Management of Aniridic Keratopathy with Allograft Limbal Stem Cell Transplantation Followed by Phacoemulsification Surgery

1
Management of Aniridic Keratopathy with Allograft
Limbal Stem Cell Transplantation Followed by
Phacoemulsification Surgery

• Sibel Aksoy, MD, Yonca A. Akova, MD
• Baskent University, School of Medicine
  Department of Ophthalmology
  Ankara-Turkey

The authors acknowledge no financial interest in
the subject matter of this presentation
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Purpose

• To report a case of aniridic keratopathy treated
  with allograft limbal stem cell transplantation
  (ALSCT) and medical theraphy including
  bevacizumab followed by phacoemulsification
  surgery for congenital cataract

3
Case

• 29 years-old female
• Presented to our clinic with blurred vision and
  photophobia in both eyes
• Visual acuities were 20/60 in the right eye and
  counting fingers at 1 meter in the left eye
• Intraocular pressures were normal
• OD Normal fundus findings
  OS
  Fundoscopic examination was not possible

4
Anterior Segment Examination

• Epithelial defects and cloudy cornea in the left
  eye
• Paracentral and 3600 peripheral corneal
  neovascularization in the left eye
• Bilateral aniridia
• Bilateral congenital lamellar cataract

OD
OS
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SURGICAL TREATMENT
MEDICAL TREATMENT

• Left eye treated with superficial keratectomy,
  allogreft limbal stem cell transplantation and
  amniotic membrane transplantation

• Topical lomefloxacin (4x1), topical dexamethasone
  (8x1), topical hydroxypropylmethilcellulose(8x1),
  oral cyclosporine A (3mg/kg) and oral
  metilprednisolone (0,5mg/kg)
• Decreased gradually

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The day after surgery

• Figures showing amniotic membrane transplantation
  and corneal sutures of the limbal graft at
  superior temporal and inferior nasal quadrants

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Postoperative 6 months

• VA Finger counting from 2 meters
• Obvious reduction in vascularity
• Increased epithelization
• Decreased haze

Pre-therapy
Post-therapy
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Postoperative 1 year

• Revascularization at the inferior and nasal
  quadrants
• Subconjunctival (0.04 mg/ 0.01ml) and topical
  bevacizumab (4x1) treatment were added

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• Follow-up reduction of vascularization

Phacoemulsification and intraocular lens
implantation was performed 1.5 years after ALSCT
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Postoperative 6 months

• Visual acuity increased to 20/200
• Corneal epithelium was intact with minimal
  surface vascularity
• No side effects of bevacizumab treatment were
  seen

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Conclusion

• Aniridia is a congenital, almost always
  bilateral, pan-ocular and non-self limiting
  disease
• ALSCT as well as systemic immunosuppression and
  bevacizumab therapy improved the aniridic
  keratopathy in our patient
• It is a progressive disease and long term
  follow-up is needed

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NAME Sibel Aksoy, M.D. TITLE Resident in
Ophthalmology DATE AND PLACE OF BIRTH
November 20, 1979, Turkey 2002 Doctor of
Medicine, Osmangazi University, School of
Medicine, Eskisehir, Turkey 2004-2009 Resident,
Baskent University, School of Medicine,
Department of Ophthalmology, Ankara, Turkey
NAME Yonca Aydin Akova, M.D. TITLE Professor in
Ophthalmology DATE AND PLACE OF BIRTH
October 21, 1960, Turkey 1983 Doctor of
Medicine, Istanbul University, School of
Medicine 1990 Istanbul University, School of
Medicine, Department of Ophthalmology 2000Profess
or of Ophthalmology, Baskent University, School
of Medicine, Department of Ophthalmology, Ankara,
Turkey 2002 Chairperson, Baskent University,
School of Medicine, Department of Ophthalmology,
Ankara, Turkey