Upper and lower conjunctival fornix depth in healthy white caucasian eyes: a method of objective assessment.

PurposeEvaluating anti-scarring therapies require objective assessment of scarring, and knowledge of normal fornix anatomy. Measurement of conjunctival scarring has focused on inferior fornix shortening, although the superior fornix is often overlooked. There are data on normal fornix depth (FD) in South Asians, but there are no studies investigating normal conjunctival FD in white Caucasians. We designed a fornix depth measurer (FDM) for objective measurement of upper and lower conjunctival FD. The purpose of this study was to evaluate intra- and inter-observer variability, and to establish a reference for normal conjunctival FD in an ethnically white Caucasian population.Patients and methodsProspective cross-sectional study evaluating conjunctival FD in 252 clinically normal white Caucasian participants aged 20-80. Paired observers evaluated inter- and intra-observer variability. Data was analyzed using Bland-Altman plots and analysis of variance.ResultsFor white Caucasian subjects, mean upper and lower conjunctival fornix depths were 15.6 mm (95% confidence interval (CI), 12.5-18.8) and 10.9 mm (95% CI, 8.0-13.7), respectively. Females have smaller FDs (upper FD 15.3 mm±1.6 females, 16.2 mm±1.4 males, P<0.001; lower FD 10.6 mm±1.3 females, 11.3 mm±1.4 males, P<0.001). There was a progressive decline in FD with age (upper fornix depth 16.3 mm±1.2 at age 20-29, and 15.0 mm±1.8 at age 80+ (P=0.04)). There was 94-100% intra-observer and inter-observer agreement for upper and lower fornix measurements.ConclusionsUsing a slightly different custom-designed FDM, central conjunctival fornix depth in white Caucasian eyes appears to be similar to data previously reported in South Asian eyes. Fornix depth measurements were repeatable and reproducible.

Incidence, presenting features, and diagnosis of cicatrising conjunctivitis in the United Kingdom.

PURPOSE: Cicatrising conjunctival disorders are uncommon, and are difficult to diagnose and manage. This study was designed to assess the annual incidence and underlying diagnosis of patients with cicatrising conjunctivitis (CC) within the United Kingdom. METHODS: Clinical data of newly diagnosed cases of CC were reported via the British Ophthalmological Surveillance Unit at diagnosis and at 12 months follow-up. RESULTS: A total of 50 (61%) ocular mucous membrane pemphigoid (OcMMP), 16 (20%) Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS-TEN) and 16 (20%) other causes of CC, equating to an incidence of 0.8, 0.2, and 0.2 per million, respectively, were reported. Although diagnosis of SJS-TEN was usually within a median of 7 days of symptom-onset, that for OcMMP and other CC was a median 225 days for both. At diagnosis, 64/163 (39%) eyes had moderate/severe conjunctival inflammation, and 102/164 (62%) had symblepharon formation. Although 43/82 (52%) patients were commenced on immunosuppression or had this therapy modified, at follow-up there was an increase in the number of symblepharon, despite control of inflammation (P<0.001). Mortality only occurred in the SJS-TEN group (4/16 (25%)). CONCLUSION: CC has a substantial morbidity and for non-SJS-TEN causes, diagnosis is frequently delayed. The proportion of patients given immunosuppressive therapy to prevent disease progression may be less than optimal. These data highlight the need for developing patient access to specialist-designated centres with expertise in CC.

Cicatrising conjunctivitis with anti-basement membrane autoantibodies in ectodermal dysplasia.

AIMS: To report circulating and mucosa-deposited anti-basement membrane zone autoantibodies in a series of six ectodermal dysplasia patients with severe bilateral cicatrising conjunctivitis and blindness due to both corneal disease and intractable surface inflammation. We also report clinical improvement with steroid-sparing systemic immunosuppression combined with clearance of bacterial colonisation. METHODS: Conjunctival and buccal immunohistopathology, and serological analysis using a panel of epithelial basement membrane zone proteins including the bullous pemphigoid antigen 180 (BP180) were carried out as part of an ocular pemphigoid work-up in each patient. The degree of photophobia, conjunctival inflammation and visual acuity were monitored to evaluate the response to immunosuppression. The mean duration of follow-up was 31 (SD 6) months. RESULTS: Four of the six patients showed positive immunopathology: direct immunofluorescence testing of the conjunctiva in one patient demonstrated linear IgA deposition along the basement membrane zone, and IgG and IgM in the buccal mucosa of another patient. Circulating autoantibodies to BP180 were detected in two other patients. Treatment with systemic immunosuppression, combined with clearance of bacterial colonisation, reduced the severity of photophobia and degree of conjunctival inflammation in 5/6 (83%) patients. CONCLUSIONS: Systemic immunosuppression, used as steroid-sparing therapy, combined with clearance of bacterial colonisation can control inflammation and disabling photophobia, and allow improvement in vision, in a subgroup of ectodermal dysplasia patients who have severe cicatrising conjunctivitis which shares clinical and immunopathological features with ocular mucous membrane pemphigoid.

Susac syndrome: microangiopathy of the retina, cochlea and brain.

BACKGROUND: Susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. METHODS: Analysis of two male and two female cases of Susac syndrome recognized in Australia. RESULTS: In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of Susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behçet's syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as multiple sclerosis. CONCLUSION: Susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.

Dural sinus thrombosis: a mechanism-based classification and review of 42 cases.

Cranial venous outflow obstruction due to dural sinus thrombosis may result in venous hypertension, cerebral infarction, cerebral haemorrhage or impaired cerebrospinal fluid (CSF) absorption with consequent pseudotumour syndrome. We propose a mechanism based classification of dural sinus thrombosis from these four outcomes. Forty two cases of dural sinus thrombosis presenting to Royal Prince Alfred Hospital between 1986-1997 were retrospectively reviewed. These cases were classified according to mechanism of presentation and relevance of this to site of thrombosis, treatment and prognosis. This study shows that the superior sagittal sinus and transverse sinus are the commonest sites of thrombosis, and multiple sites of thrombosis (69%) are more frequent than a single site. Magnetic resonance imaging (MRI) with venous flow studies is the investigation of first choice for diagnosis but angiography remains the gold standard. A pseudotumour syndrome is the commonest presentation (43%) followed by cerebral haemorrhage (31%). The overall prognosis for sinus thrombosis is good, with 71% of cases recovering to normal function.