Sterile keratitis after uneventful corneal collagen cross-linking in a patient with Axenfeld-Rieger syndrome

  • Betul Seher Uysal
  • Derya Yaman
  • Ozge Sarac
  • Emine Akcay
  • Nurullah Cagil
Case Report



To report on a keratoconus (KC) patient with Axenfeld-Rieger syndrome (ARS) who developed sterile keratitis after accelerated corneal collagen cross-linking (CXL).


An 18-year-old patient with ARS and KC who had previously undergone intrastromal ring segment implantation underwent accelerated CXL (9 mW/cm2 UVA intensity for 10 min).


After uneventful surgery, the patient presented with severe photophobia, redness of the eye, and decreased vision 72 h following the procedure. Slit-lamp examination showed anterior multiple superficial stromal infiltrates in the central cornea with an overlying epithelium defect. Due to the lack of pain and absence of any pathogen from corneal samples, a diagnosis of sterile keratitis was considered. A combination of topical antibiotic and corticosteroid regimen was administered. Three months after CXL slit-lamp examination showed a mild stromal scar overlying the central cornea, which did not decrease visual acuity.


The mechanism by which the sterile keratitis occurs following CXL remains unclear. For our case, the reason of post-CXL sterile keratitis could be considered as an immune response due to the staphylococcal antigens. Furthermore, the possible developmental disturbance of corneal stroma in ARS might have contributed to the development of post-CXL sterile keratitis.


Axenfeld-Rieger syndrome Corneal collagen cross-linking Keratoconus Sterile keratitis 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in this study involving human participant were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.

Human and animals rights

This article does not contain any studies with animals performed by any of the authors.

Informed consent

Informed consent was obtained from the participant included in this study. Additional informed consent was obtained from the participant for whom identifying information is included in this article. The participant gave written informed consent for publication which was added to the supplementary material section.

Patient consent

The patient has consented to the submission of the case report for submission to the journal.


  1. 1.
    Rabinowitz YS (1998) Keratoconus. Surv Ophthalmol 42:297–319CrossRefPubMedGoogle Scholar
  2. 2.
    Wollensak G, Spoerl E, Seiler T (2003) Riboflavin/ultraviolet-A-induced collagen crosslinking for the treatment of keratoconus. Am J Ophthalmol 135:620–627CrossRefPubMedGoogle Scholar
  3. 3.
    Edwards M, McGhee CN, Dean S (2001) The genetics of keratoconus. Clin Exp Ophthalmol 6:345–351CrossRefGoogle Scholar
  4. 4.
    Kamińska A, Sokołowska-Oracz A, Pawluczyk-Dyjecińska M et al (2007) Variability of clinical manifestations in the family with Axenfeld-Rieger syndrome. Klin Ocz 109:321–326Google Scholar
  5. 5.
    Ito YA, Walter MA (2014) Genomics and anterior segment dysgenesis: a review. Clin Exp Ophthalmol 42:13–24CrossRefPubMedGoogle Scholar
  6. 6.
    Idrees F, Vaideanu D, Fraser SG et al (2006) A review of anterior segment dysgeneses. Surv Ophthalmol 51:213–231CrossRefPubMedGoogle Scholar
  7. 7.
    Shields MB (1983) Axenfeld-Rieger syndrome. A theory of mechanism and distinctions from the iridocorneal endothelial syndrome. Trans Am Ophthalmol Soc 81:736–784PubMedPubMedCentralGoogle Scholar
  8. 8.
    Chambers D, McGonnell IM (2002) Neural crest: facing the facts of head development. Trends Genet 18:381–384CrossRefPubMedGoogle Scholar
  9. 9.
    Koller T, Mrochen M, Seiler T (2009) Complication and failure rates after corneal crosslinking. J Cataract Refract Surg 35:1358–1362CrossRefPubMedGoogle Scholar
  10. 10.
    Angunawela RI, Arnalich-Montiel F, Allan BD (2009) Peripheral sterile corneal infiltrates and melting after collagen crosslinking for keratoconus. J Cataract Refract Surg 35:606–607CrossRefPubMedGoogle Scholar
  11. 11.
    Zamora KV, Males JJ (2009) Polymicrobial keratitis after a collagen cross-linking procedure with postoperative use of a contact lens: a case report. Cornea 28:474–476CrossRefPubMedGoogle Scholar
  12. 12.
    Baum J, Dabezies OH Jr (2000) Pathogenesis and treatment of sterile midperipheral corneal infiltrates associated with soft contact lens use. Cornea 19:777–781CrossRefPubMedGoogle Scholar
  13. 13.
    Fernandes M, Vemuganti GK, Rao GN (2007) Bilateral periocular psoriasis: an initial manifestation of acute generalized pustular psoriasis with coexistent Sjogren’s syndrome. Clin Exp Ophthalmol 35:763–766CrossRefPubMedGoogle Scholar
  14. 14.
    Gokhale NS, Vemuganti GK (2010) Diclofenac-induced acute corneal melt after collagen crosslinking for keratoconus. Cornea 29:117–119CrossRefPubMedGoogle Scholar
  15. 15.
    Ficker L, Seal D, Wright P (1989) Staphylococcal infection and the limbus: study of the cell-mediated immune response. Eye 3:190–193CrossRefPubMedGoogle Scholar
  16. 16.
    Dart JKG (1993) Disease and risks associated with contact lenses. Br J Ophthalmol 77:49–53CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer Science+Business Media B.V., part of Springer Nature 2018

Authors and Affiliations

  • Betul Seher Uysal
    • 1
  • Derya Yaman
    • 1
  • Ozge Sarac
    • 1
  • Emine Akcay
    • 1
  • Nurullah Cagil
    • 1
  1. 1.Department of Ophthalmology, Atatürk Training and Research HospitalYıldırım Beyazıt University, BilkentAnkaraTurkey

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