Patterns of ocular morbidity and blindness in leprosy a three centre study in Eastern India

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1 Lepr Rev (2006) 77, Patterns of ocular morbidity and blindness in leprosy a three centre study in Eastern India KIRSTEEN J. THOMPSON*, **, GWEN M. ALLARDICE***, G. RAJAN BABU þ, HELEN ROBERTS þþ, WALTER KERKETTA þþþ & AALO KERKETTA þþþ *Purulia Leprosy Hospital, Purulia, , West Bengal, India **Tennent Institute of Ophthalmology, Gartnavel General Hospital, Great Western Road, Glasgow G12 OYN, UK ***University of Strathclyde, Richmond St, Glasgow G1 1XQ, UK þ The Leprosy Mission, South Asia, CNI Bhavan, 16, Pandit Pant Marg, New Delhi , India þþ Premananda Memorial Leprosy Hospital, 259A, APC Road, Kolkata , West Bengal, India þþþ Muzaffarpur Leprosy Mission, PO Ramna, Kanhauli, Muzaffarpur, Bihar India Accepted for publication 8 March 2006 Summary Background Eye disease and visual impairment due to leprosy are not uncommon, but their prevalence and the risk factors for such pathology have yet to be fully characterized. Methods An observational study of leprosy-affected individuals in the community, in three districts in Eastern India was performed to determine the prevalence of ocular morbidity and blindness. The subjects were chosen by stratified random sampling, according to stage of treatment, or stage of post-treatment surveillance. Each subject underwent ophthalmic examination according to a standard pro-forma. Results Thirty-three out of 1137 (2.9%) subjects were blind according to the WHO classification, and 232/1137 (20.7%) had moderate visual impairment. The prevalence of blindness and visual impairment was greatest in one centre, Jhalda. Multivariate Logistic Regression analysis showed that longer duration of disease, more advanced treatment stage and older age were independently associated with blindness. One hundred and forty-one cases (28%) in Muzaffarpur, 95 cases (63%) in Saldoha and 378 cases (78%) in Jhalda had active or treated multibacillary leprosy. Discussion The prevalence of blindness in leprosy varies in different population groups, with differing patterns of leprosy-related ocular morbidity, blindness and disease type evident even in different areas of the same region. Leprosy screening and Correspondence to: K. J. Thompson ( kirsteenjt@doctors.org.uk) /06/ $1.00 q Lepra 130

2 Ocular morbidity and blindness in leprosy 131 surveillance programmes should include ocular examination as part of routine screening, particularly in individuals with a history of multi-bacillary disease. Individuals with known sight- threatening pathology should undergo continued active follow up. Local epidemiological studies would enable appropriate programme planning and efficient allocation of resources. Introduction It is now recognized that much ocular morbidity and blindness due to leprosy are potentially avoidable by screening, detection and treatment of early ocular changes. 1 Incorporation of regular eye examination into general leprosy care and early appropriate treatment have been advocated. 2 The prevalence of ocular morbidity and blindness in leprosy worldwide is unknown, 3 as epidemiological studies from different countries give wide ranging results. The prevalence of blindness due to leprosy has been variously estimated as being 4.7% in India, 4 1.6% in Nigeria, 5 1.3% in China, 6 1.2% in Nepal 7 and 0.6% in Uganda. 8 Few of these, and other studies reflect the prevalence of visual impairment in the overall leprosy-affected population as opposed to the prevalence in selected groups of leprosy-affected individuals in hospitals or leprosaria, which are known to have a high frequency of multiple complications. 2,9,10 We examined leprosy-affected people in three control areas in Eastern India, (Muzaffarpur and Saldoha in Bihar, and Jhalda in West Bengal) to profile the patterns of eye disease, visual impairment and blindness, and to relate this information to the stage of treatment. This in turn would enable more efficient and effective planning and use of resources. The study was a cross-sectional, observational study comparing the prevalence of potentially sight-threatening complications of leprosy, in patients classified according to their stage of treatment, in the three control areas. Patients and methods The study was approved by the Project Team of The Leprosy Mission (South Asia), and was carried out during the first 6 months of 1998, when eye screening of all new leprosy patients and patients with known risk factors for eye complications had been introduced in the three control areas involved. Thus, eye examination was part of routine clinical care for many of the patients. Those for whom it was not were offered the eye examination, with a verbal explanation of the study and its aims. It was made clear that appropriate treatment would be given for any pathology detected, and that continuing management of their disease would not be affected should they decline the examination. Five paramedical workers responsible for detection and long term follow up of leprosy patients in the community in three centres were trained. They were taught to identify ophthalmic pathology, detectable by macroscopic examination under good illumination, in categories recorded on a proforma specifically designed for the purpose. Quality control of the accuracy of their clinical examination methods and their recording of the data was carried out prior to commencing the project, by carrying out a small pilot project in a leprosy colony close to the base hospital. The study sites comprised Saldoha (a rural tribal area in Bihar), Muzaffarpur, (a semi-rural non tribal area in north Bihar) and Jhalda (a semi-rural mixed

3 132 K. J. Thompson et al. tribal/non-tribal area in West Bengal). Effectively, for the leprosy-affected population, each area in the study has minimal primary eye care facilities. Each control area serves a population of approximately 3-4 million people, each in an area between 3 and 4000 km 2. A sample size of 500 subjects in each centre was planned in order to take a sample of 100 patients from each of the following five categories according to the duration of treatment at the commencement of the study. The information on treatment duration or surveillance status was obtained by patient interview and confirmed where possible from patient records:. New patients, i.e. up to 3 months on multidrug therapy (MDT).. Patients on treatment, i.e months on MDT (or longer, if not on a fixed duration regimen).. Patients released from treatment (RFT).. Patients released from control (RFC) within the preceding 5 years.. Patients released from control (RFC) more than 5 years preceding entry into the study. Patients attending the clinics during the study period were systematically sampled, (for example, every fifth patient), until 100 patients enrolled in each group. Numbers in the fifth group attending for prevention of disability follow-up were inadequate, and some had to be recalled by identification from the relevant register and were requested to attend the next clinic. The actual numbers from each centre who were enrolled in the study were: 486 patients from Jhalda, 499 patients from Muzzafarpur and 152 patients from Saldoha. All other patients received routine management by paramedical workers not involved in the study with referral to eye care services if appropriate. All study patients underwent ophthalmic examination according to a common protocol (Appendix 1). Each parameter was graded according to severity, and coded for ease of recording. Madarosis was not included as it is not sight threatening. Trichiasis, initially included in the study plan, was excluded when the study was carried out, as it was not always easily determined with the naked eye in field conditions. It is uncommon in association with facial nerve weakness and where trachoma is not endemic, as in our study area. No patients were pseudophakic. Aphakia was recorded as an alternative parameter in the lens section of the protocol. Cataract was assessed using oblique illumination to determine the presence of lens translucency and opacity (immature cataract) or of total opacity (mature cataract). Pupillary size and shape, and the presence or absence of iris atrophy, were used to assess iritis (previous or current). Normal vision was graded 0, and included visual acuities of 6/6 6/18 in the better eye. Blindness (grade 3) was defined as a visual acuity of,3/60 in the better eye, and moderate visual loss (grades 1 and 2) as a visual acuity of 6/24 3/60 in the better eye (Appendix 1). The study was conducted over a period of six months in 1998 at rural and semirural multi-drug therapy (MDT) distribution points in control group areas. The data were reviewed for recording errors. The information was recorded on Excel and statistical analyses were carried out with SPSS software. Multivariate logistic regression analyses were performed to investigate the independent effects on blindness, of gender, age, location, type of disease and duration of treatment.

4 Ocular morbidity and blindness in leprosy 133 Results The sample sizes in Jhalda and Muzaffarpur were 486 and 499, respectively, and Saldoha had fewer cases (152). Overall there were more males than females; of those from Jhalda and Muzaffarpur 335 (69%) and 324 (65%), respectively, were male, in Saldoha 78 (51%) were male. The age distributions in the three centres were similar, the proportions,40, and. 60 being 267 (55%), 180 (36%) and 14 (9%), respectively. Table 1 shows the numbers and proportions of individuals with visual impairment in each subgroup in each centre. Thirtythree people (32 from Jhalda and one from Muzaffarpur) (2.9%) from the 1137 studied were blind, and 232 (20.4%) had moderate visual impairment. The prevalence of both moderate visual impairment and blindness progressively increases with duration of disease, with large proportions of those released from control and those with leprosy for more than 5 years having significant visual dysfunction. Seventy-five (56.8%) patients released from control in Jhalda and 44 (22%) in Muzaffarpur were visually impaired or blind, and all 4 (100%) study patients released from control in Saldoha were visually impaired. One hundred and forty-one (28%) patients in Muzaffarpur, 95 (63%) patients in Saldoha and 378 (78%) patients in Jhalda were classified as having multibacillary leprosy. Table 2 gives the proportions. A higher proportion of patients in Jhalda and Saldoha had MB disease than in Muzaffarpur, where a higher proportion of patients had PB disease. Most blind individuals were those with MB leprosy in the Jhalda sample. Of the 32 blind subjects in Jhalda, 11 (34%) had had leprosy for 10 years or less, and 21 (66%) had had the disease for more than 10 years (P, ). Of the same 32 subjects, 2 (6%) had had PB leprosy, and 30 (94%) had had MB leprosy, P ¼ Table 3 shows multivariate logistic regression analysis of the factors determining blindness among the 486 subjects in Jhalda. Duration of treatment is the strongest independent factor associated with blindness. There is a rise of 3.7 in the odds of being blind Table 1. Numbers of patients with visual impairment or blindness, in each of the five categories (new patient; patients on multidrug therapy (MDT); those released from treatment; those released form control in the preceding 5 years; and those released from control over 5 years earlier) in each of the three centres, Jhalda (J), Muzaffarpur (M) and Saldoha (S) Place Treatment category No visual loss % Moderate visual impairment % Blind % Total J New patients On multi-drug therapy RFT: released from treatment RFC: released from control RFC more than 5 years M New patients On multi-drug therapy RFT: released from treatment RFC: released from control RFC more than 5 years S New patients On multi-drug therapy RFT: released from treatment RFC: released from control RFC more than 5 years

5 134 K. J. Thompson et al. Table 2. Proportions of blind patients from Jhalda (J), Muzaffarpur (M) and Saldona (S) in relation to the treatment status of the disease and disease type (multibacillary or paucibacillary) MB PB Place Treatment category Total No. blind % blind Total No. blind % blind J New patients On multi-drug therapy RFT: released from treatment RFC: released from control RFC more than 5 years M New patients On multi-drug therapy RFT: released from treatment RFC: released from control RFC more than 5 years S New patients On multi-drug therapy RFT: released from treatment RFC: released from control RFC more than 5 years when comparing patients released from treatment to those released from control within the preceding 5 years, and odds of 20 times higher when comparing patients released from control for over 5 years, to new patients. Age is other significant independent factor, with those in the age group being 7 times more likely to be blind as those under 40 years of age, and those over 60 years of age being 15 times more likely to be blind as those under 60 years old. Gender and type of disease were not independent predictors of blindness in this study. The causes of blindness in the 32 patients from Jhalda with vision less than 3/60 comprised orbicularis oculi weakness leading to impaired eyelid closure and corneal damage (14 cases, 44%), cataract in one or both eyes (32 cases, 100%), impaired corneal sensation, Table 3. Results of a multivariate logistic regression of gender, age, type of disease and duration of treatment on blindness in Jhalda subjects Variables in multivariate analysis Number of subjects Significance Odds ratio (OR) 95.0% CI for Exp(OR) Gender: baseline male Gender: female (0.34, 2.31) Age: baseline 40years Age: years (1.61, 33.9) Age: 60 years (2.9, 80.5) Disease: baseline MB Disease: PB (0.06, 1.22) Treatment group: baseline New patients On multidrug therapy (0.18, 15.7) RFT: released from treatment (0.43, 32.95) RFC: released from control (0.44, 31.7) RFC more than 5 years (2.39, 179)

6 Ocular morbidity and blindness in leprosy 135 four with corneal scarring in both eyes and two with scarring in one eye (12 cases, 37.5%), and iritis (four cases, 12.5%). Twenty-three of these patients were male over the age of 40, and seven were female over the age of 40. There were only two male and no female blind subjects under the age of 40. One patient from Muzaffarpur was blind due to mature cataracts. Patients with a best visual acuity greater than 3/60, but at increased risk of developing blindness due to the presence of ocular impairments such as orbicularis oculi weakness, impaired corneal sensitivity or iridocyclitis, were also determined. These comprised 23 (5%) patients in Muzaffarpur, 9 (6%) patients in Saldoha and 98 (20%) patients in Jhalda. The numbers of subjects in each centre with complicated cataract (cataract associated with leprosy-related corneal pathology, or intra-ocular inflammation) were determined and compared with the numbers who had cataract alone. Simple cataract was common, with 41 cases in Muzaffarpur, 11 cases in Saldoha and 118 cases in Jhalda, whereas complicated cataract was seen less frequently with two, no and six cases, respectively, in each of the centres. The relationship between visual acuity, and the presence of each type of ocular complication studied was observed, by classifying eyes as having normal visual acuity (6/18 or better) or impaired visual acuity (, 6/18). With each type of ocular complication, impaired visual acuity had a higher frequency than normal visual acuity, whereas normal visual acuity had a much higher frequency than impaired visual acuity in eyes with no complications identified. Discussion The pattern of distribution of leprosy worldwide and the epidemiological distribution of ocular complications of the disease are not coincident. 1,2 Other factors including type of disease (MB or PB), early or delayed diagnosis and treatment, and facilities for eye care influence the prevalence of ocular complications of leprosy. 11 Both historically, and to the present day in many places, leprosy affected people have been compelled, or choose themselves, to live in leprosy institutions, colonies, or among groups of similarly affected people, rather than in their original homes and communities. This has largely precluded accurate population based studies. Our study was done in rural or semi-rural centres away from cities or towns where there are large leprosy hospitals with eye departments. Regional demographic studies on other conditions, which can affect the eyes, such as tuberculosis, vitamin deficiencies and trauma could further facilitate the interpretation of our study results. The Saldoha sample was drawn from a scattered population in a remote, forested area where poverty and the need for work often necessitate moving away for months at a time, even across national borders. Hence, the population studied was smaller than the other two groups. The sample studied nevertheless provides valid information for inclusion in the study. Patients not sampled would include those with undetected leprosy, non- compliant patients on MDT and non-attenders for surveillance and prevention of disability care. The first of these are, by definition, unknown, and the latter two groups were significant only in Saldoha, and these limitations were taken into account in interpreting the results. The paramedical workers were trained to carry out the survey during routine MDT distribution and the screening of patients released from treatment (RFT) and released from control (RFC). This latter group had to be recalled. Those with visual problems may have been more likely to attend, which may have inflated the proportions in this group who were

7 136 K. J. Thompson et al. found to have visual impairment. The prevalence of blindness and visual impairment in Jhalda was higher than the other two centres. In Jhalda, a significantly higher proportion of subjects had MB leprosy and long duration of disease, although, from our analysis, only the longer duration of disease is independently contributory. In addition, Jhalda is in an area where leprosy work was well established 80 years ago, By contrast, effective leprosy control programmes were introduced in the Muzaffarpur area during the 1970s and 1980s, when dapsone and MDT came into use, and in Saldoha, in the 1950s and 1960s. This variation in the history of the three centres may partly explain the differences in the distribution and types of leprosy, but further investigation could yield additional information useful in the planning of leprosy control programmes. In Jhalda, 30 of the 32 blind subjects were over 40 years of age, and all 32 had obvious cataract in one or both eyes. Blindness due to cataract in this age group in the general population is well recognized. 12 Globally, approximately 45 million people are blind, and almost half of these are blind due to cataract. Four percent of people over 60 years old are blind, and 60% of these live in India, China and Sub Saharan Africa. 13 Minassian and Mehra s study in Central India showed an incidence of 450 new cases of cataract blindness per 100,000 people at risk, all occurring in individuals over the age of 35 years. 12 In India as a whole, 8.4 million people are said to be blind or severely visually handicapped due to cataract. 14 PB disease is commoner than MB disease in India 16 and may result in nerve damage, producing lagophthalmos and corneal anaesthesia. 1 In South East Asia and South America, MB disease is commoner than PB, with associated complications due to tissue infiltration by bacilli, and inflammation more likely to occur. 1 78% of the subjects in the Jhalda group had MB leprosy, and of the 32 blind people in the same sample, 30 (93.8%) had MB leprosy. Longstanding untreated MB leprosy is often associated with bilateral lagophthalmos and corneal anaesthesia, in addition to intra-ocular infiltration, and inflammation. Cataract associated with evidence of lagophthalmos and corneal damage, or of previous iritis or scleritis was considered to have occurred as a complication of ocular leprosy. The high prevalence of MB leprosy in Jhalda with associated features of bilateral lagophthalmos, corneal hypoaesthesia and chronic iritis is contrary to what has been documented for India as a whole. 15 The duration of leprosy in the Jhalda blind group (65.5%. 10 years, P value, ) was significantly higher than that in subjects in the same group with visual acuities better than 3/60. Accurate disease duration may be difficult to ascertain, particularly in older patients, infected before the introduction of leprosy control programmes. 15 Where there is uncertainty regarding the time of onset of the disease, it may be recorded from the time of diagnosis, giving an artificially short duration. Furthermore, following completion of a course of MDT, patients are technically classified as cured, yet relapse may occur, and some cured patients continue to have recurrences of acute or subacute iridocyclitis, 17 or scleritis, while remaining skin smear negative for Mycobacterium leprae. 3,18 Against the small but significant group of patients in Jhalda (and one in Muzaffarpur), whose blindness developed due to retrospectively identifiable risk factors, it is salutary to look at the pattern of visual acuity in relation to ocular pathology and type of leprosy across all groups of the study population. It is clear that prevention of ocular involvement, or treatment of ocular complications of leprosy in the earliest possible, reversible stages is paramount. Disabling visual impairment is common in leprosy sufferers and in those who have been released from treatment. Our results and those of others 18 show that the prevalence

8 Ocular morbidity and blindness in leprosy 137 of visual impairment in this population increases with age, particularly on account of cataract, and the onset may be earlier than in the general population. Furthermore, leprosy affected individuals do not access eye care facilities as readily as the general population, thus increasing the prevalence of preventable visual impairment and blindness. 18,19 The majority of our subjects were not aware that they had potentially treatable visual impairment. Significant cataract can be identified by testing visual acuities and seeking red reflexes. Targeted screening of the population at risk (Table 4) followed by cataract surgery for those affected clearly warrants consideration. The variation in the results of our study, and of studies in other geographical locations, suggests that the initial phase of planning a screening programme should include locally performed demographic studies as national patterns and statistics may not be indicative of the local picture. In conclusion, evidence from our study, and others, suggests that the prevalence of blindness in leprosy varies widely in different population groups, but can be significantly higher than in the general population (as determined by national or regional morbidity statistics). Leprosy screening and surveillance programmes should include ocular examination as part of the routine screening procedure, particularly in individuals with a history of MB disease. Individuals with known sight threatening complications should undergo continuing active follow-up. 1 Testing visual acuity, corneal sensation or blink pattern (noting redness of the eye also), and orbicularis oculi function should form the minimum testing undertaken to give adequate information for early detection of impairments. 20 Current ILEP guidelines for eye examination in leprosy include the determination of (1) any lid gap on gentle eye closure, (2) visual acuity greater than or less than 6/18, (3) presence or absence of a persistently red eye, and (4) presence or absence of a facial patch in reaction. 21 The presence of a facial patch in reaction, particularly adjacent to an eye, may provide early evidence of impending lagophthalmos in that eye, enabling close surveillance and early diagnosis and treatment. Similarly, corneal insensitivity, often associated with infrequent or incomplete blinking, may provide early evidence of possible corneal damage before the eye develops persistent redness. Observation of the corneal and scleral appearances, and pupil shape and size gives additional information, and takes little time. Since there is regional variation in the type and prevalence of ocular complications of leprosy, carrying out local surveys would enable greater efficiency for control programmes in planning ocular screening procedures and training staff in these procedures. It would also guide allocation of resources. The long term benefits accrued would include a reduction in patient morbidity (including possible maintenance of the ability to work or to live independently), a reduction in resource expenditure on treatment of more advanced ocular Table 4. Risk factors for the development of blindness in people with leprosy 4,8,19 Multibacillary leprosy Duration of active disease.10 years History of, or known type II reactions ^iritis Facial patch, or diffuse facial skin infiltration Orbicularis oculi weakness or lagophthalmos Reduced or absent corneal sensation History, or clinical evidence of previous scleritis Only-eyed, or grade I-II sight impaired patients Coexisting disease eg. diabetes mellitus, glaucoma

9 138 K. J. Thompson et al. complications, (including surgery) and other more prolonged or costly care in tertiary referral centres, along with a reduced need for long term care and support of visually impaired and blind leprosy-affected patients. Acknowledgements We are grateful to Mr Keshab Mahato, Mr Ranjit Murmu, Mr James Hansdak, Mr Tarapada Mahato and their colleagues for their cooperation in carrying out this study, and to Dr C. S. Walter, Director of The Leprosy Mission, South Asia, for facilitating the work and permitting presentation of the data. Our thanks are also given to Mrs S. Mondal and Ms R. Wood for secretarial help, and to Professor Gordon Dutton for help in preparing the manuscript. References 1 Lewallen S, Tungpakorn NC, Kim SH, Courtright P. Progression of eye disease in cured leprosy patients: implications for understanding the pathophysiology of ocular disease and for addressing eye care needs. Br J Ophthalmol, 2000; 84: ffytche TJ. The continuing challenge of ocular leprosy (mini review). Br J Ophthalmol, 1991; 75: Lewallen S. Prevention of blindness in leprosy: an overview of the relevant clinical and programme-planning issues. Ann Trop Med Parasitol, 1997; 91: ffytche TJ. Residual sight threatening lesions in leprosy patients completing multidrug therapy and sulphone monotherapy. Lepr Rev, 1991; 62: Malu KN, Malu AO. Blindness in leprosy patients of Kaduna state, Northern Nigeria. Trop Doct, 1995; 25: Yang Jianwen, Jian Daming, Hu Lufang et al. Blindness and low vision in leprosy patients in Sichuan province, China. Ind J Lepr, 1998; 70: Knuuttila JP, van Brakel WH, Anderson AM. Ocular impairments in an impairment survey of leprosy-affected persons in Nepal. Ind J Lepr, 1998; 70: Waddell KM, Saunderson PR. Is leprosy blindness avoidable? The effect of disease type, duration and treatment on eye damage from leprosy in Uganda. Br J Ophthalmol, 1995; 79: Mvogo CE, Bella-Hiag AL, Ellong A et al. Ocular complications of leprosy in Cameroon. Acta Ophthalmol Scand, 2001; 79: Courtright PD. Defining the magnitude of ocular complications from leprosy: problems of methodology. Int J Lepr Other Mycobact Dis, 1988; 56: Courtright P, Daniel E, Sundar Rao et al. Eye disease in multibacillary leprosy patients at the time of their leprosy diagnosis: findings from the Longitudinal Study of Ocular Leprosy (LOSOL) in India, the Philippines and Ethiopia. Lepr Rev, 2002; 73: Minassian DC, Mehra V. 3 million blinded by cataract each year; projections from the first epidemiological study of incidence of cataract blindness in India. Br J Ophthalmol, 1990; 74: Resnikoff S, Pascolini D, Etya ale D et al. Global data on visual impairment in the year Bull World Health Org, 2004; 82: Nirmalan PK, Robin AL, Katz J et al. Risk factors for age related cataract in a rural population of southern India: the Aravind Comprehensive Eye Study. Br J Ophthalmol, 2004; 88: Sundar Rao PS, Daniel E, Kurian N, Gayathri KJ. Epidemiological aspects of ocular morbidity in leprosy-affected persons. Ind J Lepr, 1998; 70: World Health Organisation. Global Leprosy Situation, Weekly Epidemiol Rec, 2005; 80: Carus NH, Raizman MB, Williams DL, Baker AS. Relapse of mycobacterium leprae infection with ocular manifestations. Clin Infect Dis, 1995; 20: Hogeweg M. Cataract: the main cause of blindness in leprosy. Lepr Rev, 2001; 72: Thompson K. Ocular morbidity in a sample of 150 treated leprosy patients. Ind J Lepr, 1998; 70: Thompson K, Daniel E. Management of ocular problems in leprosy. Ind J Lepr, 1998; 70: Courtright P, Tamplin M. Guidelines for the management of eye care in leprosy: Recommendations from ILEPsupported meeting. IAPB News, 2002, pp. 8 9.

10 Ocular morbidity and blindness in leprosy 139 Appendix THE PREVALENCE OF BLINDNESS IN LEPROSY IN JHALDA, MUZAFFARPUR AND SALDOHA Study Pro-forma Name: ::::::::: Age: ::::::, 20 ¼ 0, ¼ 1, ¼ 2,.60 ¼ 3 Sex: :::::: M ¼ 0, F ¼ 1 Centre: ::::::::: Disease classification: :::::::::.. MB ¼ 0, PB ¼ 1 Duration of disease: :::::::::::: 0 5 years ¼ 0, 5 10 years ¼ 1,.10 years ¼ 2 Duration of treatment: ::::::::::::::: New ¼ 0, on MDT ¼ 1, RFT ¼ 2, RFC ¼ 3,.5 years RFC ¼ 4 Right eye Left eye Comments 1 Visual acuity 0 ¼ 6/6 6/18 1 ¼ 6/24 6/60 2 ¼ 5/60 3/60 3, 3/60 2 Orbicularis function 0 ¼ Normal 1 ¼ Weak, no lagophthalmos 2 ¼ Mild lagophthalmos, with no corneal exposure on gentle closure 3 ¼ Moderate lagophthalmos, with,50% corneal exposure on gentle closure 4 ¼ Severe lagophthalmos, with.50% or more corneal exposure on gentle closure 3 Cornea 0 ¼ Normal appearance and sensation 1 ¼ Corneal anaesthesia/hypoaesthesia 2 ¼ Scarring present, visual axis clear 3 ¼ Severe scarring present, visual axis obscured 4 Iris and pupil e.g. patient on treatment for iritis 0 ¼ Normal 1 ¼ Small pupil 2 ¼ Irregular pupil 3 ¼ Obvious iris atrophy with irregular pigmentation and/or full thickness defects (polycoria) 4 ¼ Obscured by dense corneal opacity

11 140 K. J. Thompson et al. 5 Lens 0 ¼ Normal 1 ¼ Immature cataract 2 ¼ Mature, uncomplicated cataract 3 ¼ Complicated cataract (i.e. with findings as in 4.1-4) 4 ¼ Aphakia/pseudophakia 6 Sclera 0 ¼ Normal 1 ¼ Inflamed 2 ¼ Staphyloma Right eye Left eye Comments Examined by: :::::::::::::: Date: ::::::::::::::::::::.