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REVIEW

CURRENT
OPINION The osteo-odonto-keratoprosthesis
Mehran Zarei-Ghanavati a,b,, Venkata Avadhanam a,,
Alfonso Vasquez Perez a, and Christopher Liu a,c,d

Purpose of review
To describe the practice of and recent developments in the osteo-odonto-keratoprosthesis.
Recent findings
Formal psychological assessment and support have been described. A sub-aqua system for fashioning the
osteo-odonto-keratoprosthesis lamina; adoption of thicker laminae; use of bisphosphonate drugs; advances
in laminar imaging; and use of bone augmentation and bone morphogenetic protein have been described
for prevention, detection, and management of laminar resorption. Two systems of optical cylinders
available commercially. A stepladder approach to buccal mucous membrane overgrowth onto the optical
cylinder has been described, including use of mitomycin-C. Orbital decompression has been used for
cosmetic improvement. Detection of glaucoma may be aided by an intraocular pressure sensor, whereas
surgical treatment is mainly by way of glaucoma drainage devices, as endolaser ciliary ablation and recti
muscle disinsertion and reinsertion not having prolonged and significant long-term beneficial effect.
Sublingual timolol has been described. The use of endoscopy has been proposed for preoperative
evaluation of the posterior segment, ciliary ablation and for vitreoretinal surgery although wide-angle
viewing systems remains standard practice.
Summary
The osteo-odonto-keratoprosthesis is the procedure of choice for rehabilitation of corneal blindness for end-
stage ocular surface disease, serving a completely different patient group to the Boston Type 1 KPro.
Keywords
glaucoma, keratoprosthesis, KPro, laminar resorption, MOOKP, OOKP

INTRODUCTION PATIENT ASSESSMENT


The osteo-odonto-keratoprosthesis (OOKP) is OOKP is life-changing surgery for patients. It
the most enduring device among all keratoprosthe- requires lifelong follow up and commitment to
ses to date. Its success is attributed to the biological attend hospital visits periodically and patients
constitution of its components [1]. Strampelli should be prepared to undertake further surgical
first described the technique of using tooth as a revisions as required. Patients referred to the OOKP
keratoprosthesis (KPro) skirt and oral mucous service are assessed by a multidisciplinary team
membrane as a cover to protect the device [2]. comprising of ophthalmologists, a maxillofacial sur-
Faclinelli made several improvements to the geon, anaesthetists and a clinical psychologist. A
technique, which is now known as modified or thorough assessment is carried out including ocular
MOOKP [3]. examination, evaluation of visual potential, oral
The OOKP device is made of an alveo-dental health and availability of suitable teeth, general
lamina made from a single rooted tooth usually a
canine of the patient, which harbours an optical a
Sussex Eye Hospital, Brighton, UK, bFarabi Eye Hospital, Tehran Uni-
cylinder in its centre through which the patient can versity of Medical Sciences, Tehran, Iran, cBrighton and Sussex Medical
see. The entire complex is covered with buccal School, Brighton and dTongdean Eye Clinic, Hove, UK
mucosa (BM) after its implantation onto the eye. Correspondence to Christopher Liu, FRCOphth, Sussex Eye Hospital,
The BM is a resilient and robust membrane that Eastern Road, Brighton, UK, BN2 5BE. Tel: +44 1273 696955;
offers physical protection as well as nourishment ext: 7652; fax: +44 1273 664610; e-mail: cscliu@aol.com
to the osseous part of the lamina. The OOKP is the 
Mehran Zarei-Ghanavati and Venkata Avadhanam contributed equally to
procedure of choice for eyes with severe dryness and the manuscript.
absent lid(s) or blink. Indications and contraindica- Curr Opin Ophthalmol 2017, 28:397–402
tions for OOKP are given in Table 1. DOI:10.1097/ICU.0000000000000388

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Corneal and external disorders

Table 2. Preoperative assessment


KEY POINTS
Preoperative ophthalmological investigations
 Combination of careful patient selection, VA (intact light perception) Essential
multidisciplinary approach, meticulous surgical
Entopic phenomena Not mandatory
technique and regular follow-up have provided
satisfactory visual success rate for OOKP. Electrodiagnostic tests (flash VEP, flash ERG) Not mandatory
Ultrasonography (A-scan and B-scan) Essential
 Timely management of mucosal membrane
Digital estimation of IOP Essential
complications can prevent more serious ones.
Preoperative oral investigations
 Early diagnosis and treatment of glaucoma is the most Orthopantomography Essential
significant step to improve functional success after OOKP.
X-ray of tooth Essential
 Laminar resorption has been addressed by recent Spiral CT Not mandatory
advances in diagnosis, prevention and treatment of Preoperative psychological assessment Essential
this complication.
Preoperative anaesthesia assessment Essential
 Endophthalmitis should be ruled out and treated as an
emergency in any OOKP patient with new symptoms of CT, computed tomography; ERG, electroretinogram; IOP, intraocular pressure;
VEP, visual evoked potential.
pain or decreased vision.

support is of paramount importance. Those who are


well adjusted to blindness are best not offered the
health condition and fitness for general anaesthesia.
procedure. OOKP is performed only in one eye –
The psychologist assesses patients’ mental status,
usually to the eye with the best visual potential or to
preparedness and understanding of OOKP surgery
an only eye. Smokers are advised to quit the habit.
and its sequelae. Having adequate social and family
Potential patients are asked to pay attention to oral
hygiene. All patients must be counselled for possible
Table 1. Indications and contraindications of OOKP
loss of vision at any time after OOKP surgery and for
Indications: ocular emergencies such as endophthalmitis and
Patient should fulfil all of the following criteria: retinal detachment. Table 2 summarize preoperative
Severe visual loss (<6/60 in better or only eye) because of assessment steps.
corneal opacity (uncorrectable with scleral contact lens)
Poor prognosis of conventional keratoplasty because of high risk
of rejection or limbal stem cell deficiency SURGICAL TECHNIQUE
Severe dry eye and/or severe irreparable lid damage Details of OOKP surgery are fully described in
Common indications: previous publications, which is generally carried
Stevens–Johnson syndrome out in two stages [3,4]. In the first stage, the ocular
Mucous membrane pemphigoid surface is prepared and mucous membrane grafted
Chemical injury onto the eye. At the same or a separate sitting, the
Thermal injury (fire, liquid aluminium, etc) OOKP lamina is prepared and implanted under
Trachoma
orbicularis of the contralateral lower eyelid. In the
second stage, usually after 2–4 months after the
Contraindications:
first, the lamina is retrieved, the mucosa is lifted
Age under 18
off the globe and lamina is inserted onto the eye.
Vision  6/60
No light perception in candidate eye
Advanced glaucoma or severe optic nerve damage (relative STAGE 1
contraindication)
Irreparable retinal detachment Preparation of the globe and mucous
Evidence of phthisis membrane graft
Unrealistic expectations A BM free graft is harvested below the parotid duct.
Patients is not psychologically able to accept: Muscle and excess fat are trimmed off and the graft is
Severe multi-staged operations and possible further surgery soaked in an antibiotic solution usually cefuroxime.
Cosmetic appearance of operated eye BM is preferred over labial because the former
Life-long follow-up is thicker.
Risk of severe complications or permanent visual loss A 360-degree limbal peritomy is carried out on the
proposed eye. Superficial keratectomy is performed
OOKP, osteo-odonto-keratoprosthesis. to remove the epithelium and scar tissues over the

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The osteo-odonto-keratoprosthesis Zarei-Ghanavati et al.

cornea. The rectus muscles are isolated with bridle diameter. It passes through the lamina and projects
sutures. The BM graft is trimmed to size and sutured a 2–3 mm beyond the alveolar side. The posterior
to the sclera and rectus insertions (for vascular supply). section, 3 mm long and 4.5 to 5.25 mm wide (small
A plastic conformer is applied over the mucosa, which and large versions), is closely opposed to the dentine
stays for a month to prevent adhesions. surface of the lamina and traverses the anterior
In cases with poor oral health and mucosal chamber. A PMMA cylinder of appropriate power
disease, severe dry eye, or where viability of BM (based on A-scan biometry) is inserted into the
grafts is a concern, laminar preparation is deferred lamina and cemented, which acts as a filler rather
until the mucosa is well established on the ocular than an adhesive (Fig. 1c). If the periosteum has
surface. Thus, stage 1 could be divided into two been detached, it is reattached with fibrin glue. The
separate sittings. entire complex is implanted in a sub-muscular
pocket in the contralateral lower lid for fibrovascular
investment.
Preparation of the osteo-odonto-acrylic
lamina
A monoradicular tooth (usually a canine) and sur- Edentulous patients
rounding alveolar bone is removed en bloc (Fig. 1a). Patients requiring an OOKP but who do not have
The tooth and the surrounding bone are fashioned suitable teeth have the option of an allograft OOKP or
with a dental flywheel to an approximately 3 mm tibial bone KPro. An allograft tooth can be retrieved
thick rectangular lamella under constant irrigation from living related or nonrelated donors following
with balanced salt solution. An aperture is drilled human leukocyte antigen matching and screening
perpendicularly in its centre to accommodate the for blood-borne diseases. Although some authors
optical cylinder (it will otherwise cause cylinder tilt) have reported long term success, [7] albeit not as
(Fig. 1b). There are two systems of optical cylinders good retention results as autografts, our allograft
available. One takes advantage of a larger posterior and tibial bone patients have had poor retention.
diameter to increase visual field [5]. The other aims
for myopia and uses spectacle correction to mini-
mize the image to improve visual field [6]. The STAGE 2
current UK design of Poly(methyl methacrylate) First, the lamina is retrieved and soft tissues over its
(PMMA) cylinder, which is based on first system, osseous aspect is trimmed to reduce bulk, while the
has two sections. The anterior stem is 5.75 mm long tissue on the dentine side is totally excised (Fig. 1d).
and 3.5 to 4 mm (small and large versions) in Further surgery is only contemplated if the lamina is

FIGURE 1. (a) Upper canine tooth and surrounding jawbone extracted en bloc. (b) A hole is drilled through lamina. (c) The
optic cylinder is cemented to the hole in the lamina. (d) Removal of excess soft tissue from the retrieved lamina. (e)
Cryoextraction of lens. (f) The lamina sutured to the sclera.

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Corneal and external disorders

FIGURE 2. (a) Central mucosal membrane ulceration. (b) Implantation of Baerveldt tube. (c) Explantation of the lamina for
leakage. A channel undetectable by CT scanning had formed. CT, computed tomography.

adequate without excessive resorption. The lamina bulk and mobility. The optical cylinder is checked for
is stored in heparinized venous blood of the patient stability with the wooden end of a cotton tip. Fundo-
until the time of implantation. The BM graft is scopy is carried out with slit lamp biomicroscopy.
dissected from the superior part of the globe and Periodic visual fields and B-scanning are carried out.
lifted off to expose cornea and sclera. The centre of
the cornea is identified, marked and trephined to
match the posterior part for the optical cylinder. OUTCOMES
Two relieving radial corneal incisions are made for OOKP is highly successful and patients can achieve a
cryoextraction of lens or removal of IOL and cap- visual acuity of up to 6/4. It is also the most durable
sular bag (Fig. 1e). Iris is disinserted from its root, device with laminar retention rates of up to 80%
and core vitrectomy is performed. The lamina is over two decades [1]. In Tan’s systematic review,
positioned on the cornea with the posterior stem more than half of patients enjoy a vision better than
of the cylinder passing through the corneal open- 6/18 after OOKP [8]. In our study, the same percent-
ing. It is anchored tightly with Vicryl sutures to the age of patient gained visual acuity of 6/12 or better
globe (Fig. 1f). Centration of the optical cylinder is [9]. However, a number of complications can occur
confirmed with intraoperative indirect ophthal- related to mucosa, lamina, IOP and retina.
moscopy. The BM graft is reflected back on the BM graft problems ranging from mucous mem-
eye to cover the lamina and ocular surface. Its brane overgrowth occluding the optic to its ulcer-
central part is trephined to allow protrusion of ation and exposure of the lamina are among
the anterior optical cylinder. the commonest complications (up to 50%) [10].
Mucosal overgrowth can be treated with trimming
and cauterization. In recurrent cases, off-label appli-
FOLLOW UP CARE &&
cation of mitomycin C can be used [11 ]. Mucosal
OOKP patients are admitted for 1 week after each ulcerations occur because of ischaemia, dryness and
stage and receive systemic and local antibiotic treat- infection. They need prompt treatment to prevent
ment and oral prednisolone. Allograft patients are laminar erosion and intraocular infection (Fig. 2a).
started on systemic immunosuppression after stage 1. Mild ulcers can be treated with adequate lubrica-
Oral acetazolamide is required after stage 2 and it tion. Small areas of laminar exposure due to mucosal
should be continued until intraocular pressure (IOP) erosion can be treated with smoothening of the
is normalized. Night-time application of broad-spec- lamina’s rough edges with increased lubrication.
trum antibiotic ointment has to be continued for life. Larger ulcers could be surgically treated with tarsal
A different antibiotic ointment is used in a 3-month pedicle, mucosal rotation, and bucket handle flaps
alternate cycle to reduce bacterial resistance. The and free patch grafts. Occasionally, it is necessary
optical cylinder is cleaned with juice from a fresh to perform a new, complete BM graft following
cut lemon over sterile cotton tipped applicator. Stage 1. Recurrent ulceration following Stage 2 could
OOKP patients require life-long follow up. After be treated by through the lid conversion, pulling the
surgery, they need to be seen weekly during the upper lid down as biological cover and suturing
first month, then monthly for 3 months, then every upper and lower lid margins after removing tarsus
3–4 months. The IOP is checked by digital palpation. and deepithelialization of the ulcerated BM
The buccal mucous must be evaluated for signs of graft. Orbital decompression has been described
ischaemia, thinning, ulceration and necrosis. The for retroplacement of globe to improve exposure
lamina should be palpated with a cotton tip for its and cosmesis [12].

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The osteo-odonto-keratoprosthesis Zarei-Ghanavati et al.

Glaucoma is an important cause of visual loss in complications. Retinal detachment repair was suc-
anatomically successful OOKP eyes. Many patients cessful in only 20% of patients [19]. Endophthalmitis
have pre-existing glaucoma that is difficult to detect may present with atypical signs and symptoms.
before surgery. Falcinelli et al. reported that the overall Patient education is important for early presentation
prevalence of pre-existing glaucoma was 36.4% of retinal detachment and endoophthalmitis where
among 181 patients [1]. In our unpublished results, time is of the essence. Any acute symptom of pain or
the prevalence of de novo glaucoma and pre-existing decreased vision should be promptly evaluated to
glaucoma was 15% and 40%, respectively [4]. Perform- exclude endophthalmitis. Vitreoretinal surgery can
ing an intra-capsular lens extraction may decrease the be carried out with the aid of a temporary Ekhardt
incidence of glaucoma. After OOKP implantation keratoprosthesis, wide-angle viewing systems or
measurement of IOP is not possible with existing endoscopic vitrectomy.
technology, hence it is estimated by digital palpation. Loss of laminar volume and integrity are import-
However, monitoring of optic disc changes can be ant causes of anatomical failure of OOKP. The inci-
performed with photography, optical coherence tom- dence of laminar resorption is variably reported from
ography and Heidelberg retina tomograph. Visual 2% to 28% [1,7]. But, it is under reported since the
fields can be tested with standard perimetry such as clinical detection of resorption is often a late mani-
Goldmann and Humphrey’s analysers. Falcinelli festation. However, in the majority of the cases it is
showed that visual evoked potential is more accurate slowly progressive and compatible with laminar sur-
than electroretinogram, contrast sensitivity and auto- vival. Detection of bone resorption is difficult
mated threshold perimetry for detecting glaucoma because the process is gradual and the lamella is
after OOKP surgery [13]. Recent use of IOP sensor with hidden beneath oral mucosal membrane. Resorption
the Boston KPro brings hope for its similar use in can cause global or focal thinning of the lamina,
OOKP eyes that will aid in accurate measurement which may give telltale signs of altered refraction,
and monitoring [14]. Topical antiglaucoma drops tilting of the optical cylinder, aqueous leak and
are not usually effective after OOKP surgery because endophthalmitis.
drops are not absorbed through thick oral mucosal Imaging of the lamina is usually done with
graft; hence, medical treatment is mainly with oral multi-detector CT (computerized tomography)
acetazolamide and/or sublingual timolol [15]. Patients scan, which is indicated for confirmation of resorp-
allergic to sulfa drugs can take an off-label oral beta- tion as well as monitoring of laminar status.
blocker instead. Surgical procedures to reduce IOP Changes to linear dimensions and volumetric data
include cyclodiastasis, endoscopic cyclophotocoagu- of the lamina obtained by serial CT scanning can be
lation, disinsertion and reinsertion of recti, and tube compared to estimate laminar resorption [20]. Serial
shunts (Ahmed and Baerveldt). Endoscopic cyclopho- surveillance of the lamina with CT scanning is not
tocoagulation has a high incidence of complications indicated in every patient because of hazards of
like vitreous haemorrhage and visual loss [16]. Recti radiation and clinically may not be justifiable. We
disinsertion and reinsertion can only provide limited have observed that a clinically and radiologically
decrease of IOP for a limited time of several months. satisfactory lamina could still prove to be
Tubes are the commonly inserted after the OOKP to inadequate because of undetected fracture or chan-
reduce the IOP (Fig. 2b). Nevertheless, the results are nels allowing ingress of infection (Fig. 2c).
less efficient in OOKP eyes due to unpredictable encap- Augilar et al. [21] invented autoclavable micro-
sulation, and perhaps, because of the obliteration of milling instrument to fashion the lamina under fluid
the episcleral vessels. Additionally, tube surgery is to eliminate thermal and desiccation damage. Bone
challenging in the presence of the OOKP lamina augmentation technique has been described to boost
and thick BM graft. On the other hand, tube exposure the labial side of the lamina by mandibular bone graft
is much less common and tube insertion easier due to a [22]. Although the efficacy is uncertain, bisphospho-
single chamber eye from which much vitreous had nate drugs can be prescribed systemically to slow down
been removed. the bone (and dentine) resorption process. In case of
Although endoscopy was proposed for assess- severe resorption, the lamina needs to be exchanged
&&
ment of optic disc and macula before stage 1 [17 ], with a new one. Recently, Iyer performed augmenta-
it would only be useful in a limited number of can- tion of the bony part of the lamina by applying bone
didates with unequivocal visual potential [18] Vitre- morphogenetic protein and obtained encouraging
&&
ous haemorrhage and choroidal detachment are results in cases of laminar resorption [23 ].
usually self-resolving complications in early postop- Fabrication of synthetic OOKP laminae reduces
&&
erative period. Lim et al. [17 ] reported that up to one oral morbidity, surgical steps and costs and may
third of their OOKP patients needed vitreoretinal eliminate laminar resorption. We have investigated
surgery for either diagnosis or treatment of retinal a hydrogel-based material as a substrate for synthetic

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Corneal and external disorders

7. de la Paz MF, De Toledo JÁ, Charoenrook V, et al. Impact of clinical factors on


lamina. In in-vitro tests, the material is found to the long-term functional and anatomic outcomes of osteo-odonto-kerato-
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requirement of oral procedure and multidisciplinary && overgrowth in OOKP eyes. Cornea 2014; 33:981–984.
This study showed the effective treatment of mucous membrane overgrowth and
team involvement, the long term anatomical suc- also discussed the causes and prevention of this complication.
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Financial support and sponsorship 17. Lim LS, Ang CL, Wong E, et al. Vitreoretinal complications and vitreoretinal
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None. &&

157:349–354.
The vitreoretinal complications in OOKP patients need meticulous
approaches. In this study, the outcomes and techniques for their management
Conflicts of interest is discussed.
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