ObjectiveTo summarize the clinical features in idiopathic hypotony maculopathy.MethodsA retrospective case series study. Eighteen eyes of 18 patients who were diagnosed with idiopathic hypotony maculopathy were enrolled in the Second Affiliated Hospital of Wenzhou Medical University from August 2012 to December 2017. There were 8 males (8 eyes) and 10 females (10 eyes). All patients underwent examinations including BCVA, optometry, slit lamp microscope, fundus color photography, UBM, B-mode ultrasound, OCT, FFA and axial length (AL). BCVA was recorded with logMAR acuity. The results of affected eyes and contralateral healthy eyes were compared. Paired t test was performed to compare the intraocular pressure (IOP), diopter and AL of the affected eyes and contralateral healthy eyes.ResultsAmong 18 eyes, there were 6 eyes with logMAR BCVA＜1.0, 10 eyes with logMAR BCVA 1.0−2.0, 2 eyes with light perception. The average diopter was +2.32±1.78 D. The average IOP was 5.18±1.38 mmHg (1 mmHg=0.133 kPa). The average AL was 20.92±1.61 mm. The differences of IOP (t=21.6, P＜0.000), diopter (t=5.9, P=0.002) and AL (t=9.13, P＜0.000) between the affected eyes and contralateral healthy eyes were significant. The inflammatory reaction in the anterior segment was observed in 13 eyes (72.22%). In the posterior segment, all the eyes were documented with chorioretinal folds, optic disc swelling and retinal phlebectasia were also demonstrated in 14 eyes, while with macular uplift in 7 eyes. In the UBM and gonioscope examination, the angle chamber was open in all patients with ciliary body cyst in 6 eyes, while no ciliary body detachment was detected. All eyes had been examined with B-scan ultrasound and found the increasing thickness of eye ball in all eyes, and nodular changes in the optic papilla in 5 eyes. The chorioretinal folds were further confirmed by OCT with the appearance of the gear shape, much more obviously in the choroid than that in retina. In the FFA, fluorescein leakage was found around the optic disc in 13 eyes at the late stage, while there was no obvious abnormal leakage in the macular or poster part of retina.ConclusionsIdiopathic hypotony maculopathy could present with various clinical manifestations. The choroiretinal folds is typical clinical sign.
ObjectiveTo observe the interobserver agreement of classification of macular degeneration in severe pathological myopia (PM) by ophthalmologists with different clinical experience. MethodsA retrospective study. From January 2019 to December 2021, 171 eyes of 102 patients with severe PM macular degeneration who were examined at Eye Center of Beijing Tongren Hospital of Capital Medical University were included in the study. The clinical data such as age, gender, axial length, spherical equivalent power, fundus color photography, and optical coherence tomography (OCT) were collected in detail. Six independent ophthalmologists (A, B, C, D, E, F) classified each fundus photography based on META-PM and ATN classification of atrophy (A) system and interobserver agreement was assessed by Kappa statistics. According to the classification standard of traction (T) in the ATN classification, the OCT images were interpreted and classified, in which T0 was subdivided into retinal pigment epithelium (RPE) and choroidal thinning, choroidal neovascularization (CNV) with partial RPE and choroidal atrophy, RPE, and choroidal atrophy. Lamellar macular hole can't be classified by ATN system, which was defined as TX. Kappa (κ) test was used to analyze the consistency of classification results between physicians A, B, C, D, E and F. κ value ≤0.4 indicates low consistency, 0.4＜κ value ≤ 0.6 indicates moderate consistency, and κ value ＞0.6 indicates strong consistency. ResultsAmong the 171 eyes of 102 cases, there were 20 males with 37 eyes (19.6%, 20/102), and 82 females with 134 eyes (80.4%, 82/102); age was 61.97±8.78 years; axial length was (30.87±1.93) mm; equivalent spherical power was (－16.56±7.00) D. Atrophy (A) classification results in META-PM classification and ATN classification, the consistency of physician A, B, C, D, E and physician F were 73.01%, 77.19%, 81.28%, 81.28%, 88.89%; κ value were 0.472, 0.538, 0.608, 0.610, 0.753, respectively. In the ATN classification, the T0, T1, T2, T3, T4, and T5 were in 109, 18, 11, 12, 9, and 8 eyes, respectively; TX was in 4 eyes. ConclusionsThere are differences in the consistency of classification of severe PM macular lesions among physicians with different clinical experience, and the consistency will gradually improve with the accumulation of clinical experience.
ObjectiveTo observe the clinical features of eyes in children with methylmalonic acidemia (MMA). MethodsA retrospective clinical case study. From June 2019 to June 2022, 13 children with MMA visited on the Department of Ophthalmology of Henan Children's Hospital were included in the study. The anterior segment and fundus were examined under surface or general anesthesia. Best corrected visual acuity (BCVA) and refraction were performed in 9 cases; fluorescein fundus angiography (FFA) was performed in 3 cases; flash electroretinogram (FERG) was performed in 6 cases; flash visual evoked potential (FVEP) was detected in 6 cases; optical coherence tomography (OCT) was performed in 3 cases. ResultsAmong the 13 pediatric patients with methylmalonic acidemia, 6 cases were male and 7 cases were female. The average age at first visit was 45 months. All cases suffered from hyperhomocysteinemia; 9 cases were with epilepsy; 2 cases were with infantile spasms; 11 cases were with stunting, 13 cases were with repeated pulmonary infection during growth period; 4 cases were with hydrocephalus; 1 cases was with hypertension and renal insufficiency. Genetic dectection results of 8 cases were recorded, MMACHC:c.609G>A:p.W203* mutation site was found in all cases. One case was accompanied by corneal ulcer. There were 10 cases with nystagmus, 4 cases with macular degeneration, 3 cases with hyperopic refractive error and esotropia. Nine cases underwent BCVA examination, BCVA was light perception-0.6. In OCT, 2 cases of 3 cases showed retinal thinning and photoreceptor cell layer atrophy in the macular area. In FFA, 2 cases of 3 cases showed circular transparent fluorescence in the macular area. Five cases of 6 cases who with FVEP had different degrees of P100 peak time delay and decreased amplitude, and 4 cases of 6 cases with FERG had decrease of a and b wave in light and dark adaptation. ConclusionsThe clinical phenotypes of eyes in children with MMA are various and the severity was different; most of them are accompanied by nystagmus, and the fundus lesions are common in the characteristic bovine eye like macular region. Those with macular disease have severe visual impairment.
Maculopathy caused by various fundus diseases in the late stage is a common cause of low vision. Medical technology is difficult to reverse the loss of macular function currently, so interventions that help improve the visual system, utilize residual visual function, and improve quality of life deserve attention. Damage to the fovea of the macula does not mean that the entire retinal function is impaired. There may be one or more retinal regions adjacent to the fovea that can serve as a fixation center. It is possible to form stable paracentral fixation, complete functional remodeling of the visual system, and effectively utilize residual visual function by taking appropriate training on these potential paracentral fixation points for most patients. In 2021, a clinical guideline has been published for low vision rehabilitation in China. In order to strengthen the precise management of diseases and develop a standard operating procedure for visual training specifically for patients with low vision due to macular disease, the National Clinical Research Center for Eye Diseases initiated and organized relevant domestic experts, utilizing the latest research experience at home and abroad, and through repeated discussions, this consensus (International Practice Guideline Registration Number: PREPARE-2023CN199) was formed as a reference for ophthalmologists, optometrists and rehabilitation physicians in their clinical research and practice.