatic intubation with endotracheal tube and, in the second case, laryngeal mask airway.

Transient cystoid macular oedema in multiple choroidal naevi.

Choroidal Naevi are generally thought to be trivial. However, sometimes they cause visual loss, visual filed defect and rarely transform into malignant melanoma. We report the first case of unilateral numerous choroidal naevi in a healthy female associated with transient cystoid macular oedema and postulate about its pathogenesis.

Intracranial arachnoid cyst; an unusual cause of epiphora.

PURPOSE: To describe a case of unilateral intracranial arachnoid cyst in association with enophthalmos and epiphora on the same side. METHODS: Case report. RESULTS: A young man with symptoms of unilateral epiphora is described. He had a large intracranial arachnoid cyst with an unusually large orbit leading to enophthalmos and symptomatic epiphora on that side. Radiological features of the orbit and associated pneumosinus dilatants are described. We also offer hypotheses to describe the paradoxical occurrence of an expanding intracranial mass and inward growth of the orbit and paranasal sinuses. CONCLUSION: A new cause for enophthalmos is described.

Bilateral viral retinitis in a patient with immune deficiency because of purine nucleoside phosphorylase deficiency.

BACKGROUND: Progressive retinal outer necrosis is a devastating viral necrotizing retinitis, which affects predominantly immunocompromised patients. We report a case in which the ocular findings led to a search for an underlying immune deficiency, established as congenital purine nucleoside phosphorylase deficiency. METHODS: Retrospective, interventional case report. RESULTS: We describe the clinical features and management of the disease. CONCLUSION: This is the first report of bilateral varicella zoster virus retinitis with underlying purine nucleoside phosphorylase deficiency. It highlights the importance of thorough investigation in apparent immunocompetent patients presenting with viral retinitis, as this may indicate an undiagnosed primary immunodeficiency.

Endophthalmitis following intravitreal injections.

BACKGROUND: To report the clinical features, culture results, management and visual outcome of patients with endophthalmitis following intravitreal injections. METHODS: Retrospective review of all patients with suspected endophthalmitis after intravitreal injections treated with intravitreal antibiotics (teicoplanin and ciprofloxacin) at a referral centre between January 2003 and December 2010. RESULTS: Nineteen cases that had aqueous or vitreous biopsy were identified. Nine had negative culture. Ten had positive culture; Staphylococcus species in 4/10, Streptococcus species in 4/10, E. coli in 1/10 and gram-negative bacilli in 1/10. Symptoms developed within the first 48 hours in all. One of ten culture-positive cases had no pain on presentation, while 5/9 patients with negative culture reported pain. Initial treatment consisted of intravitreal antibiotics in all cases, 6/19 cases required a second intravitreal antibiotic injection, 4/19 underwent vitrectomy as secondary therapy. At the last follow up, 7/19 patients had visual acuity of 6/18 or better, 9/19 had visual acuity of 6/60 or worse. CONCLUSION: The overall numbers of patients with endophthalmitis following intravitreal injections has risen dramatically over the past years. In contrast to earlier reports of multicentre studies, outcome of patients is relatively poor in the current treatment settings. We did not find clinical features useful in identifying cases with negative culture.

Challenges of managing sympathetic ophthalmia in a young child and the role of infliximab.

The authors report the difficult challenges and use of infliximab in the delayed presentation of a 2½-year-old boy with a penetrating eye injury from a small ballpoint pen that led to sympathetic ophthalmia.