From Western Eye Hospital (HM), London, United Kingdom; the Ocular Oncology Service (JLH), the Cornea and External Disease Service (DSG), and the Oculoplastics Service (HMH), Moorfields Eye Hospital, London; United Kingdom; and Ocular Oncology and Vitreoretinal Surgery Services (PM), Duke University Eye Center, Durham, North Carolina.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Hemal Mehta, MA, MBBS, Western Eye Hospital, Marylebone Road, London NW1 5YE, United Kingdom.
Transpositional autokeratoplasty describes the process by which a corneal graft is obtained from the patient’s fellow eye. Transpositional autografting has been used in instances of unilateral traumatic, degenerative or congenital corneal disease where the other (donor) eye was already blind, usually due to retinal degeneration.1 We report a case of transpositional penetrating autokeratoplasty where healthy donor tissue was obtained from the contralateral eye which harboured a choroidal melanoma.
A 67-year-old woman presented to Moorfields Ocular Oncology Service with a 2-month history of painless peripheral visual field loss in her right eye. Her past medical history was significant for systemic tumors including a cerebellopontine angle meningioma resected 20 years earlier and primary right renal cell carcinoma treated by nephrectomy 6 years earlier. She had chronic exposure keratopathy in the left eye, due to facial nerve damage at the time of meningioma resection, which had failed treatment with aggressive lubrication.
On ophthalmic examination, the left eye visual acuity was hand movements with inaccurate projection. The lower eyelid malposition resulted in a significant 5 mm lagophthalmos. The tear film was dry and the cornea was opacified with extensive vascularization (Fig. 1). The posterior segment was normal by ultrasonography.
Figure 1. Pre-Surgery Image of the Left Eye Showing Lagophthalmos and Resultant Exposure Keratopathy.
The right eye visual acuity was light perception and a relative afferent pupillary defect was present. Fundus examination revealed an infero-nasal amelanotic choroidal mass with overlying exudative retinal detachment. The cornea was clear with no pannus, scarring or guttatae. Ultrasound confirmed a choroidal mass measuring 12.3 mm at greatest basal diameter and 6.3 mm in height with low to medium internal reflectivity and no extraocular extension. A choroidal biopsy was performed to exclude a metastasis and confirmed a spindle B melanoma. Systemic evaluation did not reveal any reactivation of either the renal cell carcinoma or central nervous system meningioma.
The choroidal melanoma was treated with Ru-106 episcleral plaque brachytherapy with 120 Gray delivered to the tumor apex. Eighteen months later, the vision declined to no light perception with associated iris rubeosis, choroidal tumor enlargement and extensive exudative detachment. The cornea remained clear. This right eye was scheduled for enucleation for local tumor control. In anticipation of her monocular status, penetrating keratoplasty was planned for the left eye to improve corneal clarity. A 1-gram gold weight was inserted into the left upper lid in addition to a medial canthoplasty to enhance lid closure.
The Cornea and Ocular Oncology Services decided to perform a corneal transplant for the left eye with the donor corneal button obtained from the patient’s right eye. In a staged approach, initial transpositional autokeratoplasty was followed by enucleation of the right eye 4 weeks later.
The transpositional autograft was carried out under local anaesthesia. An 8.00 mm right corneal button was trephined. An 8.25 mm donor corneal button was placed into the 8.00 mm right eye corneal rim and secured with 10-0 nylon continuous sutures. The 8.00 mm right corneal button was fitted to the left eye 7.75 mm corneal rim and secured with interrupted 10-0 nylon sutures.
1 week post-operation, the left eye visual acuity was 3/60 with intact graft interfaces bilaterally. 4 weeks later, the right eye was enucleated under local anaesthesia with sedation. Histopathology confirmed a choroidal melanoma with anterior segment neovascularisation, but no evidence of anterior chamber tumor seeding.
No systemic immunosuppression was required and topical steroid drops were stopped 3 months after the procedure. One year after the autograft procedure, lid apposition over the left eye was improved and the donor button clear with sutures intact (Fig. 2). Two years after the autograft, the visual acuity was 6/12 in the left eye and the graft was clear following suture removal and cataract extraction. No tumor seeding was detected clinically and the systemic screen remained negative for metastatic disease.
Figure 2. One Year After Operation the Graft in the Left Eye Is Clear and Sutures Intact.
Since Plange1 reported the first transpositional autograft in 1908, the procedure has been used to treat unilateral corneal disease by obtaining graft tissue from the fellow eye, which was blind usually due to degenerative posterior segment disease or advanced glaucoma. To the authors’ knowledge, this is the first reported case of transpositional penetrating autokeratoplasty with donor tissue coming from a fellow eye which harborsharbors a posterior uveal melanoma. This report raises issues about the use of melanoma eyes as a source of donor corneal tissue and the role of transpositional autokeratoplasty compared with conventional penetrating corneal allografts.
Corneas from eyes enucleated for choroidal melanoma were frequently used for allografts;2 although this is less common since the advent of testing donors for infectious agents such as HIV, because of the complex issues arising from counselling a patient about both removal of an eye and serious infectious disease.3 There have been no reported cases of transmission of uveal tract melanoma by corneal transplantation and a retrospective cohort study2 comparing outcomes with tissue from eyes with primary choroidal melanoma (n = 47) and normal eyes (n = 47) showed no evidence of tumor transmission after a limited follow up period of 5.4 years. There was no significant difference in the incidence of graft rejection, corneal thickness and endothelial cell counts between the two groups. There are no reports of subsequent ocular, orbital, or metastatic spread in patients that have received a donor button from an eye harbouring a choroidal melanoma. The Eye Bank Association of America4 (EBAA) only advises use of corneas if the uveal melanoma does not involve the ciliary body or more anterior structures of the eye.
In donor patients with systemic malignancies, the EBAA permit corneas to be transplanted provided adenocarcinoma is excluded, the anterior structures of the eye are not involved, and an infectious aetiology of the malignancy, such as leukaemia or lymphoproliferative disorders, is not suspected. There is a single case report of transmission of a systemic malignancy (disseminated colorectal adenocarcinoma with choroidal metastases) by allokeratoplasty to the recipient’s eye (iris papillary adenocarcinoma) with confirmation of tumor markers by PCR.5
Autokeratoplasty offers some advantages over allografts. Autografting reduces the chances of corneal graft rejection,6 a substantial risk to successful outcome. Autografts may require less topical immunosuppression than allografts, thereby reducing associated side-effects. Price and Hanna7 took advantage of this to carry out autografts in three patients with pre-existing glaucoma who would not have been able to tolerate topical corticosteroids.
Potential risks of this procedure include bilateral infection7 and intra-operative expulsive haemorrhage as both corneal domes would be open during the procedure. Potential damage to the corneal button during surgical manipulation would require stand-by allograft donor tissue to be available. Patients undergoing this staged corneal autotransplantation and subsequent fellow eye enucleation for a malignant melanoma must be cautioned to the risks of both surgeries.
In this report, the patient had severe corneal decompensation in one eye and a blind fellow eye with a posterior melanoma which had failed brachytherapy. The corneal transplant and enucleation surgeries were staged as the patient could not tolerate general anaesthesia or prolonged surgery due to health reasons. The staged approach was more tolerable to the patient, but had the small, but unquantifiable, risk of metastatic dissemination by introducing a 4 week delay before enucleation of the melanoma eye. Ideally, these procedures would be combined and carried out under general anaesthesia.
In this observational report, limited to 2 years follow-up, there is no evidence of an increased risk of contralateral ocular seeding or enhanced metastatic potential as a result of transpositional autokeratoplasty involving an eye with choroidal melanoma. The patient has been successfully visually rehabilitated with no evidence of graft rejection.
- Duke-Elder S., System of ophthalmology. Diseases of the cornea and sclera, epibulbar manifestations of systemic diseases, cysts and tumors. vol. VIII(2). 1965; London: Henry Kimpton. p. 263.
- Harrison D.A., Hodge D.O., Bourne W.M., Outcome of corneal grafting with donor tissue from eyes with primary choroidal melanomas. A retrospective cohort comparison. Arch Ophthalmol. 1995;113(6): 753–756.
- Maguire P., Faulkner A., Communicating with cancer patients. Bmj. 1988;297(6663):1610. doi:10.1136/bmj.297.6663.1610 [CrossRef]
- Chu W., The past twenty-five years in eye banking. Cornea, 2000;19(5): 754–765. doi:10.1097/00003226-200009000-00020 [CrossRef]
- McGeorge A.J., et al. , Papillary adenocarcinoma of the iris transmitted by corneal transplantation. Arch Ophthalmol, 2002;120(10):1379–1383.
- Boruchoff S.A., Dohlman C. H., Corneal autografts. Am J Ophthalmol. 1967;63(6):1677–1681.
- Price F.W. Jr., Hanna S.I., Bilateral penetrating autokeratoplasty. J Refract Surg. 1995;11(6):494–496.