ACTA O P H T H A L M O L O G I C A 67 (1989) Supplementum 192

The effect of corneal grafting on vision in bilateral amblyopia

Joseph Frucht-Pery, Arturo S. Chayet, Sandy T. Feldman, Daniel J. Buckley and Stuart I. Brown

Department of Ophthalmology, University of California at San Diego, San Diego, CA, USA

Abstract. Two children with bilateral congenital cor- neal opacities (sclerocornea) received unilateral corneal transplants at the ages of 4’/e and 16 years, respectively. Both developed reading vision and reduced nystagmus excursions.

Key words: corneal transplantation - sclerocornea - children - amblyopia - congenital corneal opacities.

Corneal transplantation of congenital corneal opa- cities has been performed in gradually increasing numbers since 1970 (Schanzlin et al. 1980; Wood et al. 1970; Zaidman et al. 1982; Brown 1974; Feldman et al. 1987). The visual acuity achieved in the suc- cessful cases have generally not exceeded 201200 to date. There have not been enough successful pa- tients with a sufficient difference in the age of surgery to be able to prognosticate the visual re- sults and the depth of ambylopia with age.

We report the development of vision in 2 child- ren, 4 and 16 years of age who had corneal trans- plantation of densely opacified sclerocornea.

Patients

Two patients are included in this study. The first was examined 5 years ago when he was 50 months of age. He was born with bilateral congenital sclerocornea and buphthalmos. Sometime in the latter part of his first year of life, he underwent a corneal transplantation in his right eye which

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failed and shortly thereafter the eye was enu- cleated. There was no further medical or surgical treatment to the remaining left eye. Our examin- ation showed total scclerocornea in the markedly enlarged left eye (Fig. 1). The sclera was visible only when the eye was turned. The intraocular pressure was 38 mmHg and he had acurate central and temporal light perception. Two days later, he underwent cyclocryotherapy with 4 applications at -80°C for 60 sec. Within a week, the eye was no- ticeably smaller, and the pressure was reduced to 19 mmHg, whereupon he underwent a cataract ex- traction and a penetrating keratoplasty using 8 mm donor cornea in a 7112 mm host bed. The donor cornea remained transparent with treat- ment by topical cortical steroids and 0.5% timolol (Fig. 2). The sutures were removed in the second post-operative week. At that time, it was observed that the optic nerve was white and appeared totally cupped. Four weeks post-operatively, he denied any vision until we called his attention to move- ments in front of his eye which he then realized were objects. Later, he could point to objects a few feet away. He returned to his home in another state, and his attending physicians report that the transplant has remained clear, and he has 20/400 vision with +10.50 correction. The optic nerve was said to be pale, but there was no apparent atrophic cupping. The intraocular pressure varies between 19 and 24 mmHg. Treatment is timololO.5% twice a day and topical corticosteroids 4 times a day. The

Fig. 1. Pre-operative appearance of patient 1 with buphthalmos and total sclerocornea 0s.

patient is attending school with normally sighted children.

The second patient is a 16 year old female who was born with bilateral total sclerocornea (Fig. 3). At the age of 7, she underwent a cornea transplant in the right eye which failed. She was first exam- ined by us at the age of 16, and was found to have

hand motion vision in all fields at 1 foot and rapid nystagmoid movements of both eyes and normal intraocular pressure by pneumotonometry. Four- teen months ago, a corneal transplant was per- formed on her left eye. An anterior polar cataract was noted but not removed at surgery in an other- wise normal anterior segment. The corneal trans-

Fig. 2. Post-operative transparent corneal graft of the left eye of patient 1.

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Fig. 3. Appearance of patient 2's left eye before surgery.

plant has remained transparent is spite of an epi- sode of graft rejection by vascularization to the su- tures at three weeks post-operatively (Fig. 4). The sutures were removed and the rejection success- fully treated with topical and systemic steroids. At approximately 6 weeks post-operatively, the pa- tient was questioned regarding vision. She re- sponded that she could make out lines of an E at 6 feet. She was elated and returned the next week

with a handwritten note on lined paper which de- scribed how she could see flowers on the side of the road, her dog across the room and that she could see what she wrote and realized that she did not spell very well. Seven months post-operatively, this patient's medication is 1% prednisilone acetate 3 times daily. Her distance vision is 20/400, and the intraocular pressure continues to be normal. She could read large print newspapers and magazines

Fig. 4. Post-operative transparent corneal graft of the left eye of patient 2. An anterior polar cataract is present.

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if they were held 4 inches from her eye and could walk safely without her seeing eye dog. Her nystag- moid movements had diminished but were still present.

Discussion

Two recent studies of extraction of bilateral, com- plete congenital cataracts clarified earlier reviews. They indicated that the achieved visual acuities were significantly less in eyes when surgery was de- layed after 8 weeks of age (Gelbert et al. 1982; Ro- gers et d. 1981). The latter results report the often assumed poor prognosis of transplantation of the congenitally opaque cornea and the prior failures of corneal transplantation of the first eyes of the patients in the present study, were the probable reasons that corneal transplantation of their re- maining eyes was delayed.

Aside from the treatment of congenital corneal opacities and buphthalmos, which is addressed in another report, corneal transplantation was car- ried out as previously described and succeeded without incident.

The development of sight in these eyes that pre- viously had totally opaque corneas was especially interesting. At 3 weeks post-operatively, both pa- tients were asked if they could recognize large ob- jects and both replied negatively. The 16 year old did not appreciate improved vision until we dem- onstrated to her that she could see the lines that made up an E. We had to explain to her that this was the way an E appeared although she knew how to print and write the letter. The first patient was aphakic and could not recognize the shape of a person directly in front of him until the person moved all of his limbs which seemed to allow the patient to frame the object in space. He achieved his best sight months after he returned to his home in another part of the country. Both children have had a reduction in the frequency of their nystag- mus and both feel that they would see easier if their remaining involuntary eye movements would

Finally, the achievement of sight in these 2 chil- dren who were bilaterally blind since birth is im- portant to our concept of development of sight

stop.

and brain maturation. Similar results were noted in a recent brief report concerning the transplan- tation of 2 adults with bilateral congenital corneal opacities (Noble & Easty 1985). These findings in- dicate the value of maintaining the eye and its health until transplantation can be feasible and the usefulness of transplantation in juveniles or adults with bilateral congenital corneal opacities who have not been afforded the opportunity for early surgery.

Acknowledgments

This study was supported partly by a search to Prevent Blindness, Inc.

grant from Re-

References

Brown S I (1974): Corneal transplantation of the infant cornea. Trans Acad Ophthalmol Otolaryngol78 461.

Feldman S T, Frucht J & Brown S I (1987): Corneal trans- plantation in microphthalmic eyes. Am J Ophthalmol 104 164.

Gelbert S S, Hoyt C S, Jastrebski G & Marg E (1982): Long-term visual results in bilateral congenital cata- racts. Am J Ophthalmol 93: 615.

Noble B A & Easty D L (1985): Late grafting in congeni- tally opaque corneas. Dev Ophthalmol 11: 75.

Rogers G L, TisNer C L, Tsou B H, Hertle R W & Fellows R R (1981): Visual acuities in infants with congenital cataracts operated on prior to 6 months of age. Arch Ophthalmol99: 999.

Schanzlin D J, Goldberg D B & Brown S I (1980): Trans- plantation of congenitally opaque corneas. Ophthal- mology 12: 1253.

Wood T 0 & Haufman H E (1970): Penetrating kerato- plasty in an infant with sclerocornea. Am J Ophthal- mol 70: 609.

Zaidman G W, Johnam B L & Brown S I (1982): Corneal transplantation in a patient with corneal dermoid. Am J Ophthalmol93: 84.

Author’s address: Stuart I. Brown, MD, Department of Ophthalmology, University of California at San Diego, (T-014), La Jolla, CA 92093, USA.

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