August 2014

 

RESIDENTS

 

Academic grand rounds

Artificial anterior segment


by Nathaniel Roybal, MD, PhD

 
 

Kevin Miller, MD, Kolokotrones professor of clinical ophthalmology, Jules Stein Eye Institute, Los Angeles

Nathaniel Roybal, MD, PhD, third-year resident

 

Figure 1: Appearance of the patient at the time of initial presentation to Dr. Miller. The left eye was aphakic and aniridic.

Figure 2: Appearance of the patient following Ophtec 311, Ahmed tube shunt, and Boston type 1 keratoprosthesis implantation

Introduction from Kevin M. Miller, MD

This month we present an interesting but unfortunate gentleman who was seen by multiple specialists at UCLAs Stein Eye Institute. He is a complex patient who presented emergently to our tertiary care facility for evaluation following multiple surgeries. The patient required a multispecialty surgical approach to address his visual rehabilitation. He was recently evaluated by Nathaniel Roybal, MD, PhD, one of the current third-year residents, while rotating through the cornea service. Nathaniel chose this case due to its novelty and interesting discussion. Nathaniel is headed to the University of Iowa for a vitreoretinal fellowship when he graduates. Our discussant panel includes Simon K. Law, MD, PharmD, from the glaucoma division at Stein Eye, and Sophie X. Deng, MD, PhD, from the cornea division at Stein Eye. I also provided care for this patient and was asked to share some of my perspectives.

Case presentation

A 67-year-old man of Middle Eastern descent with a history of peripheral corneal degeneration of unknown etiology underwent penetrating keratoplasty of the left eye (OS) in 1975 and right eye (OD) in 1977. He underwent repeat penetrating keratoplasty OS in 1998 for graft failure and uncomplicated bilateral cataract extraction with intraocular lens (IOL) implantation in 2002. In 2005, he presented to Stein Eye with a Paecilomyces fungal keratitis complicated by perforation requiring an emergent therapeutic penetrating keratoplasty. He then developed a fungal endophthalmitis, ciliary body detachment and hypotony, and underwent pars plana vitrectomy, IOL explantation, and ciliary body repair OS. He had persistent corneal surface disease OS including marked surface irregularity and recurrent epithelial defects secondary to limbal stem cell deficiency. Two years later, he was referred to Dr. Millers clinic. He was surgically aphakic and functionally aniridic OS at that time (Figure 1). He reported significant photophobia, glare sensitivity, dissatisfaction with his cosmetic appearance, and poor visual acuity. He was on maximal medical therapy with elevated intraocular pressure. His uncorrected distance visual acuity (UDVA) was 20/50-1 OD and counting fingers at 1 foot OS with no improvement on pinhole testing. His corrected distance visual acuity (CDVA) with spectacles was 20/50-2 OU with manifest refractions of 1.00 +4.00 x 015 OD and +9.50 sphere OS. Slit lamp biomicroscopy OD showed peripheral corneal thinning with opacification, a clear corneal graft, and a posterior chamber IOL. Biomicroscopy OS revealed peripheral corneal thinning, a clear corneal graft with temporal guttata, peripheral anterior synechiae from 9 to 11 oclock, an irregular 11 mm pupil, and aphakia. His optic nerve OS revealed mild pallor with a 0.6 cup to disk ratio. His posterior segments were within normal limits bilaterally.

Discussion

The case was presented to some of the local experts. Dr. Law commented: Glaucoma is a common outcome in eyes that have had multiple interventions. Given the patients elevated intraocular pressure, Dr. Law recommended glaucoma surgery. We have extensive experience in placing drainage devices in similar eyes following penetrating keratoplasty or after the implantation of intraocular devices such as the Ophtec 311 [Ophtec USA, Boca Raton, Fla.] and keratoprostheses. Regarding the presence of the corneal graft he stated, Studies have shown that the health of a corneal graft may be adversely affected by the placement of a glaucoma drainage device1; however, this particular eye had refractory disease as a result of multiple surgeries. Glaucoma surgery was inevitable. I asked Dr. Law how the peripheral anterior synechiae (PAS) might affect the case. He stated, The extent of PAS can increase after each additional surgery. Interestingly, he pointed out that outflow can also be adversely affected after additional surgeries even without obvious closure of the angle by the peripheral iris. I asked about his preferred surgical approach. I find that tube shunt implantation is a viable option in these cases. Dr. Miller has extensive experience with artificial iris implantation. He said, An acceptable cosmetic and refractive result could be achieved with a colored contact lens. Unfortunately, this patient was unable to tolerate a rigid gas permeable lens secondary to his irregular corneal surface and recurrent erosions. He was not a good candidate for a traditional secondary IOL because of the markedly dilated pupil. He was, however, an excellent candidate for an Ophtec 311, an iris reconstruction lens that combines an artificial iris and optic. It provides satisfactory cosmetic results and would address both the aniridia and the aphakia.2

Dr. Deng commented from the cornea and anterior segment perspective. She stated: The patient is status post corneal transplantation, aniridic and aphakic. A standard IOL will not achieve the best outcome. Regarding the patient being hyperopic and suffering from significant glare, I agree with Dr. Miller. I would address the patients symptoms and proceed with iris reconstruction and lens implantation. Unfortunately, this procedure may cause further graft decompensation. The patient already had two failed grafts and suffered from an abnormal corneal surface due to limbal stem cell deficiency. If the current graft further decompensated, he would likely benefit most from a Boston type 1 keratoprosthesis (Massachusetts Eye and Ear Infirmary, Boston). The keratoprosthesis has an expanding role in visual rehabilitation.3 All three attendings shared a common suggestion regarding preoperative counseling. The possible limited outcomes and complications should be discussed in detail with the patient. Combining several prosthetic devices in the anterior chamber will have an unknown long-term outcome. They also stated that his pinhole vision of 20/50 was an excellent indicator that he had a good possibility of visual rehabilitation.

Case outcome

Dr. Miller decided to address the patients physical, optical and cosmetic discomfort with a combined procedure. The patient underwent repeat penetrating keratoplasty OS combined with anterior synechiolysis and implantation of a 15 D brown Ophtec 311 iris reconstruction lens that was suture-fixated to the sclera. The Ophtec 311 combines a colored artificial iris with a clear intraocular lens. The patients postoperative course was complicated by persistent hyphema requiring an anterior chamber washout. He also developed medically uncontrolled secondary angle closure glaucoma for which he underwent Ahmed tube shunt (New World Medical, Rancho Cucamonga, Calif.) implantation OS. His UDVA remained 20/400 OS with a CDVA of 20/70 secondary to persistent corneal surface disease and irregularity. However, he reported a significant improvement in photophobia and glare sensitivity. As a result of the persistent corneal surface disease, a Boston type 1 keratoprosthesis was implanted OS. His immediate postoperative appearance is shown in photographs taken 1 month after surgery (Figure 2). At this point, his anterior segment was almost completely artificial. Four months after the keratoprosthesis surgery, the patient had a UDVA of 20/40 and a CDVA of 20/30. Eleven months after surgery, he had a UCVA of 20/40. Intraocular pressure (IOP) was estimated to be 10 mmHg by digital palpation and measured to be 14 mmHg by pneumotonometry at the limbus on no IOP-lowering agents. His optic nerve head revealed moderate glaucomatous cupping with mild pallor and a cup-to-disc ratio of approximately 0.55.

Take-home points

This patient presented with multiple ocular comorbidities and a long history of anterior segment degeneration. The patients visual rehabilitation required multiple surgeries and a unique combination of prosthetic devices. The case demonstrates the successful incorporation of multiple prosthetic elements to achieve an excellent visual outcome.

References

1. Sugar A, Tanner JP, Dontchev M, Tennant B, Schultze RL, Dunn SP, Lindquist TD, Gal RL, Beck RW, Kollman C, Mannis MJ, Holland EJ. Recipient risk factors for graft failure in the cornea donor study; Cornea Donor Study Investigator Group. Ophthalmology 2009; 116:10238. 2. Pozdeyeva NA, Pashtayev NP, Lukin VP, Batkov YN. Artificial iris-lens diaphragm in reconstructive surgery for aniridia and aphakia. J Cataract Refract Surg 2005; 31:17501759.

3. Aldave AJ, Kamal KM, Vo RC, Yu F. The Boston type I keratoprosthesis: improving outcomes and expanding indications. Ophthalmology 2009; 116: 640651.

Contact information

Miller
: kmiller@ucla.edu
Roybal: christopher-roybal@uiowa.edu

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