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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.
Figure 1. 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). Chromosomal linkage Axenfeld-Rieger Anomaly Axenfeld-Rieger Syndrome 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 is essential.
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- Urogenital anomalies 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. 2006; 10:193-197.
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 2002; 43:1350-1357.
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 thalmol. 2000; 238:833-839.
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- practice. Cornea. 2004; 23:565-570.
(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- lar Genetics. 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- dian Pediatr. Mar 2005; 42:277.
man W, Jaeger EA, eds. Foundations of clini- (2) HONKANEN R.A., NISHIMURA D.Y., SWIDERS- cal ophthalmology. 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. 2001; (Phe112Ser) in the FOXC1 gene. Am J Opht- halmol. 2003; 135:368-375.
(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. 1975; ly: long-term structural and functional outco- me. Ophthalmology. 2004; 111:112-117.
(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. 2001; Isabel BLEYEN Belgielei 95 -97 (5) NAJJAR D.M., CHRISTIANSEN S.P., BOTHUN B-2018 Antwerpen E.D., SUMMERS C.G. − Strabismus and am-

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