Ambiguous genitalia, giant congenital melanocytic nevus and subpulmonic outlet ventricular septal defect in an African child with Neurofibromatosis 1.

Neurofibromatosis type 1 is an autosomal dominant multisystemic disease caused by mutation of the neurofibromin (NF1) gene located on chromosome 17q11. We report a case of Neurofibromatosis 1 with ambiguous genitalia, giant congenital melanocytic nevus, and associated subpulmonic outlet ventricular septal defect, hitherto unreported in sub-Saharan Africa. In addition, a literature review of congenital heart diseases associated with Neurofibromatosis 1 is presented.

Sub-specialization among Nigerian ophthalmologists: status, disposition and barriers.

PURPOSE: To determine the status of sub-specialization among Nigerian ophthalmologists as well as their dispositions and barriers against sub-specialization with a view to providing valuable information for the purpose of human resources for eye care planning thereby providing useful insight into the future of ophthalmic practice in Nigeria. METHODS: This was a web-based, cross-sectional study conducted among ophthalmologists in Nigeria. An online questionnaire was distributed through e-mails using Qualtrics software (Qualtrics, Provo, UT, USA). Information concerning socio-demographic characteristics, type of practice, location of practice, years of practice, status and disposition to sub-specialization as well as barriers to sub-specialization were obtained through the questionnaire. RESULTS: Two hundred and four Nigerian ophthalmologists participated in the study out of which 118 (57.8%) were females. One hundred and ten (54.0%) respondents had undergone sub-specialty training. The sub-specialties with the highest number of patronage was Paediatric Ophthalmology and Strabismus (14.2%). Respondents who had practised for more than 7 years were three times more likely to have undergone sub-specialty training compared to respondents who had practised for 7 years and below [odds ratio (OR) = 3.01, 95% confidence interval (CI) = 1.33-6.83, p = 0.01]. The main barriers to the availability and uptake of sub-specialty services as well as the challenges of sub-specialty services at the centres with established sub-specialty practice were non-availability/inadequate trained specialist and inadequate equipment. CONCLUSION: Nigerian ophthalmologists are well disposed to sub-specialization although the extent of sub-specialization among them was a little above average. The main barriers to the availability and uptake of sub-specialty services as well as the challenges of sub-specialty services at the centres with established sub-specialty practice in this study were non-availability/inadequate trained specialist and inadequate equipment.

Assessment of eye drop instillation techniques among patients with primary open angle glaucoma in a Nigerian tertiary hospital.

PURPOSE: To assess the technique of eye drop instillation and its determinants among patients with primary open angle glaucoma (POAG) attending a Nigerian tertiary hospital. METHODS: This study was a cross-sectional observational study conducted among 130 participants. Systematic sampling technique was used. Inclusion criteria were the presence of POAG, in individuals ≥ 18 years, and self-instilling their ocular hypotensive medications for at least 6 months. Demographic data and clinical characteristics were obtained using an interviewer-administered questionnaire and clinical examination. All participants underwent eye drop instillation of sterile water. Administration techniques were observed and graded using a comprehensive grading scheme. Patients with poor techniques were educated. Determinants of poor eye drop administration technique were also explored. RESULTS: One hundred and thirty patients with POAG were studied. The mean age was 57.13 ± 13.20 years, and 63% percent of the participants had poor eye drop administration technique. On multivariate analysis, previous eye drop instillation education significantly influenced eye drop instillation technique (p = 0.02; OR = 3.230; 95% CI = 1.173-8.896). Among the participants, 47 (36%) touched the globe, 128 (98.5%) did not wash their hands, and 126 (97%) did not occlude the punctum. Sequel to the training, mean score of subjects improved from 2.8 ± 1.1 to 4.1 ± 1.3 (p value < 0.001). CONCLUSION: This study demonstrated that a high proportion of POAG patients had poor eye drop instillation technique despite long-term self-use of topical medication. Thus, this aspect of therapy deserves periodic scrutiny by the clinician.

Is primary open-angle glaucoma a risk factor for diabetic retinopathy?

PURPOSE: To compare the prevalences of diabetic retinopathy in diabetes mellitus patients, with and without primary open-angle glaucoma, with a view to determine if glaucoma is a risk factor for the development of diabetic retinopathy. METHODS: Cross-sectional, comparative study consisting of 86 diabetic patients with glaucoma matched with 86 diabetic patients without glaucoma. The two groups were matched by age, sex and duration of diabetes mellitus. Demographic data were obtained via patient medical records and self-administered questionnaires. Participants underwent a standardized examination protocol including blood pressure measurement and ocular examination. Main outcome measure was the presence of diabetic retinopathy. RESULTS: Two hundred and ninety-two eyes (144 glaucomatous eyes and 148 non-glaucomatous eyes) of 172 participants with diabetes mellitus were assessed. The prevalence of diabetic retinopathy among 86 participants with glaucoma comorbidity was 23.6%, while the prevalence among 86 non-glaucomatous participants was 33.8% (p = 0.06). After the regression analysis, controlling for systemic and ocular risk factors for diabetic retinopathy, the odds of developing diabetic retinopathy were significantly higher in the glaucomatous eyes compared with eyes without glaucoma (OR: 2.75; p = 0.03; 95% CI: 1.10-6.87). CONCLUSION: This study demonstrated that glaucomatous diabetic eyes were almost three times more likely to develop diabetic retinopathy compared to non-glaucomatous diabetic eyes. Prospective studies may be required to establish a risk-cause relationship. Ocular perfusion pressure control should be considered in patients with diabetes mellitus and glaucoma.

Childhood traumatic cataract in Nigeria; a multicentre study: 2017-2021.

OBJECTIVE: To determine the frequency, demography, aetiology and mechanisms of ocular injuries associated with childhood traumatic cataract in Nigeria. METHODS: A retrospective multicentre study conducted across ten child eye health tertiary facilities in Nigeria between January 2017 and December 2021. Clinic records of all children aged 0-17 years who had been diagnosed with cataract at the various participating centres were reviewed. Information collected include: biodata, mechanism of injury; laterality, place of injury; object responsible; person responsible; duration before presentation and surgical intervention. RESULTS: A total of 636 out of 1656 children (38.4%) had traumatic cataracts during the study period. Their mean age was 109.4 ± 45.2 months with a male-to-female ratio of 2:1. Most injuries were unilateral, two (0.3%) children had bilateral involvement. Only 78 (15.3%) children presented within 4 weeks of the injury. Closed globe injuries were responsible for the traumatic cataract in 475 (74.7%) children, while open globe injuries were more likely to present within 24 h (P < 0.001). The commonest objects of injury were cane, sticks, plant, wood and play materials. Self-inflicted injuries occurred in about 82 (13%) children while 407 (64.0%) were caused by close relatives and contacts. The location where trauma occurred was home in 375 (59.8%) and school in 107 (16.8%) children. CONCLUSION: This multicentre study demonstrates that more than one-third of all childhood cataracts in Nigeria are trauma-related and majority are due to closed globe injuries. Public health interventions to reduce the occurrence of ocular trauma and to encourage early presentation after trauma are advocated.

The Quality of Life of Medically Versus Surgically Treated Primary Open-angle Glaucoma Patients at a Nigerian Hospital.

PURPOSE: The purpose of this study is to compare the quality of life (QoL) of medically treated versus surgically treated primary open-angle glaucoma (POAG) patients attending Lagos University Teaching Hospital, Lagos, Nigeria. METHODS: The study was a hospital-based, comparative cross-sectional study. Consecutive consenting POAG participants who met the inclusion criteria were recruited until the sample size was achieved. QoL of all participants was assessed using the glaucoma QoL-15 and National Eye Institute Visual Function Questionnaire-25 (NEI-VFQ-25) questionnaires. Analysis was done using the IBM statistical package for the social sciences version 25.0. RESULTS: The study involved 81 participants who were only on medical treatment for POAG and 81 age- and sex-matched POAG participants who had trabeculectomy surgery. Surgically treated participants had a lower mean intraocular pressure 11.68 mmHg when compared to the medically treated participants 14.82 mmHg. Medically treated participants however had a better overall mean glaucoma QoL using the glaucoma QoL-15 (medical 34.36 ± 10.4, surgical 39.11 ± 13.9 P = 0.015) and NEI-VFQ-25 questionnaires (medical 68.32 ± 15.0, surgical 62.44 ± 18.8 P = 0.029). Controlling for the severity of glaucoma using the glaucoma QoL-15 questionnaire, medically treated participants had a better QoL only among participants with severe POAG (medical 49.13 ± 5.9, surgical 54.06 ± 4.6 P = 0.003). While using the NEI-VFQ-25 questionnaire, medically treated participants had a significantly better QoL only among participants with moderate (medical 74.70 ± 6.6, surgical 67.07 ± 15.6 P = 0.012) and severe POAG (medical 54.52 ± 6.3, surgical 45.51 ± 10.0 P = 0.004). CONCLUSION: The study demonstrated that although participants that had trabeculectomy had a lower mean intraocular pressure, their overall mean QoL was reduced compared to the medically treated participants.

Strengthening retinopathy of prematurity screening and treatment services in Nigeria: a case study of activities, challenges and outcomes 2017-2020.

OBJECTIVES: Retinopathy of prematurity (ROP) will become a major cause of blindness in Nigerian children unless screening and treatment services expand. This article aims to describe the collaborative activities undertaken to improve services for ROP between 2017 and 2020 as well as the outcome of these activities in Nigeria. DESIGN: Descriptive case study. SETTING: Neonatal intensive care units in Nigeria. PARTICIPANTS: Staff providing services for ROP, and 723 preterm infants screened for ROP who fulfilled screening criteria (gestational age <34 weeks or birth weight ≤2000 g, or sickness criteria). METHODS AND ANALYSIS: A WhatsApp group was initiated for Nigerian ophthalmologists and neonatologists in 2018. Members participated in a range of capacity-building, national and international collaborative activities between 2017 and 2018. A national protocol for ROP was developed for Nigeria and adopted in 2018; 1 year screening outcome data were collected and analysed. In 2019, an esurvey was used to collect service data from WhatsApp group members for 2017-2018 and to assess challenges in service provision. RESULTS: In 2017 only six of the 84 public neonatal units in Nigeria provided ROP services; this number had increased to 20 by 2018. Of the 723 babies screened in 10 units over a year, 127 (17.6%) developed any ROP; and 29 (22.8%) developed type 1 ROP. Only 13 (44.8%) babies were treated, most by intravitreal bevacizumab. The screening criteria were revised in 2020. Challenges included lack of equipment to regulate oxygen and to document and treat ROP, and lack of data systems. CONCLUSION: ROP screening coverage and quality improved after national and international collaborative efforts. To scale up and improve services, equipment for neonatal care and ROP treatment is urgently needed, as well as systems to monitor data. Ongoing advocacy is also essential.

Prevalence and causes of visual impairment among hearing impaired students in Lagos, Nigeria.

OBJECTIVES: To determine the prevalence and causes of visual impairment in hearing-impaired students in Lagos, Nigeria and to assess their level of ophthalmic care utilization. METHODS: Descriptive cross sectional study carried out in a primary school for hearing impaired students in Lagos, Nigeria. Socio-demographic characteristics, medical and ocular history were obtained. Ocular examination comprised of distance and near visual acuity assessment; cover tests; anterior segment examination and direct fundoscopy. Visually impaired students had cycloplegic refraction, dilated fundoscopy and subjective refraction. In-depth interviews were carried out for parents of students requiring referral for further eye care. RESULTS: One hundred and nine students participated in the study. The prevalence of visual impairment was 19%. The causes of visual impairment were refractive error (11%), amblyopia (6%), cataract (1%) and macular scarring (1%). A lower mean age (p = 0.020) and ocular misalignment (p = 0.037) were associated with visual impairment on multivariate analysis. The spectacle correction coverage was 11% and only 8% of the students had prior ophthalmic care utilization. Ignorance of ocular morbidity and perceived inaccessibility of eye care were cited by parents/guardians as reasons for poor utilization of eye-care services. CONCLUSION: A high prevalence of visual impairment was found among the hearing impaired students; uncorrected refractive error and refractive amblyopia were the most prevalent causes. The level of prior ophthalmic service utilization was found to be low. It was recommended that ophthalmic services especially refractive services should be made available in schools for the hearing impaired.

Two cases of Vogt-Koyanagi-Harada's disease in sub-Saharan Africa.

Vogt-Koyanagi-Harada's (VKH) disease has been reported to be rare in sub-Saharan Africa. Two Nigerians with the disease are presented in this report. The first patient, a 32-year-old pregnant Nigerian woman presented with a 1-month history of bilateral blurring of vision, persistent headache, and alopecia. Presenting visual acuity was 1 m counting fingers in both eyes. Examination revealed vitiligo and poliosis with bilateral panuveitis as well as bilateral exudative retinal detachment. A clinical assessment of complete VKH disease was made. The patient commenced systemic and topical steroids that resulted in remarkable recovery of vision and control of inflammation. The second patient, a 56-year-old Nigerian woman presented with severe headache, tinnitus, and visual loss in both eyes of 2 weeks duration. There was associated redness of both eyes and photophobia. Examination showed visual acuity of Hand motion (HM) and counting fingers at 1 meter (CF). in the right and left eye, respectively, with bilateral panuveitis and bilateral exudative retinal detachment. Subsequent follow-up showed poliosis, vitiligo, and sunsetting fundus appearance. The patient improved with systemic and topical corticosteroids. Developing a high index of suspicion is necessary in diagnosing VKH disease, even in sub-Saharan Africa. Prompt institution of appropriate treatment prevents blindness.