Cornea plana 1 | |
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Other names | Autosomal Dominant Cornea Plana, CNA1. |
Cornea plana 1 is inherited in an autosomal dominant manner. | |
Specialty | Ophthalmology |
Cornea plana 1 (CNA1) is a congenital disorder that causes the cornea to flatten and the angle between the sclera and cornea to shrink. [1] This could result in the early development of arcus lipoides, hazy corneal limbus, and hyperopia. [2] Cornea plana 1 is an autosomal dominant disorder. [3]
Cornea plana commonly presents as a flat cornea, early-onset arcus lipoides, low anterior chamber depth, and an indistinct border between the sclera and cornea due to a decreased angle between the two. [2] Although a small corneal diameter is anticipated, measuring it can be challenging because the scleral tissue overlaps the cornea by a few millimeters. In the patients who have been described, the anterior chamber depth has been found to vary from 0.8 to 2.1 mm. [3]
Moreover, high hyperopia, strabismus, microcornea, posterior embryotoxon, iridocorneal adhesions, iris lumps, iris wasting, and pupillary abnormalities can all be present. [4] Instead of hyperopia, myopia has been identified in a few cases. [5] As many as 90 percent of cases have a bilateral presentation, which is linked to sclerocornea. [1] Finally, congenital ptosis has also been reported, and the absence of corneal protrusion is most likely the cause. [3]
Clinical presentation, keratometry, [5] pachymetry, and endothelial imaging are used to make the diagnosis. [6]
Since the decreased refraction in the autosomal recessive form is more severe than in the autosomal dominant form, corneal refraction measurements can be used to differentiate between CNA1 and CNA2. [3]
Histology is distinguished by acanthosis, keratinization of the corneal epithelium, Bowman's membrane defects, and stromal vascularization and scar formation. [7]
Cornea plana 1 | |
---|---|
Other names | Autosomal Dominant Cornea Plana, CNA1. |
Cornea plana 1 is inherited in an autosomal dominant manner. | |
Specialty | Ophthalmology |
Cornea plana 1 (CNA1) is a congenital disorder that causes the cornea to flatten and the angle between the sclera and cornea to shrink. [1] This could result in the early development of arcus lipoides, hazy corneal limbus, and hyperopia. [2] Cornea plana 1 is an autosomal dominant disorder. [3]
Cornea plana commonly presents as a flat cornea, early-onset arcus lipoides, low anterior chamber depth, and an indistinct border between the sclera and cornea due to a decreased angle between the two. [2] Although a small corneal diameter is anticipated, measuring it can be challenging because the scleral tissue overlaps the cornea by a few millimeters. In the patients who have been described, the anterior chamber depth has been found to vary from 0.8 to 2.1 mm. [3]
Moreover, high hyperopia, strabismus, microcornea, posterior embryotoxon, iridocorneal adhesions, iris lumps, iris wasting, and pupillary abnormalities can all be present. [4] Instead of hyperopia, myopia has been identified in a few cases. [5] As many as 90 percent of cases have a bilateral presentation, which is linked to sclerocornea. [1] Finally, congenital ptosis has also been reported, and the absence of corneal protrusion is most likely the cause. [3]
Clinical presentation, keratometry, [5] pachymetry, and endothelial imaging are used to make the diagnosis. [6]
Since the decreased refraction in the autosomal recessive form is more severe than in the autosomal dominant form, corneal refraction measurements can be used to differentiate between CNA1 and CNA2. [3]
Histology is distinguished by acanthosis, keratinization of the corneal epithelium, Bowman's membrane defects, and stromal vascularization and scar formation. [7]