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      Multimodal imaging in posterior microphthalmos

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          Abstract

          Purpose

          To evaluate the multimodal imaging including optical coherence tomography angiography (OCTA) findings in patients with posterior microphthalmos (PM).

          Methods

          In an observational case series, four eyes of two patients, eight and twenty-three years old, with clinical proven PM underwent complete ophthalmic examination, including refraction, fluorescein angiography, optical coherence tomography (OCT), OCTA, B-scan ultrasonography, axial length measurement using IOL Master optical measuring, and Pentacam evaluation.

          Results

          Both patients were high hyperopic with partial thickness retinal fold in macula, retinoschisis, and foveal hypoplasia. Axial length was less than 17 mm with scleral thickening in all eyes. OCTA showed absence of foveal avascular zone (FAZ) in both superficial and deep capillary plexuses. Pentacam showed corneal steepness, shallow anterior chamber, and low anterior chamber volume.

          Conclusion

          OCTA findings showed absence of avascular zone in both superficial and deep capillary plexuses, while OCT shows partial thickness retinal fold and retinoschisis.

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          Posterior segment changes associated with posterior microphthalmos.

          Moncef Khairallah, Riadh Messaoud, Sonia Zaouali … (2002)
          To characterize and analyze the posterior segment ocular involvement in patients with posterior microphthalmos. Retrospective observational case series. Eighteen patients (8 sporadic cases and 10 siblings from 5 different families) between the age of 4 and 36 years with posterior microphthalmos. Records of patients with posterior microphthalmos over a 5-year-period were reviewed, including clinical, fundus photographic, fluorescein angiographic, and ultrasonographic findings, and management. All patients had bilateral foreshortening of the posterior ocular segment (range, 7--11.2 mm) with associated high hyperopia (range, +12.00--+19.00 diopters) and normal or slightly smaller than normal anterior segment dimensions. Visual acuity ranged from 20/200 to 20/40. Inheritance of this syndrome was compatible with an autosomal recessive pattern. Posterior segment changes included bilateral elevated papillomacular retinal fold (13 patients, 72.2%); fine retinal folds (6 patients, 33.3%); chorioretinal folds (11 patients, 61.1%); uveal effusion syndrome (3 patients, 16.7%); pigmentary retinopathy (4 patients, 22.2%), including retinitis punctata albescens in 1 patient; absence or marked reduction of the capillary-free zone (18 patients, 100%); crowded optic discs (18 patients, 100%); and sclerochoroidal thickening on ultrasonography (18 patients, 100%). Two patients with uveal effusion were successfully treated with scleral surgery. A wide variety of congenital or acquired posterior segment changes may be encountered in patients with posterior microphthalmos. Although high hyperopia and elevated papillomacular retinal fold are the main causes of visual impairment, other chorioretinal changes, such as pigmentary retinopathy, chorioretinal folds and uveal effusion syndrome, should be considered as causes of visual disturbance in patients with posterior microphthalmos. Early ultrasonographic diagnosis, close follow-up, and appropriate management are mandatory to improve or maintain visual function in such patients.
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            High-hyperopia database, part I: clinical characterisation including morphometric (biometric) differentiation of posterior microphthalmos from nanophthalmos

            N. Relhan, S Jalali, N Pehre … (2016)
            Purpose To characterise and differentiate posterior microphthalmos (PM) and nanophthalmos (NO) using morphometric parameters. Patients and methods Consecutive case database of patients with hyperopia >+7.00 D sphere was analysed retrospectively for clinical and biometric characterisation. Thirty-eight consecutive high-hyperopic subjects (75 eyes) with axial lengths <20.5 mm underwent uniform comprehensive ocular evaluation. Twenty-five subjects were diagnosed as PM and 13 as NO based on the horizontal corneal diameter. Parameters analysed included visual acuity, refraction, horizontal corneal diameter, anterior chamber depth, lens thickness, axial length, fundus changes, and associated ocular pathology. Primary outcome measures: ocular biometry difference between PM and NO. Secondary outcome measures: differences in associated ocular pathologies between PM and NO. Results Hyperopia ranged from +7 to +17 D and was similar in the two groups. Lens thickness was statistically more in NO than in PM group (4.53±0.75 mm vs 3.82±0.48 mm, P <0.001), whereas anterior chamber depth was more in the PM than in NO group (3.26±0.36 mm, vs 2.59±0.37 mm, P <0.001). NO had higher association with angle-closure glaucoma (66.7% vs 0%) and pigmentary retinopathy (38.5 vs 8.0%) but lesser association with macular folds (0% vs 24%) as compared with PM. NO was associated with poorer visual acuity. Conclusion PM and NO have significant differences in lens thickness, anterior chamber depth, prevalence of glaucoma, pigmentary retinopathy, macular pathology, and visual acuity while being similar in hyperopic refraction.
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              Hereditary posterior microphthalmos with papillomacular fold and high hyperopia.

              E Gerke, Randall J. Bateman, M Spitznas (1983)
              Five patients had a bilateral hereditary ocular syndrome composed of posterior microphthalmos with a papillomacular fold and high hyperopia. Anterior segment dimensions were near normal; the vitreous compartment was markedly fore-shortened. A papillomacular retinal fold extending from the center of the fovea toward the optic nerve head was present. Visual acuity ranged from 0.05 (20/400) to 0.6 (20/33); refractive errors ranged from + 11.25 to + 17.50 diopters. An autosomal recessive pattern of inheritance is postulated.
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                Author and article information

                Contributors
                Mojtaba Abrishami
                Journal
                Journal ID (nlm-ta): J Curr Ophthalmol
                Journal ID (iso-abbrev): J Curr Ophthalmol
                Title: Journal of Current Ophthalmology
                Publisher: Elsevier
                ISSN (Electronic): 2452-2325
                Publication date PMC-release: 04 February 2019
                Publication date Collection: September 2019
                Publication date (Electronic): 04 February 2019
                Volume: 31
                Issue: 3
                Pages: 335-338
                Affiliations
                [a ]Eye Research Center, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran
                [b ]Eye Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
                Author notes
                [∗ ]Corresponding author. Eye Research Center, Khatam-al-Anbia Eye Hospital, Qarani Blvd, Mashhad, 9195965919, Iran. mojtaba_abrishami@ 123456yahoo.com
                Article
                Publisher ID: S2452-2325(18)30203-8
                DOI: 10.1016/j.joco.2019.01.001
                PMC ID: 6742752
                SO-VID: a2a93782-0247-450b-b044-55f94c90b493
                Copyright © © 2019 Iranian Society of Ophthalmology. Production and hosting by Elsevier B.V.
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 4 September 2018
                Date revision received : 26 December 2018
                Date accepted : 4 January 2019
                Categories
                Subject: Article

                Keywords: posterior microphthalmos,optical coherence tomography angiography,retinal fold,macula
                Data availability:
                Keywords: posterior microphthalmos, optical coherence tomography angiography, retinal fold, macula

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