Acta Ophthalmologica 2016

Indications and outcomes of keratoplasties in

children during a 40-year period
Anna Majander,1 Tero T. Kivel€
a2 and Kari Krootila3
1
Paediatric Service, Department of Ophthalmology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
2
Ophthalmic Pathology Service, Department of Ophthalmology, University of Helsinki and Helsinki University Hospital, Helsinki,
Finland
3
Department of Anterior Segment and Corneal Surgery Service, Department of Ophthalmology, University of Helsinki and Helsinki
University Hospital, Helsinki, Finland

ABSTRACT. Introduction
Purpose: To report the indications and the outcomes of keratoplasties in
children over four decades. Corneal grafting in children is challeng-
Methods: A retrospective cohort study of patients aged 16 years or younger who ing not only technically but also with
underwent keratoplasty in the Helsinki University Eye Hospital during 1968– respect to allograft survival and visual
recovery. In general, the outcome of
2011. Diagnosis, preoperative status, age at the time of surgery, surgical
paediatric keratoplasties is inferior to
technique, complications and follow-up time were registered. Main outcome
that of adults but depends on the
measures were visual acuity and graft survival as assessed by Kaplan–Meier indication and the age of the patient
analysis. The independent role of risk factors on outcomes was evaluated by Cox (Erlich et al. 1991; Yang et al. 1999;
multivariate regression analysis. Patel et al. 2005; Al-Ghamdi et al. 2007;
Results: Forty-eight keratoplasties, 42 penetrating and six lamellar, were Vanathi et al. 2009; Limaiem et al.
performed in 44 eyes of 39 children at the age of 4.5 months to 16 years 2011; Lowe et al. 2011; Hovlykke et al.
(median, 12 years). Five patients had bilateral grafts, and five grafts were 2014). Ten-year graft survival varies
regrafts. The indication for keratoplasty was injury for 13 grafts, non- from 20% or less in infants grafted for
traumatic acquired corneal opacities for 11, keratoconus for eight, corneal severe developmental corneal opacities
dystrophy for seven, congenital corneal opacities for six and aniridia for three to 80% or more in adolescents grafted
grafts. The cumulative proportion of clear grafts was 46% at 5 years for keratoconus (Yang et al. 1999;
postoperatively, and the median follow-up time of clear grafts was 5.1 years Lowe et al. 2011). Indications of graft-
(range, 0.4–29 years) for 41 penetrating allografts (PKP). Simultaneous ing reflect variations in the incidence of
intraocular surgery at the time of grafting [hazard ratio (HR) 9.7], corneal corneal infections as well as those of
hereditary and acquired corneal opaci-
vascularization (HR 8.1) and regrafting (HR 5.4) were the main independent
ties, that vary from infectious corneal
risk factors for graft failure in this PKP cohort. The cumulative proportion of
opacities and congenital glaucoma to
clear grafts was 84% at 5 years in the absence of any of these risk factors. congenital hereditary endothelial dys-
PKP for keratoconus and corneal dystrophy yielded clear grafts in 83% of trophy and keratoconus depending on
the eyes, and a visual acuity ≥0.3 (Snellen) in 75% of the eyes. Seventeen of population (Patel et al. 2005; Al-
the 20 graft failures were due to rejection. Ghamdi et al. 2007; Sharma et al.
Conclusions: Favourable graft survival was obtained in primary keratoplasties 2007; Limaiem et al. 2011).
for non-vascularized corneal opacities performed without any other simulta- In addition to the risk factors for
neous intraocular surgery. Visual outcome was favourable in keratoconus and graft failure shared with adults, such as
corneal dystrophies and poor in most eyes with injury. corneal vascularization, glaucoma,
infectious corneal opacity and previous
Key words: child – graft survival – keratoplasty – penetrating – risk factors – treatment outcome grafting, paediatric keratoplasties have
a specific risk profile related to devel-
Acta Ophthalmol. 2016: 94: 618–624
opmental factors (Vail et al. 1997;
ª 2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd
Panda et al. 2007; Huang et al. 2009;
Lowe et al. 2011). Surgery may be
doi: 10.1111/aos.13040 complicated by more elastic ocular

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tissues and the smaller size of the eye. acuity. The following preoperative sutures in three eyes. The sutures were
The outcome of grafting in develop- details were registered with specific silk in two, and 10/0 Nylon in three
mental congenital corneal opacities focus on the features considered as risk eyes. During 1979–2011, 38 penetrating
depends strongly on the age of the factors for failure (Dana et al. 1995; keratoplasties, one automated lamellar
child at the time of surgery and the Vail et al. 1997; Panda et al. 2007; therapeutic keratoplasty, two deep
severity of the disorder: eyes with few Lowe et al. 2011): the extent of corneal anterior lamellar keratoplasties and
iridocorneal synechiae have a markedly opacity and vascularization, the pres- one autotransplant were performed.
better graft survival than those with ence of iridocorneal and lenticulo- Either 10/0 Nylon alone, or a combi-
lenticulocorneal synechiae (Yang et al. corneal adhesions, glaucoma, nation of 10/0 and 11/0 Nylon, mostly
1999; Michaeli et al. 2005; Zaidman intraocular inflammation, and preced- as continuous sutures, was used. After
et al. 2007; Kim et al. 2013). The visual ing intraocular or corneal surgery. 1987, the host and donor corneal but-
outcome is affected not only by the Rejections were classified according to tons were prepared by vacuum trephi-
factors common with adults such as Panda et al. (2007) as epithelial, stro- nation. The median host corneal
astigmatism, glaucoma and cataract, mal, endothelial and rejection in a button diameter was 7.5 mm (range
but also by the amblyogenic impact of regraft. The following features were 6.0–9.5), and the median graft diameter
the corneal opacity and the timing of considered diagnostic for glaucoma 7.75 mm (range 6.75–10.0). Postopera-
the surgery (Comer et al. 2001; based on Ganesh et al. (2013): progres- tive topical therapy included antibi-
McClellan et al. 2003; Lowe et al. sive cupping of optic nerve head, otics, corticosteroids and artificial tears
2011). abnormal growth of axial length or in all cases and cycloplegics in some
Attention to these factors is essen- corneal diameter in infants in associa- patients. Topical antibiotics were con-
tial for optimal targeting of resources tion with a hypertensive intraocular tinued for 2–4 weeks postoperatively.
to those patients who most likely will pressure for age (higher than 21 mmHg Topical corticosteroid was continued
benefit from the surgery. Preoperative in school-aged patients). Intraocular with decreasing dosage (beginning with
assessment of the risk profile and the pressure was measured by Goldmann six times a day) until all sutures had
predicted prognosis of each eye form or Tono-Pen (Tono-Pen XL; Reich- been removed. The timing of suture
the basis for clinical decisions regard- ert, Depew, NY, USA) applanation removal was documented for 33 grafts:
ing surgical management. This study tonometers, or by iCare rebound the first sutures were removed a mean
was performed to report the indica- tonometer (iCare TA01i; Icare Fin- of 3.6 (SD 1.6) and the last ones 7.3
tions and analyse the outcome of land, Vantaa, Finland). (SD 3.3) months postoperatively.
paediatric corneal transplantations in The main end points were graft Selected patients received systemic
our university hospital over a 40-year survival and clarity, and the best- immunosuppression postoperatively:
period. Patients who had undergone corrected visual acuity (BVCA) was six patients peroral corticosteroid
corneal grafting at the age of 16 years recorded as Snellen decimal notation (three with severe injury, one with
or younger were enrolled. The out- for patients capable of performing the suspected inflammatory corneal opac-
come and the predictive value of risk test. Spectacles were used for refractive ity and two operated early in this
factors were evaluated on the basis of correction. Snellen or letter charts were cohort) and one patient cyclosporin
observed graft survival and visual used in older and Lea symbol charts (after regrafting). All host corneal but-
acuity. (Good-Lite Co., Elgin, IL, USA) in tons underwent histopathologic inves-
younger children. Counting fingers, tigation.
hand motion and light perception were In 18 eyes, additional simultaneous
Patients and Methods converted to Snellen fractions accord- intraocular surgery was performed at
ing to Lange et al. (2009), to enable the time of primary grafting: in 14 eyes
Patients
statistical comparison of pre- and post- anterior segment surgery (cataract
This retrospective cohort study was operative visual acuities. extraction, liberation of iridocorneal
performed with the approval of the adhesions, anterior vitrectomy,
ethics committee of the Helsinki intraocular lens implantation) and in
Surgical data
University Hospital following the four eyes retinal surgery (pars plana
Tenets of the Declaration of Helsinki. Forty-eight keratoplasties were per- vitrectomy aided with an intraopera-
Patients who had undergone corneal formed in 44 eyes (22 right and 22 left tive keratoprosthesis in three eyes).
grafting at the age of 16 years or eyes). Five procedures were regrafts, Three of the regrafted eyes underwent
younger in 1968–2011 were eligible. one of which had been primarily also additional simultaneous anterior
Thirty-nine patients, 26 males and 13 grafted elsewhere. Seven corneal sur- segment surgery. Three grafts under-
females, were identified from the graft geons performed the keratoplasties. went refractive surgery postoperatively.
and surgery registries of the hospital. Donor corneas were obtained from
All except one patient had their post- the Helsinki University Eye Bank,
Statistical methods
operative follow-up in the Helsinki and they underwent appropriate
University Central Hospital. The fol- screening for infectious agents and Penetrating allografts (PKPs; 41 grafts
lowing data were collected: diagnosis, endothelial quality. During 1968– for 38 eyes of 35 patients) of this cohort
laterality, preoperative findings, age at 1978, three penetrating and three man- underwent statistical analyses regard-
the time of surgery, surgical technique, ual anterior lamellar keratoplasties ing graft survival. Kaplan–Meier sur-
any simultaneous intraocular surgery, were performed using continuous vival analysis was used to analyse time
complications, graft survival and visual sutures in three, and interrupted to graft failure. Surviving grafts were

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censored at last visit. Groups were Results tively, and nine of these eyes reached a
compared by the log-rank test. Signif- BCVA 0.3 or better. None of the grafts
icance was set at p < 0.05. Cox pro- Indications for grafting and outcomes in eyes with aniridia and two of the six
portional hazards regression analysis grafts for congenital corneal opacities
was used to identify independent risk The indications for keratoplasty, the remained clear. The only eye grafted
factors of graft failure. Significance of number of patients, eyes and grafts for a herpetic scar retained graft clarity
each variable was first studied by uni- and the age of the patients at the time for at least 25 years postoperatively
variate analysis. Regrafts underwent of surgery are presented in Table 1. with BCVA 0.3. All three modern
the univariate analysis only in the Ocular injuries were the most frequent lamellar keratoplasties and the only
entire allograft data set, and primary (27%) indication, followed by non- autotransplant were clear at the last
allografts were analysed as an indepen- traumatic acquired corneal opacities follow-up after 0.4–9.5 years.
dent data set. Variables were consid- (23%), keratoconus (17%), corneal The median BCVA improved from
ered statistically significant if p < 0.10. dystrophies (14%), congenital corneal the preoperative 0.03 (range from light
The variables that were significant on opacities (12%) and aniridia (6%). perception to 1.0) to the final median
univariate analysis in the primary allo- Deteriorated visual acuity was the BCVA 0.32 of the surviving allografts
graft data set underwent multivariate indication for grafting in eyes with (range from hand movement to 0.9,
analysis. Each significant variable was keratoconus and corneal dystrophies, n = 21; Fig. 1), whereas that of the
combined pairwise with another signif- the median best-corrected visual acui- failing allografts was a median of 0.02
icant variable in bivariate models. ties (BCVA) of which was 0.1. In (range from no light perception to 0.1,
Variables which lost significance in traumatic and non-traumatic corneal n = 20). In the surviving grafts, the
bivariate analysis were judged not to scars, either deteriorated vision or median improvement of BCVA was 0.2
be independent risk factors of graft need for intraocular surgery was the Snellen units. In eight eyes, vision
failure. Mann–Whitney independent indication for grafting. In two eyes deteriorated including one eye that
samples U-test was used to compare with congenital corneal opacity, graft- underwent simultaneous iridosclero-
graft size and age at surgery between ing was performed to prevent an corneal cyst resection with deteriora-
surviving and failing grafts. Spear- imminent spontaneous corneal perfo- tion of BCVA from 1.0 to 0.3. Graft
man’s rank correlation test was used ration. opacification was the most frequent
for the analysis of statistical depen- Graft survival and visual outcome of reason for poor vision. Amblyopia was
dence between patient’s age at the time PKPs varied depending on the indica- the reason for poor vision in seven eyes
of grafting and the length of the tion for grafting (Table 2): 10 of the 12 with clear grafts: two eyes grafted after
postoperative period to graft failure. eyes with keratoconus and corneal the period of visual plasticity, three
The Statistical Package for the Social dystrophy had clear grafts at the last eyes with long-lasting treatment for
Sciences SPSS 19 (IBM Corp., Armonk, control after a median postoperative additional posterior segment injury
NY, USA) was used for all analyses. follow-up of 11.9 and 4.7 years, respec- and two eyes with ocular developmen-

Table 1. Diagnoses, the number of patients, bilateral grafts, primary grafts, regrafts and the total number of grafts, and the age of the patients at the
time of surgery.

Age (year)
Patients Bilateral Primary allograft Regraft Other technique Total median
Diagnosis n n n n n n (range)

Keratoconus 7 1 7 0 1 8 15.0 (12–16)

Corneal dystrophy 4 2 5 1 1 7 7.6 (5–14)
Posterior polymorphous 2 2 1
Reis-B€ucklers 1
Endothelial 1
Aniridia 2 0 2 1 0 3 12.4 (10–13)
Congenital 6 0 6 0 0 6 0.4 (0.4–15)
Peters’ anomaly 3
Congenital staphyloma, cornea plana, sclerocornea 1 each
Traumatic acquired 11 0 8 2 3 13 12.0 (3–15)
Penetrating 7 6 1
Contusion 2 1 1
Chemical/combustion 2 1 2 1
Non-traumatic acquired 9 2 8 1 2 11 11.0 (3–14)
Stevens–Johnson syndrome 1 1 2
Mucopolysaccharidosis 2 1 1*
Disciform herpetic scar, dry eye, band keratopathy 1 each
of uveitis, Molteno complication, failed epikeratoplasty,
iridocorneoscleral cyst
Total 39 5 36 5 7 48 12.0 (0.4–16)

*The primary grafting was performed elsewhere.

The bold numbers indicate the numbers in each main category.

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Table 2. Survival of penetrating allografts, follow-up time of surviving grafts and visual outcome factors were associated with either of
according to diagnosis. them. This model also fitted well to our
data as evidenced by its small 2 log
Best-corrected
Surviving visual acuities ≥ 0.3 likelihood statistic as compared to the
Eyes grafts Follow-up time, years (Snellen) alternative bivariate models. Regraft-
Diagnosis n n median (range) n ing, vascularized cornea and simulta-
neous intraocular surgery were thus the
Keratoconus 7 6 11.9 (0.5–29) 5 main independent risk factors for graft
Corneal dystrophy 5 4 4.7 (2.3–25) 4 failure in our cohort.
Aniridia 2 0 0
The cumulative proportional PKP
Congenital 6 2 0.4 and 14 0
Traumatic acquired 9 4 3.4 (0.4–9.6) 1 survival at 5 years in our cohort was
Non-traumatic acquired 9 5 7.0 (0.4–25) 2 46% (Fig. 2). The median survival time
Total 38 21 5.1 (0.4–29) 12 of regrafts was only 1 month (range 1–5)
and that in eyes with vascularized
cornea 3 months (range 1–21). The 5-
year cumulative proportional primary
allograft survival for eyes without cor-
neal vascularization but undergoing
simultaneous intraocular surgery was
38% with a median survival time of
3.6 years [95% confidence interval (CI)
1.2–6.2]. The corresponding probability
of clear grafts in eyes without any of
these three risk factors (‘low-risk grafts’)
was 84%. Altogether four grafts
retained clarity at least over 20 years.
The median age at the time of
surgery was 13 years for failing and
12 years for surviving allografts
(p = 0.83, Mann–Whitney U-test).
The length of the postoperative period
to graft failure did not show any age
dependence either (p = 0.61, Spear-
man’s rho = 0.144). The three youngest
Fig. 1. Pre- and postoperative best-corrected visual acuities (BCVA, Snellen decimals). Visual patients, operated at the mean age of
acuities below chart vision are presented using the semi-quantitative clinical scale ‘counting 5 months, failed and carried multiple
fingers’ (CF), ‘hand motion’ (HM), ‘light perception’ (LP) and ‘no light perception’ (NLP). The risk factors for failure as part of their
number of the eyes included under one circle is indicated if more than one. severe developmental ocular disorders.
The median diameter of both failing
tal disorders. None of the patients one with Peters’ anomaly, the glau- and surviving grafts was 7.5 mm
grafted at the age of 7 years or younger coma was under control. (p = 0.85, Mann–Whitney U-test).
and who had clear grafts benefited Cox proportional hazards univariate Graft survival was similar also in
from amblyopia treatment other than regression of graft failure indicated patients with unilateral and bilateral
appropriate refractive correction, as that regrafting when analysing all grafts (p = 0.75, log-rank test).
they had either bilateral disease or PKPs [hazard ratio (HR) 5.4] and,
other organic defects of developmental when considering only primary PKPs,
Graft failures in the PKPs
or traumatic origin. a need for simultaneous intraocular
surgery at the time of grafting (HR Twenty allografts failed (Table 5).
9.7), preoperatively vascularized cor- Seventeen failures were diagnosed as
Risk factors for graft failure in the PKPs
nea (HR 8.1), the presence of irido- and rejections. In addition, three grafts
The potential pre- and perioperative lenticulocorneal adhesions (HR 4.3) failed due to spontaneous iris prolapse
risk factors for failure in the PKPs are and corneal opacity extending to the in host cornea, retinal detachment and
presented in Table 3 according to the limbus (HR 3.3) were significant risk uveitic band keratopathy.
indication for grafting. Regrafts were factors for failure (Table 4). Bivariate Three eyes had classical signs and
analysed as a separate group, and the regression analysis of primary allo- symptoms of endothelial rejection. In
other risk factors were analysed for grafts, adjusting for each significant two of these eyes, rejection was severe
primary grafts. Eight eyes undergoing risk factor pairwise, revealed that only and led to graft failure. A mild rejec-
primary grafting had a vascularized simultaneous intraocular surgery tion in one graft was overcome with
cornea preoperatively, in six of them in (p = 0.03, HR 5.7) and preoperatively topical treatment. All six allografts
all four quadrants. Eight eyes under- vascularized cornea (p = 0.03, HR 4.0) with stromal rejection had undergone
going primary grafting had glaucoma retained significance as independent simultaneous intraocular surgery at the
at the time of surgery. In all eyes except risk factors, whereas the other risk time of grafting. In seven allografts,

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Table 3. Risk factors for failure in penetrating allografts by indication for grafting.

Indications

All I II III IV V VI
Risk factor n = 41 n=7 n=6 n=3 n=6 n = 10 n=9

Regrafting 5 0 1 1 0 2 1
Primary grafts
Other previous surgery 16 0 2 2 1 7 4
Vascularized cornea 8 0 0 1 5 2 0
Iridolenticulocorneal adhesions 6 0 0 0 3 2 1
Opacity extending to the limbus 21 0 5 2 6 4 4
Intraocular inflammation 8 0 0 0 0 5 3
Glaucoma 8 0 0 2 3 2 1
Simultaneous intraocular surgery 18 0 0 2 4 7 5
None 7 7 0 0 0 0 0

The indications are presented as follows: I, keratoconus; II, corneal dystrophy; III, aniridia; IV, congenital opacity; V, traumatic acquired opacity; VI,
non-traumatic acquired opacity.

Table 4. Cox proportional hazards regression of failure of regrafts in the entire penetrating allograft data set and of potential risk factors in the
primary penetrating allograft data set.

Variable Hazard ratio Wald chi-square p 95% CI

Univariate analysis Regraft 5.4 9.4 <0.01 1.8–15.9

Primary graft
Other previous surgery 1.1 0.03 0.85 0.4–3.0
Vascularized cornea 8.1 11.8 0.01 2.5–26.5
Iridolenticulocorneal adhesions 4.3 7.0 0.01 1.5–12.0
Opacity extending to the limbus 3.3 3.4 0.07 0.9–11.8
Intraocular inflammation 1.9 1.3 0.26 0.6–5.4
Glaucoma 2.4 2.5 0.11 0.8–7.0
Simultaneous intraocular surgery 9.7 8.9 <0.01 2.2–43.5
Bivariate analysis Model 1 2 log likelihood = 78.6
Vascularized cornea 6.9 6.7 0.01 1.5–29.9
Iridolenticulocorneal adhesions 1.3 0.1 0.71 0.3–6.0
Model 2 2 log likelihood = 77.5
Vascularized cornea 5.8 7.3 <0.01 1.6–20.5
Opacity extending to the limbus 2.5 1.2 0.28 0.5–12.7
Model 3* 2 log likelihood = 73.1
Vascularized cornea 4.1 5.0 0.03 1.2–13.9
Simultaneous intraocular surgery 5.7 4.6 0.03 1.2–28.6
Model 4 2 log likelihood = 79.4
Simultaneous intraocular surgery 9.5 8.4 <0.01 2.1–43.7
Opacity extending to the limbus 3.6 3.6 0.06 1.0–13.6
Model 5 2 log likelihood = 82.6
Simultaneous intraocular surgery 7.2 6.2 0.01 1.5–34.2
Iridolenticulocorneal adhesions 2.0 1.4 0.23 0.3–6.1
Model 6 2 log likelihood = 86.3
Iridolenticulocorneal adhesions 4.5 6.9 0.01 1.5–13.7
Opacity extending to the limbus 3.4 3.5 0.06 0.9–12.4

*Preferred model.

poor epithelium initiated failure. Four uncertain, unless the absence of ante- Discussion
of these seven were primary grafts: rior chamber reaction suggested the
three had a vascularized cornea preop- latter diagnosis. Compared with most other published
eratively and three had putative limbal Three eyes were lost: one with severe series, indications for paediatric cor-
stem cell insufficiency, that is aniridia, initial penetrating injury and intraocu- neal grafting in our centre were char-
dry eye and chemical injury. In addi- lar foreign body, one with sclerocornea acterized by a larger proportion of
tion, three regrafts presented initially and congenital aphakia that developed traumatic corneal scars, but a lower
with epithelial insufficiency. Differenti- an inoperable retinal detachment and frequency of congenital corneal opaci-
ation between chronic epithelial rejec- one eye with Peters’ anomaly develop- ties and regrafts (Dana et al. 1995;
tion and limbal stem cell insufficiency ing spontaneous iris prolapse in the Patel et al. 2005; Al-Ghamdi et al.
as the reason for failure remained host cornea. 2007; Sharma et al. 2007; Lowe et al.

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whereas 75% of the eyes had that level

in the Australian study. The latter
cohort included patients grafted at the
age of up to 18 years, which may in
part explain the difference in the visual
outcome. On the other hand, grafting
in keratoconus appeared to be success-
ful already in the early period of
grafting as four grafts performed in
1971, 1979, 1980 and 1984 were clear
after 18–29 years. Similar finding with
even longer graft survival up to
37 years was recently reported by
Ruusuvaara et al. (2015). Grafting in
corneal dystrophies was nearly equally
successful despite the significantly
younger mean age at the time of
surgery. The youngest patient in this
group was grafted at the age of 5 years
and obtained excellent visual recovery
from 0.05 to 0.9 with 5 years of follow-
up. Graft survival and favourable
visual outcome in paediatric corneal
dystrophies have been reported also in
larger series (Javadi et al. 2003; Al-
Ghamdi et al. 2007). Therefore, instead
of using the older classification of
indications as proposed by Stulting
et al. (1984), we evaluated keratoconus
Fig. 2. The Kaplan–Meier estimate of graft survival for all grafts (dotted line with squares), the and corneal dystrophies separately
low-risk grafts (continuous line with crosses), regrafts (plain continuous line), grafts undergoing from other non-traumatic acquired
simultaneous intraocular surgery (continuous line with circles) and grafts with vascularized cornea and congenital corneal opacities that
(plain dotted line). The number of grafts at risk in each risk group is presented in the table below. carry an entirely different risk profile as
regards graft failure (Table 3).
Table 5. Types and mean time to graft failures for penetrating allografts. In our cohort, regrafting, simultane-
Grafts Time to failure, months
ous intraocular surgery and vascular-
Failure type n mean (range) ized cornea were the main risk factors
for graft failure. Regrafting and cor-
Regraft rejection 5 4 (1–12) neal vascularization are known to
Endothelial rejection 2 25 (20–29) increase failure rate also in adults (Vail
Epithelial rejection 4 14 (3–46) et al. 1997), and simultaneous intraoc-
Stromal rejection 6 7 (1–12)
ular surgery has been reported to be
Other 3 4 (1–12)
All 20 9 (1–21)
associated with failures in paediatric
keratoplasties (Comer et al. 2001). A
The failures other than rejections included band keratopathy, retinal detachment and iris prolapse. similar pattern of risk factors in paedi-
atric grafting was also identified in a
2011; Hovlykke et al. 2014). In many paediatric corneal grafting and thereby recent Danish study (Hovlykke et al.
of the eyes with injury, grafting was have suggested valuable guidelines 2014). However, unlike in the Aus-
performed to enable intraocular sur- for clinical practice (Yang et al. tralian registry study (Lowe et al.
gery. These differences reflect our con- 1999; Michaeli et al. 2005; Al-Ghamdi 2011), preceding surgery or inflamma-
servative approach to performing et al. 2007; Zaidman et al. 2007; tion did not increase failure rate in our
corneal grafting in children. Finally, Rao et al. 2008; Lowe et al. 2011; cohort. Ninety-three per cent of all
unlike in some other populations Kim et al. 2013). In accordance with failing grafts of our cohort had one of
(Sharma et al. 2007), only one graft these reports, penetrating keratoplas- the three main risk factors. This
was performed for corneal scarring ties for keratoconus had a favourable applied especially to the eyes with
from herpetic keratitis. Whether this outcome also in our cohort. Graft traumatic and non-traumatic acquired
is due to lower incidence of infectious survival was very similar to that corneal opacity, of which 92% and
scars in our population or merely reported in the large Australian Cor- 73%, respectively, had at least one of
reflects different clinical practice neal Graft Registry Study, (Lowe et al. these risk factors. This explains the
remains unknown. 2011) but visual outcome remained modest graft survival in these groups.
Many recent series have reported poorer: half of the eyes in our small Their visual outcome was also worse,
diagnosis-specific data on prognosis of cohort achieved BCVA 0.5 or better because only three of the 18 eyes had a

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BCVA 0.3 or better. Amblyopia or ment optical coherence tomography, categories “hand motion” and “counting fingers”
using Freiburg Visual Acuity Test (FrACT).
posterior segment injury explained which enable more accurate preopera-
Graefes Arch Clin Exp Ophthalmol 247: 137–142.
poor visual acuity when the grafts were tive evaluation (Majander et al. 2012), Limaiem R, Chebil A, Baba A, Ben Youssef N,
clear. Therefore, a thorough evaluation could be applied only to a few patients Mghaieth F & Al Matri L (2011): Pediatric pene-
of the preoperative ocular findings is in our cohort. trating keratoplasty: indications and outcomes.
Transpl Proc 43: 649–651.
important when predicting visual out- In conclusion, our cohort gives a
Lowe MT, Keane MC, Coster DJ & Williams KA
come after grafting in eyes with injury North European view on paediatric (2011): The outcome of corneal transplantation in
or other ocular diseases. On the other keratoplasties, which are rarely per- infants, children, and adolescents. Ophthalmology
hand, preoperative findings suggestive formed in any population and show 118: 492–497.
Majander AS, Lindahl P, Vasara LK & Krootila K
of poor outcome should not entirely highly population-based case mixes.
(2012): Anterior segment optical coherence tomog-
preclude surgical interventions. Some Thorough assessment of risk factors raphy in congenital corneal opacities. Ophthal-
of the high-risk eyes may succeed better and careful patient selection form the mology 119: 2450–2457.
than expected like in one our patient basis for successful grafting and long- McClellan K, Lai T, Grigg J & Billson F (2003):
Penetrating keratoplasty in children: visual and
with severe penetrating injury, who term survival. Such can be obtained
graft outcome. Br J Ophthalmol 87: 1212–1214.
underwent pars plana vitrectomy aided also after paediatric keratoplasty as Michaeli A, Markovich A & Rootman DS (2005):
with an intraoperative keratoprosthesis revealed in this cohort with follow-up Corneal transplants for the treatment of congenital
and lensectomy. The BCVA of this eye times up to 29 years. On the other corneal opacities. J Pediatr Ophthalmol Strabis-
mus 42: 34–44.
was 0.5 at last follow-up 6 years post- hand, a guarded prognosis based on
Panda A, Vanathi M, Kumar A, Dash Y & Priya S
operatively. preoperative assessment should be (2007): Corneal graft rejection. Surv Ophthalmol
The number of grafts associated with weighted case by case with the poten- 52: 375–396.
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McGhee CNJ (2005): The indications and out-
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several grafts had chronically poor preclude grafting in adverse clinical Zealand: 1991-2003. Br J Ophthalmol 89: 404–408.
epithelium, and in seven grafts failure situations such as injury or severe Rao KV, Fernandes M, Gangopadhyay N, Vemu-
was initiated by poor epithelium. developmental disorder in risk of spon- ganti GK, Krishnaiah S & Sangwan VS (2008):
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Whether these represented true epithe- taneous perforation.
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Sharma N, Prakash G, Titiyal JS, Tandon R &
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Primary pediatric keratoplasty: indications, graft Vajpayee RB (2007): Pediatric keratoplasty in
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Stulting RD, Sumers KD, Cavanagh HD, Waring
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CO & Gammon JA (1984): Penetrating kerato-
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Dana MR, Moyes AL, Gomes JA et al. (1995): The
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Vail A, Gore SM, Bradley BA, Easty DL, Rogers CA
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unclear cause of graft failure appears Dua HS, Miri A & Said DG (2010): Contemporary
Vanathi M, Panda A, Vengayil S, Chaudhuri Z &
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to be a common feature of paediatric Experiment Ophthalmol 38: 104–117.
Dada T (2009): Pediatric keratoplasty. Surv Oph-
keratoplasty (Lowe et al. 2011). Erlich CM, Rootman DS & Morin JD (1991):
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Yang LL, Lambert SR, Lynn MJ & Stulting RD
Our data were collected over a 40- Corneal transplantation in infants, children and
(1999): Long-term results of corneal graft survival
year period during which major young adults: experience of the Toronto Hospital
in infants and children with peters anomaly.
for Sick Children, 1979–1988. Can J Ophthalmol
advances were made in surgical tech- 26: 206–210.
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niques, graft storage and understand- Ganesh A, Mai DT & Levin AV (2013): Pediatric
Zaidman GW, Flanagan JK & Furey CC (2007):
Long-term visual prognosis in children after
ing of risk factors for failure (Vail et al. glaucoma terminology. Am J Med Genet A 161A:
corneal transplant surgery for Peters anomaly type
1997; Panda et al. 2007; Zaidman et al. 3205–3215.
I. Am J Ophthalmol 144: 104–108.
Hovlykke M, Hjortdal J, Ehlers N & Nielsen K (2014):
2007; Lowe et al. 2011). Therefore, Clinical results of 40 years of paediatric kerato-
some of the eyes included in our study plasty in a single university eye clinic. Acta Oph-
might be treated differently today with thalmol Scand 92: 370–377.
possibly better outcome. This applies Huang C, O’Hara M & Mannis MJ (2009): Primary
pediatric keratoplasty: indications and outcomes. Received on September 26th, 2015.
especially to the eyes with probably Cornea 282: 1003–1008. Accepted on January 31st, 2016.
impaired limbal stem cell function. Javadi MA, Baradan-Rafii AR, Zamani M, Karim-
Keratoplasty cannot be performed in ian F, Zare M, Einollahi B, Jafarinasab MR & Correspondence:
these eyes without previous surface Yazdani S (2003): Penetrating keratoplasty in Anna Majander, MD
young children with congenital hereditary Department of Ophthalmology
optimization, that is autologous or endothelial dystrophy. Cornea 22: 420–423. Helsinki University Hospital
allogeneic stem cell transplantation, Kim YW, Choi HJ, Kim MK, Wee WR, Yu YS &
P.O. Box 220
which has become widely recognized Oh JY (2013): Clinical outcome of penetrating
Helsinki FI-00029 HUS
only in the last 20 years (Dua et al. keratoplasty in patients 5 years or younger: peters
anomaly versus sclerocornea. Cornea 32: 1432– Finland
2010). In addition, modern partial 1436. Tel: +358405482445
thickness keratoplasties and new imag- Lange C, Feltgen N, Jubker B, Schultze-Bonsel K & Fax: +358947176544
ing technologies, such as anterior seg- Bach M (2009): Resolving the clinical acuity Email: anna.majander@hus.fi

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