Sclerocornea

Last update 01 Nov 2024

Basic Info

Synonyms

Hardening of skin and connective tissue, Sclerocornea, 巩膜化角膜

Introduction

A congenital anomaly in which a part or the whole of the cornea acquires the characteristics of sclera, resulting in clouding of the cornea. [https://orcid.org/0000-0002-0736-9199]

100 Clinical Results associated with Sclerocornea

100 Translational Medicine associated with Sclerocornea

0 Patents (Medical) associated with Sclerocornea

176

Literatures (Medical) associated with Sclerocornea

01 Nov 2024·Ophthalmology and Therapy

Preoperative Structural Risk Factors for Glaucoma After Penetrating Keratoplasty for Congenital Corneal Opacity: An Observational Study

Article

Author: Wu, Ling-Ling ; Hong, Jing ; Wu, Ling-ling ; Yu, Ting ; Qu, Jing-hao ; Wang, Xin ; Xie, Zi-Jun ; Xie, Zi-jun ; Qu, Jing-Hao

INTRODUCTIONPost-keratoplasty glaucoma (PKG) is a major complication following penetrating keratoplasty (PKP) for congenital corneal opacity (CCO). This study aims to assess the preoperative structural risk factors for PKG following PKP for CCO using ultrasound biomicroscopy (UBM).METHODSPediatric patients with CCO who underwent preoperative UBM and primary PKP were enrolled. Patients with anterior segment operation history or with a follow-up duration less than 12 months were excluded. The structural features of the anterior segment including central corneal thickness, anterior chamber depth, angle closure range (ACR), anterior synechia range, maximum iridocorneal adhesion length, abnormal iridocorneal synechia, and lens anomalies were identified on UBM images. The medical histories were reviewed to identify clinical features. The incidence of PKG was assessed to determine significant structural and clinical risk factors.RESULTSFifty-one eyes of 51 pediatric patients with CCO were included. The median age at surgery was 8.0 months, and the mean follow-up duration was 33 ± 9 months. Eleven (21.6%) eyes developed PKG. The main structural risk factors were abnormal iridocorneal synechia (P = 0.015), lens anomaly (P = 0.001), and larger ACR (P = 0.045). However, a larger range of normal anterior synechia without involvement of the angle was not a significant risk factor. Preoperative glaucoma (P < 0.001) and higher intraocular pressure (P = 0.015) were clinical risk factors. A shallow anterior chamber was a unique risk factor for sclerocornea (P = 0.019).CONCLUSIONSDetailed preoperative examination of iridocorneal synechia, lens, and angle closure using UBM is critical for PKG risk assessment, surgical prognosis evaluation, and postoperative management in patients with CCO.

01 Jun 2024·iScience

RAD21 deficiency drives corneal to scleral differentiation fate switching via upregulating WNT9B

Article

Author: Shi, Zhaoying ; Chen, Yonglong ; Chu, Wai Kit ; Liu, Hongyan ; Qi, Benxiang ; Li, Zongyi ; Liu, Guanghui ; Duan, Haoyun ; Zhang, Bi Ning ; Zhou, Qingjun

The cornea and sclera are distinct adjacent tissues, yet their stromal cells originate from common neural crest cells (NCCs). Sclerocornea is a disease characterized by an indistinguishable boundary between the cornea and sclera. Previously, we identified a RAD21 mutation in a sclerocornea pedigree. Here, we investigated the impacts of RAD21 on NCC activities during eye development. RAD21 deficiency caused upregulation of PCDHGC3. Both RAD21 knockdown and PCDHGC3 upregulation disrupted the migration of NCCs. Transcriptome analysis indicated that WNT9B had 190.9-fold higher expression in scleral stroma than in corneal stroma. WNT9B was also significantly upregulated by both RAD21 knockdown and PCDHGC3 overexpression, and knock down of WNT9B rescued the differentiation and migration of NCCs with RAD21 deficiency. Consistently, overexpressing wnt9b in Xenopus tropicalis led to ocular developmental abnormalities. In summary, WNT9B is a determinant factor during NCC differentiation into corneal keratocytes or scleral stromal cells and is affected by RAD21 expression.

01 May 2024·European Journal of Ophthalmology

Birth prevalence and characteristics of congenital corneal opacities

Article

Author: Borik, Kaitlynn ; Mohney, Brian G ; Hodge, David ; Reynolds, Margaret M

Purpose/Aim To report the birth prevalence and natural history of congenital corneal opacities among a population-based cohort of children. Materials and methods The medical records of patients <5 years diagnosed with a congenital onset corneal opacity while residing in Olmsted County, Minnesota, from January 1, 1977, through December 31, 2016, were retrospectively reviewed. Results Fourteen patients were diagnosed with a congenital corneal opacity during the 40-year study period for a birth prevalence of 1 in 5188 live births. The mean age at diagnosis was 7.5 months (range 0–48 months) and 9 (64.3%) were males. Four patients had congenital glaucoma, 4 had limbal dermoids, 2 had sclerocornea, and 1 patient each had Descemet's tear from birth trauma, herpes simplex virus type 1 keratitis, corneal leukoma, and an undiagnosed scar. Six (42.8%) patients required treatment for their underlying corneal opacity including the four patients with congenital glaucoma. The other 8 (57.1%) patients had a clear central axis. Four (28.6%) of 14 patients required amblyopia therapy, and 4 (28.6%) developed strabismus. Four (28.6%) patients had associated systemic conditions. During a mean follow up of 5.4 years (range 1.3–27.0 years), the median best corrected visual acuity (BCVA) was logmar 0.16 (20/25) (range 20/20-hand motion) with one patient with unilateral BCVA less than 20/60 and one patient with bilateral BCVA less than 20/60. Conclusions In this 40-year cohort, congenital corneal opacities were relatively rare and the result of a variety of disorders. Although amblyopia and strabismus occurred commonly, most patients had good visual outcomes.

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Sclerocornea

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