Gordon J. Johnson

The definition and classification of glaucoma in prevalence surveys

This review describes a scheme for diagnosis of glaucoma in population based prevalence surveys. Cases are diagnosed on the grounds of both structural and functional evidence of glaucomatous optic neuropathy. The scheme also makes provision for diagnosing glaucoma in eyes with severe visual loss where formal field testing is impractical, and for blind eyes in which the optic disc cannot be seen because of media opacities.

The Cardinal Manifestations of Bardet–Biedl Syndrome, a Form of Laurence–Moon–Biedl Syndrome

To determine the interfamilial and intrafamilial variation in the expression of the Bardet-Biedl syndrome (a form of Laurence-Moon-Biedl syndrome), we looked for the five recognized features of the disorder (retinal dystrophy, obesity, polydactyly, mental retardation, and hypogonadism), plus possible renal manifestations, in some or all of 32 patients with this disorder. All 28 patients examined had severe retinal dystrophy, but only 2 had typical retinitis pigmentosa. Polydactyly was present in 18 of 31 patients, but syndactyly, brachydactyly, or both were present in all. Obesity was present in all but 1 of 25 patients. Only 13 of 32 patients were considered mentally retarded. Scores on verbal subtests of intelligence were usually lower than scores on performance tasks. Seven of eight men had small testes and genitalia, which was not due to hypogonadotropism. All 12 women studied had menstrual irregularities, and 3 had low serum estrogen levels (1 of these had hypogonadotropism, and 2 had primary gonadal failure). The remaining women who were of reproductive age had endocrinologic evidence of reproductive dysfunction. Diabetes mellitus was present in 9 of 20 patients. Renal structural or functional abnormalities were universal (n = 21), and three patients had end-stage renal failure. We conclude that the characteristic features of Bardet-Biedl syndrome are severe retinal dystrophy, dysmorphic extremities, obesity, renal abnormalities, and (in male patients only) hypogenitalism. Mental retardation, polydactyly, and hypogonadism in female patients are not necessarily present.

Glaucoma in China: how big is the problem?

AIMS To derive preliminary estimates for the number of adults in China suffering from glaucoma, and project the burden of visual morbidity attributable to primary and secondary glaucoma. METHODS Age and sex specific data from two population surveys were applied to US Census Bureau population estimates for urban and rural China. It was assumed that data from Singapore were representative of urban China, and those from Mongolia were representative of rural China. RESULTS It was estimated that 9.4 million people aged 40 years and older in China have glaucomatous optic neuropathy. Of this number, 5.2 million (55%) are blind in at least one eye and 1.7 million (18.1%) are blind in both eyes. Primary angle closure glaucoma (PACG) is responsible for the vast majority (91%) of bilateral glaucoma blindness in China. The number of people with the anatomical trait predisposing to PACG (an “occludable” drainage angle) is in the region of 28.2 million, and of these 9.1 million have significant angle closure, indicated by peripheral anterior synechiae or raised intraocular pressure. CONCLUSIONS This extrapolation of data from two east Asian countries gives an approximate number of people in China suffering from glaucoma. It is unlikely that this crude statistical model is entirely accurate. However, the authors believe the visual morbidity from glaucoma in China is considerable. PACG is probably the leading cause of glaucoma blindness in both eyes, and warrants detailed investigation of strategies for prevention.

Aetiology of suppurative corneal ulcers in Ghana and south India, and epidemiology of fungal keratitis

Background: A multicentre study was carried out in Ghana and southern India to determine the aetiology of suppurative keratitis in two regions located at similar tropical latitudes. Studies of fungal keratitis from the literature were reviewed. Methods: Patients presenting at rural and urban eye units with suspected microbial keratitis were recruited to the study. Corneal ulceration was defined as loss of corneal epithelium with clinical evidence of infection with or without hypopyon. Microscopy and culture were performed on all corneal specimens obtained. Results: 1090 patients were recruited with suspected microbial keratitis between June 1999 and May 2001. Overall the principal causative micro-organisms in both regions were filamentous fungi (42%): Fusarium species and Aspergillus species were the commonest fungal isolates. Pseudomonas species were most frequently isolated from cases of bacterial keratitis in Ghana but in India the commonest bacterial isolates were streptococci. Conclusion: Infections of the cornea due to filamentous fungi are a frequent cause of corneal damage in developing countries in the tropics and are difficult to treat. Microscopy is an essential tool in the diagnosis of these infections. A knowledge of the “local” aetiology within a region is of value in the management of suppurative keratitis in the event that microscopy cannot be performed.

Central corneal thickness and intraocular pressure in a Mongolian population

OBJECTIVE The purpose of the study was to quantify the variation of central corneal thickness (CCT) in an east Asian population and to examine its relationship to estimates of intraocular pressure (IOP) made with an applanation tonometer. DESIGN The study design was a cross-sectional, population-based survey. PARTICIPANTS One thousand two-hundred forty-two residents of Hövsgöl Province, Mongolia, 10 to 87 years of age participated. MAIN OUTCOME MEASURES The CCT was measured using an optical pachymeter in all subjects. The IOP was measured using a, Goldmann-type applanation tonometer in subjects 40 years of age and older. RESULTS There was a highly significant decrease in CCT with age: 5 microns/decade in men and 6 microns/decade in women (both, P < 0.0001). A highly significant positive correlation was identified between IOP and CCT. Linear regression analysis suggests that between the ages of 40 and 80 years, an increase in CCT of 10 microns is associated with an increase in IOP measurements of 0.18 mmHg in right eyes (95% confidence interval, 0.12-0.25) and 0.24 mmHg in left eyes (95% confidence interval, 0.17-0.31). The authors calculate that interindividual differences in CCT may produce a difference in measured IOP of between 2.3 and 3.1 mmHg. CONCLUSIONS Variation in CCT is a significant source of variation in IOP measurements between individuals. The authors suggest that measurement of corneal thickness should be considered when assessing IOP as a risk factor for glaucoma in east Asians.

YAG laser iridotomy treatment for primary angle closure in east Asian eyes

AIM To assess the efficacy of Nd:YAG laser iridotomy as initial treatment for primary angle closure in a community setting in rural Mongolia. METHODS Subjects with occludable drainage angles in two glaucoma prevalence surveys in Mongolia (carried out in 1995 and 1997) were treated with YAG laser iridotomy at the time of diagnosis. These patients were re-examined in 1998. Patency of iridotomy, intraocular pressure (IOP), visual acuity, and gonioscopic findings were recorded. Iridotomy was classified unsuccessful in eyes where further surgical intervention was required or in which there was a loss of visual acuity to <3/60 from glaucomatous optic neuropathy. RESULTS 164 eyes of 98 subjects were examined. Patent peripheral iridotomies were found in 98.1% (157/160) of eyes that had not undergone surgery. Median angle width increased by two Shaffer grades following iridotomy. Iridotomy alone failed in 3% eyes with narrow drainage angles and either peripheral anterior synechiae or raised IOP, but normal optic discs and visual fields. However, in eyes with established glaucomatous optic neuropathy at diagnosis iridotomy failed in 47%. None of the eyes with occludable angles that were normal in all other respects, and underwent iridotomy, developed glaucomatous optic neuropathy or symptomatic angle closure within the follow up period. CONCLUSIONS Nd: YAG laser iridotomy is effective in widening the drainage angle and reducing elevated IOP in east Asian people with primary angle closure. This suggests that pupil block is a significant mechanism causing closure of the angle in this population. Once glaucomatous optic neuropathy associated with synechial angle closure has occurred, iridotomy alone is less effective at controlling IOP.

Detection of gonioscopically occludable angles and primary angle closure glaucoma by estimation of limbal chamber depth in Asians: modified grading scheme

AIM To evaluate the performance of limbal chamber depth estimation as a means of detecting occludable drainage angles and primary angle closure, with or without glaucoma, in an east Asian population, and determine whether an augmented grading scheme would enhance test performance. METHOD A two phase, cross sectional, community based study was conducted on rural and urban areas of Hövsgöl and Ömnögobi provinces, Mongolia. 1800 subjects aged 40 to 93 years were selected and 1717 (95%) of these were examined. Depth of the anterior chamber at the temporal limbus was graded as a percentage fraction of peripheral corneal thickness. An “occludable” angle was one in which the trabecular meshwork was seen in less than 90° of the angle circumference by gonioscopy. Primary angle closure (PAC) was diagnosed in subjects with an occludable angle and either raised pressure or peripheral anterior synechiae. PAC with glaucoma (PACG) was diagnosed in cases with an occludable angle combined with glaucomatous optic neuropathy and consistent visual morbidity. RESULTS Occludable angles were identified in 140 subjects, 28 of these had PACG. The 15% grade (equivalent to the traditional “grade 1”) yielded sensitivity and specificity of 84% and 86% respectively for the detection of occludable angles. The 5% grade gave sensitivity of 91% and specificity of 93% for the detection of PACG. The interobserver agreement for this augmented grading scheme was good (weighted kappa 0.76). CONCLUSIONS The traditional limbal chamber depth grading scheme offers good performance for detecting occludable drainage angles in this population. The augmented scheme gives enhanced performance in detection of established PACG. The augmented scheme has potential for good interobserver agreement.

Strategies for control of trachoma: observational study with quantitative PCR

BACKGROUND Antibiotics are an important part of WHOs strategy to eliminate trachoma as a blinding disease by 2020. At present, who needs to be treated is unclear. We aimed to establish the burden of ocular Chlamydia trachomatis in three trachoma-endemic communities in Tanzania and The Gambia with real-time quantitative PCR. METHODS Conjunctival swabs were obtained at examination from 3146 individuals. Swabs were first tested by the qualitative Amplicor PCR, which is known to be highly sensitive. In positive samples, the number of copies of omp1 (a single-copy C trachomatis gene) was measured by quantitative PCR. FINDINGS Children had the highest ocular loads of C trachomatis, although the amount of pooling in young age groups was less striking at the site with the lowest trachoma frequency. Individuals with intense inflammatory trachoma had higher loads than did those with other conjunctival signs. At the site with the highest prevalence of trachoma, 48 of 93 (52%) individuals with conjunctival scarring but no sign of active disease were positive for ocular chlamydiae. INTERPRETATION Children younger than 10 years old, and those with intense inflammatory trachoma, probably represent the major source of ocular C trachomatis infection in endemic communities. Success of antibiotic distribution programmes could depend on these groups receiving effective treatment.

The Spectrum of Renal Disease in Laurence–Moon–Biedl Syndrome

To determine the nature, extent, and severity of renal involvement in Laurence-Moon-Biedl syndrome (obesity, mental retardation, polydactyly, hypogonadism, and pigmented retinal dystrophy), we evaluated 20 of 30 patients with the disorder identified from ophthalmologic records in Newfoundland. The mean age was 31 years, and seven were male. All 20 patients had structural or functional abnormalities of the kidneys or both. Three had end-stage renal disease, with two requiring maintenance hemodialysis. The remaining 17 patients had normal serum creatinine values and estimated creatinine clearances. Half the subjects had hypertension. Fourteen of 17 patients could not concentrate urine above 750 mOsm per kilogram of body weight even after vasopressin, whereas all 10 normal controls could. Urinary pH decreased below 5.3 after ammonium chloride administration in all 15 normal controls, but in only 13 of 18 patients. Calyceal clubbing or blunting was evident in 18 of 19 patients studied by intravenous pyelography; 13 patients had calyceal cysts or diverticula. Seventeen of 19 patients had lobulated renal outlines of the fetal type. Four patients had diffuse renal cortical loss, but only two of these had renal insufficiency. We conclude that Laurence-Moon-Biedl syndrome includes the presence of renal abnormalities.

Prevalence of glaucoma in Thailand: a population based survey in Rom Klao District, Bangkok

Aim: To determine prevalence, demography, mechanism, and visual morbidity of glaucoma in urban Thai people. Methods: 790 subjects aged 50 years or older from Rom Klao district, Bangkok, Thailand, were enumerated in a population based cross sectional study. Each subject underwent the following investigations: visual acuity, visual field testing, slit lamp examination, applanation tonometry, gonioscopy, and an optic disc examination after mydriasis. Main outcome measures included visual acuity (logMAR), visual fields, intraocular pressure (IOP), gonioscopic characteristics, vertical cup/disc ratio (VCDR), prevalence of types of glaucoma. Glaucoma was diagnosed on the basis of optic disc appearance and visual field defects. In eyes in which the optic disc could not be examined, glaucoma was diagnosed when visual acuity was <3/60 and either IOP >99.5th percentile or there was evidence of previous glaucoma surgery. Results: 701 subjects were examined (response rate 88.7%). In eyes with “normal” suprathreshold visual fields, the mean IOP was 13.3 mm Hg (97.5th percentile = 20 mm Hg). The 97.5th and 99.5th percentiles of VCDR were 0.72 and 0.86 respectively. Of the 701 subjects examined in the clinic, 27 had glaucoma (3.8%, 95% CI: 2.5 to 5.6), 16 had primary open angle glaucoma (POAG, prevalence 2.3%, 95% CI: 1.3 to 3.7), six were primary angle closure glaucoma (PACG, prevalence 0.9%, 95% CI: 0.3 to 1.9), and five were secondary glaucoma (SecG, prevalence 0.7%, 95% CI: 0.2 to 1.7). Among the 43 unilaterally blind subjects, glaucoma was the cause in five subjects (12%). One subject was bilaterally blind due to glaucoma (prevalence 11%, 95% CI: 0.3 to 61.9). 28 people (4%) were glaucoma suspects on the basis of optic disc appearance and six on the basis of visual fields only. 98 subjects (14%) had “occludable angles” in either eye, 22 of whom had primary angle closure (PAC, prevalence 3.1%, 95% CI: 1.9 to 4.7); 14 had peripheral anterior synechiae in either eye and eight had ocular hypertension (OHT). Conclusions: POAG accounted for 67% of all glaucoma, PACG 21%, and secondary glaucomas 12%. Glaucoma was the second most common cause of severe unilateral visual loss.