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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 3  |  Page : 652-654

Rare presentation of acute hydrops in bilateral advanced keratoconus in a 5-year-old child: A case report


1 Department of Cornea and Refractive Services, The Eye Foundation, Coimbatore, Tamil Nadu, India
2 Cataract and Refractive Services, The Eye Foundation, Coimbatore, Tamil Nadu, India

Date of Submission08-Dec-2021
Date of Acceptance31-Jan-2022
Date of Web Publication16-Jul-2022

Correspondence Address:
Dr. Shreesha K Kodavoor
Department of Cornea and Refractive Services, The Eye Foundation Hospital, 582-A, Diwan Bahadur Rd, R S Puram West, Coimbatore - 641 002, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_3015_21

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  Abstract 


We report a rare case of bilateral advanced keratoconus presenting with acute hydrops in a 5-year-old child with no known syndromic association and frequent eye rubbing. Pediatric consultation revealed delayed milestones and hypothyroidism with occasional seizures. Acute Hydrops was managed conservatively since fitness for intracameral perfluoropropane injection was delayed. Subsequently, the patient underwent deep anterior lamellar keratoplasty in both the eyes with good surgical outcome. Initial presentation of keratoconus with acute hydrops in a 5-year-old patient is rare. As this case was associated with hypothyroidism, global developmental delay, and seizures, it requires further investigation for any association with keratoconus.

Keywords: Acute hydrops, DALK, keratoconus


How to cite this article:
Kodavoor SK, Rathi N, Ramamurthy D. Rare presentation of acute hydrops in bilateral advanced keratoconus in a 5-year-old child: A case report. Indian J Ophthalmol Case Rep 2022;2:652-4

How to cite this URL:
Kodavoor SK, Rathi N, Ramamurthy D. Rare presentation of acute hydrops in bilateral advanced keratoconus in a 5-year-old child: A case report. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 13];2:652-4. Available from: https://www.ijoreports.in/text.asp?2022/2/3/652/351165



Keratoconus is a bilateral, progressive, non-inflammatory ectatic disorder of the cornea. Its initial presentation is mainly noted during puberty. Sudden increase in the rate of progression is noted during adolescence, pregnancy, and during any systemic hormonal changes. Multiple syndromes are associated with keratoconus like Down, Marfan, etc.[1] It presents with decreased and fluctuating vision, astigmatism, and increased higher order aberrations.[2] Acute hydrops as an initial presentation is very rare in a young child.

Here we would like to present an uncommon presentation of bilateral advanced keratoconus with acute corneal hydrops in a 5-year-old child associated with global developmental delay without any known syndromic association.


  Case Report Top


A 5-year-old male child was brought by the mother in our out-patient department with chief complaints of whitening of the black part of the right eye since two days. Birth history of the child was uneventful with normal full-term vaginal delivery. However, developmental history revealed delayed milestones; also the child had history of episodic seizures. Family history and past history was not significant. Patient's blood investigations revealed raised thyroid-stimulating hormone (2.03 μIL/ml), indicating hypothyroidism. Patient was referred to paediatrician and on systemic examination was found to have impaired global development and epilepsy and subsequently was started on anti-epileptic medication. Retinoscopy revealed no glow in right eye and dull glow in the left eye. The child was highly uncooperative for visual acuity test.

On slit lamp examination, the right eye revealed corneal edema inferiorly and paracentrally with break in Descemet's membrane with significant corneal protrusion, while the left eye revealed Vogt striae, Bowman, Descemet's membrane scar along with central corneal protrusion [Figure 1]. Dilated fundus examination and intraocular pressure were within normal limits. Corneal topography with a rotating Scheimpflug device (Pentacam, OCULUS Optikgeräte GmbH) showed K1 value of 83.5 D (diopters), K2 value of 91.4 D, astigmatism of 7.9 D at 30.3°, and a thinnest cornea pachymetry of 356 microns in right eye; the left eye divulged K1 value of 67.9 D, K2 value of 78.8 D, astigmatism of 10.9 D at 160.0°, and a thinnest pachymetry of 379 microns [Figure 2]. However, quality of the scans was not reliable due to poor cooperation of the patient. On anterior segment optical coherence tomography (AS-OCT), the right eye showed a break in Descemet's membrane and an increase in corneal thickness at the corresponding point of break; the surrounding cornea and left eye showed compact cornea and thinning with significant scarring [Figure 3]. The patient was diagnosed with advanced keratoconus in both eyes with acute hydrops in the right eye.
Figure 1: Slit lamp image of (a) Right eye shows acute hydrops (b) Left eye shows features of advanced keratoconus

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Figure 2: Topography of (a) Right eye shows advanced keratoconus with increased corneal thickness at site of hydrops (b) Left eye shows advanced keratoconus

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Figure 3: Anterior segment optical coherence tomography image shows large Descemet's cleft in the right eye, and compact cornea and thinning with scarring in left eye

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The patient was advised to undergo intracameral injection of 14% perfluoropropane (C3F8) gas and started on hyperosmotic eye drops, soft steroids, and lubricating agents. However, Pediatrician consultation suggested to wait for a month, and to start on anti-epileptic medication. The patient returned after a month and the examination revealed spontaneous reattachment of Descemet's membrane. However they developed a paracentral corneal scar in the right eye at the site of the initial Descemet's membrane break; left eye examination appeared same as the initial presentation. The patient was advised to undergo deep anterior lamellar keratoplasty (DALK) in the left eye first, which was performed successfully with good outcome. DALK in the right eye was performed after a month with good outcome [Figure 4]. Retinoscopy revealed a refractive error of +0.25/-4.25 × 100° in right eye and -1.50/-3.75 × 120° in left eye; visual acuity could not be assessed as child was uncooperative.
Figure 4: Slit lamp image of (a) Right eye after penetrating keratoplasty shows clear graft and (b) Left eye after penetrating keratoplasty shows clear graft

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  Discussion Top


Acute hydrops is the development of marked corneal edema caused by a break in Descemet's membrane and the endothelium, allowing aqueous to enter the corneal stroma and epithelium.[3] Its presentation in keratoconus is comparatively rare (3%). However, in our case, the initial presentation of keratoconus was with acute corneal hydrops in a young patient with global developmental delay not associated with eye rubbing or any known syndrome which could likely to be associated with keratoconus. Eye rubbing is probably due to chronic traumatizing mannerisms seen in mentally retarded patients leading to this condition; however it was not associated in our case. Downie reported a case of bilateral corneal hydrops in an 8-year-old boy with atopic disease,[4] and Panahi-Bazaz et al.[5] reported another case of acute bilateral hydrops in a 7-year-old girl with vernal keratoconjunctivitis.

Bawazeer et al.[6] reported that among different risk factors associated with Keratoconus (KC) including age, sex, race, family history, mitral valve prolapse, collagen vascular disease, Marfan syndrome, Down syndrome, ocular trauma, pigmentary retinopathy, and contact lens use, eye rubbing was the only significant predictor. However, our case did not present with any such association.

Management of acute hydrops in this case was conservative as he did not get fitness immediately for intracameral perfluoropropane (C3F8) injection under general anesthesia, and was started on hyper osmotic agents along with soft steroids and tear substitutes. Hydrops resolved in a month with this treatment with a paracentral scar.

In our case, other environmental or genetic factors might have played a role in the development and progression of keratoconus. On the management perspective in our case, deep anterior lamellar keratoplasty was performed in both the eyes. Intra-operatively as full thickness penetrating keratoplasty an open globe surgery results into invariable complications in pediatric population given that the scleral rigidity is not comparable to adult population. Penetrating keratoplasty in case of advanced keratoconus gives a good result, but increased duration of visual rehabilitation and steroid requirement post surgery ultimately increases the occurrence of glaucoma and also has higher chances of graft rejection. DALK procedure overcomes all these limitations.[7] However refractive and topographic outcomes are comparable between the two methods.[8]


  Conclusion Top


Initial presentation of keratoconus with acute hydrops in a 5-year-old patient is a rare occurrence. Also, keratoconus being associated with a known syndrome, or eye rubbing, or any surface pathology was not observed in our case. However, mental retardation in our patient can be acknowledged as a cause for unawareness and late presentation with acute hydrops. To the best of our knowledge, this is the first case report of bilateral advanced KC with acute hydrops in one eye in a 5-year-old without any vernal keratoconjunctivitis or known systemic association.

As this case is associated with hypothyroidism, global developmental delay, epilepsy, and mental retardation, it requires further probing and investigation for any association with keratoconus.

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.
Rabinowitz YS. Keratoconus. Surv Ophthalmol 1998;42:297-319.  Back to cited text no. 1
    
2.
Cozma I, Atherley C, James NJ. Influence of ethnic origin on the incidence of keratoconus and associated atopic disease in Asian and white patients. Eye (Lond) 2005;19:924-5; author reply 925-6.  Back to cited text no. 2
    
3.
Stone DL, Kenyon KR, Stark WJ. Ultrastructure of keratoconus with healed hydrops. Am J Ophthalmol 1976;82:450-8.  Back to cited text no. 3
    
4.
Downie LE. The necessity for ocular assessment in atopic children: Bilateral corneal hydrops in an 8 year old. Pediatrics 2014;134:e596-601.  Back to cited text no. 4
    
5.
Panahi-Bazaz MR, Sharifipour F, Moghaddasi A. Bilateral keratoconus and corneal hydrops associated with eye rubbing in a 7-year-old Girl. J Ophthalmic Vis Res 2014;9:101-5.  Back to cited text no. 5
  [Full text]  
6.
Bawazeer AM, Hodge WG, Lorimer B. Atopy and keratoconus: A multivariate analysis. Br J Ophthalmol 2000;84:834-6.  Back to cited text no. 6
    
7.
Reinhart WJ, Musch DC, Jacobs DS, Lee WB, Kaufman SC, Shtein RM. Deep anterior lamellar keratoplasty as an alternative to penetrating keratoplasty a report by the American academy of ophthalmology. Ophthalmology 2011;118:209-18.  Back to cited text no. 7
    
8.
Mohammadpour M, Heidari Z, Hashemi H. Updates on managements for keratoconus. J Curr Ophthalmol 2017;30:110-24.  Back to cited text no. 8
    


    Figures

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



 

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