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Year : 2012  |  Volume : 5  |  Issue : 2  |  Page : 121-123  

Unilateral keratoconus presenting with acute hydrops in a patient with mental retardation, epilepsy, and bronchial asthma

1 Department of Ophthalmology, R. G. Kar Medical College and Hospital, 1, Khudiram Bose Sarani, Kolkata, India
2 Department of Ophthalmology, NRS Medical College, Kolkata, India
3 Department of Ophthalmology, ESI (SD) Barasat, Kolkata, India

Date of Web Publication4-Aug-2012

Correspondence Address:
Bandyopadhyay Sabyasachi
R. G. Kar Medical College and Hospital, Kolkata-4
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0974-620X.99379

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How to cite this article:
Sabyasachi B, Kumar GA, Kumar PS, Lila B. Unilateral keratoconus presenting with acute hydrops in a patient with mental retardation, epilepsy, and bronchial asthma. Oman J Ophthalmol 2012;5:121-3

How to cite this URL:
Sabyasachi B, Kumar GA, Kumar PS, Lila B. Unilateral keratoconus presenting with acute hydrops in a patient with mental retardation, epilepsy, and bronchial asthma. Oman J Ophthalmol [serial online] 2012 [cited 2022 Aug 11];5:121-3. Available from: https://www.ojoonline.org/text.asp?2012/5/2/121/99379

Keratoconus is a progressive condition wherein cornea assumes an irregular conical shape following non-inflammatory axial ectasia. The estimated frequency of keratoconus is approximately 50 to 230 per 100 000 population. [1] The disease is mostly bilateral with only 0.5 to 4% being unilateral. [2],[3] The important ocular associations include vernal keratoconjunctivitis, constant eye rubbing, rigid contact lens wear, blue sclera, andretinitis pigmentosa. [4],[5],[6] Atopic dermatitis, bronchial asthma, mental retardation as inDown's syndrome, Ehlers-Danlos syndrome, Marfan's syndrome, osteogenesis imperfecta type I, and mitral valve prolapse are frequently associated systemic conditions. [6],[7],[8]

Here-in we present a 19 year-old female patient with right sided keratoconus and acute hydrops. The left eye was normal. The patient had mental retardation, epilepsy, and bronchial asthma. Those conditions were reported in the past as isolated associations, but their combination in a single patient is unique to this case. [6],[7],[8]

A 19-year-old female patient came to our hospital in June 2009 with history of pain, redness, and sudden dimness of vision in her right eye for 2 days. Her best corrected visual acuity (BCVA) in right eye was only perception of light with positive projection of rays in all the four quadrants. The BCVA in left eye was 20/20. The intraocular pressure (IOP) by Schiotz tonometry was 21 mm of Hg in the right eye and 19 mm of Hg in the left eye. On slit lamp examination in the right eye there was ciliary conjunctival congestion and a conical shaped cornea with corneal edema and tears in the Descemet's membrane [Figure 1]. The left eye was normal on slit lamp examination. On videokeratography, corneal topography, and central corneal thickness (CCT) in the right eye could not be captured due to poor fixation. In the left eye corneal topography was normal and CCT was 541μ [Figure 2].
Figure 1: Slit lamp photograph of right eye showing keratoconus with acute hydrops

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Figure 2: Videokeratography of the left eye showing no evidence of keratoconus

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She had a poorly treated epileptic seizure disorder since infancy. Computerized axial tomographic (CT) scan of brain was within normal limits. Electro encephalography (EEG) showed interictal generalized seizure activity. The patient was also suffering from mental retardation and bronchial asthma. Cardiology and gynecology consultations revealed no abnormalities. Electrocardiography (ECG) and echocardiography were normal. The ultrasonography (USG) of pelvic area and thyroid function tests were normal. Orthopedic evaluation was unremarkable.

The acute hydrops in the right eye was treated with 0.5% betoxolol eye drops twice daily, 5% hypertonic saline eye drops four times daily, 6% hypertonic saline eye ointment at bed time, and carboxymethyl cellulose eye drops four times daily. She was on treatment with carbamazepine and clobazam for epilepsy. Budesonide and formetorol fumerate inhalers and expectorant containing bromhexine, guifenesin, and terbutaline were given for bronchial asthma.

The acute hydrops stage subsided and the BCVA in right eye improved to 20/60 (with - 2.00Dsph and - 2.50Dcyl at 170°) after 3 months [Figure 3]. Follow up videokeratography in January 2010 showed keratoconus in the right eye and the normal left eye [Figure 4] and [Figure 5]. During one and half year follow up, the left eye showed no evidence of keratoconus and the BCVA remained at 20/20 (with -0.50Dsph). The bronchial asthma was well controlled and there was no further seizure attack.
Figure 3: Slit lamp photograph of right eye showing resolution of acute hydrops during follow up

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Figure 4: Videokeratography of the right eye showing keratoconus during follow up

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Figure 5: Follow up videokeratography of the left eye still showing no evidence of keratoconus

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Unilateral keratoconus is rare disease (frequencies range from 0.5 to 4%) and even rarer is the initial presentation of that single eye at the stage of acute hydrops. [2],[3] Though, the associated mental retardation, epilepsy, and bronchial asthma have been described in the past, all these associations in a single patient are unique to this case. [6],[7],[8] In differential diagnosis, other causes of unilateral corneal edema like HSV disciform keratitis, acute congestive glaucoma, and chemical burns needed to be excluded. The resolution of acute hydrops was in accordance with the favorable sequelae in younger than elderly patients. [9]

In conclusion, the possibility of unilateral keratoconus should be kept in mind in a young patient presenting with unilateral sudden corneal edema particularly associated with mental retardation, epilepsy, and bronchial asthma.

   References Top

1.Rabinowitz YS. Keratoconus. Surv Ophthalmol 1998;42:297-319.  Back to cited text no. 1
2.Rabinowitz YS, Nesburn AB, McDonnell PJ. Videokeratography of the fellow eye in unilateral keratoconus. Ophthalmology 1993;100:181-6.  Back to cited text no. 2
3.Holland DR, Maeda N, Hannush SB, Riveroll LH, Green MT, Klyce SD, et al. Unilateral keratoconus. Incidence and quantitative topographic analysis. Ophthalmology 1997;104:1409-13.  Back to cited text no. 3
4.Cameron JA, Al-Rajhi AA, Badr IA. Corneal ectasias in vernal keratoconjunctivitis. Ophthalmology 1989;96:1615-23.  Back to cited text no. 4
5.Koeing SB, Smith RW. Keratoconus and corneal hydrops associated with compulsive eye rubbing. Refract Corneal Surg 1993;9:383-4.  Back to cited text no. 5
6.Kenyon KR, Hersh PS, Starck T. Corneal dysgeneses, dystrophies and degenerations. In: Albert DM, Jacobiec FA, Azar DT, Gragoudas ES, Power SM, Robinson NL, editors. Principles and Practice of Ophthalmology. Vol 2. 2 nd ed. Philadelphia: W.B. Saunders Company; 2000. p. 735-7.  Back to cited text no. 6
7.Weed KH, MacEwen CJ, Giles T, Low J, McGhee CN. The Dundee university scottish keratoconus study: Demographics, corneal signs, associated diseases, and eye rubbing. Eye (Lond) 2008;22:534-41.  Back to cited text no. 7
8.Pierse D, Eustace P. Acute keratoconus in mongols. Br J Ophthalmol 1971;55:50-4.  Back to cited text no. 8
9.Al Suhaibani AH, Al-Rajhi AA, Al-Motowa S, Wagoner MD. Inverse relationship between age and severity and sequelae of acute corneal hydrops associated with keratoconus. Br J Ophthalmol 2007;91:984-5.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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