NEWBORN WITH BILATERAL HAZY CORNEAS
BLEYEN I.* BARTELS M. C.*WOLFS R.C.W.*
In this case report we present a preterm born babywith bilateral hazy corneas and initially normal in-traocular pressures. After birth, the corneal opacifi-cation increased and a progressive buphthalmos be-came evident in the right eye. A trabeculectomy wasperformed in the right eye. Our final diagnosis wassclerocornea in combination with Peters' anomaly.
Nous présentons une prématurée avec deux cornéesopaques et des tensions intra-oculaires initiallementnormales. Après la naissance, la cornée s'est opa-cifiée encore plus et l'oeil droit a développé une buph-talmie progressive. Une trabéculectomie a été pra-tiquée à l'oeil droit. Notre diagnostic final est unesclérocornée combinée avec une anomalie de Pe-ters.
Peters' anomaly, sclerocornea, buphthalmos,corneal opacification, intraocular pressure
Anomalie de Peters, sclérocornée, buphtalmie,opacification de la cornée, tension intra-oculaire
* Erasmus MC, University Hospital, Rotterdam
received: 04.09.06accepted: 21.10.06
Bull. Soc. belge Ophtalmol., 303
, 29-32, 2007.
On the second day after birth, a 28 weeks oldpreterm baby (46XX, weight of 935 grams andApgar score at birth of 3/4/6) was presentedto the ophthalmologist because of bilateral hazycorneas. The mother (G4, P0, A3) suffered fromschizophrenia and was known to use Haldoland cannabis during this pregnancy. There wasno known malnutrition. The father had also apsychiatric history and suffered from bilateralcongenital cataract. There was no anterior seg-ment dysgenesis described in either parent.
Sclerocornea with also central opacity caused by
Clinical examination of the preterm was nor-
Peters'anomaly. Here the limbus is better recognizable, pro-
mal except for the bilateral hazy corneas. Ex-
bably because of the older age of the patient.
tensive screening for metabolic disorders andinfections was negative. Toxic screening wasnegative for alcohol but positive for cannabis.
Genetic examination could not yet be performeddue to legal issues.
Peters'anomaly, sclerocornea and congenital
During the clinical ophthalmological examina-
endothelial dystrophy represent mesenchymal
tion the anterior chambers appeared normal
dysgeneses of the cornea. In sclerocornea there
and the lenses were presumably clear. An echo
is a peripheral, white, vascularized corneal rim
B scan examination showed an attached reti-
that blends with sclera obliterating the limbus.
na bilaterally. Retinopathy of prematurity could
The central cornea is generally normal. In total
not be assessed due to the hazy corneas. It was
sclerocornea, the entire cornea is involved with
not possible to measure the intraocular pres-
a clearer central part. The opacification affects
sure with a Tonopen, but intraocular pressure
the full thickness of the stroma. Potentially co-
was initially normal on palpation in both eyes.
existing abnormalities include shallow anterior
The central corneal opacification increased slight-
chamber, iris abnormalities and microphthal-
ly and 4 weeks after birth the left cornea showed
mos. Systemic abnormalities include limb de-
a central perforation with iris prolaps. This was
formities, craniofacial and genitourinary de-
treated conservatively with chloramphenicol
fects (1, 9).
ointment and drops. Two weeks later the in-
In Peters'anomaly there is a congenital central
traocular pressure had increased on palpation
corneal opacity with corresponding defect in the
in both eyes, more in the right than the left, and
posterior stroma, Descemet's membrane and
a progressive buphthalmos became evident in
endothelium. The cornea is rarely vascularized
the right eye. The left eye was treated with
and usually clear at the periphery, although
timolol 0,1%. At the age of 6 1/2 weeks a tra-
scleralization of the limbus is common. Other
beculectomy was performed in the right eye. No
associated anterior segment abnormalities in-
defined limbus could be seen and a smooth
clude glaucoma (50-70% of cases), anterior
continuation between sclera and cornea made
polar cataract and less often microcornea, mi-
the exact measurement of the corneal diame-
crophthalmos, cornea plana, sclerocornea,
ter difficult (Figure 1). After the iridectomy a
colobomata and mesodermal dysgenesis of the
small vitreous prolaps was removed with Van-
angle and iris (3). Developmental mechanisms
nas scissors. Postoperatively the intraocular
include faulty separation of the lens vesicle from
pressure remained stable around 14 mmHg in
the surface ectoderm, primary abnormal mi-
both eyes. Both corneas remained hazy, but the
gration of neural crest cells into the cornea and
patient showed signs of searching for light. Our
intrauterine corneal inflammation (4, 12).
final diagnosis was sclerocornea in combina-tion with Peters' anomaly.
Table 1. Previously mapped loci and genes known to cause anterior segment anomalies (2,4,10).
Posterior polymorphous corneal dystrophy
Primary congenital glaucoma
in humans and the numerous possible effectson ocular development. Cannabis, however, is
Development of the anterior segment of the eye
described as an unlikely teratogen (11).
is a complex process that depends on multiple
Anterior segment morphogenesis appears to be
inductive events and coordinated interactions
particularly sensitive to deviations in expres-
between cells of ectodermal, neuroectodermal,
sion levels of the regulatory genes on which it
and neural crest origin. The neural crest is a
depends. Mutations in a number of transcrip-
specialized population of mesenchymal cells
tion factor genes - all of which are involved in
that emigrates from the dorsal margin of the
the control of developmental processes in oth-
neural folds at the time of neural tube closure.
er organs as well - cause congenital anterior
Cranial neural crest cells migrate and differen-
segment malformations in the heterozygous
tiate into various ocular tissues, such as the
state. Genetic defects causing many of the an-
corneal endothelium and stroma, the iris stro-
terior segment disorders have been mapped to
ma, the trabecular meshwork and the ciliary
various chromosomal regions as shown in Ta-
body stroma (7). It has been found that the cra-
ble 1 (4, 6).
nial neural crest is especially vulnerable to ter-atogens and that the same malformation can
be caused by many different agents. Strom-land et al.
describe various ocular teratogens
Peters' anomaly is usually seen as an isolatedocular defect, but associated ocular and sys-temic anomalies are described (Table 2). Most-
Table 2. Associated ocular and systemic anomalies (8, 5).
ly, the associated ocular and systemic anoma-lies in patients with Peters' anomaly are relat-
ed to maldevelopment of the neural crest cells.
Early treatment for those systemic anomalies
Conotruncal anomalies of
Ozeki et al. revealed that Peters'anomaly, with
corneolenticular adhesion, other ocular anom-
Persistent fetal vasculature
Central nervous system ano-
alies or glaucoma, was accompanied by sys-
temic anomalies more frequently than not. There-
Optic nerve staphyloma
fore, these cases especially need to be evalu-
ated for the presence of systemic anomalies (8).
MacroglossiaLimb malformationsJoint laxity
Surgical management and
blyopia in bilateral Peters anomaly. J Am Ass
Pediatr Ophthalmol Strabismus.
Surgical management is mainly focused on cor-
(6) ORMESTAD M., BLIXT A., CHURCHILL A.,
neal transplantation and glaucoma surgery.
MARTINSSON T., ENERBACK S., CARLSSON
Yang et al. showed that long-term graft clarity
P. − Foxe3 haploinsufficiency in mice: a model
could be achieved in 36% of eyes, 93% of which
for Peters' anomaly. Invest Ophthalmol Vis Sci.
were first grafts. Surgical intervention (one or
more procedures) is effective in controlling IOP
(7) OZEKI H., SHIRAI S. − Developmental eye ab-
in 32% of eyes with associated congenital glau-
normalities in mouse fetuses induced by reti-noic acid. Jpn J. Ophthalm.
1998; 42: 162-
coma, often requiring adjunctive medical ther-
(8) OZEKI H., SHIRAI S., NOZAKI M., SAKURAI
Visual outcome is guarded in children with
E., MIZUNO S., ASHIKARI M., MATSUNAGA
Peters'anomaly. This may be explained, in part,
N., OGURA Y. − Ocular and systemic features
by the high incidence of postoperative compli-
of Peters' anomaly. Graefes Arch Clin Exp Oph-
cations. Pervasive neurologic impairment - brain
abnormalities, developmental delay, mental re-
(9) REZENDE R.A., UCHOA U.B., UCHOA R., RA-
tardation, and other types of cognitive dysfunc-
PUANO C.J., LAIBSON P.R., COHEN E.J. − Con-
tion - may also play an influential role in deter-
genital corneal opacities in a cornea referral
mining visual results. Furthermore, both ante-
(10) SEMINA E.V., BROWNELL I., MINTZ-HITTNER
rior and posterior segment pathology and sen-
H.A., MURRAY J.C., JAMRICH M. − Mutations
sory aberrations - strabismic, anisometropic,
in the human forkhead transcription factor
and deprivational amblyopia - may also play a
FOXE3 associated with anterior segment ocu-
role as well as the inability of examiners to fol-
lar dysgenesis and cataracts. Human Molecu-
low refractions, to institute refractive correc-
. 2001; 10: 231-236.
tion, and to enforce compliance (14).
(11) STROMLAND K., MILLER M., COOK C. − Ocu-
lar teratology. Surv. ophthalm.
1991; 35: 429-
(12) WARING G.O., RODRIGUES M.M. − Congeni-
(1) BHAT Y.R., SANOJ K.M. − Sclerocornea. In-
tal and neonatal corneal abnormalities. In: Tas-
Mar 2005; 42:277.
man W, Jaeger EA, eds. Foundations of clini-
(2) HONKANEN R.A., NISHIMURA D.Y., SWIDERS-
. Vol 1, Chap 9. Philadel-
KI R.E., BENNETT S.R., HONG S., KWON Y.H.,
phia: Lippincott, 1993; 1-38.
STONE E.M., SHEFFIELD V.C., ALWARD W.L.M.
(13) YANG L.L., LAMBERT S.R. − Peters' anomaly.
− A family with Axenfeld-Rieger syndrome and
A synopsis of surgical management and visual
Peters Anomaly caused by a point mutation
outcome. Ophthalmol Clin North Am.
(Phe112Ser) in the FOXC1 gene. Am J Opht-
(14) YANG L.L., LAMBERT S.R., LYNN M.J., STUL-
(3) KENYON K.R. − Mesenchymal dysgenesis in
TING R.D. − Surgical management of glauco-
Peters' anomaly, sclerocornea and congenital
ma in infants and children with Peters' anoma-
endothelial dystrophy. Exp Eye Res.
ly: long-term structural and functional outco-
(4) MATSUBARA A., OZEKI H., MATSUNAGA N.,
NOZAKI M., ASHIKARI M., SHIRAI S., OGU-RA Y. − Histopathological examination of two
cases of anterior staphyloma associated with
Correspondence and reprints
Peters' anomaly and persistent hyperplastic pri-mar y vitreous. Br J Ophthalmol.
Belgielei 95 -97
(5) NAJJAR D.M., CHRISTIANSEN S.P., BOTHUN
E.D., SUMMERS C.G. − Strabismus and am-
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