The Boston Keratoprosthesis (KPro) is the most widely used artificial cornea or keratoprosthesis.

It
is a treatment option for corneal disease not amenable to standard penetrating keratoplasty (PKP) or
corneal transplant. Continued advances in design and superior postoperative care have resulted in
improved outcomes and an exponential increase in the use of the device in recent years.[1] In 2010,
1188 procedures were performed compared to fewer than 50 in 2002 - to date more than 4500
procedures have been performed worldwide. [2]

History
The concept of an artificial cornea is over 200 years old.[3] The first keratoprosthesis was described in
1789 during the French Revolution by Guillaume Pellier de Quengsy.
The first reported human KPro surgery with a quartz crystal implant was performed by Nussbaum
[4]

in 1855, although some modern KPro experts note that while Nussbaum may
have reported the first human surgery, in fact, Guillaume Pellier de Quengsy’s brother, also an
ophthalmologist, may have actually been the first to perform the surgery in a human.
In recent decades multiple synthetic corneas have been pioneered and developed, though only three
are principally used in practice: the Boston Keratoprosthesis (Massachusetts Eye & Ear Infirmary,
Boston, MA), the AlphaCor (Addition Technology Inc., Des Plaines, IL) [5] and the osteo-odonto
keratoprosthesis also known as the ‘OOKP’ (originally described by Strampelli, modified by
Falcinelli).[6]
The Boston Keratoprosthesis has evolved from original concept to an established device over the
past fifty years under the lifetime leadership of Claes Dohlman, MD, PhD. The device was approved
by the FDA for marketing in 1992. An active keratoprosthesis research program continues in Boston,
MA and other centers worldwide fostering continued innovation with the device.

Design
The Boston Keratoprosthesis is a collar button design keratoprosthesis. It consists of three
components: a front plate with optical stem, a back plate and titanium locking c-ring (See Additional
Resources 1). The most recent design is threadless; during assembly the front and back plates are
snapped together with corneal tissue sandwiched in-between, which is then used to suture the
device to the eye. It is available in type I and type II formats. (The type II format is reserved for
severe end-stage ocular surface disease desiccation, and is similar to the type I device but requires
a permanent tarsorrhaphy to be performed through which a small anterior nub of the type II model
protrudes). This wiki page will focus on the more commonly used type I device. The type I Boston
KPro is available in either a single standard pseudophakic plano power or customized aphakic
powers (based on axial length) with adult (8.5 mm diameter) and pediatric (7.0 mm diameter) sized
back plates. The device is currently machined from medical grade polymethylmethacrylate (PMMA)
by a small, family owned and operated precision machine shop (J.G. Machine Co. , Inc.) in Woburn,
Massachusetts .
Recent advances in design have contributed to improved outcomes. First, the addition of holes (at
present 16 holes) in the back plate allows diffusion of nutritive aqueous to support donor graft stroma
and keratocytes.[7] [8] Second, in 2004, a titanium locking c-ring was added to prevent intraocular
disassembly of the device. Third, in 2007, the design was changed from a threaded (screw-type)
assembly to a threadless design which simplified assembly and produced less damage to the donor
endothelium. [9] The most recent advance in design is the implementation of a titanium back plate
which likely improves biocompatibility and retention, and may reduce complications such as
retroprosthetic membranes (RPM) and stromal corneal melts.[10] [11] [12]
Indications
Although traditional penetrating keratoplasty (PKP) or corneal transplantation is an established
treatment for some forms of corneal blindness, some conditions are not amenable to PKP. The
Boston KPro has been used include multiple graft failure, Stevens-Johnson syndrome (SJS)[13] ,
ocular cicatricial pemphigoid (OCP), other autoimmune diseases[14] , ocular burns (acid and
alkali)[15][16] and other conditions with poor prognosis with traditional PKP. Currently, the Boston KPro
is considered by some to be a primary treatment option in cases of repeat graft failure[17] and
aniridia[18], and, by a smaller percentage of surgeons, to be a primary option in cases of herpetic
keratitis[19] and pediatric corneal opacities[20] . Pediatric KPro use in particular is still a controversial
topic that is widely debated among corneal specialists because of the high level of care needed
postoperatively and the possibly devastating complications of the procedure.

Surgical Procedure
Prosthokeratoplasty is the term for a procedure in which a damaged cornea is replaced with an
artificial cornea. During implantation of the Boston KPro, the device is assembled with a donor
corneal graft positioned between the front and back plate, that is then sutured into place in a similar
fashion to PKP. The surgical procedure may be carried out by any surgeon familiar with PKP, and
without the worry of astigmatism due to the rigidity of the assembled device. The current
recommendation is removal of the crystalline lens at the time of KPro surgery given the inevitable
development of cataract as a result of several factors, including long-term postoperative topical
steroid use, surgical trauma and other factors[21] . The primary keratoprosthesis surgery is often
combined with other procedures including iridoplasty, glaucoma filtration devices, IOL and lens
capsule removal and core vitrectomy. Please view accompanying video for a detailed review of
device assembly and surgical procedure.

Preoperative & Postoperative Photo

On the left, preoperative photo of an eye with keratoconus and hand motion vision after multiple
failed grafts. This eye had a history of multiple filtration surgeries for advanced glaucoma. On the
right, seven month postoperative photo after implantation of Boston type 1 KPro. Preliminary vision
was 20/30 despite an early RPM.
Prognostic Categories
The Boston KPro can provide visual rehabilitation for severely diseased corneas at high risk for
failure with PKP. However, preoperative diagnosis influences clinical outcome after KPro
surgery.[22] Many studies have shown the incidence of repair procedures and worse final vision
outcomes were higher in groups with autoimmune conditions (SJS, OCP). The difference in
outcomes appears to be related to the degree and cumulative past period of inflammation. Overall
most favorable outcomes are achieved in non-cicatrizing conditions, followed by ocular burns and
OCP with the worst outcomes in SJS patients.

Postoperative management
Closely monitored, long-term follow-up with an ophthalmologist familiar with the KPro is a necessity
with the device. Due to the substantial risks of glaucoma and endophthalmitis, it may be best to
manage these patients in a team setting consisting of a corneal specialist, gluacoma specialist, and
retinal specialist. It must be stressed that KPro eyes require life-long follow-up entailing the
commitment of the both the patient and ophthalmologist. The rigorous follow-up should guide patient
selection. Furthermore, patient compliance with postoperative medication is of paramount
importance. KPro surgery in a historically non-compliant patient should proceed with extreme
caution.
Due to incomplete biointegration of hardware in the eye there is a significant risk of
infectious endophthalmitis[23] , a devastating complication which often results in a substantial loss of
vision. The incidence of IE has been significantly reduced by life-time daily topical antibiotic
prophylaxis.[24] The optimal postoperative antibiotic prophylaxis is not universally agreed upon.
Typical regimes include a topical fourth generation fluoroquinolone (moxifloxacin or gatifloxacin) with
or without topical vancomycin. The increase in incidence of gram-positive IE cases in one study was
the basis for the addition of topical vancomycin (preserved with 0.005% benzalkonium chloride
which allows storage at room temperature for up to 60 days – this lowered cost and improved patient
compliance) to the prophylaxis regime in some patients. Most surgeons recommend a topical
fluoroquinolone and topical vancomycin in the initial postoperative period of all KPro eyes and then
continuing dual agent coverage in autoimmune (a group at higher risk for IE) or monocular patients.
In lower risk groups, a single agent topical fluoroquinolone or polymyxin B/trimethoprim 2-3 times
daily can provide effective long-term antibiotic prophylaxis. Some KPro surgeons recommend
antibiotic cycling to prevent microbial resistance, although there are no published studies to support
this. Regardless of regime employed, the incidence of this devastating complication is reduced with
permanent topical antibiotic prophylaxis and patients should be educated on the important of
compliance.
Another postoperative management intervention is the indefinite placement of a bandage contact
lens (BCL).[25] Placement of a BCL is needed to maintain adequate ocular surface hydration and
prevent stromal melt, dellen formation and necrosis. The BCL has many other added benefits
including improving patient comfort and protecting from possible exposed sutures. The benefit of the
BCL is multifactorial and can include other therapeutic, refractive and cosmetic roles. Ocular surface
exposure can lead to KPro melt and failure and sometimes a tarshorraphy is necessary in addition to
the BCL. The downside to BCL use is the increased risk of infection associated with contact lens
use, especially when used on a chronic, extended wear basis.
Life-long topical steroids such as prednisolone acetate is necessary in all KPro eyes to prevent
inflammation and has been successfully utilized since inception of the device.[8][26]Since steroids can
 cause IOP elevation, reduce host defenses and inhibit wound healing, KPro patients require close
follow-up and monitoring for these and other potential complications.

Complications
The three most commonly reported postoperative complications are retroprosthetic membrane
(RPM), elevated intraocular pressure/glaucoma and infectious endophthalmitis (IE) which will be
discussed here.[27] [28] [29] [30] Other less frequent complications include sterile vitritis, stromal melt,
retinal detachment and vitreous hemorrhage.
The incidence of RPM in clinical series is reported to be between 25 and 65%. It is believed
inflammation is the most important factor for RPM formation [11]. Most cases are successfully treated
with simple, single session YAG membranotomy. Typically surgical membranectomy is reserved for
cases refractory to YAG laser treatment. The YAG laser should be used with caution at energy
greater than 3.0 mJ as it can crack or pockmark the KPro optic and appropriate technique and laser
offsets should be used. [31] To avoid surgical membranectomy, RPM should be treated before it
progresses, thickens and becomes progressively more vascularized rendering it less suitable to
YAG membranotomy. Despite best efforts at RPM monitoring and treatment with YAG laser, some
patients will ultimately still progress and require more invasive surgical intervention.
With the significant reduction in IE with current antibiotic regimens, glaucoma is now the most
significant threat to vision in KPro patients. The reported incidence of glaucoma preoperatively in
KPro eyes is 60-76% and postoperative high IOP has been shown in 15-38% of KPro eyes. Gradual
closure of the anterior chamber angle is suspected as the etiology.[26] Monitoring IOP is challenging
as traditional tonometry cannot be used with the device in place and measurements rely on digital
palpation of the sclera. This complication of KPro surgery is optimally managed in close consultation
with an experienced glaucoma specialist and often requires filtration surgery with aqueous shunts
and aggressive topical IOP-lowering agents.[26][32] [33]
Infectious endophthalmitis is a catastrophic complication often resulting in loss of vision. The most
comprehensive series reports the overall incidence of IE as 2.7% per patient year[24]--in effect one of
the highest endophthalmitis rates of any ophthalmic surgical procedure currently performed. For
perspective, this is 67.5x's higher than the rate for cataract surgery, and 13.5x's higher than the rate
for glacuoma filtering surgery. Clinically this complication presents as a sudden onset of ocular pain,
scleral injection, intraocular inflammation manifested by anterior and/or vitreous turbidity (often with
hypopyon) and decreased vision. This is in contrast to sterile vitritis which also presents with a
decrease in vision. On exam, flocculent vitritis is seen but typically without the pain, tenderness or
conjunctival injection seen with IE.[26][34] Final vision after an episode of IE is closely correlated with
the causative agent - eyes infected with Streptococci or Staph. Aureus (especially methicillin-
resistant strains (MRSA)) result in poorer vision when compared to Staph. epidermis or Strep.
viridians infections. If IE occurs, immediate evaluation and treatment is required. Aqueous and/or
vitreous samples should be examined and cultured for bacteria and fungi. Intravitreal injection of
vancomycin, amikacin and dexamethosone is recommended and pars plana vitrectomy is often
necessary. Hospitalization should be considered in order to guarantee compliance with aggressive
topical treatment, post-surgical care and possible IV antibiotics.[26

References
1. Jump up↑ Klufas, M.A. and C.E. Starr, The Boston Keratoprosthesis : An update on recent
advances. Cataract and Refractive Surgery Today September 2009. 9(9).
2. Jump up↑ Klufas, M.A. and K.A. Colby, The Boston keratoprosthesis. Int Ophthalmol Clin, 2010.
50(3): p. 161-75.
3. Jump up↑ Chirila, T.V. and C.R. Hicks, The origins of the artificial cornea: Pellier de Quengsy and
his contribution to the modern concept of keratoprosthesis. Gesnerus, 1999. 56(1-2): p. 96-106.
4. Jump up↑ Pellier de Quengsy, G., Précis au cours d'operations sur la chirurgie des yeux. 1789,
Paris: Didot.
5. Jump up↑ Hicks, C.R., et al., Outcomes of implantation of an artificial cornea, AlphaCor: effects of
prior ocular herpes simplex infection. Cornea, 2002. 21(7): p. 685-90.
6. Jump up↑ Liu, C., et al., The osteo-odonto-keratoprosthesis (OOKP). Semin Ophthalmol, 2005.
20(2): p. 113-28.
7. Jump up↑ Khan, B.F., et al., Advances in Boston keratoprosthesis: enhancing retention and
prevention of infection and inflammation. Int Ophthalmol Clin, 2007. 47(2): p. 61-71.
8. ↑ Jump up to:8.0 8.1 Harissi-Dagher, M., et al., Importance of nutrition to corneal grafts when used as a carrier
of the Boston Keratoprosthesis. Cornea, 2007. 26(5): p. 564-8.
9. Jump up↑ Dohlman, C. and M. Harissi-Dagher, The Boston Keratoprosthesis: A New Threadless
Design. Digital Journal of Opthalmology, 2007. 13(3).
10. Jump up↑ Ament, J.D., et al., The Boston Keratoprosthesis: Comparing Corneal Epithelial Cell
Compatibility with Titanium and PMMA. Cornea, 2009.
11. ↑ Jump up to:11.0 11.1 11.2 Dohlman, C.H., et al., Titanium vs. PMMA Backplates for Boston Keratoprosthesis:
Incidence of Retroprosthetic Membrane. ARVO, 2009. Program Number 1505: p. Poster A415.
12. ↑ Jump up to:12.0 12.1 Newsletter VII, in Boston Keratoprosthesis Update, R. Walcott-Harris and C. Dohlman,
Editors. 2010.
13. Jump up↑ Sayegh, R.R., et al., The Boston keratoprosthesis in Stevens-Johnson syndrome. Am J
Ophthalmol, 2008. 145(3): p. 438-44.
14. Jump up↑ Ciralsky, J., et al., Keratoprosthesis in autoimmune disease. Ocul Immunol Inflamm, 2010.
18(4): p. 275-80.
15. Jump up↑ Tuft, S.J. and A.J. Shortt, Surgical rehabilitation following severe ocular burns. Eye, 2009.
16. Jump up↑ Harissi-Dagher, M. and C.H. Dohlman, The Boston Keratoprosthesis in severe ocular
trauma. Can J Ophthalmol, 2008. 43(2): p. 165-9.
17. Jump up↑ Ma, J.J., J.M. Graney, and C.H. Dohlman, Repeat penetrating keratoplasty versus the
Boston keratoprosthesis in graft failure. Int Ophthalmol Clin, 2005. 45(4): p. 49-59.
18. Jump up↑ Lee, H., R. Khan, and M. O'Keefe, Aniridia: current pathology and management. Acta
Ophthalmol, 2008. 86(7): p. 708-15.
19. Jump up↑ Khan, B.F., et al., The Boston keratoprosthesis in herpetic keratitis. Arch Ophthalmol,
2007. 125(6): p. 745-9.
20. Jump up↑ Aquavella, J.V., et al., Pediatric keratoprosthesis. Ophthalmology, 2007. 114(5): p. 989-
94.
21. Jump up↑ Harissi-Dagher, M. and K.A. Colby, Cataract extraction after implantation of a type I
Boston keratoprosthesis. Cornea, 2008. 27(2): p. 220-2.
22. Jump up↑ Yaghouti, F., et al., Keratoprosthesis: preoperative prognostic categories. Cornea, 2001.
20(1): p. 19-23.
23. Jump up↑ Nouri, M., et al., Endophthalmitis after keratoprosthesis: incidence, bacterial causes, and
risk factors. Arch Ophthalmol, 2001. 119(4): p. 484-9.
24. ↑ Jump up to:24.0 24.1 Durand, M.L. and C.H. Dohlman, Successful prevention of bacterial endophthalmitis in
eyes with the Boston keratoprosthesis. Cornea, 2009. 28(8): p. 896-901.
25. Jump up↑ Harissi-Dagher, M., J. Beyer, and C.H. Dohlman, The role of soft contact lenses as an
adjunct to the Boston keratoprosthesis. Int Ophthalmol Clin, 2008. 48(2): p. 43-51.
26. ↑ Jump up to:26.0 26.1 26.2 26.3 26.4 Dohlman, C.H., et al., Introduction to the use of the Boston keratoprosthesis.
Expert Review of Ophthalmology, 2006. 1(1): p. 41-48.
27. ↑ Jump up to:27.0 27.1 Chew, H.F., et al., Boston keratoprosthesis outcomes and complications. Cornea, 2009.
28(9): p. 989-96.
28. ↑ Jump up to:28.0 28.1 Bradley, J.C., et al., Boston type 1 keratoprosthesis: the university of california davis
experience. Cornea, 2009. 28(3): p. 321-7.
29. ↑ Jump up to:29.0 29.1 Aldave, A.J., et al., The Boston type I keratoprosthesis: improving outcomes and
expanding indications. Ophthalmology, 2009. 116(4): p. 640-51.
30. Jump up↑ Akpek, E.K., et al., Outcomes of Boston keratoprosthesis in aniridia: a retrospective
multicenter study. Am J Ophthalmol, 2007. 144(2): p. 227-231.
31. Jump up↑ Chak, G. and J.V. Aquavella, A safe Nd:YAG retroprosthetic membrane removal
technique for keratoprosthesis. Cornea, 2010. 29(10): p. 1169-72.
32. Jump up↑ Dohlman, C.H., et al., Shunts to divert aqueous humor to distant epithelialized cavities
after keratoprosthesis surgery. J Glaucoma, 2010. 19(2): p. 111-5.
33. Jump up↑ Rivier, D., et al., Glaucoma and keratoprosthesis surgery: role of adjunctive
cyclophotocoagulation. J Glaucoma, 2009. 18(4): p. 321-4.
34. Jump up↑ Nouri, M., M.L. Durand, and C.H. Dohlman, Sudden reversible vitritis after
keratoprosthesis: an immune phenomenon? Cornea, 2005. 24(8): p. 915-9.
35. Jump up↑ Zerbe, B.L., M.W. Belin, and J.B. Ciolino, Results from the multicenter Boston Type 1
Keratoprosthesis Study. Ophthalmology, 2006. 113(10): p. 1779 e1-7.
36. Jump up↑ Ament, J.D., et al., Global corneal blindness and the Boston keratoprosthesis type I. Am J
Ophthalmol, 2010. 149(4): p. 537-9.
37. Jump up↑ Ament, J.D., et al., Role for ipsilateral autologous corneas as a carrier for the Boston
keratoprosthesis: the Africa experience. Arch Ophthalmol, 2010. 128(6): p. 795-7.
38. Jump up↑ Ament, J.D., et al., Cost-effectiveness of the Boston keratoprosthesis. Am J Ophthalmol,
2010. 149(2): p. 221-228 e2.
39. ↑ Jump up to:39.0 39.1 Ciolino, J.B. and C.H. Dohlman, Biologic keratoprosthesis materials. Int Ophthalmol
Clin, 2009. 49(1): p. 1-9.
40. Jump up↑ Ament, J.D., et al., The Boston Keratoprosthesis: comparing corneal epithelial cell
compatibility with titanium and PMMA. Cornea, 2009. 28(7): p. 808-11.
41. Jump up↑ Todani, A., et al., Measurement of IOP With Intraocular Pressure Transducer. ARVO,
2009. Program Number 5220: p. Presentation Abstract.
42. Jump up↑ Ciolino, J.B., et al., A drug-eluting contact lens. Invest Ophthalmol Vis Sci, 2009. 50(7): p.
3346-52.
43. Jump up↑ Pineles, S.L., et al., Binocular Visual Function in Patients With Boston Type I
Keratoprostheses. Cornea, 2010.