Ker

SURVEY OF OPHTHALMOLOGY
VOLUME 54 NUMBER 2 MARCHAPRIL 2009
MAJOR REVIEW
Pediatric Keratoplasty
M. Vanathi, MD,1 Anita Panda, MD, FICS, FAMS, MRCOphth,1 Sujith Vengayil, MD,1
Zia Chaudhuri, MS, FRCS,2 and Tanuj Dada, MD1
1
Cornea and Refractive Surgery Services, Dr Rajendra Prasad Centre for Ophthalmic Sciences,
All India Institute of Medical Sciences, New Delhi, India; and 2Pediatric Ophthalmology Services,
Maulana Azad Medical College, New Delhi, India
Abstract. Penetrating keratoplasty in children is a highly challenging and demanding procedure

associated with a high risk of graft failure or failure of amblyopia therapy in clear grafts. Nonetheless,
keratoplasty remains the surgery of choice for the management of pediatric corneal stromal opacities
or edema. Allograft rejection, graft infection, corneal neovascularization, glaucoma, trauma to the
anterior segment, vitreous pathology, and additional surgical interventions, especially those related to
glaucoma management, are important risk factors. Successful penetrating keratoplasty in children
requires careful preoperative evaluation and selection of patients follow-up by well-motivated parents,
an expert corneal transplant surgeon, and a devoted pediatric ophthalmologist. (Surv Ophthalmol
54:245--271, 2009. 2009 Elsevier Inc. All rights reserved.)
Key words. acquired non-traumatic opacity acquired traumatic opacity anterior segment
dysgenesis congenital corneal opacity congenital glaucoma congenital hereditary
endothelial dystrophy corneal transplantation endothelial rejection keratoplasty in
children pediatric keratoplasty Peters anomaly
I. Introduction
in pediatric patients with bilateral corneal involvement.159 Technical advances, however, have now
lowered the age at which keratoplasty is performed
and indications have increased with improvement in
surgical techniques and therapies.
Special problems in corneal transplantation in
children include difficult preoperative evaluation;
intraoperative problems such as low scleral rigidity,
increased fibrin reaction, and positive vitreous
pressure; the need for frequent examinations under
anesthesia for postoperative follow-up evaluations;
frequent loosening of sutures necessitating replacement/early removal; increased risk of rejection and
infections; and the difficulties with repeated refractive error assessments, and reversal of amblyopia.
Congenital eye disorders, although infrequent, are

important causes of childhood blindness. Visual
deprivation due to corneal opacification can lead to
long-term changes in the central nervous system.161
In order to achieve optimal visual results and avoid
visual-deprivation amblyopia, corneal transplantation must be performed in the early months of life.
Penetrating keratoplasty in children has a higher
rate of graft failure and a poorer visual prognosis
than adult keratoplasty. Improved understanding of
intraoperative and postoperative problems has
resulted in more successful pediatric corneal grafts.
Pediatric keratoplasty was performed infrequently
prior to the mid 1970s18 and was recommended only
245
2009 by Elsevier Inc.
All rights reserved.
0039-6257/09/$--see front matter

doi:10.1016/j.survophthal.2008.12.011
246
Surv Ophthalmol 54 (2) March--April 2009
Even with increased anatomic success of pediatric corneal grafts, visual rehabilitation remains
a concern.
In developing nations an increasing number of
grafts are performed for infectious keratitis, postinfectious keratitis corneo-iridic scars, or keratomalacia. Measures to maintain clear graft and successful visual rehabilitation following keratoplastyare
required to achieve a successful anatomical and
functional outcome. This review of corneal grafting
in infants and children evaluates the various
indications, as well as factors affecting graft prognosis, technique, special problems, and outcome of
pediatric keratoplasty.
II. Indications
Pediatric corneal opacities have been classified
into three categories:133,134 congenital, traumatic,
and acquired non-traumatic. Indications for pediatric corneal transplantation (Table 1) vary widely; the
proportion of keratoplasties performed for congenital indications range from 14--64%, for acquired
non-traumatic conditions they range from 19--80%,
and for acquired traumatic conditions they range
from 6--29%.1,31--34,95,134 Al-Ghamdi et al5 propose
a newer classification that takes into account visual
prognosis in pediatric keratoplasty:
A. Congenital opacities--Congenital hereditary
endothelial dystrophy (CHED)
B. Congenital opacities--Non-CHED
B1. Frequently associated with glaucoma
B2. Infrequently associated with glaucoma
TABLE 1
Indications for Pediatric Keratoplasty

A. Congenital opacities: Congenital hereditary endothelial dystrophy
B. Congenital opacities: Non-CHED
1. With associated glaucoma
a. Congenital glaucoma
b. Peters anomaly
c. Other anterior segment dysgenesis
2. Without glaucoma
a. Sclerocornea
b. Dermoid
c. birth trauma
d. Metabolic diseases
e. Keloid
f. Aniridia
C. Acquired traumatic
D. Acquired non-traumatic
a. Keratoconus
b. Infectious keratitis with or without perforation
c. Postinfectious corneal/corneo-iridic scars
d. Keratomalacia
VANATHI ET AL
Developmental influences affecting anterior segment differentiation between the 6th to 16th week of
gestation result in congenital corneal opacities.110,162
These influences may be genetic, infectious, traumatic, toxic or a combination of these factors. The
prevalence of congenital corneal opacities is approximately 3/100,000. With congenital glaucoma
included this rises to 6/100,000.92,142 The most
common primary cause of congenital corneal abnormalities in the developed nations is Peters anomaly
(40.3%), followed by sclerocornea (18.1%), dermoid
(15.3%), congenital glaucoma (6.9%), microphthalmia (4.2%), birth trauma, and metabolic disease
(2.8%).110
A. CONGENITAL OPACITIES: CHED
CHED presents as bilaterally symmetrical diffuse

corneal opacification and edema of varying
degree.39,74,99,158 The stromal opacity is thought to
result from terminal misdifferentiation of the
endothelial cells. Corneal clouding in the autosomal
recessive type of congenital hereditary endothelial
dystrophy is present at birth or within the neonatal
period. Nystagmus is often present, and there are no
other signs or symptoms. The autosomal recessive
form is more prevalent in countries where consanguineous marriage is frequent.6,115 Patients with the
autosomal dominant type of endothelial dystrophy
usually have clear corneas early in life, with corneal
opacification being slowly progressive and nystagmus infrequent. Photophobia and epiphora, may be
the first indications of the dystrophy. As the opacity
develops later in the dominant type, infantile or
autosomal dominant hereditary endothelial dystrophy have been considered to be more appropriate
names for this variant.64
CHED may be associated with congenital glaucoma.
Deafness may accompany the autosomal dominant
type. Corneal sensitivity is usually normal. On slit-lamp
evaluation, Descemets membrane is thickened and,
on retroillumination, has a beaten copper appearance. Increase in thickness of Bowmans layer has also
been reported.75 Widespread edema with hyaline
degeneration, superficial vascularization, scarring,
and calcium deposits may occur.99 Band keratopathy
and spheroidal degeneration have also been reported
in some cases of CHED.115 The most important
pathologic finding is the increased thickness of the
non-banded portion of the Descemets membrane.
The autosomal dominant (AD) form of CHED has
been mapped to the pericentromeric region of
chromosome 20.26 Mutations in the SLC4A11 gene
cause autosomal recessive CHED.65,77
247
PEDIATRIC KERATOPLASTY
The primary defect in patients with CHED is

a degenerated and dysfunctional corneal endothelium, characterized by an increased permeability
and an abnormal and accelerated Descemets
membrane secretion.9,70 The underlying pathophysiological mechanism may be related to an abnormal
endothelial barrier function, leading to secondary
swelling of the stroma and epithelium. Histopathologic examination shows an absence of the endothelial cell layer with presence of a variably thick
collagenous layer posterior to the anterior banded
zone of Descemets membrane.
CHED is not generally thought to be associated
with other ocular abnormalities. An ultrasonographic study of 20 eyes (10 patients) with CHED
showed ocular enlargement similar to that occurring in uncomplicated axial myopia with an inverse
relationship between the degree of enlargement
and the visual acuity or visual result following
penetrating keratoplasty. This suggests that infantile
corneal edema sufficient to cause stimulus deprivation may result in abnormal enlargement of the
globe.143
The dystrophy may be misdiagnosed as congenital
glaucoma. Birth trauma, mucopolysaccharidosis,
and intrauterine infections should also be considered in the differential diagnosis. Visual acuity may
be surprisingly better than the clinical appearance
of the eyes,117,131 but CHED can lead to ambylopia.
Penetrating keratoplasty in congenital hereditary
endothelial dystrophy is moderately successful, and
graft survival and visual outcome is better in cases
with delayed onset.
of the Descemets membrane. Abnormalities of crest

cell migration, proliferation, and differentiation
contribute to disorders of the corneal stroma,
endothelium, trabecular meshwork, and iris. The
coexistence of different anterior segment anomalies
has been termed combined mesenchymal dysgenesis.100
Concurrent management of glaucoma and corneal
opacification is sometimes required. CHED should
be suspected if persistent and total corneal opacification fails to resolve after normalization of
IOP.89,106 Corneal decompensation due to congenital glaucoma is a rare indication for corneal
transplantation in childhood13,18,31,42,44,46,134,161
and usually confers a poor prognosis.
Preoperative control of intraocular pressure is
required before penetrating keratoplasty. Cyclodestructive procedures for control of pharmacologically
resistant elevated intraocular pressure (IOP)46 may
be required to reducing the size of eyes with
buphthalmos before performing keratoplasty. Mitomycin C trabeculectomy and glaucoma drainage
implant surgery are other options in treatment of
postkeratoplasty glaucoma. These penetrating surgeries may predispose to graft failure by disruption of
blood--aqueous barriers. The simultaneous placement of glaucoma-filtering implant at the time of
primary penetrating keratoplasty has been described.15 Reports of corneal grafting for congenital
glaucoma after treatment with cyclophotocoagulation46 and glaucoma filtering implants have appeared
infrequently.5
B. CONGENITAL OPACITIES: NON-CHED
Peters anomaly is one of the most common

congenital corneal opacification encountered in
corneal practice. It is bilateral in approximately
80% of the cases. This congenital malformation of
the anterior segment is characterized by a central
corneal opacity with corresponding defects in the
posterior stroma, Descemets membrane, and endothelium. Iris strands typically arise from the collarette and extend to the periphery of the corneal
leucoma. The peripheral cornea is usually relatively
clear. Peters anomaly is associated with a wide range
of congenital ocular and systemic abnormalities and
commonly occurs as a sporadic disorder. A few cases
have autosomal recessive and autosomal dominant
inheritance.36,110 The co-existing glaucoma can
complicate the graft and visual prognosis in the
management of these cases.
The extent of ocular involvement varies from mild
to severe.149 Mild disease is defined by the presence
of normal iris and lens. Moderate disease is defined
by the presence of central iridocorneal adhesions
(anterior synechiae), or other iris defects such as
The non-CHED congenital corneal opacities can

be subdivided into those that are frequently
associated with glaucoma and those that are
infrequently associated with glaucoma.
1. Frequently Associated with Glaucoma
a. Congenital Glaucoma
The causes of congenital corneal opacification
associated with glaucoma include congenital glaucoma, Peters anomaly with glaucoma, and CHED
with glaucoma. CHED with congenital glaucoma
occurs infrequently.89,100 The combination of
CHED or Peters anomaly with congenital glaucoma100 originates from defects in the neural crest
cell contribution with abnormal neural crest cell
migration, resulting in congenital glaucoma and
neural crest cell differentiation, which results in
CHED.16 Histopathological evaluation reveals
absence of the endothelial layer with variably
thickened collagenous posterior nonbanded zone
b. Peters Anomaly
248
atrophy, abnormal vasculature, or coloboma. Severe

disease is defined by the presence of corneolenticular adhesion, or by the presence of corneal
staphyloma with or without corneal adhesions.167
Peters anomaly has been associated with congenital anterior and posterior segment anomalies as
well as systemic abnormalities.165 Glaucoma is the
most common of the ocular anomalies, observed in
30--70% of eyes, and is considered the most difficult
to control among all the childhood glaucoma types.
Peters anomaly patients are at risk for developing
glaucoma even if they present without glaucoma at
the outset. Eyes presenting with glaucoma usually
are found to have the lens cataractous with or
without adherence to the posterior cornea. Microphthalmia occurs in 25--50% of the eyes. Iris
abnormalities, chorioretinal coloboma, staphyloma,
retinal dysplasia, cataract, ptosis, persistent hyperplasic primary vitreous, optic nerve hypoplasia,
foveal hypoplasia, macular pigment epitheliopathy,
and colobomas are sometime associated. Systemic
anomalies, seen in 60% of the patients, include
developmental delay, central nervous system defects,
craniofacial abnormalities, microcephaly, seizure
disorder, fetal alcohol syndrome, and autism.
Congenital cardiac malformations, skeletal deformities, genitourinary malformations, ear defects, as
well as cleft lip and palate may also be present.
Histopathological evaluations132 indicate a central
absence of Bowmans membrane and iris synechia to
the periphery of the central corneal leucoma.
Extensive keratolenticular adhesions with retrocorneal fibrous tissue fill the central defect of endothelium and Descemets membrane, suggesting
a late anterior displacement of the normally
developed lens leading to a secondary endothelial
degeneration. Attenuated endothelium and abnormally composed Descemets membrane indicates
primary dysgenesis of the endothelium. Extensive
defects of posterior stroma with anterior stromal
disorganization and endothelial metaplasia suggest
dysgenesis of both the keratocytic and endothelial
mesoderm. Although a unified pathogenic mechanism is not consistently applicable, either primary or
secondary dysgenesis of the corneal mesoderm may
be responsible for the occurrence of Peters
anomaly.
c. Other Mesenchymal Dysgenesis
The terms mesenchymal dysgenesis and anterior
chamber cleavage syndrome refer to a spectrum of
congenital ocular disorders ranging from posterior
embryotoxon in its simplest form to Peters anomaly
at its most complex. Other conditions that are
included in this spectrum of congenital ocular
disorders are Axenfeld anomaly, Reiger anomaly,
VANATHI ET AL
and syndrome and posterior keratoconus.157 All

patients with the Axenfeld-Reiger (A-R) syndrome,
irrespective of their ocular manifestations, share the
same general features: a bilateral developmental
disorder of the eyes, a frequent family history, no sex
predilection, frequent systemic developmental
defects, and a high incidence of associated glaucoma. The age at which A-R syndrome is diagnosed
varies from birth to late childhood, most commonly
early infancy or childhood. Clinical features include
peripheral anterior segment anomalies and iridocorneal abnormalities with or without other associated ocular and systemic anomalies.
2. Infrequently Associated with Glaucoma
a. Dermoids
Dermoids are classified as choristomas, and the
opacification is usually peripheral. They present as
round or oval, whitish or yellowish cones protruding
on the anterior surface of the eyeball. They consist
of ectodermal (keratinized epithelium, hairs, sebaceous and sudoriferous glands, nerves, smooth
muscles, and, less frequently, teeth) and mesodermal elements (fibrous tissue, fat, blood vessels, and
cartilage) combined in different proportion.124
Indications for surgery are astigmatism and cosmesis. Ultrasound biomicroscopy (UBM) evaluation
can be helpful in determining the depth of
dermoids. Most cases may require simple excision
with or without amniotic membrane or only lamellar
keratoplasty.
b. Metabolic Causes
Most metabolic causes are of autosomal recessive
inheritance. The corneas are usually clear at birth,
followed by progressive opacification. Corneal clouding may be a part of many metabolic disorders,
including those involving amino acids, lipids, carbohydrates, purines, and so forth. Systemic mucopolysaccharidoses (MPS) are lysosomal storage disorders
that affect the glycosaminoglycan catabolism. Seven
types of MPS have been described, based on the
enzymes affected and the clinical manifestations.
Because cornea contains glycosaminoglycans, corneal
clouding is a part of many of these MPS syndromes.
MPS I-H (Hurler), MPS I-S (Scheie), MPS- IV
(Morquio), MPS VI (Maroteaux-Lamy) and MPS VIII
(Sly) are associated with variable amounts of corneal
clouding. Hurlers syndrome and Scheies syndrome
share the deficiency of same enzyme (alpha-iduronidase). The corneal clouding in the former is
more diffuse and central, whereas the latter has
peripheral clouding progressing centrally with
age.118 Open-angle glaucoma may occur in
both.105,130 Intelligence, stature, and lifespan are
249
increased in Scheies syndrome compared to Hurlers

syndrome. Pigmentary retinopathy and optic atrophy
are complicate the visual prognosis in these
syndromes.43
Morquio syndrome and Maroteaux-Lamy syndrome
presents with marked dwarfism, chest wall deformities,
cardiovascular abnormalities, and corneal clouding.
There is diffuse ground glass corneal stromal opacification in Morquio syndrome155 and is of varying
severity in Maroteaux-Lamy syndrome. The more
recently described MPS VIII (Sly syndrome) also has
corneal clouding (mild to severe), papilledema, and
retinal pigmentary degeneration.43
c. Sclerocornea
Sclerocornea is a primary, nonprogressive anomaly
in which scleralization of part or all of the cornea
occurs. In the peripheral type of sclerocornea, the
affected area is vascularized with peripheral arcades of
superficial scleral vessels. The corneas in sclerocornea
may be smaller in diameter with diffuse anterior
stromal opacification and may be associated focal
nebular densities and extensive superficial
vascularization.
Sclerocornea can present either as a primary anomaly or in association with cornea plana17,40,72 and has
been reported to occur in isolation or with associated
ocular and systemic anomalies.48,55,60,81,88,113,116,138
Histopathologically, vascularized collagenous tissue
occupies the anterior one-fourth of the corneal
stroma, with bundles of collagen fibrils 75--90 nm in
diameter. Descemets membrane shows abnormal
anterior lamination.102 The results of keratoplasty in
sclerocornea have not been encouraging.37,52,163,172
Frucht-Pery45 observed reduced nystagmus excursions
and attainment of reading vision in two children with
bilateral sclerocornea after unilateral corneal transplants at the ages of 4.5 and 16 years, respectively.
d. Birth Trauma
Birth trauma caused by forceps blade placement
across the orbit and globe during delivery can result
in blunt trauma and rupture of Descemets membrane. Descemets tears in birth trauma are usually
central and unilateral in a vertical or oblique
pattern. Diffuse stromal and epithelial edema due
to birth trauma usually clears within weeks or
months. The residual high astigmatism necessitates
penetrating keratoplasty when contact lens wear is
not possible.11
e. Corneal Keloid
Congenital corneal keloids are a rare entity that
present as white glistening benign protuberant
masses, appearing as single, solitary nodules or
involving the entire corneal stroma. Keloids occur

more frequently in males than females. Congenital
keloids usually occur in the presence of other ocular
anomalies,150 including peripheral iridocorneal
adhesions, anterior segment mesenchymal dysgenesis, aniridia, and cataract with subluxated lenses.
Corneal keloids also have been described in
children with Lowes syndrome, Rubinstein Taybi
syndrome, and fibrodysplasia ossificans progressive.
Corneal keloid may mimic a limbal dermoid.
Congenital keloids have been attributed to
undifferentiated hyperplasia of opaque corneal
and scleral tissue. Corneal keloid in Lowes syndrome is major cause of visual disability in children
over the age of 6 or 7 years in whom glaucoma and
cataract have treated surgically. In cases of Lowes
syndrome, it has been suggested that amino acids
filter into the cornea from abnormal vessels or that
substances from within the anterior chamber go
through a defective endothelium. Histopathologically, keloids are characterized by a haphazard
arrangement of fibroblasts, collagen bundles, and
blood vessels.
f. Aniridia
Absence of iris tissue is the sentinel finding, but
additional ocular structures are often affected.
Mutations of the Pax 6 gene have been identified
also in families affected by aniridia. Corneal lesions
in aniridia include peripheral pannus and epithelial
abnormalities that may advance centrally, resulting
in the need for penetrating keratoplasty.79 Aniridic
keratopathy remains a significant cause for visual
loss. Poor vision in aniridic eyes may be the result of
macular hypoplasia, nystagmus, amblyopia, cataracts, glaucoma, and corneal disease, termed aniridic
keratopathy. Congenital aniridia rarely requires keratoplasty at an early age except when associated with
significant opacification or with glaucoma leading
to corneal decompensation. Penetrating keratoplasty alone is not adequate treatment for severe
stromal scarring in aniridia, as it does not treat the
underlying epithelial disease, which requries limbal
stem cell transplantation.57,76,82,139
g. Posterior Polymorphous Dystrophy (PPMD)
PPMD56 is autosomal dominant in inheritance
with high penetrance. It is a bilateral disease, but
may be asymmetrical. Although it typically occurs in
the second or third decade of life, it may also be
congenital or develop early in life. It may be seen in
Alport syndrome. The corneal lesions in PPMD are
the level of the Descemets membrane and endothelium and may be vesicle-like, bands, or diffuse
opacities. The vesicular lesions that are the hallmark
250
of PPMD are seen as a transparent cyst with

a surrounding gray halo at the level of the
Descemets membrane and the endothelium,
appearing in the entire cornea or as isolated lesions.
Sinous brad bands and gray thickened Descemets
membrane may be seen. Endothelial guttae can also
be seen in PPMD. These may produce corneal
edema ranging from severe stromal edema to
bullous keratopathy.
Peripheral anterior synechia, raised intraocular
pressure, corectopia, calcific band keratopathy,
hydroxyapatite deposition in the corneal stroma,
and posterior keratoconus occur in PPMD. PPMD
may be confused with iridocorneal endothelial
syndrome (ICE), but the sporadic nature and
unilateral involvement distinguishes ICE. The characteristic pathologic changes in PPMD are the
appearance of epithelial like cells on the posterior
corneal surface.
C. ACQUIRED TRAUMATIC
Penetrating injuries remain an important cause of

acquired corneal scarring in the pediatric age
group.159 Acquired traumatic corneal scars are the
indication for 8--26% of penetrating keratoplasties
in children.5,20,32,33,95 The most important issue to
be considered in the timing of keratoplasty for
penetrating trauma is the threat of amblyopia.
D. ACQUIRED NON-TRAUMATIC
One of the most common causes of acquired

corneal scarring in children under the age of 6 years
is Herpes simplex keratitis.159 In developing nations,
other infectious keratitides remain the main
indications for pediatric keratoplasty.1,32,123,146--148
Penetrating keratoplasty for post keratomalacia
corneal melts are also common.21,126,148
Keratomalacia from vitamin A deficiency as an
important cause of preventable corneal opacification has a reported incidence varying between 8%
and 27.3%71,108,109,128 and remains a major cause of
pediatric ocular morbidity and severe visual impairment in developing countries.108,109,127 Ocular
surface changes include xerosis, keratinised plaques, stromal punched out ulcers, and focal or
diffuse stromal melting.127 Malnutrition, systemic
diseases, and lack of immunization predispose to
keratomalacia. Acute corneal melting results from
the ocular pathological changes in severe vitamin A
deficiency.128
Acquired non-traumatic corneal opacities were
the major indication (71.32%) for pediatric keratoplasty in a tertiary care center in north India,32
which is in contrast to series from the western world.
Infectious keratitis (72.6%) and keratomalacia
VANATHI ET AL
(27.36%) constituted the major causes for keratoplasty. Fever, diarrhea, and malnutrition were the
commonly associated systemic presentations in the
acquired non-traumatic group in this large retrospective series. Poverty and low socioeconomic
status prevalent in developing nations predispose
those individuals to corneal infection and malnutrition.32,122,146,148 Keratomalacia in children is hastened by protein-caloric malnutrition precipitated
by childhood communicable diseases such as
measles.129
III. Technique
Pediatric keratoplasty was considered previously
to be contraindicated because of its technical
challenges due to a low scleral rigidity and forward
displacement of lens--iris diaphragm.13,47,103,115
Although pediatric keratoplasty is now considered
to be a safe and effective procedure, specific
problems do exist in the management of children
who undergo corneal transplantation.
A. PREOPERATIVE EVALUATION AND DECISIONMAKING
An examination under anesthesia (EUA), including A and B scans, is usually done prior to
penetrating keratoplasty. A portable hand-held slitlamp evaluation immediately preoperatively can
eliminate the need for another EUA. In cases with
posterior segment pathology, electrophysiological
tests, such as visual evoked responses and electroretinography, can help decide on the need to
proceed with surgery. The decision of surgery at
an earlier age will depend on the laterality of the
corneal condition and its severity, the risks of
general anesthesia for initial surgery and for
repeated postoperative EUAs, and the associated
systemic abnormalities and metabolic conditions.
The commitment of parents to the long-term care
of the child after surgery plays a crucial role. Hence,
proper counseling for the social, economic, and
psychological demands on them following surgery is
vital for a successive outcome in pediatric
keratoplasty.
Early penetrating keratoplasty for congenital
corneal opacity may prevent deprivation amblyopia.
However the increased risk of failure, especially in
neonatal and infant eyes, requires careful case
selection. Younger age at the time of surgery
appears to increase the risk of failure due to
difficulties
with
intraand
postoperative
management.
The decision regarding surgery in CHED cases is
difficult. Although the cornea appears hazy and no
251
red reflex is seen, these patients seem to see much

better than would be predicted. If the patient has
good fixation and the eyes are orthophoric, surgery
may be delayed. However, if fixation is lost and
nystagmus develops, penetrating keratoplasty
should be performed promptly. When the child
presents with nystagmus, some recommend performing keratoplasty in one eye with the hope of
achieving better visual acuity.115
In cases of Peters anomaly, the extent of ocular
involvement is to be graded preoperatively. The
association of central nervous system abnormalities
with in Peters anomaly, should prompt the treating
ophthalmologist to look for signs of central nervous
system problems (developmental delay, structural
defects, seizure disorders, fetal alcohol syndrome) as
these increase the difficulty of caring for the child. A
pediatric neurological examination and neuroimaging may be required in such cases.
The important issue considered in keratoplasty
for traumatic corneal scarring is the threat of
amblyopia. It has been recommended not to delay
keratoplasty for penetrating trauma in children
once the decision is made to perform the surgery.34
The optimal timing and sequence of penetrating
keratoplasty and glaucoma drainage implant surgery
in refractory congenital glaucoma patients who
require corneal and glaucoma surgery is still
unclear.8
The decision to perform regrafts is made in the
light of factors such as age, risk of glaucoma, and
risk of amblyopia. When graft failure occurs,
regrafting is required to visually rehabilitate the
child. The causes of the corneal graft failure
influence the outcome of the regraft. When the
risk of surgical complications and glaucoma outweigh the benefits of the surgery, it is wise to avoid
regrafting.
Lamellar keratoplasty should be considered in
order to obtain desired results in conditions such as
superficial scars or limbal dermoid because of the
significantly lower risk of rejection and avoidance of
intraocular surgery. Tectonic patch grafts,148,152
rotational keratoplasty,84,90 and optical iridectomy
may also be considered in selective cases of pediatric
corneal opacification as an effective alternative
management option to keratoplasty.
B. ANESTHESIA
When performing pediatric keratoplasty, it is

desirable to have an anesthesiologist with experience in pediatric anesthesia. It is imperative to be
able to maintain the optimal depth of anesthesia
throughout the entire procedure. Use of a nonpolarizing muscle relaxant with a peripheral nerve
stimulator can help eliminate the risk of movement

and contraction of the extraocular muscles.47
Hyperventilation of the patient can help reduce
intraocular pressure.7 A slightly higher positioning
of the head aids in lowering positive pressure.
C. PREPARATION OF GLOBE
Increased positive pressure during surgery is

a major problem in pediatric keratoplasty. After
anesthesia is induced, 20% intravenous mannitol
(0.5--1.5 g/kg body weight over a period of 20--30
minutes) and/or digital massage aid in achieving
optimal ocular hypotony during surgery. Some
surgeons prefer to use preoperative Honan balloon
and intravenous mannitol to reduce positive vitreous pressure and the possible risk of anterior
displacement of the lens--iris diaphragm. Preoperative mydriatics or miotics may be used depending on
the procedure. The surgical table should contain all
instruments necessary to deal with an unexpected
lens loss in the even of raised positive pressure. The
surgeon should ensure that the donor button has
been punched and is ready before anterior chamber
entry.
A lid speculum with or without Flieringa ring is
used. Care should be taken to ensure that the
speculum, surgeon, and assistant do not apply
pressure on the globe. A lateral canthotomy may
be considered. As pediatric eyes have an increased
scleral elasticity and decreased scleral rigidity, the
use of a Flieringa ring provides scleral support for
the immature and lax infant tissue, thereby preventing collapse of sclera after host trephination. The
Flieringa ring must be sutured with care in infants
because the needle can penetrate the thin sclera of
pediatric eyes and cause retinal tears. In addition,
unequal placement of the fixation sutures can result
in irregularity of the graft recipient bed and
considerable astigmatism.
D. TREPHINATION
The recipient cornea is trephined to 70--80%

depth by using disposable suction trephines. Corneal trephination is technically demanding in
younger patients because of the increased elasticity
of infant tissue.18,116 Anterior chamber entry is
made with an MVR blade through the incision, and
the chamber is filled with viscoelastic to protect the
lens and iris from injury. The incision is then
completed with scissors. The donor cornea is
punched out from the endothelial side, and a graft
host disparity of 0.2--0.5 mm is used by most corneal
surgeons. Rapid formation of peripheral anterior
synechias (PAS) and difficulty reforming the chamber are also important problems faced while
252
performing pediatric keratoplasty. The use of 100

U/ml of heparin solution to irrigate the anterior
chamber to prevent fibrin formation and subsequent synechia is preferred by some. The injection
of sodium hyaluronate into the angle after anterior
chamber is entered may decrease the risk of PAS
formation and secondary glaucoma. Performing
several peripheral iridectomies has been found to
be important in preventing synechia formation.
Hemorrhage in the anterior chamber should be
controlled and clots removed as fibrin remaining
can lead to formation of posterior synechia and PAS
formation. Although would closure is faster with
a running suture, interrupted single sutures for
keratoplasties is usually preferred in children as they
allow for an earlier suture removal to avoid suturerelated problems.
Oversized corneal grafts (1 mm) have been used
in keratoplasty in an attempt to increase the
morphologic success of corneal grafting in children.147 In a prospective, nonrandomized clinical
trial, 40 pediatric patients with unilateral or bilateral
corneal opacification of congenital or acquired
origin (post infectious keratitis corneal opacification, 25%; traumatic corneal scars, 20%; sclerocornea, 20%) underwent corneal grafting surgery with
donor corneal buttons oversized by 1 mm. At the
end of 1 year, 85% of grafts remained clear,
providing an adequate anterior chamber depth
(2.20 0.612 mm in the congenital group and
2.36 0.302 mm in the acquired group). Although
the authors concluded that oversizing donor corneal buttons achieves adequate anterior chamber
depth in pediatric cases and can help prevent
postkeratoplasty glaucoma, a longer follow-up will
be required to confirm these conclusions.
Larger grafts have been used in patients with
keratoconus and buphthalmos.31,140 Failed regrafts
in congenital glaucoma have been found to be
associated with smaller diameter of the graft. Transplanted endothelial cells migrate over graft--host
junction to the recipient rim; the fewer number of
transplanted endothelial cells in small-sized grafts
migrating over to the relatively larger sized recipient
rim in buphthalmos has been thought to be
responsible for graft failure. Toker et al140 therefore
recommend adjusting the graft size in each eye
before surgery. Surgery in buphthalmic eyes can be
complicated as the corneas are thinner.
VANATHI ET AL
required. If vitreous prolapse occurs, anterior

vitrectomy with an automated vitreous cutter must
be performed. Any vitreous strands adherent to the
wound or iris are removed to prevent vitreous
adhesive syndromes and PAS formation. Planned
additional procedures, such as lens extraction, IOL
implantation, synechiolysis, and anterior vitrectomy
may be performed as required. Performance of an
additional surgical procedure at the time keratoplasty is strongly associated with decreased graft
survival rate.1,8,31 Among the variables analyzed,
corneal ulceration, vitrectomy-lensectomy, persistent inflammation, posterior segment anomalies,
regrafts, and postoperative complications have been
found to be associated with poor visual outcome and
allograft survival.33,34
F. SIMULTANEOUS KERATOPLASTY WITH
GLAUCOMA FILTERING DEVICE IMPLANTATION
Upon completion of the keratoplasty, a limbalbased conjunctival flap is created in the superotemporal quadrant. The plate of the primed
drainage device is sutured to the sclera with 8.0
non-absorbable sutures, 8--10 mm posterior to the
limbus. The tube is cut to an appropriate length
with an anterior bevel and inserted into the anterior
chamber through a 23-gauge needle track and is
covered by a donor scleral patch. In patients
younger than 6 months with anteroposterior
diameter less than 22 mm, a pediatric-sized implant
is preferred.7 When cyclocryotherapy is necessary in
cases of refractory glaucoma requiring penetrating
keratoplasty, it is typically performed in two or three
quadrants with two to four spots in each quadrant.
Problems in pediatric keratoplasty can be subdivided into those arising in the preoperative,
intraoperative, and postoperative periods.18
Preoperative problems
1. Complete preoperative evaluation of the

corneal pathology is usually not possible.
2. Need for specialized investigations such as
ultrabiomicroscopic examination.
3. IOP evaluation usually not accurate in opaque
corneas.
4. Patient should be evaluated for systemic associations in cases of congenital corneal opacities.
Intraoperative problems
E. CONCOMITANT PROCEDURES
Loss of crystalline lens and vitreous at the time of

transplantation may occur despite rigorous preventive measures. When the posterior capsule is
intact, a thorough aspiration of cortical remains is
1. Small size of the palpebral fissure reduces the

working space available for manipulations.
2. Excessive lowering of the intraocular pressure
is to be avoided as severe hypotony prevents
optimal trephination of the recipient cornea.
253
3. Caution is to be exercised while performing

the scleral fixation due to the higher risk of
perforation as the sclera is thinner in pediatric
eyes.
4. Use of Flieringa rings with unequal placement
of fixation sutures may also result in increased
distortion resulting in difficulty while
suturing.
5. Need for performing associated procedures
such as lensectomy, anterior vitrectomy, glaucoma procedures, and so on, is high.
6. Increased positive pressure of vitreous with
forward shift of lens--iris diaphragm due to the
low scleral rigidity and increased elasticity of
pediatric eyes.
7. Increased difficulty in suturing and cheese
wiring due to the thin peripheral corneal
tissue in certain cases.
G. PEDIATRIC KERATOPROSTHESIS
Use of keratoprosthesis to treat pediatric corneal

opacity11,25,114 offers an alternative treatment option
for those eyes with poor prognosis for graft survival.
A retrospective review of 22 eyes of 17 pediatric
patients with a history of corneal opacification due
to primary congenital disease and/or previous failed
keratoplasty treated with keratoprosthesis surgery by
Aquavella et al11 explores the option of keratoprosthesis in pediatric patients. Over a mean follow-up
of 9.7 months (range 1--37 months), all 21 Boston
keratoprostheses were retained without dislocation
or extrusion. In the two cases with AlphaCor
implants, the keratoprosthesis was not retained
(spontaneous extrusion in one case and traumatic
dislocation in the other) and had to be replaced
with a Boston keratoprosthesis. The visual axis
remained clear in all cases, with five eyes having
undergone retroprosthetic membranes removal.
Reoperation for management of concurrent glaucoma (three eyes) or retinopathy (two eyes) was
sometimes required. Although the authors11 conclude that the Boston keratoprosthesis implantation
helps to restore a clear visual axis without extrusion
or rejection and may be an appropriate alternative
for the management of pediatric corneal opacity,
keratoprosthesis in pediatric cases should be considered only as the last resort. Keratoprosthesis may
offer the possibility of rapid visual rehabilitation due
to high optical quality, which enables early amblyopia treatment.
H. EPIKERATOPLASTY
Epikeratoplasty has been performed to provide

refractive correction of pediatric aphakia.
Epikeratoplasty in children is more successful if risk
factors, such as younger patient age, microcornea,

corneal endothelial cell dysfunction, mental
retardation, and combined cataract surgery, are
avoided.28 Complications such as residual refractive
error, epithelial defect, interface opacities, graft
vascularization and graft infection, graft necrosis,
graft haziness or opacification, and graft dehiscence
have made this procedure less desirable.
IV. Postoperative Management

Postoperative management of pediatric corneal
grafts demands dedicated follow-up evaluations
under anesthesia, monitoring of postoperative
medications for frequency alterations, appropriate
management of sutures, close watch for rejection,
frequent correction of refractive errors, initiation of
amblyopia therapy, and ensuring compliance to
long-term amblyopia therapy. Hence, the need to
emphasize on the biphasic approach of 1) maintaining a clear graft, and 2) reversing amblyopia, is
of paramount importance in the postoperative
management.
A. IMMEDIATE POSTOPERATIVE MANAGEMENT
Postoperative treatment regimen involves topical

corticosteroid along with antibiotics and lubricants.
Topical steroids are given more frequently in the
initial postoperative period and gradually tapered
and changed to less potent steroids such as
fluoromethalone in 3--6 months. Topical CsA 2%
when used in pediatric keratoplasty can help reduce
frequency and duration of postoperative topical
steroids. EUAs in the early postoperative period are
important in order to assess the graft status, assess
intraocular pressure, and initiate prompt treatment,
if required.
B. SUTURE REMOVAL
Loosening of sutures or vascularization requires

an urgent EUA. Frequent EUAs during the first 2
months after pediatric keratroplasty in children less
than 6 months of age is mandatory until all sutures
are removed and at monthly intervals for 6 months
and less frequently thereafter. Frequent EUAs are
also required to detect problems such as glaucoma
and retinal detachment.
All sutures are usually removed within 3 months
in children younger than 8 years and by 6 months in
older children. Different centers follow their own
set routines for suture removal in pediatric keratoplasty. An increased frequency of topical steroid and
antibiotics is required for a week after suture
removal. Suture loosening and graft rejection can
occur insidiously in young children who cannot
254
communicate discomfort and vision changes. Graft

rejection is thought to occur more quickly in
children due to an amplified wound healing
response.24
C. REFRACTIVE CORRECTION
Refraction is done after suture removal for optical

correction, and amblyopia therapy is initiated as
soon as possible. In cases of potential risk of dense
amblyopia, early refractive correction may be provisionally prescribed with frequent changes as
required in an attempt to increase the efficacy of
amblyopia therapy. Early refractive rehabilitation by
spectacle correction or contact lenses to correct
residual astigmatism and contact lenses or intraocular lens implants for aphakia are required.
D. AMBLYOPIA MANAGEMENT
The neurological basis of amblyopia is related to

the concept of cortical competition. Visual cortical
cells are potentially connected to both the eyes
equally, provided both eyes are functioning.49,59,135,153
If one eye predominates, these cortical cells are
stolen by the dominating side. The dominance of
one eye over the other is usually a result of better
visual acuity in that eye, especially if primary
strabismus is not present. It is postulated that
strabismic amblyopia is initiated as a maladaptive
differentiation in the ocular dominance columns,
whereas the non-strabismic amblyopias (anisometropic and the deprivation amblyopias) may be
initiated from the malfunctioning of the ganglion
cell population of the amblyopic eye.41,69,135 Thus
the non-strabismic amblyopias are caused by optical
degradation of one retinal image while in strabismic
amblyopias both retinal images are initially clear.
The total clinical picture is confusing because of
secondary changes in other parts of the central
nervous system that occurs subsequently. The
manifest features can be due to a slower, more
enduring type of change (pooling, loss, and
re-wiring of the neurons) as well as a more transient,
adaptive type of response (such as suppression of
diplopia). Thus, mechanisms leading to amblyopia
have been divided into two basic types, those
causing form deprivation and those resulting in
abnormal binocular interaction.86,120,135,136,153,154 It
is of importance to realize that the process of visual
maturation and development of amblyopia becomes
especially significant in the early period of visual
development, also called the critical period, when
neural plasticity makes the visual system vulnerable.
This may last is up to 7--8 years in
humans.69,135,153,154 Once this period is over amblyopia cannot occur. This is the time when amblyopia
VANATHI ET AL
therapy is maximally effective as the immature visual

system can be modulated.41,49,69,135,153,154
As the visual deprivation in amblyopia is more
related to the competitive interaction between both
the eyes rather than disuse in most cases, results of
treatment are better if started within the critical
period as this is the time when changes in the lateral
geniculate body and the visual cortex are partially or
completely reversible.135,153 The key for the management of amblyopia is equalization of visual acuity
in both the eyes so that they can function together.
The modalities include a high degree of suspicion of
the condition, early detection, observation of
associated abnormal eye movements in the form of
roving eye movements, nystagmus, abnormal head
postures, and so forth, removal of any media opacity,
correction of refractive errors, and providing the
worse eye a competitive advantage over the better
eye by occluding the better eye, either physically
with a patch or with the help of cycloplegic drugs.
Strict vigilance and monitoring of therapy is
important. Aggressive amblyopia management is
mandatory for good visual outcomes in pediatric
patients undergoing keratoplasty.29,33,34,135,153,171
Occlusion of the better eye by direct patching
forms the mainstay of the treatment for amblyopia.
A patch applied over the skin is preferred to a patch
over the spectacles as the child can easily take off the
spectacles or look outside through the sides of the
occluded spectacle. The principle of this therapy is
to provide a competitive advantage to the worse eye,
which will eliminate the components of abnormal
binocular interaction and the inhibitory influences
of the better eye on the receptive fields of the worse
eye. Occlusion should be started as soon as possible.
The family should be educated to recognize the
fixating eye and guide the patient toward free
alternation.19,85,101,104,111,112
E. REGRAFTS
Repeat penetrating keratoplasty is quite often

required in pediatric eyes as there is high chance of
failure of the primary graft. The graft survival rate is
lower in eyes undergoing multiple regrafts.151
Regrafts in congenital glaucoma tend to fail earlier
than primary grafts.2,140 In the heterogenous group
studied by Dana et al,33 20% of eyes had undergone
regrafting at a mean of 17 months after the first
surgery, of which 22% had a second regraft at
a mean of 62 months after the original procedure.
Anatomical success of the grafts dropped from 78%
in the eyes that had one graft, to 19% in those eyes
that had two grafts, and to nil in those eyes that had
three grafts.
255
Parmley et al94 also had a high rate of regrafts in

26 grafts on 16 eyes in 10 patients of which six eyes
that were grafted two or more times over a mean
follow-up of 30 months. Of the six eyes that were
regrafted, only one child obtained ambulatory
vision.4 Apart from the eight primary keratoplasties,
three repeat keratoplasties were required in the
series of Peters anomaly described by Althaus and
Sundmacher.10
The Kaplain-Meier survival curve showed a highly
significant difference in the likelihood of maintaining a clear graft with initial grafts compared with
second, third, and fourth grafts in the large series by
Yang et al.167 Thirty-six percent of first grafts
maintained long-term clarity compared with just
6% of second grafts. The probability of second or
subsequent grafts surviving for 3 years was less than
10%. Ten out of 26 transplants in the series of
Comer et al29 were regrafts, of which seven subsequently failed. Of 58 eyes (the majority of which
had Peters anomaly or sclerocornea) 23 eyes
required regrafting between 2 weeks and 110
months postoperatively.44 The probability of maintaining a clear graft, calculated by survival analysis,
was 75% (SE, 6%) at 1 year and 58% (7%) at 2
years. Rejection reversals tend to be more successful
in the primary grafts compared to that in regrafts.68
The increased need for regrafting, besides the high
incidence of complications in pediatric corneal
transplantation, calls for a cautious approach to
decision-making before attempting surgical intervention. Repeat grafts may still be indicated in
infants or children in the amblyogenic age group as
even if the regraft survives for only 1 year, this will
enhance the visual development of the child and
reduce the risk of amblyopia.47
V. Complications
Acquired corneal scars, later corneal decompensation in older children, and phakic eyes have the
best prognosis. Corneal perforations, active inflammation or infection, and infants with multiple
ocular anomalies have the poorest prognosis.
Children undergoing combined procedures have
been found to have a less favorable result than those
undergoing a single- or two-staged procedure.31
Complications such as cataract development, secondary glaucoma, epithelial defects, band keratopathy, retinal detachment, wound leakage,
retrocorneal membrane, and microbial keratitis
make the postoperative course complex often
necessitating regrafting.44 Preoperative vascularization of the cornea, persistent epithelial defects, and
performance of lensectomy-vitrectomy were factors
most highly correlated with poor graft survival.134

Postoperative shallowing of the anterior chamber
and the occurrence of anterior synechiae leading to
secondary glaucoma are other causes of graft failure
in the pediatric age group.147 Other factors limiting
visual outcome include glaucoma, hemorrhage, and
retinal complications.18
A. GRAFT REJECTION
Pediatric corneal transplantation has an increased

rejection rate because of the more active immune
system in younger patients.9 Endothelial immune
rejection165 leading to graft failure is one of the
main causes for graft failure. Well-established graft
rejection in children is usually irreversible.18
Increased risk of allograft rejection after bilateral
keratoplasty is controversial.9,12,38 Early intervention
may be considered in both eyes in an attempt to
provide useful vision and avoid irreversible amblyopia. In infants with an amplified inflammatory
response, graft rejection can occur rapidly and be
less responsive to treatment. Early symptoms of graft
rejection, such as reduced visual acuity and ocular
discomfort, cannot be communicated, resulting in
a delay in the diagnosis and treatment and, hence,
a higher degree of graft failure. The reported
percentages of graft rejection in pediatric keratroplasty vary between 22%146 and 43.4%.1,62 Graft
rejection was the cause for all graft failures in series
of pediatric keratoplasty in CHED reported by
Javadi et al62 in which rejection had occurred in
10 eyes (43.4%), of which endothelial rejection
accounted for 30.4% of eyes.
Yang et al observed graft rejection to be the most
frequent cause for graft failure in their series (25%),
with 48% of the rejection episodes involving the first
graft.167 Rejection was reversible in only 28% of
episodes, showing a much lower reversal rate in
pediatric grafts compared to that of 50--78% in adult
grafts.9 In the series by Comer et al,29 53% of the
rejection episodes were not reversible and resulted
in failure. Vajpayee et al146 also noted rejection in
22.5% of cases with 55% of them not being
reversible, and all of these children had reported
late for management. Seventeen of 19 grafts with
rejection failed in the series reported by Cowden.31
Stulting et al134 reported 11% of graft failures to be
related to allograft rejections; whereas more than
50% of the graft failures in the series by Dana et al33
were attributed to graft rejection. Although graft
rejection was the primary cause for graft failure in
their series, Dana et al 35 conclude that rejection is
not a significant predictor of failure as most of their
rejection episodes were successfully treated.
256
VANATHI ET AL
McClellan et al83 were successful in retaining clear

grafts with five out of six rejections.
Schonherr et al119 reported results of 71 keratoplasties in 66 eyes of 61 patients (15 lamellar
(homologous not human leucocyte antigen
[HLA]-matched) keratoplasties (11 autologous penetrating rotating, 42 penetrating (homologous not
HLA-matched), and 3 penetrating homologous
HLA-matched keratoplasty) with the main indications being traumatic scarring (22 eyes), corneal
dystrophy (13 eyes), scarring after keratitis (10
eyes), graft failure (7 eyes), and chemical burn (5
eyes). Even though 40 of 42 eyes after PK had a clear
graft, only 21 of the 42 eyes obtained a visual acuity
of 0.5 or better. Graft rejection was noted in 20% of
the homologous PKs. Sufficient thought must go
into evaluating tissue matching in PK.18
Topical cyclosporine (CsA) 2% is used four times
a day along with systemic steroids as a routine in PK
by some surgeons. It is then tapered over 3 months
to once a day. CsA is a potent immunomodulator
that affects early stages of antigenic sensitization and
subsequent proliferation of immunocompetent
cells. The available reports in literature on efficacy
and safety of topical CsA in PK are few. Cosar et al30
observed that the rejection-free graft survival rate in
their study was higher with use of topical CsA.
Although the difference in graft survival rates
between the CsA cases and control group was not
statistically significant, Cosar et al30 consider their
results to be impressive as use of topical CsA had
prolonged graft survival rates in the corneal grafts of
much younger children who have a high risk of graft
rejection. Prolonged use of topical corticosteroids is
associated with increased risk of cataract formation,
glaucoma, and delayed wound healing. Use of
topical CsA eliminates these risks of topical steroids.
Whereas steroids induce a general ocular immunosuppresion with an enhanced risk for secondary
infection, topical CsA, being a specific immunomodulator by nature of its action on T lymphocytes
only, does not affect the antimicrobial arm of the
immune system. Therefore there is less risk of graft
infection. Early suture removal can also be done
with use of topical CsA as this does not delay wound
healing, which is beneficial in pediatric corneal
grafts as the suture related problems can be
minimized.
limited.5,33,134 Reported incidences of graft infection vary from 10--50% in pediatric grafts.156
Graft survival prognosis becomes bleak after onset
of bacterial infection and hence calls for aggressive
preventive measures. Most pediatric graft infections
are attributed to suture-related causes. Completion
of suture removal as early as possible should be
considered. A close follow-up is required even after
removal of sutures, especially in eyes with glaucoma
or ocular surface disorders. Prolonged use of
antibiotics until all sutures have been removed
may decrease the risk of development of graft
infection. Non-compliance to follow-up, resulting
in the failure to recognize irritation due to loose
sutures, is the most important risk factor for graft
infection in developing countries. Lower socioeconomic status and long distance from the treating
referral center contributes to delay in diagnosis and
treatment.1 There is a higher prevalence of graft
infection in eyes with congenital corneal opacity
(especially in eyes with congenital glaucoma)
compared to acquired causes. The requirement to
perform multiple glaucoma and other surgical
diagnostic procedures may explain the increased
prevalence of graft infection in congenital glaucoma
eyes undergoing penetrating keratoplasty.
Wagoner et al156 report culture-positive bacterial
keratitis in 35 (17.3%) of their 202 primary pediatric
keratoplasties
with Gram-positive
organisms
accounting for infection in 91.4% cases and 77.6%
of isolates. Streptococcus pneumoniae is the most
common organism causing pediatric graft infection
in most studies. Final visual outcome is poor in
grafts affected by bacterial infection, with 65.7%
retaining a visual acuity of hand movement or less.
Infectious corneal ulcer resulted in graft failure in
six eyes (30%) of cases with loose sutures accounting
for infection in four eyes (67%). Wagoner et al156
also report a higher prevalence of bacterial keratitis
in eyes with congenital than in acquired opacities.
In eyes with endophthalmitis secondary to graft
infection, Haemophilus influenza and Streptococcus
pneumoniae have been isolated from the vitreous.8
Frequent postoperative examinations as long as
sutures are present will help in reducing risk of
infections due to neglected sutures. Prolonged use
of broad spectrum prophylactic antibiotic therapy
until all sutures removed can also help in reducing
suture-related complications.
B. GRAFT INFECTION
C. PERSISTENT EPITHELIAL DEFECT
Bacterial keratitis after primary penetrating keratoplasty in children is a serious complication

resulting in graft failure and poor visual outcome.
Data on pediatric corneal graft infections is
Persistent epithelial defects (PED) in pediatric

corneal grafts47,133,134 can result in graft failure.
PED resulting from poor graft host junction
apposition and faulty suturing, early suture
257
loosening, drug toxicity, tear, and surface abnormalities may predispose to graft infection. Prolonged
epithelialisation, subsequent to PED also lead to
significant graft haze compromising optical quality
of vision.
D. WOUND DEHISCENCE
Wound leak and dehiscence (2--10%)18,31,33,42

due to suboptimal suturing can lead to postoperative shallowing of the anterior chamber necessitating immediate postoperative suturing under
anesthesia. With improved surgical technique and
suture materials, postoperative wound leak and
dehiscence is now rarely seen.
E. CATARACT
The reported rates of cataract vary between 2%

and 7%,18,33,42 with as much as 18%166 in eyes with
multiple interventions.
F. ENDOPHTHALMITIS
The reported rate of endophthalmitis following

pediatric keratoplasty is about 2%.31,42,134 The
incidence of ocular infections (4--9%)31,42,134 following pediatric keratoplasty is higher in cases of
children undergoing multiple procedures, as in
glaucoma patients where there is a need for
multiple intraocular interventions.
VI. Outcome of Pediatric Keratoplasty

Comparison of graft survival outcomes among the
reported studies is difficult due to the heterogeneity
of the involved conditions, varying size of the study
group, and varying periods of follow-up (Table 2).
Poor results in corneal grafting in congenital,
central corneal opacities prompts surgeons to avoid
penetrating keratoplasty in patients with unilateral,
congenital corneal opacities.61,96,126,134,159,164 Irreversible amblyopia, glaucoma, other structural
abnormalities of the anterior segment, and mental
retardation further worsens visual rehabilitation in
the congenital corneal opacity cases.134 However,
encouraged by their good results, Frueh and
Brown44 advocated corneal grafting for congenital
opacities in infants prompting an early intervention
in unilateral as well as bilateral involvement.
Pediatric keratoplasty is associated with an excellent
prognosis for graft survival in eyes with CHED and
a fair prognosis for graft survival in eyes with nonCHED congenital opacities and acquired opacities.
However, even with increasingly better anatomical
success of corneal grafts in children, visual outcome
continues to remain less than satisfactory.
With results varying in different etiological
conditions for which corneal transplantation is
done in the children, it seems more logical to
analyze the outcomes in accordance to the
indications for which the grafts are performed.
A. CONGENITAL OPACITIES: CHED

G. GLAUCOMA
The incidence of post-penetrating keratoplasty

glaucoma has been found to be 5--9%.47 Yang et al
reported166 126 glaucoma procedures performed in
34 eyes children with Peters anomaly following
keratoplasty.
H. RETINAL DETACHMENT AND PHTHISIS
Other vitreoretinal complications are expulsive

choroidal hemorrhage (2--3%), retinal detachment
(3--5%), and phthisis (4--13%).47 Yang et al166
reported a relatively high rate of retinal detachments (35%) and phthisis (18%) in eyes with Peters
anomaly that underwent multiple intraocular procedures for IOP control and visual rehabilitation.
I. AMBLYOPIA
As already discussed amblyopia is the most

important factors for visual outcome in anatomically
successful grafts.
Graft survival rates in cases of CHED vary widely

with reported percentages ranging from 25% to
90% in most series.5,6,62,75,99,115,117Keratoplasty for
CHED has a higher degree of success when
compared with transplantation for other causes of
corneal opacification, as the pathology in CHED is
usually limited to the cornea only. When performed
early, the prognosis for improved visual acuity in
children appears to be good.6 Two series of CHED
have proposed delaying surgery in patients whenever possible,75,115 whereas a third series6 has
recommended early surgical intervention. The
series by Schaumberg et al117 dealt with only those
cases of CHED in which surgery was performed at
a young age (predominantly autosomal recessive
type). Although the threat of dense amblyopia in
untreated eyes and good surgical success rates in
very young children favor consideration of relatively
early surgical intervention in the most severely
affected cases, there seems to be evidence to support
delaying surgery in some cases.117 Consideration of
how differences in age of onset, severity of the
disease, and timing of surgery influence the
258
TABLE 2
Outcome of Pediatric Keratoplasty in Reported Major Studies
Study
Zaidman
2007170
Sharma
et al
2007122
No
of Eyes
Peters anomaly
5 mo
168 eyes
of 154
children
Acquired
nontraumatic53.4%
Congenital - 33.7%
Acquired
traumatic - 14%
Congenital
CO-78.8%
Traumatic - 10.9%
Acquired
nontraumatic 10.3%
Congenital CO
5.4 3.9 yr
86 grafts in
63 eyes
65 grafts in
58 eyes of
52 children
!14 yr
Mean
Follow-Up
78.9 mo
14.52 8.54 mo
50 mo
40.4 mo
Congenital CO - 16% Congenital

Acquired
CO - 3 yr
nontraumatic - 74% Acquired
Traumatic - 10%
nontraumatic
- 12.4 yr
Traumatic 10.8 yr
20 eyes of
Congenital
17 children
glaucoma
!14 mo
(simultaneous
AGV PK)
Congenital CO
McClellan et 19 grafts in
18 eyes of
Acquired
al 200383
16 children
!15 yr
Al Torbak
20048
Mean Age
at Surgery
38 eyes
Al Ghamadi 165 grafts

20075
in 134
children
!12 years
Michalli
200587
Patel et al
200598
Indications
11.7 mo
9.24 yr
--
30.8 ( 11.1) mo
6.6 years
Anatomical
Success
90%
-
Functional
Success
54%
(O20/200)
30.1%
(O20/200)
1 Year
2 Years
3 Years or
More
--
--
--
--
--
77%
44.2%
Graft Survival
78%
--
60% (O6/18)
35%
(35% had
ambulatory
vision)
--
Cong.
-CO - 14.4%
(O6/60)
--
43%
17%
at
4 yr
--
--
VANATHI ET AL
73.7%
(Congenital
CO 71.4%
Acquired
75%)
82%
-(Congenital
CO 78%
Nontraumatic
85%
Traumatic
100%)
24 eyes of
CHED
15 children
!12 yr
8.1 ( 2.5)yr
Comer et
al 200129
26 grafts in
Congenital CO
16 eyes of
11 children
154 grafts in
Congenital CO 140 children
30.5%
!14 yr
Traumatic - 14.2%
Nontraumatic
acquired - 55.1%
13 weeks
Aasuri et al
20001
Dada et al
415 grafts
199932
(!12 yr)
(study on
indications)
Yang et al
1999167
144 grafts
in 72eyes of
47children
!12 yr
6.5 yr
Congenital CO 12.28%
Acquired nontraumatic - 71.3%
Regrafts - 10.8%
Acquired traumatic 5.4%
Peters anomaly
4.4 mo
Schaumberg 21 grafts in
CHED
40 mo
et al
16 eyes of
1999117
9 children
!12 yr
47 grafts
Peters anomaly (83%) 7 mo
Dana
(36 eyes
&Mesenchymal
et al
of 29
dysgenesis
199735
children
!12 yr)
56 eyes of
CHED
11.8 yr
Al-Rajhi
40 children
and
Wagoner
19976
Freuh and
58 eyes
Sclerocornea 17/58 6.3 mo
Brown
Peters 17/58
199744
Partialsclerocornea
12/58
Congenital glaucoma
2/58
35.5 ( 36.2) mo
1.3 yr
--
11.1
yr
79.1%
94.7%
(* 19 eyes,
O20/120)
62.5%
68.75%
61%
(ambulatory
vision)
43.8%
(Congenital
-(* 121 eyes
CO - 63.8%
O20/400)
Traumatic 54.5%
Acquired
nontraumatic 70.6%)
----
66.2%
--
88%
88%
88% at
3 yr
74% at
5 yr
--
--
39%
-(35% primary grafts)
49%
70 mo
69%
40%
(* 10 eyes,
O20/200)
--
38 mo
61%
50%
(* 24 eyes,
O20/200)
79%
37 mo
62.5%
69.8%
(O20/300)
92%
72%
56.5% at
5 yr
75%
58%
--
40m
Overall 83%
Sclerocornea 70%,
Partial sclerocornea - 83%,
Peters anomaly
- 100%
Javadi et al
200362
44% at
3
yr
35% at
10 yr
71%
--
62%
259
(Continued)
260
TABLE 2 Continued
Graft Survival
Dana et al
199534
Dana et al
199533
No
of Eyes
Indications
25 grafts in
Ocular trauma
25 children
! 12 yr
Congenital
164 grafts
CO -64%
(131
Traumatic - 17%
eyes/108
Nontraumatic
children
acquired - 19%
!12 yr)
Sajjadi et al 37 eyes of
CHED
1995115
21 children
Ariyasu et al 9 grafts in 8 Congenital glaucoma
eyes of 6
199413
infants
Parmley
26 grafts in
Peters anomaly
199394
16 eyes
Erlich et al
199142
85 grafts in
54 patients
Mean Age
at Surgery
70 mo
(# 12 mo)
Mean
Follow-Up
42.5 mo
Anatomical
Success
-62%
9.5 yr
3 yr
92%
!2 yr
24 mo
67%
30 mo
15.3%
18 weeks at
time of 1st
graft
Peters (16/54, 27
Peters
anomalyPKs)
Congenitalglaucoma
12.3 mo
(8/54, 13 PKs)
Cong.
glc - 22.8m
HSK (5/54, 10 PKs)
C Dystrophy
HSK - 5.9
(8/54, 9 PKs)
yr
Trauma (17/54, 26
C Dystrophy PKs)
11.4 yr
Trauma - 6y
20.5 mo
83%
(* 18 eyes,
O20/200)
33%
(* 84 eyes,
(O20/200)
1 Year
2 Years
84%
72.9%
-(O20/200)
75%
-ambulatory
vision
19.2%
-(ambulatory
vision)
--
Congenital CO
- 80%
Traumatic 84%
Acquired
nontraumatic 76%
(overall
80%)
3 Years or
More
70%
--
67%
--
--
--
--
--
--
--
VANATHI ET AL
Overall 22%
(Peters
44% @ 22.3
mo,
Congenital
glaucoma
0% @16.6
mo
HSK 40% @
12.2 mo
Dystrophy
75% @
27.3 mo
Trauma 71%
@ 3 mo
Functional
Success
Study
66 grafts in
57 eyes of
50 children
!14 yr
Cong. CO
-Corneal
decompensation
Keratoconus 10
Regrafts 10
Stulting
1984134
152 grafts in
107 eyes of
91 children
!14 yr
Congenital CO
Acquired
traumatic
Acquired
nontraumatic
2 mo--10 yr
98 mo
(# 20 mo)
30.1 mo
--Overall 48.4% Cong. CO - 39%

Keratoconus 70%
Acquired
traumatic 88%
Acquired
non-traumatic
- 33%
Regrafts - 11%
Congenital
glaucoma 100%
-Cong. CO - 60%, Cong. CO Cong. CO Trauma - 70%
*29%,
60%
Acquired nonO20/400
Traumatic traumatic Traumatic 70%
73%
45%, O20/ Acquired
400
nontraumatic 73%
Acquired
nontraumatic 67%,
O20/400
--
Cowden
199031
--
AGV 5 Ahmed glaucoma valve; C glc 5 congenital glaucoma; CHED 5 congenital hereditary endothelial dystrophy; CO 5 corneal opacification; Cong. 5 congenital;
HSK 5 herpes simplex keratitis; mo 5 months; PK 5 pediatric keratoplasty; yr 5 years.
*
With measurable preoperative and postoperative visual acuity.
#
Mean interval between trauma and first PK.
261
262
outcome is important when comparing results of

different series.
Pearce et al99 reported a 25% survival of first
grafts and an overall success rate of 43% (including
five regrafts) after a 3-month follow-up. Kirkness et
als75 results of keratoplasty for CHED in 31 eyes of
20 patients (median age at first keratoplasty of 13.5
years) reported good surgical success. A retrospective analysis by Groh et al of penetrating keratoplasty
outcome in 13 eyes of 8 children of CHED with
a mean age of 6.0 years (range, 3--14 years) over
a mean follow-up of 4.0 years saw a good prognosis
for graft survival and visual outcome.53
Results of penetrating keratoplasty in 37 eyes of
21 patients with CHED of the autosomal recessive
type (mean age at surgery 9.5 years) showed good
visual and anatomical success.115 Al-Ghamdi et als5
study also found significantly higher graft survival in
eyes with CHED than for other surgical indications.
Review of the outcome of penetrating keratoplasty
in CHED patients6 showed that 62.5% of the
primary corneal transplants remained grafts at
a mean follow-up of 37 months with graft survival
analysis indicating better graft survival in eyes with
delayed onset of the disease, with use of younger age
donor corneas (between 5 and 30 years), and in
patients compliant to follow-up. Results of Schaumberg et al117 and Sajjadi et al115 are consistent with
those of Al-Rajhi et al.6
Javadi et al reviewed the results of corneal
transplants in 24 eyes of 15 patients of CHED
(mean age of 8.1 years at the time of the primary
graft)62 found no relationship between age at initial
PKP and final visual outcome. In view of the
difficulties in pediatric keratoplasty and the absence
of a relationship between postoperative visual outcome and age at keratoplasty, a risk:benefit ratio
evaluation and conservative approach can perhaps
be adopted in decision-making on the timing of
keratoplasty in patients with CHED.62 It is to be
pointed out that graft survival is significantly higher
at all postoperative intervals in eyes with CHED than
for other surgical indications.5
Reaching a consensus regarding the crucial factor
of timing of surgery in CHED seems to be difficult
due to the heterogenic nature of the study groups
involved. The mean age at diagnosis and mean age
at surgery vary in most of the series. The grade of
severity of disease also influences the decision of the
timing of surgery in patients in CHED apart from
the type of the CHED being dealt with. Visual
outcomes are also affected by the relative difficulty
in assessing visual acuity in infants and children. A
more severe grade of corneal opacification associated with nystagmus is associated with a denser
ambylopia, thereby further influencing the visual
VANATHI ET AL
outcomes compared to that of the autosomal

dominant type in which opacification is usually
milder, and not associated with nystagmus; in those
cases surgery is usually delayed. It can be unanimously agreed upon, however, that eyes with CHED
are significantly more likely to achieve ambulatory
vision or vision O20/200, than eyes with other
indications.5
1. CHED with Glaucoma
Congenital glaucoma and congenital hereditary
endothelial dystrophy may coexist with the the need
for subsequent keratoplasty for visual rehabilitation
in these cases.106
Mullaney et al89 performed keratoplasty in three
children (ranging in age from 2 to 6 months) with
diffuse and homogeneously opaque corneas after
the haze failed to improve following glaucoma
surgery and histopathology evaluation of the excised
corneal buttons showed findings consistent with
CHED.
B. CONGENITAL OPACITIES: NON-CHED
1. Frequently Associated with Glaucoma

a. Congenital Glaucoma
The efficacy of corneal transplantation in infants
with corneal opacity secondary to congenital glaucoma has not been clearly established as the
percentage of clear grafts in children with congenital glaucoma varies significantly due to the small
number of cases reported.13,168 The visual prognosis
following penetrating keratoplasty in congenital
glaucoma is generally poor.42,46,159
The Cowden et als results were encouraging in
seven cases of congenital glaucoma eyes with
corneal grafts when IOP was controlled before the
keratoplasty procedure.30 However, Erlich et al
found dismal results, with none of the 13 keratoplasties in eight patients doing well.42 Ariyasu et al13
reported results of nine corneal grafts in eight eyes
of six patients with congenital glaucoma with
multiple risk factors for poor prognosis (age ! 2
years at the time of grafting, uncontrolled glaucoma
in four eyes, concurrent lensectomy, retinal, or
glaucoma surgery in five eyes, aphakia in five eyes
and an acute perforation in one eye) which had
undergone previous glaucoma surgeries. Despite
earlier glaucoma surgeries, five eyes needed simultaneous glaucoma filtering implant at the time of
keratoplasty with only 67% (nine grafts) found to
remain clear at 30 months postoperatively with six
eyes achieving ambulatory vision.
Congenital glaucoma has a 50% chance of
success, with the requirement of regrafts in several
eyes.87 Keratoplasty may be associated with

improved visual acuity in eyes with marked buphthalmos and congenitally opaque corneas treated
with cyclocryotherapy.46
Simultaneous penetrating keratoplasty and
Ahmed glaucoma valve (AGV) implant surgery with
mitomycin C can be successful but multiple
interventions for glaucoma control may be
required.168 The use of a valved implant can be
considered in patients who require emergency
simultaneous corneal and glaucoma surgery for
severe congenital glaucoma. This could help in
early postoperative control of aqueous outflow
thereby enhancing long-term graft survival in such
difficult cases. When the AGV implant is placed at
the time of the keratoplasty, it is usually effective in
controlling intraocular pressure over a span of 3
years, whereas the success of the transplanted
corneas remains poor.7,8 Long-term success rates
of simultaneous AGV implants and penetrating
keratoplasty in refractory congenital glaucoma with
corneal opacity is low, with high risk for
complications.8
Corneal grafts in eyes with congenital glaucoma
with various risk factors at the time of the
keratoplasty, such as younger age group, uncontrolled IOP, multiple intraocular surgeries, concurrent lensectomy, and retinal or glaucoma surgery,
are associated with a less favorable outcome. Useful
vision can be achieved after penetrating keratoplasty
even in some high-risk infants with congenital
glaucoma, but the risk of development of complications and graft failure is very high. Good control of
intraocular pressure before and after corneal grafting is mandatory in eyes with buphthalmos in order
to avoid graft failure and progress of glaucomatous
optic nerve atrophy.140 Effective intraocular pressure control before and after corneal grafting is
imperative in eyes with buphthalmos in order to
prevent graft failure and progress of glaucomatous
optic nerve atrophy, which will further increase
ocular morbidity in these eyes.
b. Peters Anomaly
Severe Peters anomaly with dense corneal opacities leads to blindness unless corneal transplantation is performed. The timing for keratoplasty is still
not very clear. In the multicenter study by Dana et
al,33 the congenital opacification group comprised
predominantly of anterior segment dysgenesis
(30%) and others (25%) with 62% of eyes retaining
full graft clarity. They found no significant difference noted in the retention of clarity among
diagnostic groups, whereas other studies 31,134
report that the probability of retaining clear grafts
263
in eyes with congenital corneal opacification was

found to be less compared to that in eyes with
acquired corneal opacities. Complicated cases
requiring additional surgical procedures are however associated with a poor prognosis.35
Rezende et al110 had obtained good results in
their small series of patients with Peters anomaly
with 9 out of 10 eyes retaining clear grafts at a mean
follow-up of 64.2 months. However other studies
warn of a more guarded prognosis in most cases of
Peters anomaly.31,35,51,167,170 A large series of Peters
anomaly by Yang et al167 found 54% of eyes received
one graft, 18% received two grafts, and 28%
received three or more grafts. Initial grafts were
more likely to fail during the first two postoperative
years, with more than half of all the failures
occurring within the first three postoperative
months.167 To be taken into further consideration
is the fact that substantial time and investment is
required in additional ocular surgeries besides
keratoplasty in these children.165 Hence the need
for frequent evaluation in the initial postoperative
months along with counseling of the parents is
imperative.
Parmley et al94 reported dismal results in his
series with a high incidence of graft rejection in
cases requiring cyclodestructive procedure for glaucoma control, resulting in partial or complete graft
failure shortly after the procedure. In another small
series of penetrating keratoplasty in newborns with
severe Peters anomaly,10 repeat keratoplasties,
lensectomies, and numerous glaucoma operations
had to be performed. The results obtained in
newborns remain very poor. The performance of
penetrating keratoplasty in patients with Peters
anomaly early after birth is associated with a multitude of problems, especially glaucoma, making it
difficult to retain clear grafts for an extended
period. Althaus and Sundmacher10 have suggested
postponing surgery until the patient is about 1 year
old in order to obtain a better graft survival,
although persistent amblyopia might be quite severe
and limit the functional success.
In Zaidman et als170 study of 30 eyes with Peters
anomaly type I who had undergone corneal transplantation, five of six grafts were clear (83%) in the
younger group of children although the older
group of 24 eyes fared better with good visual and
anatomical outcome. Major complications in keratoplasty in Peters anomaly included cataract,
secondary glaucoma, epithelial defects, band keratopathy, retinal detachment, wound leakage, retrocorneal membranes, and microbial keratitis.44 A
retrospective review of records of 11 patients with
congenital corneal opacity who had undergone
penetrating keratoplasty as infants by Comer et
264
al29 revealed a poor graft survival and a low final

visual acuity for Peters anomaly.
Corneal transplantation for congenital corneal
opacities (non-CHED) has the best prognosis for
the dystrophy group (posterior polymorphous dystrophy), followed by patients with Peters anomaly.87
In contrast to penetrating keratoplasty, sector
iridectomy for congenital corneal opacification
secondary to Peters anomaly is not followed by
secondary glaucoma postoperatively. The visual
outcome has been found to be comparable to that
after early keratoplasty. Junemann et al63 recommend optical sector iridectomy as an alternative
surgical approach to early penetrating keratoplasty
in patients of Peters anomaly. Optical sector
iridectomy was performed in 13 patients with
Peters anomaly (with diameter of corneal opacification greater than half of the corneal diameter) at
a mean age at surgery of 1 year and 9 months. Over
a mean follow-up of 3 years and 6 months, nine
(47%) eyes had achieved a visual acuity was 20/500
to 20/200.63 When the peripheral cornea is clear
and cataract is not associated, an optical sector
iridectomy is an effective alternative to penetrating
keratoplasty.27,134,148
VANATHI ET AL
strategies was not possible in this study,166 it is to

be noted that better visual outcome is associated
with a clear graft, phakic eye, and moderate grade of
Peters anomaly whereas graft failure, surgical
aphakia, and severe grade of Peters anomaly has
poor visual outcome and also has the coexistence of
devastating postoperative complications.
Zaidman et als170 study on 30 eyes of Peters
anomaly (type I) with corneal grafts showed that 15
(50%) required treatment for glaucoma of which
only four eyes were able to achieve good visual
acuity. Eyes with glaucoma have a poorer visual
prognosis in Peters anomaly. Secondary glaucoma
seems to be the limiting prognostic factor in the
long run with uncontrolled intraocular pressure
despite multiple surgical interventions, and graft
prognosis remains poor in the long run.10 Most
cases of Pters anomaly may develop glaucoma
postoperatively or preexisting preoperative glaucoma might worsen and medical control is usually
unsuccessful necessitating cyclodestructive or glaucoma filtering implants or both, for control of
intraocular pressure.94
2. Infrequently Associated with Glaucoma
a. Dermoid, Birth Trauma, and Metabolic Diseases
c. Peters Anomaly with Glaucoma

Yang et al report166 of a total of 79 penetrating
keratoplasties in 34 eyes of Peters anomaly with
glaucoma (median 2 surgeries; range 1--7) reported
the need for glaucoma surgery before first keratoplasty in 17 eyes, simultaneously with first keratoplasty in 8 eyes, and after the first keratoplasty in 9
eyes. Although no graft infection was noted in their
series, major postoperative complications included
graft failure, retinal detachment, phthisis and
cataract. Eyes with vision of 20/400 or better were
observed in those with clear grafts with an intact lens
and in moderate grade of Peters anomaly. Glaucoma surgery combined with medical therapy results
in adequate long-term control of IOP in only 32% of
the eyes with glaucoma in Peters anomaly. Despite
the large number of cases, prognostic indicators for
visual outcome in cases of Peters anomaly with
glaucoma could not be given in the study due to the
limitations of multiple procedures performed per
eye (1--14).166 Among the various glaucoma procedures performed in Peters anomaly with glaucoma,166 long-term IOP control was found to be
maintained successfully in one of 11 eyes that
underwent cyclocryotherapy, in all four eyes that
underwent Molteno implantation, in two of seven
eyes that underwent trabeculectomy, and in none of
the five eyes that underwent goniotomy. Although
a possible comparison of glaucoma treatment
Conditions such as dermoid, birth trauma, and

metabolic diseases constitute about 15%, 2.8%, and
2.8%, respectively, of the cases with congenital corneal
abnormalities.110 Dermoids not involving the visual axis
can be effectively managed by simple excision or
combined with lamellar grafting in cases of extension
into the deeper tissue. Dermoid infrequently may involve
the entire cornea, associated with adherence of the
atrophic iris to the posterior corneal surface and cataract.
Surgical excision of dermoid and penetrating keratoplasty for tectonic reconstruction is required in such
cases.50 According to the size and location, the dermoids
may be managed either by sectoral, annular, or central
lamellar keratoplasty.14,93,97,121,160 Corneoscleral lamellar grafts may be required in advanced cases, especially
when the whole thickness of the corneal stroma is
involved or when the tumor deeply extends around the
limbus. Dermoid excision with a 12-mm lamellar
keratectomy and followed later by a smaller (8-mm)
penetrating keratoplasty has also been reported to
provide good results. This staged procedure helps in
minimizing the complications associated with large
corneal transplants and increases the chance of longterm success.169 The use of of full-thickness central
corneal grafts in lamellar keratoscleroplasty for limbal
dermoids123 has also been found to achieve good
cosmetic results and less corneal astigmatism.
In mucopolysaccharidosis (MPS), the outcome of
keratoplasty is weighed in view of the expected
265
lifespan of the patient and the particular syndrome

involved. Because clinically significant corneal
clouding does not appear in Hunters and Sanfillipos syndrome, no therapeutic intervention in terms
of penetrating keratoplasty is warranted. In other
MPS the decision to do keratoplasty and the timing
depends on the particular syndrome and the
individual case. The visual prognosis following
keratoplasty is hampered by the coexisting retinopathy and optic nerve involvement apart from the
shortened life-span from the disease itself. Clouding
of the transplant is often observed and is related to
storage of glycosaminoglycans in the donor button.67 Surgery should be done at an early age,
especially in Hurler syndrome and Morquio syndrome, which is associated with a short life-span, so
that the child can be visually and vocationally
rehabilitated in the limited time available.67,91
Bergwerk et al reported good visual outcome
following penetrating keratoplasty in a patient with
Sly disease, in which the cornea remained clear for 2
years following surgery.22 Kasmann-Kellner reported
postoperative visual acuity improvement for nearly
a year, followed by progressive re-opacification of
the corneal graft in a case of Morquio syndrome that
was also complicated by tapetoretinal degeneration
and optic atrophy.67 Naumann and Rummelt
observed a partial, circular clearing of the hosts
cornea adjacent to the transplant in three children
with Maroteaux--Lamy syndrome in which the transplants remained clear during the follow-up of 2.5--5
years following penetrating keratoplasty.91
Recent advances in systemic treatments for MPS have
led to therapies that improve the multiple somatic
features of this disease, such as bone marrow transplantation (BMT) and enzyme replacement.141 Tokic et
al141 report improvements in cardiac function, stool
habits, visual acuity, corneal clouding, and hearing after
enzyme replacement but the therapeutic effect on ocular
manifestations, such as corneal clouding, is reported to
be not satisfactory. Huang et al also reported limited role
of BMT in clearing of corneal clouding.58
Injection of adenovirus expressing human betaglucuronidase (AxCAhGUS) into the anterior chamber
or intrastromal region of the cornea in mice with MPS
type VII (B6/MPS VII) has shown successful results with
clearing of corneal clouding.66 Intrastromal vector
administration did not generate significant levels of
anti-adenovirus neutralizing antibodies, and secondary
vector administration was also found effective.66
Ucakhan reports the longest follow-up of MPS type VI
who underwent BMT for gene transfer at the age of 13,
and penetrating keratoplasty at the age of 17, and
maintained clear corneal grafts bilaterally for 13 years.144
Corneal clearing is not considered an appropriate index
for measuring the success of systemic therapy in MPS VI
as clearing of the host cornea or opacification of donor

cornea was not observed following reciprocal corneal
transplantation in an animal experiment.3
b. Sclerocornea
The rate of success for keratoplasty in sclerocornea is significantly lower than those with other
congenital abnormalities. In Rezendes110 series, the
need for regrafts was significant in eyes with
sclerocornea. The chance of success of the corneal
graft in cases of sclerocornea is about 50%, with
requirements of repeated transplants in several
eyes.87
In Frueh and Browns series,44 the overall success
(including regrafts) was found to be 70% in eyes
with sclerocornea and 83% for partial sclerocornea.
They recommend early keratoplasty for congenital
opacities with unilateral as well as bilateral
involvement in infants, as they were able to achieve
an excellent long-term survival; however, the
increased need for regrafting and a high incidence
of complications in these cases is to be given careful
consideration before decision making for surgical
intervention in congenital opaque corneas. The
better prognosis in Peters anomaly and partial
sclerocornea is related to the lack of severe
intraocular anomalies and the subsequent lower
incidence of glaucoma along with lensectomy and
anterior vitrectomy not being required in these eyes.
A prospective, nonrandomized clinical trial by
Vajpayee et al147 reports on 40 pediatric patients
with unilateral or bilateral corneal opacification,
with corneal grafting using 1-mm oversized donor
corneal buttons for the congenital opacities (largely
comprising of sclerocornea) with visual acuity found
to be 20/80 or better in only 30% of cases.
Sclerocornea is associated with a poorer prognosis
due to the higher incidence of major anatomical
alterations that are associated with it. Sclerocorneas
heal approximately twice as fast as non-vascularized
corneas. An increased fibrin exudation is noticed in
these infant eyes, leading to broad-based anterior
synechia formation, resulting in early postoperative
glaucoma.
c. Congenital Corneal Keloid
Removal of the corneal keloid with superficial
keratectomy or treatment with lamellar or
penetrating keratoplasty may be performed for
visually significant lesions and is to be limited to
symptomatic patients.23,107,150 Rao et al107
performed multiple penetrating keratoplasties to
treat a case of congenital corneal keloid with
anterior segment mesodermal dysgenesis in a patient
266
with Rubinstein-Taybi syndrome, all of which proved

unsatisfactory. Repeated epithelial breakdown leading to graft failure, led them to conclude that
a possible limbal stem cell deficiency could be
associated. Tectonic penetrating keratoplasty in
a patient with unilateral congenital corneal keloids
with bilateral anterior segment mesodermal dysgenesis with subluxated lenses was also not successful.150
C. ACQUIRED TRAUMATIC
Reported success rates for pediatric grafts in

acquired traumatic conditions vary from 55% to
100%.1,31,34,42,132,133 Visual prognosis is better in
corneal opacification due to trauma or dystrophy
whereas congenital glaucoma has the worse prognosis. This perhaps is due to the fact that older
children tend to have less dense amblyopia as they
have had good vision in their earlier years before
trauma with less formed vision deprivation A better
outcome is achievable for pediatric corneal grafts
when performed early as it helps to prevent formed
vision deprivation and the patients are compliant
with long-term follow-up.122 Older children tend to
fare better in terms of better functional success than
the younger ones (who have dense amblyopia due to
the congenital nature of their pathologies) and than
those with postoperative complications. Stulting et
al134 reported a mean 70% probability of a clear
graft after 1 year in children aged 14 years or
younger with traumatic corneal scars. They found
vitreoretinal pathology, fibrous ingrowth, and optic
nerve damage from glaucoma as the main limiting
factors for good visual prognosis. Cowden et al31
also obtained a better graft outcome in traumatic
phakic eyes in their series. In Dana et als34
retrospective multicentric evaluation of results of
corneal grafting following corneal injury visual
acuity improvement was seen in 83% of the cases
in which visual assessment could be done. Patel et
al95 reported a 100% success rate in their grafts for
traumatic causes.
Penetrating keratoplasty for corneal trauma is
successful in the pediatric age when trauma is
limited to the anterior segment. Posterior segment
injury before keratoplasty results in poor graft and
visual outcome. Timely visual rehabilitation with
optimal amblyopia therapy enhances visual outcome. Overall, in traumatic opacities, children with
shorter time intervals between trauma and rehabilitation of the optical axis have better visual outcomes, including avoidance of uncorrected aphakia
by early implantation of posterior chamber
intraocular implant.
VANATHI ET AL
D. ACQUIRED NON-TRAUMATIC
Pediatric corneal grafts done for acquired nontraumatic causes have a high success rate. Reported
success rates for pediatric grafts in acquired nontraumatic conditions are between 40% and
85%.1,20,31,33,34,42,73,83,95,134,137 Better graft survival
rates and good visual outcome are usually achievable
in corneal opacification due to non-traumatic
acquired conditions as most of the cases in this
group include keratoconus patients.78,80,83,95
In developing countries, infectious keratitis and
keratomalacia is seen as the most common
indication for pediatric keratoplasty.1,32,122,146,148
Results of surgical management of keratomalacia
in children are not very encouraging.21,126,148
Bilateral keratomalacia can be a rarely induced by
metabolic disorders such as uncontrolled phenylketonuria. Habot-Wilner et al54 were successful in
managing the condition with amniotic membrane
transplantation in one eye and penetrating keratoplasty in the other.
Ipsilateral rotational autokeratoplasty may be considered in selective cases of central corneal opacity in
infants and children. This may be particularly applicable to patients in the developing world where there is
a great demand for donor corneal tissue. Meiser et als
series84 of 20 keratoplasties in infants and children (2
weeks to 6 years) with central corneal opacities of
herpetic or microbial etiology, had autorotation grafts
done for five cases resulting in satisfactory visual acuity
achievement. Irregular astigmatism is a common
problem in autorotation grafts.
VII. Conclusion
Early penetrating keratoplasty in infants with
congenital corneal opacities is required to prevent
amblyopia and allow normal development of vision. In
the past corneal grafting in children for congenital
corneal opacities was delayed until early childhood
because of the difficulty in operating upon infant eyes.
Recent studies stress on the need for early grafting in
order to prevent amblyopia. Technical advancements
have made corneal grafting possible at a younger age
group. Clear graft rates vary accordingly to the nature
of primary corneal pathology of the patient. Patients
with acquired opacities have better visual outcomes
than those with congenital opacities. Prognosis for
graft success is guarded in sclerocornea and congenital
glaucoma, with a 50% chance of success and an
increased need for repeated transplants. Prolonged
corneal graft survival can be achieved in children, but
successful restoration of visual acuity depends upon
a period of normal visual development before the
onset of corneal opacification. Failure to promptly
267
report to the treating surgeon in incidences of suturerelated problems is an important risk factor for graft
infection and rejection in pediatric keratoplasty cases.
Lower socioeconomic status and long distance from
referral centers also contribute to this delay in
developing countries. As most of the graft failures
due to infection occur in the early postoperative
months, pediatric keratoplasty mandates more frequent follow-ups than adult keratoplasty in the initial 6
months of the postoperative period. Increase graft
rejection rates have been noted with pediatric corneal
transplants due to the more active immune system in
the younger patients.9 Topical CsA 2% drops have
been found to be help in reducing the risk of allograft
rejection in pediatric recipients. Sustained efforts at
visual rehabilitation are obstructed by visual deprivation, high, unsymmetrical refractive errors, by strabismus,
nystagmus,
neurologic
deficits,
and
noncompliance to follow-up and amblyopia therapy.
Poor visual outcome in pediatric corneal grafts can
be attributed to amblyopia, frequent graft failures,
post keratoplasty astigmatism, and associated ocular
pathology. Amblyopia treatment has been reported to
be the only independently significant prognosticator
for visual improvement after surgery. Visual acuity
determination in all cases of clear grafts is not possible
due to the young age of infants and children. With
improved and more predictable anatomic success of
pediatric corneal grafts in recent times, the need to
achieve better optical success still remains a challenge
to corneal transplant surgeons. All these results
perhaps go on to show that in choosing treatment
optionshomologous penetrating keratoplasty,
autologous ipsilateral rotation grafts, and optical
sector iridectomydetails of the patient and family
selection are key to success.84,90,145 Allograft rejection,
graft infection, and glaucoma remain important
causes for concern for graft morbidity in the younger
age group. Pediatric keratoplasty, hence, continues to
be a highly challenging and demanding procedure.
Given the heterogeneity of the involved conditions,
a comparable analysis of graft survival outcomes
among the various studies becomes improbable. It is
therefore recommended that results are analyzed in
the format as suggested in this review to enable a more
uniform analysis in future.
VIII. Method of Literature Search

The PubMed database was searched electronically
for the years 1950--2007 with the keywords penetrating
keratoplasty in children, pediatric keratoplasty, corneal
transplantation.
Manual cross reference search: Some articles that
were not found by our Medline search were taken
from the references from other articles and books.
English abstracts available on PubMed were

included in certain important non-English language
articles on pediatric penetrating keratoplasty.
Inclusion of the articles was based on their
importance to the subject and exclusion was to
avoid redundancy.
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Outline
I. Introduction
II. Indications
A. Congenital opacities: CHED
B. Congenital opacities: non-CHED
1. Frequently associated with glaucoma
b. Peters anomaly
c. Other mesenchymal dysgenesis
2. Infrequently associated with glaucoma
a.
b.
c.
d.
e.
f.
g.
Dermoids
Metabolic causes
Sclerocornea
Birth trauma
Corneal keloid
Aniridia
Posterior polymorphous dystrophy
III. Technique
A. Preoperative evaluation and decisionmaking
B. Anesthesia
C. Preparation of globe
D. Trephination
E. Concomitant procedures
F. Simultaneous keratoplasty with glaucoma
filtering device implantation
G. Pediatric keratoprosthesis
H. Epikeratoplasty
IV. Postoperative management
A. Immediate postoperative management
B. Suture removal
The authors reported no proprietary or commercial interest in

any product mentioned or concept discussed in this article, The
authors reported no proprietary or commercial interest in any
product mentioned or concept discussed in this article.
Reprint address, Reprint address: M. Vanathi, MD, Asst. Prof of
Ophthalmology--Cornea, Ocular Surface and Refractive Surgery
Services, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All
India Institute of Medical Sciences, New Delhi 110029, India.
e-mail: vanathi_g@yahoo.com.
C. Refractive correction
D. Amblyopia management
E. Regrafts
V. Complications
A.
B.
C.
D.
E.
F.
G.
H.
I.
Graft rejection
Graft infection
Persistent epithelial defect
Wound dehiscence
Cataract
Endophthalmitis
Glaucoma
Retinal detachment and phthisis
Amblyopia
VI. Outcome of pediatric keratoplasty

A. Congenital opacities: CHED
1. CHED with glaucoma
B. Congenital opacities-NonCHED
1. Frequently associated with
glaucoma
b. Peters anomaly
c. Peters anomaly with glaucoma
2. Infrequently associated with
glaucoma
a. Dermoid, birth trauma, and metabolic
diseases
b. Sclerocornea
c. Congenital cornealkeloid
VII. Conclusion
VIII. Method of literature search

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SURVEY OF OPHTHALMOLOGY

VOLUME 54 NUMBER 2 MARCHAPRIL 2009

Abstract. Penetrating keratoplasty in children is a highly challenging and demanding procedure

Congenital eye disorders, although infrequent, are

0039-6257/09/$--see front matter

Surv Ophthalmol 54 (2) March--April 2009

Indications for Pediatric Keratoplasty

CHED presents as bilaterally symmetrical diffuse

The primary defect in patients with CHED is

of the Descemets membrane. Abnormalities of crest

B. CONGENITAL OPACITIES: NON-CHED

Peters anomaly is one of the most common

The non-CHED congenital corneal opacities can

Surv Ophthalmol 54 (2) March--April 2009

atrophy, abnormal vasculature, or coloboma. Severe

and syndrome and posterior keratoconus.157 All

increased in Scheies syndrome compared to Hurlers

involving the entire corneal stroma. Keloids occur

Surv Ophthalmol 54 (2) March--April 2009

of PPMD are seen as a transparent cyst with

Penetrating injuries remain an important cause of

One of the most common causes of acquired

red reflex is seen, these patients seem to see much

When performing pediatric keratoplasty, it is

stimulator can help eliminate the risk of movement

Increased positive pressure during surgery is

The recipient cornea is trephined to 70--80%

Surv Ophthalmol 54 (2) March--April 2009

performing pediatric keratoplasty. The use of 100

required. If vitreous prolapse occurs, anterior

1. Complete preoperative evaluation of the

Loss of crystalline lens and vitreous at the time of

1. Small size of the palpebral fissure reduces the

3. Caution is to be exercised while performing

Use of keratoprosthesis to treat pediatric corneal

Epikeratoplasty has been performed to provide

factors, such as younger patient age, microcornea,

IV. Postoperative Management

Postoperative treatment regimen involves topical

Loosening of sutures or vascularization requires

Surv Ophthalmol 54 (2) March--April 2009

communicate discomfort and vision changes. Graft

Refraction is done after suture removal for optical

The neurological basis of amblyopia is related to

therapy is maximally effective as the immature visual

Repeat penetrating keratoplasty is quite often

Parmley et al94 also had a high rate of regrafts in

most highly correlated with poor graft survival.134

Pediatric corneal transplantation has an increased

Surv Ophthalmol 54 (2) March--April 2009

McClellan et al83 were successful in retaining clear

C. PERSISTENT EPITHELIAL DEFECT

Bacterial keratitis after primary penetrating keratoplasty in children is a serious complication

Persistent epithelial defects (PED) in pediatric

Wound leak and dehiscence (2--10%)18,31,33,42

The reported rates of cataract vary between 2%

The reported rate of endophthalmitis following

VI. Outcome of Pediatric Keratoplasty

A. CONGENITAL OPACITIES: CHED

The incidence of post-penetrating keratoplasty

H. RETINAL DETACHMENT AND PHTHISIS

Other vitreoretinal complications are expulsive

As already discussed amblyopia is the most

Graft survival rates in cases of CHED vary widely