Gillian G.W. Adams

Botulinum Toxin A-induced Protective Ptosis in Corneal Disease

Botulinum toxin A produces a temporary, flaccid ptosis when injected into the levator palpebrae superioris muscle. The resulting protective ptosis was used to aid healing in 21 cases of indolent ulceration, and, prophylactically, in 4 cases of neuroparalytic keratitis. Of the indolent ulcers, 90% healed completely. In all but one case, the cornea was covered completely by the lid and complete ptosis was produced in 75% of cases in an average of 3.6 days, lasting for 16 days on average before recovery began. Recovery of levator function was complete in 8.5 weeks on average. Superior rectus underaction was seen in 68% of cases but this recovered completely in all cases in an average of 6 weeks. Impression cytology showed a trend toward normal conjunctival morphology as healing progressed.

Glaucoma following congenital cataract surgery – the role of early surgery and posterior capsulotomy

BackgroundTo determine the rate of glaucoma following congenital cataract surgery at Moorfields Eye Hospital (MEH), and to investigate potential risk factors for glaucoma in our case series.MethodsA retrospective case notes review was undertaken of all congenital cataract lensectomies performed at MEH between 1994 and 2000. The following parameters were ascertained: age at surgery, unilateral or bilateral cataract, whether a posterior capsulotomy (PC) was performed at the time of surgery, whether an intraocular lens (IOL) was inserted, duration of follow-up, and if aphakic glaucoma (AG) developed. All lensectomies were performed through a limbal incision by a single consultant surgeon.ResultsA total of 47 subjects were identified – 40 patients with bilateral cataracts and 7 with unilateral. Of the 40 bilateral cataract patients, 76 eyes had lensectomies; with 37 of these patients (71 lensectomies) having at least 5 year follow-up. Based on patient count, the 5 year risk of AG in at least one eye following surgery was 21.6%. Based on eye count, the 5 year risk of AG after lensectomy was 15.5%. The average age at surgery of patients who did not develop AG, and had at least 5 years follow-up, was 28.7 months (range 2 weeks to 6 years), with 20% having surgery within the first month of life. In comparison, the average age at surgery of patients with at least 5 years follow-up, who developed AG was 1.6 months (range 2 weeks to 7 months), with 60% having surgery within the first month of life. In subjects with at least 5 years follow-up, a PC rate of 100% was identified in the eyes that developed AG, compared to 61% in eyes that did not develop AG. An IOL was inserted in O% of eyes with AG, compared to 57% in eyes that did not develop AG. Onset of AG ranged from one month post surgery to 7 years, with an average yearly incidence of 5.3%.ConclusionEarly surgery in patients with bilateral cataracts is associated with a marked increase in risk of AG. Our data suggest that an intact posterior capsule may be associated with a lower rate of AG.

The role of botulinum toxin A in acute-onset esotropia.

OBJECTIVE To establish the effectiveness of botulinum toxin A (BTXA) in the treatment of patients with acute acquired concomitant esotropia. DESIGN Retrospective, interventional, noncomparative case series. PARTICIPANTS Fourteen patients presenting to the Strabismus and Pediatric Service at Moorfields Eye Hospital with acute-onset esotropia over a 6-year period (1991-1997). INTERVENTION 2.5 units of BTXA injected into the unilateral medial rectus muscle of the deviating eye under electromyographic control. MAIN OUTCOME MEASURES Pre- and postinjection angle of deviation, pre- and postinjection stereopsis, final level of stereopsis achieved, and whether corrective squint surgery was later required. RESULTS Fourteen patients were identified, of whom eight were male and six female. The mean age at presentation was 5.4 years, and the average time from onset to attending the clinic was 18 weeks. The mean time from onset of acute esotropia to injection was 32.5 weeks. All patients, except one, showed considerable improvement in their manifest deviation after one injection of BTXA. Eight patients (57%) maintained high-grade stereopsis of 120 seconds of arc or better and long-term ocular alignment with toxin treatment alone. In total, 11 patients (79%) gained improved stereopsis and maintained satisfactory ocular alignment with toxin therapy and did not require squint surgery. Two patients (14%) did not maintain a stable ocular position after toxin treatment and later required squint surgery, gaining good ocular alignment and high-grade stereopsis. The one patient who did not respond to the initial BTXA injection refused all further treatment. The mean follow-up time was 22 months. CONCLUSIONS Botulinum toxin therapy has a definite role in the treatment of children with acute-onset esotropia. It may well obviate the need for squint surgery. The safety and ease of administration of this treatment add to its merits.

Intensive occlusion therapy for amblyopia

AIM To study the effects of supervised inpatient occlusion treatment for amblyopia in children who had failed to respond to outpatient treatment. METHODS A retrospective study of 39 children admitted to a paediatric ophthalmic ward for 5 days of supervised intensive occlusion therapy having previously failed to respond to outpatient occlusion. Visual acuity of amblyopic and fellow eyes was recorded at each clinic visit before admission, daily during admission, and at each outpatient visit after discharge. RESULTS There was no significant overall improvement in visual acuity during a mean of 9 months of attempted outpatient occlusion before admission. During the 5 days of admission 26 children (67%) gained at least one line of acuity in their amblyopic eye and five (13%) gained three or more lines (mean gain 1.03 Snellen lines). The acuities of both the amblyopic and fellow eyes subsequently improved with continuing part time patching as an outpatient, including in nine of the children who did not respond during admission. At the last recorded visit, at a median time of 14 months after discharge, 13 (33%) of the patients had an acuity of at least 6/12 in their amblyopic eye. CONCLUSIONS The acuity of amblyopic eyes did not improve without effective treatment. Subsequent supervised inpatient occlusion therapy was effective in the majority of the children.

Batten disease: features to facilitate early diagnosis

Aims: To ascertain the clinical and electrophysiological features in patients with juvenile neuronal ceroid lipofuscinosis (jNCL/Batten disease) and to identify those features that facilitate early diagnosis. Methods: Nine patients with jNCL were identified retrospectively and their case notes reviewed. All had undergone an extensive clinical examination, including electrophysiology. Blood and molecular genetic testing confirmed the diagnosis. Results: Age at onset ranged from 4–8 years. At presentation, two of nine patients had normal fundi; only two of nine patients had a bull’s eye maculopathy. The electroretinogram (ERG) findings in this series included undetectable rod specific ERGs, an electronegative maximal response, reduced and delayed cone flicker ERGs, reduction in the b:a ratio in the photopic single flash ERG, and an undetectable pattern ERG. Vacuolated lymphocytes on peripheral blood film testing were present in eight of nine patients. Five of eight patients were homozygous for the 1.02 kb deletion on the CLN3 gene on molecular genetic testing; two of eight patients were heterozygous for that deletion. Conclusion: jNCL should be considered in children of 10 years and under presenting with visual loss and fundal changes ranging from normal through to pigmentary/atrophic changes or a bull’s eye maculopathy. Electrophysiology may suggest jNCL. Although currently untreatable, early diagnosis is important to institute appropriate counselling and support.

In vitro fertilisation and stage 3 retinopathy of prematurity

Purpose: To re-examine the risk of children born by assisted conception developing stage 3 retinopathy of prematurity (ROP) and to define whether the risk of ROP varies with the method of assisted conception.Methods: This was a retrospective study carried out between December 1995 and December 1998 of infants in a single neonatal unit serving the Brent and Harrow area of North West Thames requiring screening and treatment of ROP. The infants screened were identified from the ROP screening database. Those conceived by in vitro fertilisation (IVF) and other forms of assisted conception were identified by reviewing the neonatal notes and the maternal obstetric records. Birth weight, gestational age and the type of assisted conception were recorded. The presence or absence of any stage of ROP, its location and severity and the cases requiring treatment were recorded.Results: One hundred and seventy-nine infants fulfilled the screening criteria during this period. Acute ROP was detected in 32.4% (58 infants) and stage 3 ROP developed in 15.6% (28 infants). Twenty-one infants (11.7%) were born after assisted conception, with 12 (6.7%) being conceived by IVF. The others were conceived on clomiphene (8) or after intrauterine insemination (1). Assisted conception accounted for 21.4% of all those reaching stage 3 disease and 28.6% of those infants requiring treatment. Of the 12 infants conceived by IVF, 41.6% (5 infants) developed acute ROP which progressed to threshold ROP in all infants (100%). Of the assisted conception babies requiring treatment for ROP, 83.3% were conceived by IVF. The other child had been conceived on clomiphene. The gestational age and birth weight of the IVF infants reaching stage 3 ROP were 26.6 ± 0.89 weeks and 937 ± 170.2 g. The gestational age and birth weight in the rest of the infants reaching stage 3 ROP were lower than in those conceived by assisted conception (25.739 ± 1.13 weeks and 735.29 ± 117.70 g); however, this did not approach statistical significance (p = 0.35 and p = 0.13, respectively).Conclusions: In this study 11.7% of the group requiring screening were conceived by assisted conception. Of all babies requiring treatment for ROP, 28.6% were born after assisted conception. Of the assisted conception group, 83.3% were conceived by IVF. Assisted conception using IVF rather than other techniques appears to be the major risk factor for the development of threshold ROP. We would advise increased vigilance when screening babies conceived by the IVF methods of assisted conception.

Relationships between Maternal Ethnicity, Gestational Age, Birth Weight, Weight Gain, and Severe Retinopathy of Prematurity

OBJECTIVE To develop an algorithm that allows advanced identification of infants requiring treatment for retinopathy of prematurity (ROP). STUDY DESIGN A retrospective observational study was performed at 2 tertiary neonatal units serving a multiethnic population in the UK, using data on 929 infants eligible for ROP screening. The relationships between study variables and the risk of developing ROP requiring treatment were analyzed using multiple logistic regression. RESULTS After applying exclusion criteria, data from 589 infants were analyzed; of these, 57 required laser treatment. The proportion of treated infants was 5.9% of those born to black mothers, 9.39% of those born to white mothers, and 12.8% of those born to Asian mothers (P = .047). Multiple logistic regression showed that gestational age, birth weight, maternal ethnicity, and early weight gain were predictors for the development of ROP requiring treatment, with maternal ethnicity having greater predictive power compared with early weight gain. We developed an algorithm for predicting the development of ROP requiring treatment with sensitivity, specificity, and positive and negative predictive values of 100%, 65.7%, 23.8%, and 100%, respectively. CONCLUSION Gestational age, birth weight, early weight gain, and maternal ethnicity are important predictors for the development of ROP requiring treatment. In a multiethnic population, an algorithm to predict development of ROP requiring treatment should include maternal ethnicity. If confirmed through prospective studies, this algorithm could reduce the number of opthalmologic examinations performed for ROP screening.

Anomalies of binocular function in patients with longstanding asymmetric keratoconus.

AIMS To study binocular function in patients with longstanding asymmetric keratoconus. METHODS In 20 adult patients with longstanding asymmetric keratoconus managed with a scleral contact lens a full clinical and orthoptic assessment was performed with and without the scleral contact lens in the poorer eye. RESULTS All 20 patients had a corrected acuity of at least 6/9 in their better eye. With the scleral lens in situ the acuity of the poorer eye ranged from 6/6 to 6/60 and without the lens from 6/18 to hand movements. Patients were aged from 18 to 68 years and had worn a scleral contact lens for between 3 and 106 months. Without the contact lens in their poorer eye all patients had a small exotropia and all showed suppression, with the exception of one patient who had a right hypertropia with diplopia. With the scleral lens in situ 12 patients had an exophoria or esophoria, six a microexotropia, and two a manifest exotropia with suppression. CONCLUSIONS Binocular function breaks down in some adult patients with longstanding asymmetric keratoconus. This is probably caused by longstanding unilateral visual deprivation. There are similarities to the breakdown of binocular function seen in some patients with a longstanding dense unilateral adult onset cataract who can develop intractable diplopia following cataract surgery.

Retinal haemorrhages in an infant following RetCam screening for retinopathy of prematurity

Retinal haemorrhages in an infant following RetCam screening for retinopathy of prematurity

Factors associated with quality of life and mood in adults with strabismus

Background/Aims To explore the factors associated with the mood and quality of life (QoL) of patients with strabismus due to undergo realignment surgery. Methods A cross-sectional study was undertaken with adult patients. Along with demographic, clinical and psychosocial process variables, the Hospital Anxiety and Depression Scale and AS-20 QoL measures were administered. Regression models were used to identify the factors associated with QoL and mood. Results Of the 220 participants, 11% were experiencing clinical levels of depression, and 24% clinical anxiety. This is in line with other forms of facial disfigurement but higher than other chronic diseases. Although mood and QoL were associated with age and diplopia, it was beliefs and cognitions which were more consistently associated with well-being. This included feelings of social anxiety and avoidance, a belief that strabismus has negative consequences, poor understanding of strabismus, social support, fear of negative evaluation and the perceived visibility of their condition. Conclusions Psychosocial rather than clinical characteristics were identified as determinants of well-being in this population. It is important for clinicians planning surgery to be aware of these factors which could influence outcomes. Longitudinal studies need to be conducted to explore the direction of causality before interventions to improve well-being are developed and evaluated.