Long-Term Outcome of Staged Socket Surgery for Acquired Anophthalmos.

PURPOSE: To evaluate long-term outcomes of staged volume rehabilitation for acquired anophthalmos. METHODS: Case-note review of patients who had preceding i) eye removal without implant, ii) eye removal with an intraconal implant, but ball-related problems, or iii) removal of exposed implant. Secondary interventions were a) a first-time ball implant, b) dermis-fat graft, c) ball repositioning, d) ball replacement after prior removal, or e) orbital floor implantation. RESULTS: Secondary volume-augmenting surgery was performed in 175 sockets at a mean age of 42.7 years (range 2-91), with 62% secondary ball implants, 3% dermis-fat grafts, 6% ball repositioning, 19% ball replacement after prior removal for exposure, and 10% having orbital floor implantation. After this surgery, further volume enhancement was required in 21% of sockets, this being 40% for spheres ≤18 mm diameter, in contrast to 6% for those ≥20 mm ( p < 0.001). Exposure or malposition of the secondary implant occurred in 8% (12/151) and was unrelated to implant type, size, wrapping, or prior irradiation. Tertiary surgery addressed lining deficiency (18%) or eyelid malposition (25%). Overall, 92/175 (53%) had tertiary surgery to improve cosmesis and comfort, with 49% (36/92) being related to small implants. At a mean follow-up of 9.1 years, 82% of sockets had adequate volume, 79% had excellent lining, and 93% were comfortable. Prosthetic fit was satisfactory in 96% of cases, and 97% reported improved cosmesis. CONCLUSION: Over half of the sockets having planned 2-stage volume enhancement may need further procedures, especially after small-volume secondary implants, but, with meticulous surgery, reasonable long-term results can be achieved with few complications.

Orbital Exenteration for Advanced Periocular Adnexal Malignancies: Curative Versus Palliative Surgical Intent.

PURPOSE: To evaluate the survival benefit of orbital exenteration in periocular malignancy, taking account of preoperative intent. PATIENTS AND METHODS: Patients undergoing exenteration had retrospective chart review for demographics, clinical features, radiology, histology, and outcome. Based on systemic tumor status, the patient was either "Class I" (with absent or well-controlled systemic disease) or "Class II" (incurable active metastatic disease), and based on the extent of orbital disease and exenteration intent , was classed as either "Group A" (locally curative) or "Group B" (locally palliative). RESULTS: One hundred thirty-three patients (78 females; 59%) underwent exenteration at an average age of 61 years (median 64; range 5-91) for sebaceous, squamous and basal cell carcinomas, or for melanoma (22%, 19%, 11%, and 28%, respectively). There were 20% systemically incurable patients (26/133; Class II), and incurable local disease ("Group B") in 5% (5/107) of Class I and 15% (4/26) Class II patients. The overall survival (OS) was 88% at 12 months, 57% at 5 years, and 41% at 10 years, prognosis being worse with age more than 70 years ( p = 0.005), prior local radiotherapy ( p = 0.005) or positive resection margins ( p = 0.002). The mean OS for Type IA exenteration (145 months; 95% CI 122-168) was significantly different to 50 months for Type IB (95% CI 22-79; p = 0.02); likewise, OS for Type IIA procedures (31 months; 95% CI 11-51) was different to Type IIB (19 months; 95% CI 2-36) ( p = 0.001). CONCLUSION: Exenteration confers a significant survival with advanced periocular malignancies, even in patients with uncontrollable systemic disease, or where the local disease is deemed incurable.

Periosteal muscle anchoring for large angle incomitant squint.

PURPOSE: To describe a surgical technique for correcting very large angle exodeviations and determine long term outcomes leading to the evolution of surgical technique. METHODS: A consecutive series of seven patients with large angle, incomitant squint were operated on at Moorfields Eye Hospital over a five-year period using periosteal anchoring by a joint Strabismus/Adnexal team. All patients underwent anchoring of the insertion of the medial rectus (MR) muscle to the periosteum of the medial orbital wall via a retrocaruncular approach, with or without lateral rectus (LR) disinsertion and suturing to the lateral orbital rim connective tissue. Retrospective analysis of notes assessed previous procedures, prism dioptre (PD) deviations and complications. RESULTS: Included were seven eyes (five right, two left) of seven patients (two female, five male). Median age at first anchoring procedure was 25 years (range 4-57). Mean follow up was 34 months (range 8-66). Six patients had long-standing third nerve palsy, one patient had Moebius syndrome. Pre-operative exotropia ranged from 45 to 115 PDs (mean 76, median 75). At the final follow up, all patients had reduced horizontal deviations ranging from 0 to 80 PDs (mean 34, median 30). Further surgery was required in three patients all of whom did not undergo release of the LR muscle at the first procedure. CONCLUSION: Large angle incomitant divergent squints present a particularly difficult surgical challenge. We advocate a combined bi-rectus fixation approach to hold the globe in the primary position.

Lateral canthotomy orbitotomy: a rapid approach to the orbit.

PurposeThe lateral compartment of the orbit can readily be accessed through a horizontal lateral canthotomy without the need to swing the lid or remove bone. In this paper the technique, accessible orbital territory, and duration of surgery are presented.Patients and methodsRetrospective, non-interventional descriptive case series for patients who underwent a lateral canthotomy to access pathology within the lateral orbit.ResultsA series of 18 patients are included, all presenting with pathology lateral to, or within, the optic nerve. Pathologies included amyloidosis (1), lymphoma (4), metastatic adenocarcinoma within the optic nerve (1), idiopathic lateral rectus muscle mysositis (4), meningothelial meningioma of the optic nerve (1), intraconal orbital meningioma (1), reactive lymphoid hyperplasia (1), optic nerve glioma (3), optic nerve meningioma (1), and cavernous haemangioma (1). The median surgical time was 36 min (range 23-75 min). No patient required detachment of the lower lid, the technique leaving both upper and lower 'arms' of the lateral canthal tendon attached to Whitnall's tubercle.ConclusionsThe lateral canthotomy approach orbitotomy is a rapid, safe, and minimally disruptive approach for accessing pathology in the lateral orbit and optic nerve. The lateral canthal tendon is split along the horizontal raphe without detachment of either limb from Whitnall's tubercle, no bone is removed, and the post-operative recovery is rapid with minimal associated inflammation or chemosis. This approach is also flexible, permitting the clinician to increase exposure to the orbit peroperatively by swinging the lower lid if required.

Can Lester Jones tubes be tolerated for decades?

PurposeLester Jones described canalicular bypass tubes 50 years ago. We present a cohort of patients with Jones' tubes first placed between 1969 and 1989, and who were reviewed within the last 15 years.Patients and methodsRetrospective case-note review for living patients identified as having had Jones' tube placement prior to 1990. The duration of Jones' tube usage was noted and the number of replacements recorded.ResultsTwenty-nine patients (33 eyes) had maintenance of their Jones' tube(s) within the last 15 years, and had first tube placement before 1990. The average follow-up was 29.5 years (median 28.8 years, range 17-45.7 years). The original tube was present in 8/33 (24%) of eyes, at a mean survival of 34 years (33.3 years; range 29.4-44.4 years). The number of tube replacements during follow-up ranged from 0 to 9 (mean 1.7; median 1). When considering the initially placed tube in all 33 eyes, however, the survival ranged between 18 days and 44.4 years (mean 13.6 years; median 6.9 years). At last follow-up, 11/33 (33%) of eyes had lost their tubes, with 9 having minimal or no symptoms.ConclusionsThese patients with Jones' tube placement before 1990 provides the first recorded evidence that the device can be tolerated for at least four decades, and that some patients will-with appropriate outpatient maintenance-retain their originally placed tube. This information may be useful in counselling patients about the lifetime expectation for bypass tubes.

Upper lid ptosis surgery: what is the optimal interval for the postoperative review? A retrospective review of 300 cases.

PurposeCorrection of upper eyelid ptosis is one of the most commonly performed oculoplastic procedures on the NHS but there is currently no data in the literature informing the surgeon of the optimal time for the first postoperative review. Our aim was to investigate how often a complication that warranted intervention occurred in the first 6 weeks after surgery and whether such a complication could have been predicted preoperatively.Patients and methodsA retrospective review was performed of 300 operations in 239 patients over a 9-month period at Moorfields Eye Hospital, London. Electronic medical record software was used to extract data regarding the timing of first postoperative review, complications, any return to theatre, and any underlying risk factors or co-morbidities.ResultsAt 1 week 44 % (133) cases were reviewed, 30% (89) at 2 weeks, 17% (50) at 3 weeks, and 9% (28) at 4 or more weeks. The overall complication rate at any time during the 6-week follow-up interval was 8%. The majority of these complications were minor (24 eyes, 8%) and 1 was major (0.3%). Of the 25 complications, an underlying risk factor was identified in 14 cases.ConclusionsThese data indicate that postoperative complications are very low in the absence of preoperative risk factors. In our institution, as the risk of overcorrection is low, most patients without risk factors for exposure (51% in this series) can safely be reviewed later than 1 week after surgery, but for those with risk factors earlier follow-up is warranted.

The incidence, embryology, and oculofacial abnormalities associated with eyelid colobomas.

PURPOSE: To describe a cohort of patients with congenital eyelid coloboma, to identify associated ocular and craniofacial abnormalities, and to establish any correlation between the size and location of eyelid colobomas and the presence of such abnormalities. METHODS: An observational case series of 55 patients with eyelid coloboma treated by a single surgeon (JROC) between 1985 and 2005. RESULTS: Eyelid colobomas predominantly affected the upper lids (93%), and were typically unilateral (76%). About a third (29%) were an isolated finding, with the remainder associated with other ocular (62%) and/or craniofacial (53%) abnormalities. Of those with ocular abnormalities; 19 (56%) had conjunctival traction bands, 16 (47%) choristomas, and 8 (24%) an abnormal globe. Of those with craniofacial abnormalities; 13 (45%) had Goldenhar Syndrome, 10 (35%) clefting disorders, and 4 (14%) Fraser Syndrome. Clefting disorders were typically associated with more severe colobomas and a higher incidence of conjunctival traction bands, first arch syndromes with smaller colobomas and more choristomas. Overall large colobomas were significantly associated with the presence of other craniofacial defects compared with small colobomas (P<0.01, χ(2)), but coloboma size did not correspond with the presence of other ocular abnormalities. CONCLUSIONS: Coloboma size, location, and associations in this series are consistent with our current understanding of eyelid embryogenesis. It is likely that those colobomas associated with other craniofacial and ocular abnormalities are those which result from errors earlier in embryogenesis during eyelid specification, growth, and closure, whereas isolated colobomas arise later during eyelid separation, and after codependent structures have developed.

Periocular tuberculous disease: experience from a UK eye hospital.

BACKGROUND/AIMS: To describe our experience of patients presenting to a tertiary referral adnexal department with orbital or periocular tuberculosis (TB) over a 10-year period. METHODS: We reviewed all patients with a diagnosis of orbital or periocular TB from 2001 to 2011 in Moorfields Eye Hospital. RESULTS: Nine patients were identified over the 10-year period. Three cases of cutaneous TB, two cases of TB dacryocystitis and four cases of diffuse orbital TB were identified. All patients lived in the UK, but were born in the African or Asian subcontinents. Three patients had known prior (and treated) pulmonary TB and all were immunocompetent.All patients presented with periocular discomfort. After tissue diagnosis, all patients were referred for triple antituberculous therapy (ATT); all patients completed their course of ATT, with resolution of all orbital and lacrimal masses. There were no recurrences at a median follow-up of 26 weeks (range 1 month-5 years). One patient, who required later evisceration, was the only case with loss of vision. CONCLUSIONS: Orbital and periocular TB can be difficult to diagnose and lead to diagnostic delays, with emphasis on clinical suspicion rather than a positive culture result; the management of such cases is not only surgical, but also medical and social. Although surgical intervention can alleviate symptoms and prevent visual loss, the use of a complete course of ATT is paramount for disease management and the patient and their family need to be counselled about the associated public health issues.

Factor V Leiden mutation is associated with ocular involvement in Behçet disease.

PURPOSE: Behçet disease is a systemic disease of young adults characterized by venous occlusion in both the deep venous and retinal circulations. In severe ocular disease, blindness may occur despite immunosuppressive treatment. The most common inherited risk factor for the development of idiopathic venous thrombosis is the presence of the Factor V (FV Leiden) mutation, which confers resistance to activated protein C. The association of FV Leiden with Behçet disease has been reported, but its influence on ocular disease is not known. We therefore investigated the prevalence of this mutation in patients with Behçet disease to determine its contribution to the presence and severity of ocular disease. METHODS: One hundred and six Middle Eastern patients satisfying international criteria, and 120 healthy control subjects without a history of venous thrombosis were included in the study, and patients underwent standard examination by two ophthalmologists with an interest in Behçet disease. Genomic DNA was extracted from peripheral blood leukocytes and screened for the FV Leiden mutation with the polymerase chain reaction method with sequence-specific primers (PCR-SSP). RESULTS: FV Leiden was detected in 19% (23/120) of the control population compared with 27% (29/106) of all patients with Behçet disease (P = .13). However, among patients with Behçet disease who had ocular disease (75/106), the prevalence of FV Leiden was significantly higher (32%) than it was in control subjects (P = .04). Furthermore, ocular patients with Behçet disease in whom retinal occlusive disease was observed (25/75) had the highest expression of FV Leiden (44%). CONCLUSIONS: These data suggest that FV Leiden may be an additional risk factor for the development of ocular disease and, in particular, retinal vaso-occlusion, and it may contribute to the poor visual outcome in these patients.

Behçet's disease in patients of west African and Afro-Caribbean origin.

AIM: To report the presence of Behçet's disease with ocular involvement in patients of west African or Afro-Caribbean origin. METHODS: Case series of eight patients reporting to a tertiary uveitis service. RESULTS: Eight patients with typical features of the disease are presented. Six of the eight patients were tested and found to be HLA-B51 negative. CONCLUSION: Behçet's disease has only been reported in sporadic case reports in the indigenous west African and Afro-Caribbean populations, in whom the incidence of HLA B51 is also very low. A series of patients from the London region presented with the typical symptoms and signs of disease, most of whom were also HLA B51 negative. The presence of disease in this population, when absent in the indigenous population, suggests either that ascertainment of disease is poor in the indigenous population or that acquired factors may be important in the aetiology of the disease.

Behçet's disease: from Hippocrates to the third millennium.

Behçet's disease (BD) is characterised by recurrent episodes of orogenital aphthae, systemic vasculitis, and systemic and retinal venous thrombosis. An association between HLA-B51 and BD was first identified over 20 years ago, but recently identified gene associations implicate regions both within and without the MHC in the immunological events underlying the lesions in BD. These include allelic variants within the tumour necrosis factor gene region and within the MHC class I chain related gene region, the factor V Leiden mutation, which is associated with retinal vascular occlusion, and alleles of the intercellular adhesion molecule gene. No single causative gene for BD has emerged; the evidence indicates that the underlying immune events in BD are triggered by a microbial antigen and subsequently driven by genetic influences which control leucocyte behaviour and the coagulation pathways. Knowledge of these risk factors may permit a more accurate prognosis for a given patient, and identify new pathways for more targeted intervention than is currently available.

Hypopyon uveitis and iris nodules in non-Hodgkin's lymphoma: ocular relapse during systemic remission.

We report the case history of a patient with peripheral non-Hodgkin's lymphoma in systemic remission, who presented with a pseudohypopyon and iris nodules. Iris biopsy confirmed the presence of intraocular tumour, which subsequently responded to orbital radiation. Ocular remission, however, was soon followed by a systemic relapse, suggesting that the eye may act as a sanctuary site for tumour cells during chemotherapy, and that cells may metastasize from the eye back to the periphery during systemic remission.

Soluble adhesion molecules in Behçet's disease.

BACKGROUND: Behçet's disease (BD) may lead to blindness in up to 25% of eyes. Soluble (s)ICAM-1 but not sVCAM-1 is associated with relapse in idiopathic uveoretinitis and is reported to be raised in BD patients. We have investigated the levels of sICAM-1 and sVCAM-1 in Palestinian patients with BD and related them to both ocular and systemic disease activity and to immunosuppressive treatment. METHODS: A total of 51 patients (43 male, 8 female; mean ages 29.8 & 31.9 yr) were examined at the St John Ophthalmic Hospital during a one year period (135 consultations). Disease activity was determined from history and standard ocular examination. Anterior uveitis, vitritis and retinal vasculitis acted as markers of ocular inflammation. Peripheral venous sICAM-1 and sVCAM-1 levels were determined by standard ELISA. A total of 53 healthy age- and sex-matched clinic staff members acted as controls. RESULTS: sICAM-1 and sVCAM-1 were both significantly lower in patients on systemic immunosuppression than in those off treatment (p < 0.001). Among patients off systemic treatment, sICAM was higher in the group with active systemic disease but quiet eyes (p = 0.003), but not in those with active ocular disease (p = 0.09), compared to controls. sVCAM was not raised in either group. CONCLUSION: Systemic immunosuppression was associated with reduced sICAM-1 and sVCAM-1, supporting a role for adhesion molecules in the pathogenesis of BD. sICAM-1 levels were raised in association with inflammatory features implicating endothelial activation in active BD. The mean sICAM-1 was higher in active ocular patients than controls.

Periocular implantation cysts: a late complication of ophthalmic surgery.

PURPOSE: To describe the clinical characteristics for a group of patients presenting with acquired implantation cysts following periocular surgery. METHODS: Retrospective, non-comparative series of patients who underwent surgery at Moorfields Eye Hospital for orbital implantation cysts, with review of clinical case notes and imaging. RESULTS: Twenty-three patients (13 male; 57%) were treated between 1997 and 2011 with cystic lesions that presented at an average interval of 22.7 years after previous periocular surgery. The age at presentation ranged from 14 to 76 years (mean 45.3; median 46 years). Five patients had a history of prior enucleation or evisceration (with ball implantation in 4/5 cases) and six had undergone orbital fracture repair with either a silicone or titanium plate. Eight patients had prior strabismus surgery, three had retinal detachment repair with either a scleral buckle or vitrectomy, and one patient had repair of a conjunctival laceration in childhood. All but one patient underwent either wide marsupialisation or complete excision of the implantation cysts, with no record of recurrence. Visual acuity improved after surgery in a half of the patients, whereas remaining unchanged in the remainder. CONCLUSION: Orbital implantation cysts constitute a relatively rare complication of periocular surgery and can present decades after the causative procedure.

Acute presentation of vascular disease within the orbit-a descriptive synopsis of mechanisms.

Vascular events in the orbit can present with an acute onset of symptoms, and, if untreated, raised orbital pressure and reduced arterial perfusion can lead to loss of orbital functions. Such events are commonly due to haemorrhage, but can also be due to arterio-venous shunts and very rarely arise from intraorbital vascular occlusion. The likely diagnosis is often evident on taking a thorough history and examination and in most cases, after appropriate imaging, the process can be monitored for progression. Visual failure is usually due to high pressure at the orbital apex, resulting in ischaemic optic neuropathy, and any evidence of a persistent significant visual impairment should prompt intervention.

Acute thyroid eye disease (TED): principles of medical and surgical management.

The active inflammatory phase of thyroid eye disease (TED) is mediated by the innate immune system, and management is aimed at aborting this self-limited period of autoimmune activity. In most patients with TED, ocular and adnexal changes are mild and management involves controlling thyroid dysfunction, cessation of smoking, and addressing ocular surface inflammation and exposure. In patients with acute moderate disease, this being sufficient to impair orbital functions, immunosuppression reduces the long-term sequelae of acute inflammation, and adjunctive fractionated low-dose orbital radiotherapy is used as a steroid-sparing measure. Elective surgery is often required following moderate TED, be it for proptosis, diplopia, lid retraction, or to debulk the eyelid, and this should be delayed until the disease is quiescent, with the patient stable and weaned off all immunosuppression. Thus, surgical intervention during the active phase of moderate disease is rarely indicated, although clinical experience suggests that, where there is significant orbital congestion, early orbital decompression can limit progression to more severe disease. Acute severe TED poses a major risk of irreversible loss of vision due to marked exposure keratopathy, 'hydraulic' orbital congestion, or compressive optic neuropathy. If performed promptly, retractor recession with or without a suture tarsorrhaphy protects the ocular surface from severe exposure and, in patients not responding to high-dose corticosteroid treatment, decompression of the deep medial orbital wall and floor can rapidly relieve compressive optic neuropathy, as well as alleviate the inflammatory and congestive features of raised orbital pressure.

'Stalled' periocular necrotising fasciitis: early effective treatment or host genetic determinants?

BACKGROUND: Necrotising fasciitis (NF) is a devastating disease with considerable mortality and morbidity, and early aggressive surgical debridement of devitalised necrotic tissues has traditionally been advocated. METHODS: We describe three patients who were referred from other units several weeks after developing periocular necrotising fasciitis; in all the three, the disease had been managed medically without surgical debridement, with apparent 'stalling' of the inflammatory process despite persistent necrotic periocular tissue. RESULTS: Following 'elective debridement' of the devitalised tissues and reconstruction with local flaps, all achieved a satisfactory aesthetic result. DISCUSSION: The role of host genetic determinants, polarised cytokine responses, and early, effective medical treatment in patients with atypical 'disease phenotypes' in NF are discussed.

Cranio-orbital primary intraosseous haemangioma.

PURPOSE: Primary intraosseous haemangioma (IOH) is a rare benign neoplasm presenting in the fourth and fifth decades of life. The spine and skull are the most commonly involved, orbital involvement is extremely rare. We describe six patients with cranio-orbital IOH, the largest case series to date. PATIENTS AND METHODS: Retrospective review of six patients with histologically confirmed primary IOH involving the orbit. Clinical characteristics, imaging features, approach to management, and histopathological findings are described. RESULTS: Five patients were male with a median age of 56. Pain and diplopia were the most common presenting features. A characteristic 'honeycomb' pattern on CT imaging was demonstrated in three of the cases. Complete surgical excision was performed in all cases with presurgical embolisation carried out in one case. In all the cases, histological studies identified cavernous vascular spaces within the bony tissue. These channels were lined by single layer of cytologically normal endothelial cells. DISCUSSION: IOCH of the cranio-orbital region is rare; in the absence of typical imaging features, the differential diagnosis includes chondroma, chondrosarcoma, bony metastasis, and lymphoma. Surgical excision may be necessary to exclude more sinister pathology. Intraoperative haemorrhage can be severe and may be reduced by preoperative embolisation.

Discoid lupus erythematosus of the periorbita: clinical dilemmas, diagnostic delays.

PURPOSE: Untreated periocular discoid lupus erythematosus (DLE), though very rare, may lead to significant morbidity with lid deformities, trichiasis, and symblepharon formation. We present the largest reported cohort of patients with biopsy-proven DLE solely affecting the periorbital region. METHODS: Observational case series of patients managed over a 7-year period (2004-10). RESULTS: Seven patients (one male) presented to the Adnexal Service at Moorfields Eye Hospital at a median age of 47 years (range 23-71 years); median interval from symptom onset to biopsy-proven diagnosis was 38 months (range 6-86 months). Changes in peripheral skin were present in 1 patient (occurring after the initial eyelid presentation) and the presenting periocular features were dissimilar across the group, these included: chronic blepharo-conjunctivitis, madarosis, atypical chalazia, depigmentation of the eyelid margin, or marked, persistent periocular oedema with dacryoadenitis.Two cases settled spontaneously, but five required systemic hydroxychloroquine or intralesional corticosteroid injections. CONCLUSION: Periorbital DLE is rare and very varied in its presentation, the protean manifestations often resulting in significant diagnostic delay. All patients with unusual periocular skin disease and those with a refractory inflammatory dermopathy, should undergo biopsy of involved tissue(s), thus leading to earlier diagnosis and prevention of permanent cicatricial periocular changes.