|Year : 2021 | Volume
| Issue : 3 | Page : 393-394
Multimodal imaging of autosomal recessive cornea plana associated with hard mature and polar cataract
Vidya S Raja, Vijayalakshmi A Senthilkumar, Kavya Kondepati, Techi D Tara
Department of Glaucoma, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India
|Date of Submission||03-Jan-2021|
|Date of Acceptance||24-Feb-2021|
|Date of Web Publication||02-Jul-2021|
Dr. Vijayalakshmi A Senthilkumar
Department of Glaucoma, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu - 625020
Source of Support: None, Conflict of Interest: None
Keywords: Anterior segment OCT, autosomal recessive, cornea plana, polar cataract, ultrasound biomicroscopy
|How to cite this article:|
Raja VS, Senthilkumar VA, Kondepati K, Tara TD. Multimodal imaging of autosomal recessive cornea plana associated with hard mature and polar cataract. Indian J Ophthalmol Case Rep 2021;1:393-4
|How to cite this URL:|
Raja VS, Senthilkumar VA, Kondepati K, Tara TD. Multimodal imaging of autosomal recessive cornea plana associated with hard mature and polar cataract. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Sep 25];1:393-4. Available from: https://www.ijoreports.in/text.asp?2021/1/3/393/320001
A 39-year lady presented with drop in visual acuity in the left eye (OS) since 3 months. There was a history of defective vision in both eyes (OU) since childhood; she could read only first line in OS and first 3 lines in the right eye (OD) of vision testing chart. There were no similar complaints in the family members. At presentation, best-corrected visual acuity and intraocular pressure was 6/36 and 14 mm Hg and hand movements and 16 mm Hg in OD and OS, respectively. Slit-lamp examination OU revealed microcornea, cornea plana, central corneal opacity, widened limbal zone, early-onset arcus senilis and clear lens in OD and hard mature cataract with an anterior polar component in OS [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. Anterior segment OCT (AS-OCT) and ultrasound biomicroscopy (UBM) OU revealed shallow anterior chamber depth (2.4 mm OD/ 2.2 mm OS), clear lens in OD and anterior polar as well as posterior polar cataract in OS [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]b. Her axial lengths were OD––20.58 mm and OS––20.26 mm and keratometry readings were K1/K2-30.43D/ 35.42D OD, 27.10D/29.43D OS. Her central corneal thickness were OD 460 microns and OS 442 microns. B-scan and fundus findings were unremarkable. She underwent small incision cataract extraction with +40D PMMA rigid intraocular lens (Aurolab, Madurai, India). Her postoperative BCVA OS was 6/60 [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]b.
|Figure 1: (a) Slit lamp image OD by diffuse illumination showing corneal stromal opacity (white arrowheads), microcornea, widened limbal zone, early onset arcus senilis (white arrow), (b) slit image showing cornea plana with relatively moderate anterior chamber depth, (c and d) -slit lamp image OS showing the hard mature cataract with an anterior polar component.|
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|Figure 2: (a) AS-OCT Visante (Carl Zeiss Meditec, Inc., Dublin, CA, USA) image OD showing a slightly cone-shaped, central, corneal plaque with flattened posterior corneal surface, increased reflectance and a sharp boundary to the midperipheral cornea, (b) OS ASOCT image showing an anterior polar cataract (pyramidal cataract) (c) showing cornea plana with flattened posterior corneal surface in OS, As the patient had poor fixation in OS, motion artefacts were noted|
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|Figure 3: (a) UBM (Accutome by Keeler Plus) image of OD showing cornea plana (white arrows) and shallow AC depth both centrally & peripherally, clear lens, (b) UBM image of OS showing flat corneal curvature, shallow AC depth with anterior (white arrowheads) & posterior polar cataract (white arrows)|
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|Figure 4: (a) Slit lamp image OS at 3 weeks postoperative period showing a well apposed SICS wound with 2 tunnel sutures with pseudophakia, (b) enlarged image of the same showing central corneal stromal opacity (white arrowheads)|
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| Discussion|| |
Cornea plana is a rare condition that describes a flat cornea, where the radius of curvature is less than 43 Diopters (D) and most commonly in the range of 30–35 D. Cornea plana (CNA) is characterized by decreased corneal curvature, high hypermetropia, corneal clouding and early onset arcus senilis. Eriksson et al. described the autosomal recessive form of CNA (CNA2) that has more severe manifestations than the dominant form (CNA1) in terms of reduced visual activity, extreme hypermetropia (usually + 10 D or more), hazy corneal limbus, opacities in the corneal parenchyma, and marked arcus senilis. CNA2 has been linked to missense mutations in KERA gene encoding a cornea-specific proteoglycan, keratocan. Tahvanainen et al. described a round and opaque thickening, approximately 5 mm wide in the central cornea, occurs in most cases of CNA2 but never in CNA1. Hence, we presumed our diagnosis as CNA2 based on the typical clinical findings alone as the patient did not consent to genetic analysis. Additional anomalies such as malformations of the iris, a slit-like pupil, and adhesions between the iris & cornea, open angle glaucoma, angle closure glaucoma are more prevalent in CNA2, but these were not seen in our patient. Measuring the real IOP of such eyes is difficult since tonometers are affected by corneal curvature.
Cataract surgery in these patients are challenging even in experienced hands due to limited space in the anterior chamber as a result of flat cornea and microcornea., Imaging modalities like AS-OCT and UBM are imperative in these complicated clinical associations as they not only complement the clinical findings, also exclude the associated glaucoma, study the angle parameters, type of cataract, decide the treatment protocol and for better patient counselling. Failure to diagnose and correctly manage individuals with cornea plana at an early age can result in the development of ametropic amblyopia.
Dr. Madhu Sekhar, Chief of Cataract Clinic, Aravind Eye Hospital, Madurai
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]