Ocular Findings in Posterior Microphthalmos

Overview

Journal Saudi J Ophthalmol

Specialty Ophthalmology

Date 2019 Apr 2

PMID 30930662

Citations 3

Authors

Kursad Ramazan Zor

Erkut Kucuk

Nesrin Tutas Gunaydin

Feyza Onder

Affiliations

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Abstract

Aim: To report a critical case series of six patients with posterior microphthalmos (PM).

Method: Complete ophthalmologic examinations of all patients were performed using best-corrected visual acuity (BCVA), cycloplegic refraction, applanation tonometry, slit lamp biomicroscopy of the anterior segment, fundoscopy, A and B mode ultrasonography (USG), keratometry, and optic coherence tomography (OCT).

Results: The most significant clinical characteristics of male patients aged 10-25 years was the presence of shorter posterior segments (mean: 15.27-18.91 mm) accompanying high hyperopia (mean +9.00 - +18.50 diopter) despite the normal anterior segment findings. The BCVA ranged between 20/320 and 40/100. Retinal folds were detected bilaterally on the papillomacular band in all patients. Although neurosensory retina was included in the fold in OCT images, retinal pigment epithelium, choroid, and sclera were not included in the fold. Pigmentary retinopathy was detected in one patient.

Conclusion: Despite normal anterior segment, posterior microphthalmos is characterized with high hyperopia, and shorter axial length and bilateral papillomacular retinal fold. Refractive amblyopia, uveal effusion syndrome, retinal detachment and macular hole are complications that can be corrected. Posterior microphthalmos must be kept in mind in patients with a normal anterior segment, and high hyperopia.

Citing Articles

Insight into small eyes: a practical description from phenotypes presentations to the management.

Niazi S, Dhubhghaill S, Doroodgar F, Gatzioufas Z, Dehghan M Int J Ophthalmol. 2024; 17(2):380-391.

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Comparisons of size of foveal avascular zone area among children with posterior microphthalmos, high hyperopia, and normal eyes.

Sasaki K, Sasaki K, Hirota M, Hayashi T, Mizota A Int Ophthalmol. 2022; 42(8):2599-2607.

PMID: 35366136 DOI: 10.1007/s10792-022-02250-4.

Posterior segment abnormalities in posterior microphthalmos.

Raval N, Zhang C, Yao W, Lin J Am J Ophthalmol Case Rep. 2020; 20:100915.

PMID: 32964171 PMC: 7490723. DOI: 10.1016/j.ajoc.2020.100915.

References

Boynton J, PURNELL E . Bilateral microphthalmos without microcornea associated with unusual papillomacular retinal folds and high hyperopia. Am J Ophthalmol. 1975; 79(5):820-6. DOI: 10.1016/0002-9394(75)90743-6. View

Franceschetti A, GERNET H . [ULTRASONICS DIAGNOSIS OF MICROPHTHALMIA WITHOUT MICROCORNEA, WITH MACROPHAKIA, HIGH HYPERMETROPIA ASSOCIATED WITH TAPETO-RETINAL DEGENERATION, A GLAUCOMATOUS PREDISPOSITION AND DENTAL ANOMALIES (NEW FAMILIAL SYNDROME)]. Arch Ophtalmol Rev Gen Ophtalmol. 1965; 25:105-16. View

Yu S, Gao Y, Liang X, Huang Y . Acquired retinoschisis resolved after 23Gage pars plana vitrectomy in posterior microphthalmos. BMC Ophthalmol. 2014; 14:65. PMC: 4045141. DOI: 10.1186/1471-2415-14-65. View

Khairallah M, Messaoud R, Zaouali S, Ben Yahia S, Ladjimi A, Jenzri S . Posterior segment changes associated with posterior microphthalmos. Ophthalmology. 2002; 109(3):569-74. DOI: 10.1016/s0161-6420(01)00996-4. View

Jackson T, Yang Y, Shun-Shin G . Spectral domain optical coherence tomography findings in retinal folds associated with posterior microphthalmos. J AAPOS. 2012; 16(4):389-91. DOI: 10.1016/j.jaapos.2012.02.020. View

Kida Y, Kurome H, Hayasaka S . Bilateral microphthalmos with poor visual acuity, high hyperopia, and papillomacular retinal folds in siblings. Jpn J Ophthalmol. 1995; 39(2):177-9. View

Ayala-Ramirez R, Graue-Wiechers F, Robredo V, Amato-Almanza M, Horta-Diez I, Zenteno J . A new autosomal recessive syndrome consisting of posterior microphthalmos, retinitis pigmentosa, foveoschisis, and optic disc drusen is caused by a MFRP gene mutation. Mol Vis. 2006; 12:1483-9. View

Lee S, Ai E, Lowe M, Wang T . Bilateral macular holes in sporadic posterior microphthalmos. Retina. 1990; 10(3):185-8. View

Kiratli H, Tumer B, Kadayifcilar S . Bilateral papillomacular retinal folds and posterior microphthalmus: new features of a recently established disease. Ophthalmic Genet. 2000; 21(3):181-4. View

10.

Ryckewaert M, Zanlonghi X, CONSTANTINIDES G . High hyperopia with papillomacular fold. Ophthalmologica. 1992; 204(1):49-53. DOI: 10.1159/000310266. View

11.

Nowilaty S, Khan A, Aldahmesh M, Tabbara K, Al-Amri A, Alkuraya F . Biometric and molecular characterization of clinically diagnosed posterior microphthalmos. Am J Ophthalmol. 2012; 155(2):361-372.e7. DOI: 10.1016/j.ajo.2012.08.016. View

12.

Elder M . Aetiology of severe visual impairment and blindness in microphthalmos. Br J Ophthalmol. 1994; 78(5):332-4. PMC: 504780. DOI: 10.1136/bjo.78.5.332. View

13.

Tekin K, Teke M, Citirik M . CLINICAL APPRAISAL AND RETINAL IMAGING IN POSTERIOR MICROPHTHALMOS. Semin Ophthalmol. 2017; 33(3):412-418. DOI: 10.1080/08820538.2017.1284869. View

14.

Relhan N, Jalali S, Pehre N, Rao H, Manusani U, Bodduluri L . High-hyperopia database, part I: clinical characterisation including morphometric (biometric) differentiation of posterior microphthalmos from nanophthalmos. Eye (Lond). 2015; 30(1):120-6. PMC: 4709547. DOI: 10.1038/eye.2015.206. View

15.

Hmani-Aifa M, Salem S, Benzina Z, Bouassida W, Messaoud R, Turki K . A genome-wide linkage scan in Tunisian families identifies a novel locus for non-syndromic posterior microphthalmia to chromosome 2q37.1. Hum Genet. 2009; 126(4):575-87. DOI: 10.1007/s00439-009-0688-8. View

16.

Park S, Ahn Y, Shin S, Lee Y . Clinical features of posterior microphthalmos associated with papillomacular fold and high hyperopia. Clin Exp Optom. 2016; 99(6):590-593. DOI: 10.1111/cxo.12371. View

17.

Spitznas M, Gerke E, BATEMAN J . Hereditary posterior microphthalmos with papillomacular fold and high hyperopia. Arch Ophthalmol. 1983; 101(3):413-7. DOI: 10.1001/archopht.1983.01040010413014. View