Open Access

Keratoglobus: a close entity to megalophthalmos

SpringerPlus20165:634

https://doi.org/10.1186/s40064-016-2307-1

Received: 17 March 2016

Accepted: 9 May 2016

Published: 17 May 2016

Abstract

Background

Keratoglobus closely resembles buphthalmos and anterior megalophthalmos.

Findings

A 45-year-old man presented with gradually progressive, painless, diminution of vision in both eyes since childhood. On examination, visual acuity of right (RE) and left eye (LE) was 20/60 and 2/20 respectively. Clinical pictures of the patient are shown in panel A, B, C, D. Keratometry values were 46.47/47.94 D at 42/132° in RE and 46.90/47.23 D at 174/84° in LE, signifying steep, ectatic cornea. Axial lengths, anterior chamber depth and corneal thickness in RE/LE was 23.53/27.12 mm, 5.18/4.48 mm and 413/420 μm respectively. Iridodonesis was noted in left eye. Retinal evaluation of LE revealed retinal detachment (RD) with posterior staphyloma due to high myopia, hereas RE was within normal limits. Intraocular pressure was normal in both eyes. Final diagnosis was keratoglobus with LE myopic RD. The patient improved to 20/30 in right eye with no improvement in LE with scleral contact lens.

Conclusion

Keratoglobus, Megalophthalmos and Buphthalmos are exceedingly close entities and it is very essential to make correct diagnosis, as management options differ significantly for all three diseases.

A 45-year-old male patient presented with gradual, progressive and painless diminution of vision since childhood. On ocular examination, visual acuity of right eye (RE) and left eye (LE) was 20/60 and 2/20 respectively. Slit lamp evaluation revealed bilateral, diffuse corneal thinning in BE with outward, globular protrusion of the cornea (shown in panel A, B, C, D). The intraocular pressure (IOP) recorded with applanation tonometer was in the normal range. Keratometric values, depicting the corneal curvature, were 46.47/47.94 dioptre (D) at 42/132° and 46.90/47.23 D at 174/84° in RE and LE respectively, indicating steep and ectatic cornea bilaterally. Corneal topography assessed by Pentacam Scheimpflug Imaging system revealed, sim K values of 46.70/47.32 (steep axis at 60.9°) in RE and 45.9/47.7 (steep axis at 172.9°) in LE along with a posterior elevation of +12/+25 in RE/LE respectively. The recorded values of white-to-white diameter, axial length, corneal thickness and anterior chamber depth in RE/LE were 14.59/14.15 mm, 23.53/27.12 mm, 413/420 µm and 5.18/4.48 mm respectively. Retinal evaluation of LE revealed retinal detachment with posterior staphyloma, whereas RE was unremarkable. A diagnosis of bilateral keratoglobus with myopic retinal detachment in the LE was made. The risk of corneal perforation, even on minimal trauma was explained to the patient. On refraction no improvement in visual acuity was noted. Furthermore, the patient improved to 20/30 in the RE with a scleral contact lens while no improvement was noted in LE. The patient wilfully gave his consent to publish his medical details and images in a medical journal (Fig. 1).
Fig. 1

a Clinical picture of the patient showing diffuse thinning of cornea with outward globular protrusion. b Indentation of lower lids is shown on downward gaze, due to the protruded cornea. c, d Clinical pictures of the patient, showing bilateral clear cornea with deep anterior chamber with and stretched-out limbus

Comment

Keratoglobus is a bilateral, non-inflammatory, ectatic disorder of the cornea that is characterized by globular protrusion of the cornea (Smolek and Klyce 2000). Interestingly, this disease closely resembles buphthalmos and anterior megalophthalmos, where abnormal, large eyes with enlarged cornea as well as increased axial lengths are seen in the presence and absence of glaucoma respectively (Table 1) (Dua et al. 1999). Although our patient presented with features, indicative of megalophthalmos i.e. presence of enlarged corneas, increased axial lengths, iridodonesis and absence of increased IOP (Tsai et al. 2005), but in view of steep corneal curvatures, abnormal and thin cornea, increased white to white diameter (Lockington and Ramaesh 2015), normal intraocular pressure, absence of both miosis and ciliary ring enlargement, a diagnosis of buphthalmos or anterior megalophthalmos was excluded.
Table 1

Enumeration of differentiating points between keratoglobus, anterior megalophthalmos and buphthalmos

 

Keratoglobus

Anterior megalophthalmos

Buphthalmos/infantile glaucoma

Inheritance

No definite pattern

X-linked recessive

Sporadic

Age of presentation

Puberty

Congenital

First year of life

Natural History

Progressive

Non-progressive

Progressive

Symptoms

Frequent change of glasses

Variable and nonspecific

Watering, photophobia

Intra ocular pressure (IOP)

Normal

Normal

Elevated

Corneal diameter

>13mm; symmetric with increased WTW diameter

>13mm; symmetric

Variable, depends upon severity of glaucoma

Axial length

Increased axial length

Normal

Increased axial length

Iris

Iridodonesis ±

Iridodonesis; iris stromal hypoplasia

Normal with high insertion

Optic disc

Usually normal

Normal with increased propensity for glaucoma

Cup:disc ratio increased

Treatment

Refractive error correction and keratoplasty in advanced cases

Refractive errors correction

Control of IOP (medically/surgically)

Conclusion

Keratoglobus, Megalophthalmos and Buphthalmos are exceedingly close entities and it is very essential to make a correct diagnosis, as management options differ significantly for all three diseases (Table 1).

Declarations

Authors’ contributions

Both the authors, mentioned for this manuscript contributed considerably in clinical evaluation of the patient and manuscript writing. Both authors read and approved the final manuscript.

Acknowledgements

The authors acknowledge the experts from the Cornea and Refractive Surgery Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi for their guidance.

Competing interests

The authors declare that they have no competing interests.

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

Authors’ Affiliations

(1)
Cornea and Refractive Surgery Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences

References

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Copyright

© The Author(s) 2016