The ophthalmic diagnosis and management of four siblings with Werner syndrome.

INTRODUCTION: Werner syndrome is a rare autosomal recessive disorder caused by mutations in the Werner syndrome WRN gene, on chromosome 8. Those affected manifest early the features of ageing. DISCUSSION: Cataract surgery is prone to post-operative complications in those with Werner syndrome. The development of cystoid macular oedema (CMO) is likely multifactorial. Patients with WS have diabetes mellitus type 2 which can contribute to macular oedema. There is a deposition of abnormal WRN proteins in the macula which also predisposes to macular oedema. The trauma of cataract surgery appears to be the main stimulus for the development of CMO. CMO may, as a result, be difficult to manage in Werner syndrome patients. CONCLUSION: Further study is needed to elucidate the precise role of retinal WRN protein expression in the development of CMO in those with Werner syndrome. A tailored and more successful approach to the treatment of CMO in such patients may result.

Bilateral failure of adduction following orbital decompression.

We report a case of bilateral complete failure of adduction following bilateral translid antralethmoidal orbital decompression. We believe the probable mechanism is neuropraxia (temporary dysfunction) of the third cranial nerves' supply to the medial recti, owing to these nerves' occupying an anatomically abnormal position. Partial recovery of adduction occurred over the ensuing six months.

Angioid streaks in beta thalassaemia minor.

We report what we believe to be the first recorded case of angioid streaks in a patient with beta thalassaemia minor. The occurrence of angioid streaks in a patient with a relatively normal iron balance and only very mild haemolysis may be explained by the combination of pregnancy with associated multiple transfusions in a myopic patient where an inherent defect in Bruch's membrane may exist.

Delayed ciliochoroidal detachment following intraocular lens implantation.

Three patients who had extracapsular cataract extractions with intraocular lens implants developed delayed ciliochoroidal detachments, which responded to systemic steroid therapy. This rare complication may have been due to ciliary sulcus fixation of the implant. The possible mechanism and treatment are discussed.

Visual impairment in a case of juvenile Paget's disease with pseudoxanthoma elasticum: an eleven year follow up.

An 11-year follow-up of a child presenting with Paget's disease of bone, and who subsequently developed pseudoxanthoma elasticum, is described. The influence of therapy on the ocular manifestations was disappointing. Calcitonin did not prevent the occurrence or progression of angioid streaks, and Argon laser photocoagulation of complicating subretinal neovascular membranes failed to prevent visual loss.

Student nurse satisfaction: implications for the common foundation programme.

This decade has seen a major restructuring of pre-registration nurse education within the higher education system that was costly and initially largely unpopular with qualified nurses and students. Major flaws have been identified in the common foundation programme (CFP) and student attrition rates remain too high. Our detailed survey of student satisfaction within the CFP shows that clinical placement is the most popular course component and that poor organization remains the worst aspect of the course. Teaching was more highly regarded than the organizational component of the course, although students found room for improvement. Core teaching modules such as bioscience and nursing interventions achieved better ratings than supporting modules, for example social policy or research and reflection on practice. The establishment of contact between personal tutors and students appeared to be slow. The described deficiencies have since been addressed in curriculum planning and the development of new course structure.

Post-transplant T cell chimerism predicts graft versus host disease but not disease relapse in patients undergoing an alemtuzumab based reduced intensity conditioned allogeneic transplant.

In this multicentre retrospective study we have studied the impact of T cell chimerism on the outcome of 133 patients undergoing an alemtuzumab based reduced intensity conditioning allograft (RIC). The median age of the patients was 50 years (range 42-55 years). 77 patients were transplanted using an HLA identical sibling donor while 56 patients received a fully matched volunteer unrelated donor graft. 64 patients had a lymphoid malignancy and 69 were transplanted for a myeloid malignancy. 38 patients (29%) relapsed with no significant difference in risk of relapse between patients developing full donor and mixed donor chimerism in the T-cell compartment on D+90 and D+180 post transplant. Day 90 full donor T cell chimerism correlated with an increased incidence of acute GVHD according to NIH criteria (p=0.0004) and the subsequent development of chronic GVHD. Consistent with previous observations, our results confirmed a correlation between the establishment of T cell full donor chimerism and acute GVHD in T deplete RIC allografts. However our study failed to identify any correlation between T cell chimerism and relapse risk and challenge the use of pre-emptive donor lymphocyte infusions (DLI) in patients with mixed T cell chimerism transplanted using an alemtuzumab based RIC regimen.

Management of endophthalmitis with retained intraocular foreign body.

AIMS: To assess the impact of primary injection of intravitreal antibiotics and delayed pars plana vitrectomy with removal of intraocular foreign body (IOFB) in patients with clinical features of bacterial endophthalmitis and retained IOFB. METHODS: Retrospective review of all patients with clinical features of infective endophthalmitis and a retained IOFB who had immediate injection of intravitreal antibiotics and delayed pars plana vitrectomy with removal of IOFB in two vitreo-retinal centres during 1995-2001. Nine patients were identified and minimum follow-up was 3 months. RESULTS: Four of the nine patients had a final visual outcome of 6/18 or better. One patient developed total retinal detachment. CONCLUSIONS: The current series suggests that immediate injection of intravitreal antibiotics with delayed removal of IOFB in eyes with clinical features of infective endophthalmitis and a retained IOFB is a possible alternative to immediate removal of IOFB. This management may be associated with preservation of the eye and restoration of useful visual acuity.

Incidence of idiopathic full-thickness macular holes in fellow eyes. A 5-year prospective natural history study.

PURPOSE: This study aimed to determine the incidence of idiopathic full-thickness macular hole (FTMH) in normal fellow eyes and to evaluate the role of electrodiagnostic and psychophysical tests in identifying eyes at risk. PATIENTS AND METHODS: A prospective longitudinal natural history study of a cohort of patients with unilateral holes and normal, asymptomatic fellow eyes without posterior vitreous detachment was conducted. Subjects underwent baseline examination, pattern reversal electroretinography, electro-oculography (EOG), and color contrast sensitivity (CCS) testing for protan, deutan, and tritan thresholds and were recalled for clinical examination at 18 months and 5 years. RESULTS: At baseline, 114 patients were examined. Eighty were available for review at 18 months, of whom 6 had full-thickness macular holes develop in the fellow eye. At 5 years, 67 of the remaining 74 patients who had not developed holes at 18 months were re-examined and a further 5 were found to have holes develop in the fellow eye. A posterior vitreous detachment without hole formation had developed in 20 fellow eyes at 5 years. Although mean pattern reversal electroretinography and EOG responses were within normal limits in affected and fellow eyes at baseline, mean CCS protan, deutan, and tritan thresholds were elevated significantly in affected eyes at baseline (P = 0.0001). Unaffected fellow eyes showed normal mean protan and deutan thresholds, but significantly elevated mean tritan thresholds (P = 0.01) at baseline. Mean tritan CCS loss was, however, similar in fellow eyes in which holes later developed and in fellow eyes in which holes did not. CONCLUSIONS: The Kaplan-Meier estimated risk of fellow eye involvement is 15.6% (range, 8.4%-22.3%; P = 0.05) at 5 years. Although electrodiagnostic and psychophysical testing was not predictive of fellow eye involvement, tritan CCS loss at baseline, in apparently normal fellow eyes, may indicate subclinical foveal dysfunction, the nature of which is unclear.