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OCULAR ONCOLOGY CASES FROM COLE
Section Editor: Arun D. Singh, MD
The Blind Painful Eye
Ciliary staphyloma is a rare clinical entity arising from various etiologies that poses
a significant management challenge to the ophthalmologist.
BY MARY E. TURELL, MD; LYNN SCHOENFIELD, MD; AND ARUN D. SINGH, MD
Welcome to the inaugural issue of Advanced Ocular
Care. “Cases From Cole” will be a regular ocular oncology
column presenting interesting cases from the Department
of Ophthalmic Oncology at the Cole Eye Institute,
Cleveland Clinic Foundation. I hope you find “Cases From
Cole” to be a valuable source of information.
—Arun D. Singh, MD, section editor
CASE PRESENTATION
A 48-year-old male with diabetes presented to our clinic in February 2009 for a consultation regarding the management of a blind, painful left eye. He reported a history
of trauma and loss of vision in the eye during early childhood. Over time, he gradually developed increasing ocular discomfort and became dissatisfied with the cosmetic
appearance of the eye. Aside from well-controlled diabetes and mild hypertension, he had no significant past
medical history and a negative oncologic history. His
family and social history were also unremarkable.
On examination, the BCVA was 20/20 in the patient’s
right eye and no light perception in the affected left eye.
A complete ocular examination of the right eye was nor-
Figure 1. The patient’s left eye exhibited extensive scleral
thinning, bulging of uveal tissue, diffuse corneal scarring, and
an expansion of the palpebral fissure.
mal. An external examination of the left eye revealed ptosis and marked stretching of the eyelid skin with significant widening of the palpebral fissure secondary to an
enlarged globe. Diffuse bullous keratopathy with corneal
scarring and pannus formation was present. Circumferential thinning of the sclera in a perilimbal distribution
was most prominent at the 12-o’clock position. Marked
scleral ectasia with outward bulging of the underlying
uveal tissue was present in all quadrants (Figure 1).
Corneal scarring precluded visualization of the fundus
with ophthalmoscopy.
SURGICAL COURSE AND OUTCOME
Given the patient’s long-standing history of no light
perception vision, the presence of pain, and his desire for
improved cosmesis, we recommended enucleation. The
patient underwent enucleation of the left eye with insertion of a 22-mm Medpor implant (Porex Surgical
Products, Newnan, GA) followed by a ptosis repair of the
upper eyelid using external levator advancement. After
the enucleation and eyelid reconstructive surgical procedures, the patient was fitted with a prosthesis that result-
Figure 2. Enucleated left globe with scleral ectasia and
cornea with bullous keratopathy, neovascularization, and
calcification.
JANUARY/FEBRUARY 2010 ADVANCED OCULAR CARE 29
OCULAR ONCOLOGY CASES FROM COLE
Figure 3. Light microscopy of sectioned left globe with
marked scleral ectasia lined by uveal tract (4X magnification).
Figure 4. Light microscopy demonstrating severe cupping of
the optic nerve with optic atrophy (4X magnification).
ed in an excellent cosmetic outcome.
On pathologic evaluation, we found severe scleral ectasia in the regions observed clinically and corresponding
bulging of the underlying uvea (Figure 2). The cornea
exhibited bullous keratopathy with neovascularization
and calcification, and the scleral ectatic regions were lined
by hyperplastic retinal pigment epithelium (Figure 3).
There was marked cupping of the optic disc with atrophy
and fibrosis of the optic nerve (Figure 4). Atrophy and
gliosis of the retina were also present; intraocular tumor
was absent. Based on the clinical and pathological findings, a final diagnosis of ciliary staphyloma was made.
cess rates for primary repair. Surgical options include scleral resection to close the defect, surface diathermy aimed at
reducing the size of the lesion prior to closure, surgical
buckling, and the use of scleral patch grafts.6
In this case, we recommended enucleation because the
eye was blind and painful. In addition, we were concerned
about globe rupture after minor trauma. ■
DISCUSSION
Ciliary staphyloma is a rare clinical entity characterized
by an ectatic sclera attached to the underlying uvea. The
term staphyloma was first used by Scarpa in 1801; the
Greek word staphylos literally means “a bunch of grapes.”1
Anterior staphyloma can be congenital, or it can occur following necrotizing scleritis, scleromalacia perforans, penetrating ocular trauma, or iatrogenic surgical trauma such
as trabeculectomy.2 In this case, the patient had experienced penetrating trauma to the globe at the age of
2 years. Perforation of the globe can result in traumatic
uveal tears. The uvea develops new adhesions to the overlying sclera, resulting in a disruption of normal nutritional
processes within both the uvea and the sclera with eventual thinning of the overlying sclera.3-5
Scleral staphylomas can be a challenge for the ophthalmologist to manage and often are associated with low suc30 ADVANCED OCULAR CARE JANUARY/FEBRUARY 2010
Lynn Schoenfield, MD, is in the Department of
Anatomic Pathology at the Cleveland Clinic
Foundation. She acknowledged no financial interest in the product or company mentioned herein.
Dr. Schoenfield may be reached at (216) 839-3061;
[email protected]
Section editor Arun D. Singh, MD, is director of
the Department of Ophthalmic Oncology at the
Cole Eye Institute, Cleveland Clinic Foundation. He
acknowledged no financial interest in the product
or company mentioned herein. Dr. Singh may be
reached at (216) 445-9479; [email protected].
Mary E. Turell, MD, is a senior resident at the
Cole Eye Institute, Cleveland Clinic Foundation. She
acknowledged no financial interest in the product
or company mentioned herein. Dr. Turell may be
reached at (216) 956-6712; [email protected].
1. Scarpa AA.A Treatise on the Principal Diseases of the Eyes.2nd ed.London,UK;Cadell and W.Davies;1818.
2. Spaeth GL,Rodrigues MM.Staphyloma as a late complication of trabeculectomy.Ophthalm Surg.1977;8:81-85.
3.Watzke RC.Scleral staphylomas and retinal detachment.Arch Ophthalmol.1963;70:796-804.
4.Vall D.Scleral staphylomas and retinal detachment.Trans Am Ophthalmol Soc.1948;46:58-72.
5. Mattice AF.On the pathogenesis of scleral staphyloma.Arch Ophthalmol.1913;42:612-617.
6.Watson PG,Hazleman BL,Pavesio CE,Green WR .The Sclera and Systemic Disorders.2nd ed.London,UK;Butterworth
Heineman;2003.