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PHOTO ESSAY
Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 393-394

Multimodal imaging of autosomal recessive cornea plana associated with hard mature and polar cataract


Department of Glaucoma, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India

Date of Submission03-Jan-2021
Date of Acceptance24-Feb-2021
Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. Vijayalakshmi A Senthilkumar
Department of Glaucoma, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu - 625020
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_26_21

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  Abstract 


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 Top


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.[1] 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.[2] CNA2 has been linked to missense mutations in KERA gene encoding a cornea-specific proteoglycan, keratocan.[3] 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.[4] 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.[2] 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.[5],[6] 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.[7] Failure to diagnose and correctly manage individuals with cornea plana at an early age can result in the development of ametropic amblyopia.

Acknowledgement

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Forsius H, Damsten M, Eriksson AW, Fellman J, Lindh S, Tahvanainen E. Autosomal recessive cornea plana. A clinical and genetic study of 78 cases in Finland. Acta Ophthalmol Scand 1998;76:196-203.  Back to cited text no. 1
    
2.
Eriksson AW, Lehmann W, Forsius J. Congenital cornea plana in Finland. Clin Genet 1973;4:301-10.  Back to cited text no. 2
    
3.
Pellegata NS, Dieguez-Lucena JL, Joensuu T, Lau S, Montgomery KT, Krahe R, et al. Mutations in KERA, encoding keratocan, cause cornea plana. Nat Genet 2000;25:91-5.  Back to cited text no. 3
    
4.
Tahvanainen E, Forsius H, Kolehmainen J, Damsten M, Fellman J, de la Chapelle A. The genetics of cornea plana congenita. J Med Genet 1996;33:116-9.  Back to cited text no. 4
    
5.
Larsen V, Eriksen A. Cornea plana. Acta Ophthalmol (Copenh) 1949;27:275-86.  Back to cited text no. 5
    
6.
Fogla R, Indumathy TR. Customized toric intraocular lens implantation in cornea plana. J Cataract Refract Surg 2020;46:e11-4.  Back to cited text no. 6
    
7.
Majander AS, Lindahl PM, Vasara LK, Krootila K. Anterior segment optical coherence tomography in congenital corneal opacities. Ophthalmology 2012;119:2450-7.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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