Abstract
Aim Congenital corneal opacities (CCOs) are the major causes of early visual deprivation in infants. Balloon ultrasound biomicroscopy (UBM) examination is an effective method to diagnose CCO. However, whether it is suitable for children examination is still unknown. Methods 26 Peters' anomaly (PA) or Rieger's anomaly (RA) infants with congenital corneal opacities (CCO) (40 eyes) underwent UBM examinations to study their imaging features. Results Based on the results, they were divided into UBM Dx-Type I: Descemet's membrane (DM) and endothelium have heterogenous or discontinuous echo accompanied with corneal stroma echo-enhanced or shallow anterior chamber. Type II: Type I alteration plus abnormal strand of iris extended to the border of the posterior corneal defect or iridocorneal adhesion. Type III: Type I or II combined with the abnormal hyperechoic lens, lens luxation, or keratolenticular adhesion. Type IV: echoes of the DM and the endothelium are continuous, corneal stroma echo is enhanced, and an abnormal strand of peripheral iris extends to the prominent Schwalbe line, accompanied by iris stroma or pupil heteromorphism and a shallow or flat anterior chamber. Conclusion UBM not only could accurately evaluate the anterior segment abnormalities in CCO infants but also would be a step forward for the management of PA- and RA-associated CCO.
Highlights
Congenital corneal opacities (CCOs) are uncommon, occurring in only 2.2–3.1 per 100,000 newborns each year
They were divided into ultrasound biomicroscopy (UBM) Dx-Type I: Descemet’s membrane (DM) and endothelium have heterogenous or discontinuous echo accompanied with corneal stroma echo-enhanced or shallow anterior chamber
Peters’ anomaly (PA) and Rieger’s anomaly (RA) are associated with the abnormal differentiation of embryonic neural crest cells, which affect the structural development of the cornea, iris, and trabecular meshwork. erefore, it is often accompanied by secondary glaucoma [7]
Summary
Congenital corneal opacities (CCOs) are uncommon, occurring in only 2.2–3.1 per 100,000 newborns each year. Anterior segment dysgenesis is one of the primary causes, among which, Peters’ anomaly (PA), sclerocornea, and Rieger’s anomaly (RA) may be considered as the major distinct entities of primary disorders [4,5,6]. The early diagnosis of PA and RA with CCO is challenging [8]. E slit lamp procedure limits the early diagnosis of PA and RA due to the different opacities of the cornea as it is difficult to clearly define the lesion scope of the anterior segment structure. E oculists could not acquire enough data to analyze the anterior chamber, resulting in missed diagnosis or misdiagnosis of PA and RA during early disease stages. Erefore, the discovery of a method to diagnose PA and RA early is a tricky topic for ophthalmologists Few infantile patients with PA and RA could be diagnosed early with the disease, until the histological examination after penetrating the keratoplasty were performed [9]. erefore, the discovery of a method to diagnose PA and RA early is a tricky topic for ophthalmologists
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