management of aniridic keratopathy with allograft limbal stem cell transplantation followed by...

Management of Aniridic Management of Aniridic Keratopathy with Keratopathy with Allograft Limbal Stem Allograft Limbal Stem Cell Transplantation Cell Transplantation Followed by Followed by PhacoemulsificationPhacoemulsification SurgerySurgery

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

PurposePurpose

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

CaseCase29 years-old female Presented to our clinic with blurred

vision and photophobia in both eyesVisual 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

Anterior Segment Anterior Segment ExaminationExaminationEpithelial 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

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

SURGICAL TREATMENT MEDICAL TREATMENT

The day after surgery

Figures showing

amniotic

membrane

transplantation

and corneal

sutures of the

limbal graft at

superior temporal

and inferior nasal

quadrants

• VA: Finger counting from 2 meters

• Obvious reduction in vascularity

• Increased epithelization

• Decreased haze

Pre-therapy

Post-therapy

Postoperative 6 months

• Revascularization at the inferior and nasal quadrants

• Subconjunctival (0.04 mg/ 0.01ml) and topical bevacizumab (4x1) treatment were added

Postoperative1 year

Follow-up reduction of vascularization

Phacoemulsifica

tion and

intraocular lens

implantation

was performed

1.5 years after

ALSCT

Postoperative 6 Postoperative 6 monthsmonthsVisual acuity

increased to 20/200

Corneal epithelium was intact with minimal surface vascularity

No side effects of bevacizumab treatment were seen

ConclusionConclusionAniridia 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

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 Aydın 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

2000:Professor of Ophthalmology, Baskent University, School of Medicine, Department of Ophthalmology, Ankara, Turkey

2002: Chairperson, Baskent University, School of Medicine, Department of Ophthalmology, Ankara, Turkey