Optic neuritis in a child with biotinidase deficiency case report and literature review

Abdul-Aziz Hayati1, Wan-Hazabbah Wan-Hitam1, Min-Tet Cheong1, Rohaizan Yunus2, Ismail Shatriah11Department of Ophthalmology, School of Medical Sciences, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan, Malaysia; 2Department of Radiology, School of Medical Sciences, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan, MalaysiaAbstract: Optic atrophy has often been reported in children with biotinidase deficiency. The visual prognosis is usually poor. This report is of a 6-year-old boy with an early onset of biotinidase deficiency who presented with acute profound visual loss in both eyes. Fundoscopy revealed swollen discs in both eyes, and the imaging was consistent with bilateral optic neuritis. He was treated with systemic corticosteroid, and commenced on oral biotin. The final visual outcome was promising.Keywords: optic neuritis, children, biotinidase deficienc

Comparison of retinal vascular geometry in obese and non-obese children

PURPOSE: Childhood obesity is associated with adult cardiometabolic disease. We postulate that the underlying microvascular dysfunction begins in childhood. We thus aimed to compare retinal vascular parameters between obese and non-obese children. METHODS: This was a cross-sectional study involving 166 children aged 6 to 12 years old in Malaysia. Ocular examination, biometry, retinal photography, blood pressure and body mass index measurement were performed. Participants were divided into two groups; obese and non-obese. Retinal vascular parameters were measured using validated software. RESULTS: Mean age was 9.58 years. Approximately 51.2% were obese. Obese children had significantly narrower retinal arteriolar caliber (F(1,159) = 6.862, p = 0.010), lower arteriovenous ratio (F(1,159) = 17.412, p < 0.001), higher venular fractal dimension (F(1,159) = 4.313, p = 0.039) and higher venular curvature tortuosity (F(1,158) = 5.166, p = 0.024) than non-obese children, after adjustment for age, gender, blood pressure and axial length. CONCLUSIONS: Obese children have abnormal retinal vascular geometry. These findings suggest that childhood obesity is characterized by early microvascular abnormalities that precede development of overt disease. Further research is warranted to determine if these parameters represent viable biomarkers for risk stratification in obesity

Unilateral rhino&ndash;orbital&ndash;cerebral mucormycosis with contralateral endogenous fungal endophthalmitis

Hao Chi Ho,1,2 On Heong Liew,2 Swee Sew Teh,2 Hashim Hanizasurana,2 Mohtar Ibrahim,1 Ismail Shatriah1 1Department of Ophthalmology, School of Medical Sciences, Universiti Sains Malaysia, Kelantan, 2Department of Ophthalmology, Hospital Selayang, Lebuhraya Selayang-Kepong, Selangor Darul Ehsan, Malaysia Abstract: Rhino&ndash;orbital&ndash;cerebral mucormycosis (ROCM) is an uncommon but fatal fungal infection. We report a rare case of unilateral ROCM with ipsilateral central retinal artery occlusion and contralateral choroiditis, which later progressed to endogenous fungal endophthalmitis. The patient was successfully treated with sinuses debridement, systemic liposomal amphotericin B, and intravitreal amphotericin B. The endophthalmitis completely resolved with good vision, but the ROCM eye remained blind due to central retinal artery occlusion. Keywords: rhino&ndash;orbital&ndash;cerebral mucormycosis, central retinal artery occlusion, chorioditis, endogenous fungal endophthalmitis, Rhizopu

Comparison of retinal vascular geometry in obese and non-obese children

10.1371/journal.pone.0191434PLoS ONE132e019143

Choroidal neovascularization secondary to Best&rsquo;s vitelliform macular dystrophy in two siblings of a Malay family

Koh Alisa-Victoria,1 Tan Jin-Poi,1 Ismail Shatriah,1 Embong Zunaina,1 Nor Fariza Ngah2 1Department of Ophthalmology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Kelantan, 2Department of Ophthalmology, Hospital Selayang, Lebuhraya Selayang-Kepong, Batu Caves, Selangor, Malaysia Abstract: Best&rsquo;s vitelliform macular dystrophy complicated with choroidal neovascularization is rare in children. We report three children from a Malay family of five siblings with Best&#39;s vitelliform macular dystrophy, in which two of them subsequently developed choroidal neovascularization. The possible pathogenesis of this rare condition is described and highlighted in this report. Keywords: Best&rsquo;s vitelliform macula dystrophy, choroidal neovascularization, children, Mala

Effect of axial eye length on retinal vessel parameters in 6 to 12-year-old Malay girls

10.1371/journal.pone.0170014PLoS ONE121e017001

Effect of Axial Eye Length on Retinal Vessel Parameters in 6 to 12-Year-Old Malay Girls

PURPOSE: Retinal vessel analysis is affected by both systemic and ocular factors. Malays are the major ethnicity in South East Asia. Data on the retinal microvasculature in Malays is limited, especially among children. We aim to evaluate the influence of ocular biometry on retinal vessel parameters in young Malay girls. METHODS: This was a cross-sectional, hospital-based study involving 86 Malay girls aged 6 to 12 years old in Hospital Universiti Sains Malaysia from 2015-2016. Ocular examination, refraction, biometry, retinal photography, and anthropometric measurements were performed. The central retinal arteriolar equivalent (CRAE), central retinal venular equivalent (CRVE) and overall fractal dimension (Df) were measured using validated computer-based methods (Singapore I vessel analyzer, SIVA version 3.0, Singapore). The associations of ocular biometry and CRAE, CRVE and Df were analyzed using multivariable analysis. RESULTS: The mean CRAE, CRVE and Df in Malay girls were 171.40 (14.40) um, 248.02 (16.95) um and 1.42 (0.05) respectively. Each 1 mm increase in axial length was associated with a reduction of 4.25 um in the CRAE (p = 0.03) and a reduction of 0.02 in the Df (p = 0.02), after adjustment for age, blood pressure and body mass index. No association was observed between axial length and CRVE. Anterior chamber depth and corneal curvature had no association with CRAE, CRVE or Df. CONCLUSION: Axial length affects retinal vessel measurements. Narrower retinal arterioles and reduced retinal fractal dimension were observed in Malay girls with longer axial lengths

Optic disc topography in Malay patients with normal-tension glaucoma and primary open-angle glaucoma

Abdul Rahim Adlina,1,2 Koh Alisa-Victoria,1 Ismail Shatriah,1 Ahmad Tajudin Liza-Sharmini,1 Mt Saad Ahmad2 1Department of Ophthalmology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia; 2Department of Ophthalmology, Hospital Sultanah Bahiyah, Alor Setar, Kedah, Malaysia Background: There are limited data concerning the optic disc topography in normal-tension glaucoma (NTG) and primary open-angle glaucoma (POAG) patients living in Southeast Asian countries. This study aims to compare optic disc parameters in patients with NTG and POAG in Malaysia and to discuss the results in comparison with studies of NTG and POAG in other Asian countries.Methods: This prospective cross-sectional study was performed in two hospitals with glaucoma service in Malaysia from 2010 to 2012. Seventy-seven patients of Malay ethnicity were enrolled in this study, including 32 NTG patients and 45 POAG patients. Using the Heidelberg Retinal Tomograph III, we measured optic disc area, cup area, rim area, cup volume, rim volume, cup-to-disc area ratio, mean cup depth, maximum cup depth, cup shape measure, height variation contour, mean retinal nerve fiber layer thickness, and retinal nerve fiber layer cross-sectional area. Results: The eyes for NTG patients had significantly larger optic disc areas (2.65 [standard deviation, 0.41] vs 2.40 [standard deviation, 0.36] mm&sup2;, respectively; P=0.006) and cup areas (1.54 [standard deviation, 0.43] vs 1.32 [standard deviation, 0.40] mm&sup2;, respectively; P=0.027) compared with the eyes of POAG patients. Comparison of the other parameters between the two groups revealed no significant difference (P&gt;0.050). The moderate and severe NTG patients showed significantly deeper cups and larger disc and cup areas when compared with the moderate and severe POAG patients (P&lt;0.050).Conclusion: The NTG patients in this study have notably larger optic disc and cup areas than the POAG patients. Our observations are consistent with those reported in studies of NTG and POAG patients in Korea. The deeper cups and larger disc and cup areas may serve as indicators of severity when comparing NTG with POAG. However, these findings require verification with IOP and visual field results. Keywords: normal-tension glaucoma, primary open-angle glaucoma, Malay ethnicity, optic disc topography, Heidelberg Retina Topography II