"Where Does it Come from?" Experiences Among Survivors and Parents of Children with Retinoblastoma in Kenya.

Genetic testing and counseling have become integral to the timely control of heritable cancers, like the childhood eye cancer retinoblastoma. This study aimed to determine attitudes, knowledge and experiences related to retinoblastoma genetics, among survivors and parents of children with retinoblastoma in Kenya. This qualitative study used focus groups as the primary data collection method, coupled with a brief demographic questionnaire. Study settings were Kenyatta National Hospital and Presbyterian Church of East Africa Kikuyu Hospital. Thematic analysis was used to identify key themes. Thirty-one individuals participated in five focus groups. Two main concepts emerged: (1) the origins of retinoblastoma are unclear, and (2) retinoblastoma is associated with significant challenges. The lack of clarity surrounding the origins of retinoblastoma was linked to limited knowledge of retinoblastoma genetics, and limited genetic counseling delivery and uptake. The challenges associated with retinoblastoma were discussed in terms of the impact of the diagnosis on individuals and families, and unmet healthcare needs related to the diagnosis. Next steps will incorporate these findings to develop evidence-informed and accessible cancer genetic services in Kenya.

Delay along the care-seeking journey of patients with ocular surface squamous neoplasia in Kenya.

BACKGROUND: In Africa, accessing eye health services is a major challenge. Ocular surface squamous neoplasia (OSSN) is a substantial ocular health problem in Africa related to solar UV light exposure and HIV infection among other risk factors. The disease causes visual loss and even death in advanced cases. This study was conducted to assess referral pathway and treatment delay for patients with OSSN in Kenya. METHODS: Adults with conjunctival lesions presenting to four eye centres were asked about their occupations, when they noticed the growth, health facilities visited in seeking care, cost of consultation, surgery, medicines and histopathology and dates at each step. The time-to-presentation was divided into quartiles and correlates analysed using ordinal logistic regression. RESULTS: We evaluated 158 first-time presenters with OSSN. Most were women (102 [65%]), living with HIV (78/110 tested [71%]), with low to medium income (127 [80%]). Most of the HIV patients (49/78 [63%]) were in antiretroviral care programs. About half (88/158, [56%]) presented directly to the study centres while the rest were referred. Indirect presenters sought care earlier than direct presenters (median 2.0 months vs 5.5 months) and travelled a shorter distance to the first health facility (median 20 km vs 30 km) but had surgery later (median 12.5 months vs 5.5 months). Visits beyond the first health facility for indirect presenters markedly increased delay (median 7.3, 29.0, 37.9, and 32.0 months for 1-4 facilities, respectively). Delay was associated with number of health facilities visited (adjusted ordered OR = 9.12; 95%CI 2.83-29.4, p < 0.001) and being female (adjusted ordered OR = 2.42; 95%CI 1.32-4.44, p = 0.004). At the time of presentation at the study centres for surgery the median tumour diameter in both directly and indirectly presenting patients was 6 mm (p = 0.52) and the histological spectrum of OSSN was similar between the groups (p = 0.87). CONCLUSIONS: Referral delays definitive treatment for OSSN. Women were more likely to experience delay. Despite regular contact with the health system for those with known HIV infection, delays occurred. Early detection and referral of OSSN in the HIV service might reduce delays, but reassuringly delay did not give rise to a larger proportion with more advanced grade of OSSN.

Risk factors for ocular surface squamous neoplasia in Kenya: a case-control study.

OBJECTIVE: To determine modifiable risk factors of ocular surface squamous neoplasia (OSSN) in Kenya using disease-free controls. METHODS: Adults with conjunctival lesions were recruited at four eye care centres in Kenya and underwent excision biopsy. An equal number of controls having surgery for conditions not affecting the conjunctiva and unrelated to ultraviolet light were group-matched to cases by age group, sex and eye care centre. Associations of risk factors with OSSN were evaluated using multivariable logistic regression to estimate odds ratios (ORs) and 95% confidence intervals (95% CIs). Continuous variables were compared using the t-test or the Wilcoxon-Mann-Whitney U-test depending on their distribution. RESULTS: A total of 131 cases and 131 controls were recruited. About two-thirds of participants were female, and the mean age of cases and controls was 42.1 years and 43.3 years, respectively. Risk factors for OSSN were HIV infection without antiretroviral therapy (ART) use (OR = 48.42; 95% CI: 7.73-303.31) and with ART use (OR = 19.16; 95% CI: 6.60-55.57), longer duration of exposure to the sun in the main occupation (6.9 h/day vs. 4.6 h/day, OR = 1.24; 95% CI: 1.10-1.40) and a history of allergic conjunctivitis (OR = 74.61; 95% CI: 8.08-688.91). Wearing hats was protective (OR = 0.22; 95% CI: 0.07-0.63). CONCLUSION: Measures to prevent and control HIV, reduce sun exposure such as wearing hats and control allergic conjunctivitis are recommended.

Topical fluorouracil after surgery for ocular surface squamous neoplasia in Kenya: a randomised, double-blind, placebo-controlled trial.

BACKGROUND: Ocular surface squamous neoplasia (OSSN) is an aggressive eye tumour particularly affecting people with HIV in Africa. Primary treatment is surgical excision; however, tumour recurrence is common. We assessed the effect of fluorouracil 1% eye drops after surgery on recurrence. METHODS: We did this multicentre, randomised, placebo-controlled trial in four centres in Kenya. We enrolled patients with histologically proven OSSN aged at least 18 years. After standard surgical excision, participants were randomly allocated to receive either topical fluorouracil 1% or placebo four times a day for 4 weeks. Randomisation was stratified by surgeon, and participants and trial personnel were masked to assignment. Patients were followed up at 1 month, 3 months, 6 months, and 12 months. The primary outcome was clinical recurrence (supported by histological assessment where available) by 1 year, and analysed by intention to treat. The sample size was recalculated because events were more common than anticipated, and trial enrolment was stopped early. The trial was registered with Pan-African Clinical Trials Registry (PACTR201207000396219). FINDINGS: Between August, 2012, and July, 2014, we assigned 49 participants to fluorouracil and 49 to placebo. Four participants were lost to follow-up. Recurrences occurred in five (11%) of 47 patients in the fluorouracil group and 17 (36%) of 47 in the placebo group (odds ratio 0·21, 95% CI 0·07-0·63; p=0·01). Adjusting for passive smoking and antiretroviral therapy had little effect (odds ratio 0·23; 95% CI 0·07-0·75; p=0·02). Adverse effects occurred more commonly in the fluorouracil group, although they were transient and mild. Ocular discomfort occurred in 43 of 49 patients in the fluorouracil group versus 36 of 49 in the placebo group, epiphora occurred in 24 versus five, and eyelid skin inflammation occurred in seven versus none. INTERPRETATION: Topical fluorouracil after surgery substantially reduced recurrence of OSSN, was well-tolerated, and its use recommended. FUNDING: British Council for Prevention of Blindness and the Wellcome Trust.

Clinical Presentation of Ocular Surface Squamous Neoplasia in Kenya.

IMPORTANCE: There is a trend toward treating conjunctival lesions suspected to be ocular surface squamous neoplasia (OSSN) based on the clinical impression. OBJECTIVE: To describe the presentation of OSSN and identify clinical features that distinguish it from benign lesions and subsequently evaluate their recognizability. DESIGN, SETTING, AND PARTICIPANTS: Prospective multicenter study in Kenya from July 2012 through July 2014 of 496 adults presenting with conjunctival lesions. One histopathologist examined all specimens. Six additional masked ophthalmologists independently examined photographs from 100 participants and assessed clinical features. EXPOSURES: Comprehensive history, slitlamp examination, and photography before excision biopsy. MAIN OUTCOMES AND MEASURES: Frequency of clinical features in OSSN and benign lesions were recorded. Proportions and means were compared using χ2, Fisher exact test, or t test as appropriate. Interobserver agreement was estimated using the κ statistic. Examiners' assessments were compared with a reference. RESULTS: Among 496 participants, OSSN was the most common (38%) histological diagnosis, followed by pterygium (36%) and actinic keratosis (19%). Patients with OSSN were slightly older (mean [SD] age, 41 [11.6] vs 38 [10.9] years; P = .002) and tended to have lower levels of education than patients with benign lesions (P = .001). Females predominated (67% of OSSN vs 64% of benign lesions; P = .65). Human immunodeficiency virus infection was common among patients with OSSN (74%). The most common location was the nasal limbus (61% OSSN vs 78% benign lesions; P < .001). Signs more frequent in OSSN included feeder vessels (odds ratio [OR], 5.8 [95% CI, 3.2-10.5]), moderate inflammation (OR, 3.5 [95% CI, 1.8-6.8]), corneal involvement (OR, 2.7 [95% CI, 1.8-4.0]), leukoplakia (OR, 2.6 [95% CI, 1.7-3.9]), papilliform surface (OR, 2.1 [95% CI, 1.3-3.5]), pigmentation (OR, 1.5 [95% CI, 1.0-2.2]), temporal location (OR, 2.0 [95% CI, 1.2-3.2]), circumlimbal location (6.7% vs 0.3%; P < .001), severe inflammation (6.7% vs 0.3%; P < .001), and larger mean (SD) diameter (6.8 [3.2] vs 4.8 [2.8] mm; P < .001). All OSSN signs were also observed in benign lesions. There was slight to fair interobserver agreement in assessment of most signs and diagnosis (κ, 0.1-0.4). The positive predictive value of clinical appearance in identifying OSSN was 54% (interquartile range, 51%-56%) from photographs in which prevalence was 32%. CONCLUSIONS AND RELEVANCE: With overlapping phenotypes and modest interobserver agreement, OSSN and benign conjunctival lesions are not reliably distinguished clinically. Point-of-care diagnostic tools may help.

Toluidine Blue 0.05% Vital Staining for the Diagnosis of Ocular Surface Squamous Neoplasia in Kenya.

IMPORTANCE: Clinical features are unreliable for distinguishing ocular surface squamous neoplasia (OSSN) from benign conjunctival lesions. OBJECTIVE: To evaluate the adverse effects, accuracy, and interobserver variation of toluidine blue 0.05% vital staining in distinguishing OSSN, confirmed by histopathology, from other conjunctival lesions. DESIGN, SETTING, AND PARTICIPANTS: Cross-sectional study in Kenya from July 2012 through July 2014 of 419 adults with suspicious conjunctival lesions. Pregnant and breastfeeding women were excluded. EXPOSURES: Comprehensive ophthalmic slitlamp examination was conducted. Vital staining with toluidine blue 0.05% aqueous solution was performed before surgery. Initial safety testing was conducted on large tumors scheduled for exenteration looking for corneal toxicity on histology before testing smaller tumors. We asked about pain or discomfort after staining and evaluated the cornea at the slitlamp for epithelial defects. Lesions were photographed before and after staining. MAIN OUTCOMES AND MEASURES: Diagnosis was confirmed by histopathology. Six examiners assessed photographs from a subset of 100 consecutive participants for staining and made a diagnosis of OSSN vs non-OSSN. Staining was compared with histopathology to estimate sensitivity, specificity, and predictive values. Adverse effects were enumerated. Interobserver agreement was estimated using the κ statistic. RESULTS: A total of 143 of 419 participants (34%) had OSSN by histopathology. The median age of all participants was 37 years (interquartile range, 32-45 years) and 278 (66%) were female. A total of 322 of the 419 participants had positive staining while 2 of 419 were equivocal. There was no histological evidence of corneal toxicity. Mild discomfort was reported by 88 (21%) and mild superficial punctate keratopathy seen in 7 (1.7%). For detecting OSSN, toluidine blue had a sensitivity of 92% (95% CI, 87%-96%), specificity of 31% (95% CI, 25%-36%), positive predictive value of 41% (95% CI, 35%-46%), and negative predictive value of 88% (95% CI, 80%-94%). Interobserver agreement was substantial for staining (κ = 0.76) and moderate for diagnosis (κ = 0.40). CONCLUSIONS AND RELEVANCE: With the high sensitivity and low specificity for OSSN compared with histopathology among patients with conjunctival lesions, toluidine blue 0.05% vital staining is a good screening tool. However, it is not a good diagnostic tool owing to a high frequency of false-positives. The high negative predictive value suggests that a negative staining result indicates that OSSN is relatively unlikely.

Ocular rhinosporidiosis mimicking conjunctival squamous papilloma in Kenya - a case report.

BACKGROUND: Ocular rhinosporidiosis is a chronic granulomatous infection caused by a newly classified organism that is neither a fungus nor bacterium. It often presents as a benign conjunctival tumour but may mimic other ocular conditions. It is most often described in India. In Africa cases have been reported from South Africa, Kenya, Tanzania, Malawi, Uganda, Congo and Ivory Coast. CASE PRESENTATION: A 54 year old man was seen in Kenya with a lesion that resembled a conjunctival papilloma. We report resemblance to conjunctival papilloma and the result of vital staining with 0.05% Toluidine Blue. CONCLUSION: Ocular rhinosporidiosis occurs in East Africa. It may resemble conjunctival squamous papilloma. Vital staining with 0.05% Toluidine blue dye did not distinguish the two lesions well.

A rare case of orbital haemangiopericytoma arising in childhood.

Haemangiopericytoma (HPC) is a rare soft tissue tumour of fibroblastic origin and is part of the solitary fibrous tumour spectrum. The tumour is generally considered to be benign, but can behave clinically as if sarcomatous -- with relentless infiltrative local growth. HPC generally presents in adulthood (median age 45 years for orbital disease) and is equally frequent in both sexes. HPC can arise in any site in the body and presents as a slowly growing, painless mass. We report a case of a 20 year old African male seen at Kikuyu Eye Unit, Kenya, with a 12 year history of a gradually enlarging, painless orbital mass. The patient underwent skin-sparing orbital exenteration with complete tumour excision; histology confirmed diagnosis of HPC.

Alveolar soft-part sarcoma of the orbit.

Alveolar soft-part sarcoma (ASPS) is a rare soft tissue tumour of uncertain cellular origin. It accounts for only 1% of all sarcomas, which themselves represent only a small proportion of human tumours. ASPS can arise in any soft tissue of the body, but there is an unexplained predilection for the right side. The most common site for paediatric ASPS is in the head and neck region, although involvement of the orbit is rare, with fewer than 30 reported cases. A case of a 5-year-old Kenyan boy with left-sided orbital ASPS is reported and the difficulties of diagnosing rare tumours are discussed.

Outcomes of bilateral cataract surgery in Tanzanian children.

OBJECTIVE: To investigate outcomes of bilateral pediatric cataract surgery in east Africa. DESIGN: Retrospective interventional case series. PARTICIPANTS: Two hundred forty-three children who underwent bilateral cataract surgery at the Comprehensive Community-Based Rehabilitation for Tanzania Disability Hospital between 2001 and 2004. METHODS: Demographic, surgical, preoperative, and postoperative clinical characteristics obtained from patient records were entered into a database (Microsoft Excel; Microsoft, Redmond, WA), and statistical analysis was conducted using SPSS software for Windows (SPSS, Inc., Chicago, IL). MAIN OUTCOME MEASURES: Postoperative visual acuities and factors affecting them and postoperative refraction results. RESULTS: Intraocular lenses were inserted in the first eyes of 232 children (149 Alcon AcrySof [Alcon Laboratories, Fort Worth, TX], 83 polymethyl methacrylate [PMMA]). Fifty-eight (62%) of 94 patients with final follow-up acuities recorded in both eyes achieved 20/60 or better in their better eye and 13 (13%) of 94 patients were blind. Of the various predictors of good visual outcome identified for children or eyes, only absence of preoperative blindness (odds ratio [OR], 7.3; 95% confidence interval [CI], 3.0-18.0; P<0.0005) remained significant in a multivariate logistic regression model. One hundred nine (51%) of 212 refracted first eyes had early postoperative refractive error spherical equivalent magnitudes of 2 diopters (D) or more. Ninety-nine (47%) of 212 eyes had initial postoperative cylinders of 3 D or more, dropping to 30 (18%) of 164 of those who had later follow-up refraction. Presence of biometric data was not associated with smaller postoperative refractive errors. Eyes with AcrySof lenses were less likely (OR, 2.5; 95% CI, 1.04-6.06) to have more than 3 D of astigmatism at latest follow-up. AcrySof lenses also were more likely (OR, 2.1; 95% CI, 1.2-3.7) to be fixated in the bag than PMMA lenses. Acute fibrinous uveitis occurred in 30 cases (12%), and transient corneal haze occurred in 20 cases (8%). Twenty-seven (11%) had chronic complications, 69 (28%) underwent a further general anesthetic procedure, and 9 (4%) underwent yytrium-aluminum-garnet capsulotomy. CONCLUSIONS: Preoperative blindness was the strongest predictor of poor postoperative visual outcome; the use of AcrySof lenses as opposed to PMMA lenses made in-the-bag fixation more likely and also reduced postoperative astigmatism.

Audit of trabeculectomy at a tertiary referral hospital in East Africa.

AIM: To audit medium-term results of trabeculectomy at a tertiary referral unit in east Africa. METHODS: A retrospective review of the files of all patients who underwent trabeculectomy surgery at our hospital during the year 2001. RESULTS: One hundred fifty-seven patients were included. Mean age was 61 years (SD = 12). Thirty-one patients (20%) were women; 120 of 145 (83%) eyes had cup/disc ratios of 0.8 or greater preoperatively. Intraoperative 5-fluorouracil (5FU) was used in 57 cases (36%). Mean follow-up was 8 months (0-28, SD = 7). Mean intraocular pressures (IOP) was 25 mm Hg (6-70, SD = 10) preoperatively, 11 mm Hg (0-32, SD = 5) at 2 weeks postoperatively, and 13 mm Hg (0-42, SD = 6) at latest follow-up. One hundred fifteen patients (73%) had intraocular pressure of 15 mm Hg or less at latest follow-up and 141 (90%) had intraocular pressure of 21 mm Hg or less. Twenty (13%) had restarted antiglaucoma medication during the follow-up period. Thirty-seven of 148 (25%) lost 2 lines of Snellen acuity or equivalent between preoperative measurement and latest follow-up. Early postoperative complications (usually leaking bleb or shallow anterior chamber with hypotony) occurred in 18 patients (12%) and this was associated with postoperative loss of 2 or more lines of Snellen acuity (OR = 1.11 95% CI 1.02-1.22, P= 0.02). Patients having had 5FU had lower (t = 3.11, P= 0.02) mean intraocular pressure at 2-month follow-up (12 mm Hg, SD = 5) than those who did not receive it (16 mm Hg, SD = 6) but there was no significant difference at later follow-ups. Men (mean 14 mm Hg, SD = 6) had higher (t = 2.71, P= 0.01) intraocular pressures at latest follow-up than women (mean 11 mm Hg, SD = 3). CONCLUSIONS: The results of surgery compare well with other reported series. 5FU did not demonstrate significant benefit in terms of intraocular pressure lowering beyond 2 months postoperatively. Early postoperative hypotony should be avoided. The gender difference in intraocular pressure results has not previously been reported and deserves further investigation.