[Low complication rate with cataract operations carried out by registrars in ophthalmology]. / Laag complicatierisico bij cataractoperaties uitgevoerd door artsen in opleiding tot oogarts.

OBJECTIVE: To determine the complication rate of cataract operations and learning curve of registrars in ophthalmology. DESIGN: Retrospective, descriptive investigation. METHOD: 982 consecutive cataract operations performed by registrars in ophthalmology at University Medical Centre Utrecht and the Meander Medical Centre, Amersfoort, the Netherlands, were evaluated for complications. Of patients in whom a complication occurred, the nature of the complication, any possible follow-up operation, and best corrected visual acuity before and following operation were assessed, as well as the stage of training the registrar was at. RESULTS: The most important complications occurred in the following frequencies: endophthalmitis 0%, posterior capsule rupture with vitreous loss 1.32%, dropped nucleus 0.2%. Four patients required a second operation. There were no statistically significant differences in complication rate between the three phases of the training. In all patients with complications the best corrected visual acuity was more than 0.5. CONCLUSION: The complication rate was smaller than described in the literature and comparable with rates known for ophthalmologists. Training in cataract surgery does not lead to irresponsible risks for the cataract patient. The combination of training in both a university hospital and a general hospital has several advantages for a registrar in ophthalmology.

[Determining factors for deciding whether or not to treat refractive errors and cataract in people with a learning disability]. / Determinanten van het al dan niet behandelen van refractieafwijkingen en cataract bij mensen met een verstandelijke beperking.

OBJECTIVE: A study into the treatment of refractive errors and cataract in a selected population with learning disabilities. Design. Retrospective. METHOD: In the years 1993-2003, 5205 people (mean age: 39 years) were referred to the visual advisory centre of Bartiméus (one of three institutes for the visually impaired in the Netherlands) by learning disability physicians and were assessed ophthalmologically. This assessment consisted of a measurement of visual acuity and refractive error, slitlamp examination and retinoscopy, and was performed at the client's accommodation. Advised treatment for spectacle prescriptions and referral for cataract surgery were registered. RESULTS: Refractive errors were found in 35% (1845/5205) of the patients with learning disabilities; 49% (905/1845) already wore spectacles; another 14% (265/1845) were prescribed spectacles for the first time. Of those with presbyopia, 12% (232/1865) had reading glasses and 10% (181/1865) were given a first prescription for spectacles. The most important determinant for not prescribing spectacles was: presence of severe learning disability (odds ratio (OR): 3.7). Cataract was present in 10% (497/5205) of the population; 399 patients were advised to be referred for surgery, 55% (219/399) were referred ofwhom 26% (57/219) had surgery. Moderately severe bilateral cataract was the only determinant of cataract surgery (OR: 7.8). CONCLUSION: Refractive errors and cataract were not always treated in this group. One of the reasons for non-treatment of refractive errors was a severe learning disability. The reason for treatment or non-treatment in patients with cataract was less clear.

6,220 institutionalised people with intellectual disability referred for visual assessment between 1993 and 2003: overview and trends.

AIMS: To summarise the results of visual performance tests and other data of institutionalised people with intellectual disability referred to a visual advisory centre (VAC) between 1993 and 2003, and to determine trends in these data. METHODS: A retrospective medical record review was undertaken of 6,220 consecutive people examined ophthalmologically according to a standard protocol by one VAC that specialised in visual assessment and treatment of people with intellectual disability, between 1993 and 2003. chi2 test for linear trend was used and linear regression coefficients were calculated. RESULTS: The proportion of people aged > or =50 years increased from 19.3% to 34.2% between 1995 and 2003 (p<0.001); the combined figure of severe or profound intellectual disability decreased from 80.0% to 52.6% (p<0.001); the proportion of mobile people increased from 52.1% to 98.0% (p<0.001); the combined proportion of people with visual impairment or blindness decreased from 70.9% to 22.9% (p<0.001), and that of people with visual disorders decreased from 89.6% to 75.3% (p<0.001). Causes of intellectual disability were identified in 58.4% people; 20.8% had Down's syndrome. CONCLUSION: Many ocular diagnoses were found, indicating the need for ophthalmological monitoring. Specialised centres are helpful, because assessment and treatment of people with intellectual disability is complicated and time consuming. Protocols for efficient referral will have to be developed. A major task lies ahead to improve the treatment rates of refractive errors, cataract and strabismus, and to find specific causes of intellectual disability.

Chiasmal misrouting and foveal hypoplasia without albinism.

BACKGROUND/AIMS: To present the ophthalmological and electrophysiological characteristics of three darkly pigmented, female patients with misrouting and foveal hypoplasia. One of the patients had primary ciliary dyskinesia and situs inversus totalis (Kartagener syndrome). METHODS: Fundus photographs were taken and the angles at which the main temporal arterial branches leave the optic nerve head (ONH) were analysed. Optical coherence tomography (OCT) was performed through the presumed foveal region. Pattern onset visually evoked potentials (VEPs) (check sizes 60', 40/400 ms) were recorded and the chiasmal coefficient was calculated to detect misrouting. RESULTS: Fundus photography showed normally pigmented fundi with absence of the usual foveal hyperpigmentation, foveal avascular zone, and macular and foveal reflexes. On OCT no foveal pit was found. The VEP recordings showed the largest positive CI component over the right hemisphere for the left eye, and over the left hemisphere for the right eye, with the CI almost absent over the ipsilateral hemispheres. The differential derivations showed opposite polarity for the recordings of the two eyes. The chiasmal coefficients of all three patients were significantly indicative of misrouting (-0.99, -0.91, and -0.99, respectively). CONCLUSION: Based on the investigations in these patients the authors propose the hypothesis that foveal hypoplasia and misrouting exist as a distinct entity, and do not comprise the exclusive hallmark of albinism. The findings suggest that misrouting may exert a retrograde influence on foveal development.

[Optical coherence tomography, an important new tool in the investigation of the retina]. / Optische-coherentietomografie: een belangrijke aanwinst voor het onderzoek van het netvlies.

Optical coherence tomography, an important new tool in the investigation ofthe retina Recently optical coherence tomography (OCT) has been introduced into the field of ophthalmology to enhance visualization ofthe various retinal layers. OCT uses interference of near infra-red, low-coherent light reflected from the retina and from a reference mirror, to create transverse sections of the retina. The strength of the signal is a measure of the reflection of the retinal layer which is situated at the same distance from the light source as the mirror. Accurate placement ofthe mirror gives a transverse view of the retina with an axial resolution of 10 microm, with which the resolution of a histological section can be approximated. OCT is used in ophthalmic diseases such as macular oedema, macular holes and other abnormalities on the retinal-vitreous border, following photodynamic therapy and in treating glaucoma. OCT has been used to detect early glaucoma by measuring the thickness of the retinal nerve fibre layer. OCT and fluorescence angiography complement one another as diagnostic methods but OCT is superior in the diagnosis of macular oedema and macular holes. OCT provides images of retinal pathology which can easily be interpreted by both ophthalmologists and patients.

Cell-mediated immunity against human retinal extract, S-antigen, and interphotoreceptor retinoid binding protein in onchocercal chorioretinopathy.

Autoimmune mechanisms are thought to be involved in the pathogenesis of onchocercal chorioretinopathy. Cell-mediated immune responses to human retinal S-antigen, interphotoreceptor retinoid binding protein (IRBP), and crude retinal extract were investigated in patients with onchocerciasis from Sierra Leone, West Africa using a two-step migration-inhibition factor assay. Patients were subdivided into three groups: (1) without ocular involvement (n = 10), (2) with ocular onchocerciasis limited to the anterior segment (n = 19), and (3) with onchocercal chorioretinopathy (n = 21). A group of endemic controls (n = 25) from Sierra Leone were also studied. The cellular immune response to concanavalin A (Con A) was measured to assess the general capacity of lymphocytes to respond to a mitogen. Four of 50 (8%) patients with onchocerciasis and four of 25 (16%) endemic controls reacted with at least one retinal antigen. From the patients with onchocercal chorioretinopathy two of 21 (10%) showed a positive cellular response. The general mitogen response tested with Con A was positive in all these individuals. A role for an antiretinal autoimmune mechanism in the pathogenesis of onchocercal chorioretinopathy, as studied with human S-antigen, IRBP, or crude retinal extract, could not be shown because the cellular response to these antigens did not differ in patients with or without onchocercal chorioretinopathy or in endemic controls.

Humoral autoimmune response against S-antigen and IRBP in ocular onchocerciasis.

Autoimmune mechanisms are thought to play a role in the pathogenesis of the chorioretinal changes in ocular onchocerciasis. In this study, the involvement of autoimmunity against retinal antigens in developing chorioretinitis was investigated. Serum levels of autoantibodies, directed against human S-antigen and interphotoreceptor retinoid-binding protein (IRBP), were determined in patients with onchocerciasis (n = 46) and endemic controls (n = 38) from Sierra Leone with the use of an enzyme immunoassay. In both groups high levels of anti-human S-antigen and IRBP antibodies were detected. No relationship could be demonstrated between the antiretinal antibody level and the occurrence of chorioretinitis in onchocerciasis. The levels of both anti-human S-antigen and IRBP antibodies were significantly higher in patients with onchocerciasis compared with endemic controls (P less than 0.001). Cross-reactivity of antiretinal antibodies with parasitic antigens could not be demonstrated as a possible explanation for the higher levels in patients with onchocerciasis. No correlation was found between the levels of antibodies of different classes against the crude Onchocerca volvulus, the egg antigen, or the microfilariae and the antiretinal antibody levels. Furthermore, in a panel of 13 different monoclonal antibodies directed against O. volvulus, only one showed a slight anti-human IRBP reactivity and none reacted with S-antigen. The immune response against the two retinal antigens investigated was not specific for onchocerciasis because high antibody levels were also found in patients with Bancroftian filariasis from Papua, New Guinea, and Surinam.(ABSTRACT TRUNCATED AT 250 WORDS)

Concurrent parasitic infections in onchocerciasis and the occurrence of adverse reactions after ivermectin treatment.

Ivermectin is a safe, effective, and relatively well-tolerated drug for the treatment of human onchocerciasis. However, due to side effects of the drug, large-scale ivermectin distribution without medical supervision is not recommended. The mechanisms involved in the pathogenesis of ivermectin-induced adverse reactions are not yet known. Since onchocerciasis patients are likely to have concurrent parasitic infections, we investigated whether side effects that occur after ivermectin treatment could be related to the presence of parasite eggs and cysts in stool samples prior to treatment. One hundred twenty-nine onchocerciasis patients were treated with a single dose of ivermectin (150 micrograms/kg) and side effects were graded according to the classification of Greene and others. Stool samples were collected before and three days after treatment. A high percentage (80.5%) of the patients reported adverse effects (57% mild, 14.1% moderate, and 9.4% severe reactions). Most (95.1%) of the patients had one or more concurrent parasitic infections. No relationship could be found between the occurrence and extent of side effects and the severity of concurrent intestinal parasitic infections. However, side effects were significantly correlated with pretreatment microfilarial counts. Ivermectin treatment did not induce significant short-term changes in Trichuris trichiura or Schistosoma mansoni egg counts. However, a significant reduction in Ascaris lumbricoides egg counts and Entamoeba coli cyst loads was observed; a cure rate of 46% for cysts was reached. In contrast, hookworm egg production increased after ivermectin treatment. Further studies are required to verify ivermectin-induced changes in cyst and hookworm loads as well as the significance of these findings.

Ivermectin detection in serum of onchocerciasis patients: relationship to adverse reactions.

Ivermectin treatment of onchocerciasis patients can be accompanied by adverse reactions. Not much is known concerning the pathogenesis of these reactions. Previous studies have demonstrated that the occurrence and extent of adverse reactions are related to infection intensity. However, some severely infected patients experience relatively few adverse effects. The aim of the present study was to investigate whether this seeming discrepancy could be due to diminished ivermectin absorption. Ivermectin concentrations one and two days after treatment were measured by high-performance liquid chromatography in sera of 71 skin snip-positive onchocerciasis patients (21 without reactions, 25 with mild reactions, 14 with moderate reactions, and 11 with severe reactions). The overall mean +/- SD ivermectin concentrations one and two days after a single oral dose (150 micrograms/kg) were 16.4 +/- 6.4 and 6.6 +/- 3.1 ng/ml, respectively. The overall mean +/- SD half-life was estimated to be 19.9 +/- 8.6 hr. The data presented did not show a relationship between ivermectin concentrations and the grade of adverse reactions.

Ocular involvement in patients with onchocerciasis after repeated treatment with ivermectin.

We assessed ocular changes after therapy at six and 12 months with ivermectin (150 micrograms/kg of body weight) in a 12-month prospective study of 29 patients with ocular onchocerciasis and 15 patients with onchocerciasis without ocular involvement. The patients lived in a hyperendemic area in Sierra Leone, West Africa, where no vector control was instituted. Five months after initial treatment, the microfilarial load in skin and eyes had decreased significantly (P less than .0000), but 28 of 44 (63%) patients had positive skin-snip test results and nine of 29 (31%) patients with ocular involvement had active ocular disease. Twelve months after initial treatment, 15 of 41 (37%) patients had positive skin-snip test results and eight of 26 (31%) showed active ocular involvement. All patients with persistent ocular disease after therapy showed evidence of active onchocerciasis at that time, which suggests that a dose of ivermectin at six-month intervals is not sufficient for intensely infested patients with severe ocular disease. We developed an ocular involvement score to evaluate the patient's total ocular status and observed a significant relation between the pretreatment severity of ocular involvement and the persistence of active ocular disease after treatment with ivermectin.

Conjunctival excision or lamellar scleral autograft in 38 Mooren's ulcers from Sierra Leone.

Ulcus rodens corneae (Mooren's ulcer) was studied in 30 patients with 38 corneal ulcers. Although the clinical picture resembled Mooren's description, the West African type of ulcus rodens is different in that it has a higher prevalence, a tendency towards perforation, is most frequent in the age group 20-40 years, and is often associated with ankylostomiasis. Conjunctival excision with thermocoagulation gave some relief at the site of the ulcers, but recurrences at other places occurred in at least 52% of cases. Six eyes with a progressive iris prolapse and a flat anterior chamber were reconstructed with lamellar scleral autografts in the absence of donor corneas.

Circulating antibodies against corneal epithelium and hookworm in patients with Mooren's ulcer from Sierra Leone.

A relationship between the occurrence of Mooren's ulcer and hookworm infections was suggested by Kuriakose in 1963. Sixteen patients with clinical diagnosis of Mooren's ulcer and 15 local controls from Sierra Leone were tested with respect to serum immunoglobulin levels, circulating antibody to hookworm, circulating antibodies to corneal epithelium, stool smears, and eosinophil and lymphocyte levels. Both patients and healthy controls had circulating antibodies to corneal epithelium and to hookworm. In the controls the titres of hookworm antibodies were significantly lower than in the patients, though in both groups most people had intestinal parasite infestations as detected by the stool smear. Further investigation failed to demonstrate any other significant immune alteration in the patients as compared with local controls.

In-toto removal of a subretinal Cysticercus cellulosae by pars plana vitrectomy.

A case of subretinal cysticercosis was treated with laser coagulations round the cyst prior to surgery. In-toto removal of the living cysticercus was performed by pars plana vitrectomy. Two weeks after surgery 25/20 vision was regained. Histopathology of the cyst confirmed the clinical diagnosis.

Blindness from uveitis in a hospital population in Sierra Leone.

A retrospective study was conducted to assess the causes of blindness and visual impairment in patients who visited an eye hospital in Sierra Leone, West Africa, in 1989 and 1992. These data were compared with figures from 1981. Throughout the years, senile cataract was the major cause of blindness, followed by uveitis (including onchocerciasis). Uveitis remained the second most important cause of blindness in this population, despite the significant decrease in blindness from onchocerciasis (from 30% in 1981 to 15% in 1992). An increasing number of patients with uveitis from non-onchocercal origin was observed: almost 10% of the blindness found in 1992 was due to uveitis of non-onchocercal origin. A reduction in visual handicap in patients with non-onchocercal uveitis could be achieved if local hospitals could obtain more accurate diagnoses in these patients.

Aetiology of uveitis in Sierra Leone, west Africa.

BACKGROUND: In 1992, non-onchocercal uveitis caused 9% of blindness, 8% of visual impairment, and 11% of uniocular blindness among patients visiting an eye hospital in Sierra Leone, west Africa. The aim of this study was to determine the aetiology of uveitis in this population. METHODS: General and ophthalmic examination complemented by serum and aqueous humour analyses for various infectious agents was performed for 93 uveitis patients and compared with serum (n = 100) and aqueous humour (n = 9) analysis of endemic controls. RESULTS: At the initial examination, 45 patients (48%) proved to be severely visually handicapped. After clinical and laboratory analyses, an aetiological diagnosis was established for 49 patients (52%). Toxoplasma gondii was the most important cause of uveitis (40/93; 43%). Anti-toxoplasma IgM antibodies were detected in serum samples of seven of 93 patients (8%) compared with one of 100 controls (1%, p < 0.05). At least six patients (15%) with ocular toxoplasmosis had acquired the disease postnatally. Antibodies against Treponema pallidum were detected in 18 of 92 patients (20%) and in 21 controls (21%). Other causes of uveitis were varicella zoster virus (one patient), herpes simplex virus (two patients), and HLA-B27 positive acute anterior uveitis with ankylosing spondylitis (one patient), while one patient had presumed HTLV-I uveitis. CONCLUSIONS: In a hospital population in Sierra Leone, west Africa, uveitis was associated with severe visual handicap and infectious diseases. Toxoplasmosis proved to be the most important cause of the uveitis. Although the distribution of congenital versus acquired toxoplasmosis in this population could not be determined, the results indicate an important role of postnatally acquired disease. The results further suggested minor roles for HIV, tuberculosis, toxocariasis, and sarcoidosis as causes of uveitis in this population.

Long term changes in the visual fields of patients with temporal lobe epilepsy using vigabatrin.

AIM: To study the long term changes in the concentric contraction of the visual field in patients with temporal lobe epilepsy on vigabatrin medication. METHODS: Repeated Goldmann visual field examinations were compared in 27 patients with drug resistant temporal lobe epilepsy and concentric contraction of the visual field. Two groups were studied: 16 patients who had already stopped vigabatrin medication before surgery and 11 patients who continued vigabatrin medication. RESULTS: Concentric contraction of the visual field did not change in 16 patients who stopped vigabatrin before the first examination; there was slight but significant progress in visual field loss in 11 patients who continued the use of vigabatrin. CONCLUSION: Long term follow up of concentric contraction in this selected group of patients indicates that vigabatrin associated visual field loss is not reversible and that progression is possible when vigabatrin is continued.

Occurrence of retained lens fragments after phacoemulsification in The Netherlands.

PURPOSE: To determine the incidence of retained lens fragments after phacoemulsification in The Netherlands and to evaluate the effect of vitrectomy on this complication. SETTING: Eleven vitreoretinal centers in The Netherlands. METHODS: We performed a retrospective analysis of all patients with retained lens fragments (N = 70) who were referred for vitreoretinal surgery to 11 specialized centers. Seven patients (10%) were treated with medication alone, and 63 (90%) had pars plana vitrectomy. Minimum follow-up after vitrectomy was 3 months. RESULTS: The incidence of retained lens fragments in The Netherlands was calculated at 0.9/1000 cataract operations. Retained lens fragments occurred during the learning curve and with experienced surgeons. After medical or surgical treatment, visual acuity was 20/40 or better in 43 of 70 patients (61%). Uveitis disappeared in all cases. Retinal detachment occurred in 10 patients (14%). Attached retinal breaks were treated in an additional 5 patients. Corneal grafting was performed in 2 patients. Patients who had immediate vitrectomy did not have better functional results than patients in whom vitrectomy was delayed. The iris-fixated claw lens was implanted successfully when capsular support was insufficient. CONCLUSIONS: The introduction of phacoemulsification in The Netherlands is associated with an increase of patients with retained lens fragments. Retained lens fragments are complicated by an increased risk for retinal detachment and corneal decompensation. Vitrectomy resulted in a marked improvement of visual acuity and clearing of uveitis.

Analysis of aqueous humour in ocular onchocerciasis.

The development of an onchocercal chorioretinopathy from the first detectable signs to a full blown oncho fundus is not fully understood. We investigated the intraocular humoral immune response against Onchocerca volvulus, human S-antigen, IRBP and crude retinal extract (using an ELISA) by examining paired aqueous humour and serum samples obtained from onchocerciasis patients (without [n = 10] and with ocular symptoms [n = 8]) and endemic controls [n = 14] from Sierra Leone (West Africa). A local intraocular anti-retinal IgG antibody production could not be demonstrated in onchocerciasis patients, whether they had ocular symptoms or not. A significantly higher level of O. volvulus antibodies and IgG was measured in the aqueous of onchocerciasis patients with ocular involvement, as compared to patients without ocular symptoms (Mann-Whitney ranksum test; p less than 0.001 and p less than 0.02 respectively). Since interleukin-6 (IL-6) plays an essential role in the differentiation of B cells into immunoglobulin producing plasma cells, we therefore measured this cytokine in paired aqueous and serum samples. Elevated IL-6 levels were found in the aqueous of two out of eight onchocerciasis patients tested. In view of these findings it seems improbable that retinal autoimmunity is a major factor in the pathogenesis of onchocercal chorioretinopathy. The high intraocular levels of antibodies against the parasite suggest a direct involvement of the parasite in the pathogenesis of onchocercal chorioretinopathy.

Fatal rhinocerebral mucormycosis and diabetic ketoacidosis.

The typical features of a fulminating rhinocerebral infection by the fungus Rhizopus in a patient with diabetic ketoacidosis are described. The clinical presentation was characterized by an infection of the face, followed by rapid bilateral visual loss and massive cerebral infarction due to extensive vascular involvement. Early diagnosis is a prerequisite for successful treatment, which consists of control of the underlying disease, surgical debridement and systemic antifungal therapy.

Visual performance in specific syndromes associated with intellectual disability.

PURPOSE: To report visual performance in adults with specific causes of intellectual disability (ID) and to compare the test results to published reports. METHODS: In a large-scale multicenter epidemiologic study of sensory impairments in 1598 adults with ID, the authors performed ocular assessments in 1539 persons. They compared the test results of those with five specific genetic disorders (Angelman syndrome, Prader-Willi syndrome, fragile X syndrome, Williams-Beuren syndrome, and tuberous sclerosis). RESULTS: An overrepresentation of strabismus, low vision, and refractive errors was found. Apart from fragile X syndrome and Prader-Willi syndrome (with in general mild to moderate ID), the other syndrome groups contained one or more subjects with visual impairment or blindness. A number of them had never been seen by an ophthalmologist. CONCLUSIONS: The authors confirm a number of ocular features previously reported by other studies and suggest some additional ocular features. They found increased frequencies of treatable ophthalmologic conditions in the subgroups. Because reliable ocular assessment is feasible for 85% of persons with ID, the results are an incentive to address visual functioning in people with ID in order to correct ocular problems and maximize their possibilities.