Approaches to the epidemiology of NOHL in the region of Madrid: Survey of neuro-ophthalmologists.

OBJECTIVE: To estimate the epidemiology of Leber's optic neuropathy (NOHL) in the Region of Madrid. MATERIAL AND METHODS: The neuro-ophthalmologists who work at public hospitals of the CAM were interviewed by telephone. They were asked about the number of patients with NOHL that they had diagnosed during the time that they had been responsible for the neuro-ophthalmology department of that public hospital. The time worked and the population attended by the hospital were used to calculate the number of inhabitant-years in follow-up by each center during the corresponding period. The basic information of each case (date of birth, mutation, date of visual loss) was registered to avoid duplications. RESULTS: Our work estimates a global incidence of 2.34 cases for 10,000,000 inhabitants-year and a prevalence estimated from incidence of one case for each 106.682 inhabitants. This prevalence was very similar in all the studied areas and considerably lower than that reported by other studies. CONCLUSION: This work constitutes the first approach to the epidemiology of this disease in Spain. The prevalence of LHON in the region of Madrid, is probably lower than that reported in the literature in other regions. The prevalence and the incidence were homogeneously low in the 26 studied areas.

Agreement and differences between macular values in children using two types of spectral optical coherence tomography.

OBJECTIVES: To determine the degree of agreement and differences between macular parameters according to sex, age, laterality, ethnicity and type of optical coherence tomography (OCT) in paediatric patients. MATERIALS AND METHODS: Prospective, transversal, and comparative study. OCT Spectralis and OCT Cirrus were used to obtain the macular values of both eyes in 100 patients between 5 and 15 years without ocular pathology. Due to the similarity between the values of both eyes, in the final sample we only considered the right eyes (OD) (n = 100). RESULTS: The average central macular thickness (CMT) was 244.2 µm with Cirrus and 261.7 µm with Spectralis. The degree of agreement for the GMC was moderate (Intraclass Correlation Coefficient: 0.66) and from low to very low in the other quadrants. The GMC values of both systems were significantly higher in men than in women (P < .05). The macular retinal nerve fibre layer (mRNC), ganglion cell layer (GCC), the inner plexiform layer (IPLC), and the inner nuclear layer (INFL) had significantly higher values in men than in women (P < .01). There were no statistically significant differences in the GMC by age and laterality, however it was significantly higher in ethnic European children than in ethnic Latin American children; Cirrus (P = .042) and Spectralis (P = .033). CONCLUSIONS: Macular parameters obtained by OCT Spectralis and OCT Cirrus should not be interchangeable in pediatric patients.

Agreement in retinal nerve fiber layer values and comparison in children using two optical coherence tomography devices. / Grado de concordancia y diferencias entre los valores de capa de fibras nerviosas de la retina peripapilar en niños mediante dos tipos de tomografía de coherencia óptica espectral.

OBJECTIVE: To assess absolute agreement and differences in peripapillary retinal nerve fiber layer (pRNFL) values according to sex, age, laterality, origin and optical coherence tomography (OCT) type in normal pediatric patients undergoing OCT scans using both Cirrus and Spectralis. MATERIAL AND METHODS: Prospective, cross-sectional, and comparative study. We used Spectralis OCT and Cirrus OCT to measure optic disc values of both eyes in one hundred pediatric patients (age 5 to 15years), with no previous ocular pathology or systemic disease that could affect the eye. Due to the similarity between eyes, only right eyes were considered (n=100). RESULTS: The average peripapillary retinal nerve fiber layer (pRNFL) values were 99.6µm with Cirrus and 106.7µm with the Spectralis system. The four quadrants followed the ISNT rule (inferior-superior-nasal-temporal). The average pRNFL measures correlated negatively with the AL (P<.01) in both OCTs, and the degree of agreement between OCTs for pRNFL and inferior RNFL was moderate (CCI: 0.67 and 0.61, respectively), and low in the rest of the sectors. The average pRNFL values of both OCTs were higher in women than in men (P>.05) and there were also no statistical differences in the pRNFL according to age or laterality (P>.05). All RNFL values were significantly different between both types of OCTs (P<.05) and average pRNFL values were significantly thicker (P<.05) in Latin American than in European children in both eyes. CONCLUSIONS: We suggest that the respective peripapillary RNFL values obtained using these two OCTs should not be considered interchangeable in pediatric patients.

[Pseudo-vitelliform maculopathy and bilateral choroidal folds: differential diagnosis]. / Maculopatía pseudoviteliforme asociada a pliegues coroideos bilaterales. Diagnóstico diferencial.

CLINICAL CASE: A 64 year old woman. Best corrected acuity right eye (RE) 0.5 and 0.7 left eye (LE). Bilateral pseudophakia. No inflammatory signs. Normal IOP. RE fundus showed a rounded, yellow and excessive subfoveal deposit with positive autofluorescence. Multiple equatorial drusen and choroidal folds in both eyes. Fluorescein angiography of RE showed early foveal hypofluorescence and delayed hyperluorescence. Optical coherence tomography revealed a hiperreflective deposit over the foveal epithelium pigment. Visual fields, ocular ultrasounds and electrooculograms were normal. Non-specific alterations in colour tests. CONCLUSION: Vitelliform maculopathy and choroidal folds are very rare diseases and, exceptionally, appear together.