my clouding cornea

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CONGENITAL CLOUDING OF CORNEA Causes and Management Presenter : Dr. Rujuta Moderator : Dr. Monica, Dr. Sangit

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Page 1: My Clouding Cornea

CONGENITAL CLOUDING OF CORNEA

Causes and Management

Presenter : Dr. RujutaModerator : Dr. Monica, Dr. Sangit

Page 2: My Clouding Cornea

DEFINITIONThe term “congenital” refers to any condition

that is present in the newborn.“Clouding” refers to loss of transparency.

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“STUMPED” ClassificationS – SclerocorneaT – Tears in descemet’s membrane

Congenital GlaucomaBirth trauma

U – UlcerHerpes simplex virusBacterialNeurotrophic

M – Metabolic (rarely present at birth)MucopolysaccharidosesMucolipidosesTyrosinosis

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Classification continued…P – Posterior corneal defect

Peter’s anomalyPosterior keratoconusStaphyloma

E – Endothelial dystrophyCongenital hereditary endothelial dystrophyStromal : Congenital hereditary stromal dystrophy

D- Dermoid

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INCIDENCEThe most common primary cause of

congenital corneal abnormalities was *Peters anomaly (40.3%), followed by Sclerocornea (18.1%) Dermoid (15.3%)Congenital glaucoma (6.9%)Birth trauma, and metabolic disease (2.8%)

* Rezende RA, Uchoa UB, Uchoa R, Rapuano CJ, Laibson PR, Cohen EJ. Congenital corneal opacities in a cornea referral practice. Cornea. 2004 Aug;23(6):565-70.

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SCLEROCORNEASclerocornea is a

primary anomaly in which scleralization of the peripheral part of cornea, or of the entire tissue, occurs.

A type of mesodermal dysgenesis

Non progressive, usually bilateral, asymmetric

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SCLEROCORNEA continued…ISOLATED PERIPHERAL

SCLEROCORNEA:Abrupt change from scleral-like tissue to clear cornea without any other ocular abnormalities.

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SCLEROCORNEA continued…SCLEROCORNEA PLANA:

Flat cornea with high hyperopia. Pseudoptosis can be seen because the flat cornea poorly supports the upper lid.

SCLEROCORNEA ASSOCIATED WITH ANTERIOR CHAMBER CLEAVAGE ANOMALIES

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SCLEROCORNEA continued…TOTAL SCLEROCORNEA:

Most common form causing congenital corneal opacity.

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SCLEROCORNEA continued…HISTOPATHOLOGY : Precise arrangement of stromal lamellae

absentDiameter of collagen fibrils is increased

upto 1500A resembling that of scleral fibrils

Stromal vascularisation present

Normal cornea Abnormal cornea

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SCLEROCORNEA continued…INVESTIGATIONS : UBM for Diagnosis

Identifying potential structural abnormalities

Surgical planning by identification of pupil

MANAGEMENT : Unilateral disorder: Surgery can be

performed only if other ocular structures are normal

Bilateral disorder: Penetrating keratoplasty

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TEARS IN DM : CONGENITAL GLAUCOMA

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CONGENITAL GLAUCOMA continued…

Cause :Sporadic

ORMutation in CYP1B1 gene on Chr 2p21

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CONGENITAL GLAUCOMA continued…

SIGNS : Raised IOP Corneal enlargement and clouding Corneal diameter >10-10.5mm Optic nerve cupping Increased axial length Gonioscopic abnormalities

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CONGENITAL GLAUCOMA continued…

PATHOPHYSIOLOGY : Raised IOP

Rapid enlargement

of eye

Stretching of cornea

Breaks in Descemet’s membrane

Endothelial barrier is disturbed

Corneal oedema and

clouding

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CONGENITAL GLAUCOMA continued…

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CONGENITAL GLAUCOMA continued…

MANAGEMENT : Goniotomy and Trabeculotomy

Success rate of 80% in infantile glaucoma, esp. when performed between 1st month and one year of age

if failedTrabeculecomy/ Shunt procedures

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CONGENITAL GLAUCOMA continued…

OUTCOME OF SURGERY :Primary combined trabeculotomy-

trabeculectomy offers a viable surgical option in infants that have cloudy corneas at birth as a result of congenital glaucoma. It is associated with a favourable visual outcome and a low rate of anaesthetic complications in an Indian population. *

*Mandal AK, Gothwal VK, Bagga H, Nutheti R, Mansoori T. Outcome of surgery on infants younger than 1 month with congenital glaucoma. Ophthalmology. 2003 Oct;110(10):1909-15.

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CONGENITAL GLAUCOMA continued…

OUTCOME OF SURGERY :Simultaneous bilateral primary combined

trabeculotomy-trabeculectomy is safe and effective for developmental glaucoma. It obviates the need for long second anaesthesia with its attendant risks.*

* Mandal AK, Bhatia PG, Gothwal VK, Reddy VM, Sriramulu P, Prasad MS, John RK, Nutheti R, Shamanna BR. Safety and efficacy of simultaneous bilateral primary combined trabeculotomy-trabeculectomy for developmental glaucoma. Indian J Ophthalmol. 2002 Mar;50(1):13-9.

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BIRTH TRAUMA continued…Acute elevation in IOP

Globe distends

Exceeds elasticity of Descemet’s membrane

Descemet’s tears

Cornea imbibes aqueous

Stromal and epithelial oedema

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BIRTH TRAUMA continued…

Endothelium resurfaces the posterior cornea

Synthesizes a new thick basement membrane

Fills in the gap due to tears

Corneal oedema disappears in weeks to months

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BIRTH TRAUMA CONGENITAL GLAUCOMANormal IOP High IOPNormal corneal diameter Large corneal diameter with

buphthalmosCorneal oedema in the immediate postpartum period

Corneal oedema weeks to months after birth

Corneal oedema clears after weeks to months

Corneal oedema clears after lowering IOP

Tears in DM are vertical or oblique

Tears in DM are horizontal or concentric to limbus

Left eyes are more frequently affected and other soft tissue injuries may accompany the trauma

No preference for either eye

Usually no photophobia Photophobia

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BIRTH TRAUMA continued…MANAGEMENT : 1st choice of treatment is RGP contact lens

and patchingRefraction for glasses or contact lens should

be performed as soon as possible > prevents amblyopia secondary to severe astigmatism

Patching is necessary to treat amblyopiaLater in life, if endothelium decompensates,

penetrating keratoplasty to restore vision

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BIRTH TRAUMA continued…MANAGEMENT AT A LATER AGE:Sub-Bowman keratomileusis for high

cylindrical error secondary to birth trauma-related Descemet’s scars appears to have a stable, safe and effective follow-up over 1 year. However, longer follow-up and more cases are required to conclusively predict the usefulness of this procedure.*

*Amar Agarwal. Case study: Obstetric forceps injury leads to Descemet’s membrane scar. Sub-Bowman keratomileusis was utilized in a 30-year-old man who had high cylindrical error secondary to birth trauma-related Descemet’s scars. Ocular Surgery News U.S. Edition, March 10, 2011

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ULCERSHerpes simplex virusRubellaBacterialNeurotrophic

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ULCER - HSVNEONATAL OCULAR HSV INFECTION:

This diagnosis must be considered in any newborn with conjunctivitis or keratitis.

Risk factors : History of genital herpes in the mother

Use of a fetal scalp monitorOcular manifestations : within 2 days to 2

weeks of life

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ULCER – HSV continued…

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ULCER – HSV continued…DIAGNOSIS : Scrapings from the cornea or conjunctiva

may demonstrate the virus by flourescein antibody or peroxidase antibody staining

Electron microscopy can detect virus particles in tears

Immunologic testing using commercial kitsAcute and convalescent serum titers to

confirm primary infection

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ULCER – HSV continued…MANAGEMENT : Prophylactic : Mother with genital herpes : Cesarean

delivery and limiting the use of invasive monitors at the time of labor.*Antiviral treatment using acyclovir, penciclovir, valacyclovir, and famciclovir in third trimester prior to delivery**

*Brown ZA, Wald A, Morrow RA, et al. Effect of serologic status and cesarean delivery on transmission rates of herpes simplex virus from mother to infant. JAMA 2003; 289:203.**Hollier LM, Wendel GD. Third trimester antiviral prophylaxis for preventing maternal genital herpes simplex virus (HSV) recurrences and neonatal infection. Cochrane Database of Systematic Reviews 2008, Issue 1. Art. No.: CD004946. 

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ULCER – HSV continued…MANAGEMENT : Prophylactic : Neonate : Intravenous acyclovir treats

systemic disease, but therapeutic levels are also achieved in aqueous and tears.

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ULCER – HSV continued…Additional topical therapy : Trifluorothymidine 5-9 times/dayVidarabine or Acyclovir ointment (Idoxuridine

is not effective)Skin lesions : Warm compresses,

Topical acyclovir ointment Topical antibiotic ointments like

bacitracin, erythromycinMonitor for CNS or disseminated disease

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ULCER – RUBELLACONGENITAL RUBELLA :Uncommon cause of congenital corneal

opacityIt is acquired by the fetus transplacentally

during 1st trimester of gestationDiagnosis : History

Typical visceral, radiographic anomalies

Viral cultures of the throat, urine, secretions

Confirmatory test : Rubella specific IgM in the cord serum

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ULCER – RUBELLA continued…MANAGEMENT : Isolated opacification resolves spontaneously.If persists, penetrating keratoplasty can be

performed.

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ULCER – BACTERIAL continuedIn developing countries the prevalence of

gonorrhoea in pregnant women is between 3% and 15%. The rate of transmission from mother to newborn is between 30% and 50%.*

Prophylaxis by instillation immediately after birth of either 1% silver nitrate eye drops or 1% tetracycline eye ointment is very effective. This reduces the GCON incidence by 80% to 95%* *M. Laga, A. Meheus, and P. Piot. Epidemiology and

control of gonococcal ophthalmia neonatorum. Bull World Health Organ. 1989; 67(5): 471–477.

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ULCER – BACTERIAL continuedMANAGEMENT :Systemic treatment with Penicillin GSaline irrigation of fornicesBeta lactamase producing N. gonorrhoeae

- Intravenous cefotaximePseudomonas – Fortified gentamicin or

tobramycin dropsChlamydia – Systemic erythromycin

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ULCER - NEUROTROPHIC

Deficient corneal innervation

Decreased tearing, Decreased corneal sensation

Sterile corneal ulceration

Familial dysautonomia : Generalized dysfunction of the autonomous nervous system

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METABOLIC DISEASESMUCOPOLYSACCHARIDOSIS :Inherited lysosomal enzyme deficiencies

leading to accumulation of GAGs in the cells and extracellular matrix of the cornea.

I-H: Hurler VI: Maroteaux

Lamy

I-S: Sheie IV: Morquio

AR AR AR AR

Severe clouding within 1st few years

Severe clouding within 1st few years

Corneal clouding from birth, slowly progresses

Corneal clouding after 10 years of age

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METABOLIC DISEASESMUCOLIPIDOSIS :Neuraminidase deficiency resulting in

accumulation of sphingolipids, glycolipids, and acid mucopolysaccharides .

4 types : Type I, II, III, IV out of which type IV causes the most severe corneal clouding

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PETER’S ANOMALYMost common congenital opacity requiring

penetrating keratoplastyIt is a congenital disorder characterised by

central corneal opacity with corresponding defects in posterior stroma, Descemet’s membrane and endothelium.

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PETER’S ANOMALY continued..Peripheral cornea is

relatively clear.Synechiae extend from iris

collarette to the edge of he posterior corneal defect.

Associated with lenticular abnormalities like cataract, corneolenticular adhesions.

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PETER’S ANOMALY ClassificationType I Corneal Opacity + Iridocorneal Adhesions

Type II Corneal Opacity + Iridocorneal Adhesions + Lens

abnormalityUnilateral involvement Frequently bilateralCentral opacity bordered by iris strands that cross the AC from iris collarette

Dense opacity with lens directly adherent to posterior corneal surface

Peripheral cornea usually clearLens usually clear May be clear or cataractousGood visual acuity potentialSystemic abnormalities uncommon

Severe ocular and systemic malformations

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PETER’S ANOMALY continued..CAUSES : Incomplete central migration of the neural

crest cellsTeratogenic exposures, like intrauterine

infection, and maternal alcoholism

Incomplete development of angle is common, leading to glaucoma.

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PETER’S ANOMALY continued..MANAGEMENT : If the anomaly is bilateral and visually

disturbing, corneal transplantation is often required.

Additional surgical procedures including lensectomy, vitrectomy, total iridectomy, regrafting, glaucoma surgery are required. But they worsen the prognosis.

Hence if peripheral cornea is clear, and there is no cataract, a peripheral iridectomy can be done to create a new visual axis.

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POSTERIOR KERATOCONUSVery uncommonLocal conical internal

protrusion of posterior corneal curvature with concomitant stromal thinning and haze.

Non progressive disorder. Cause unclear.

Vision is usually acceptable; keratoplasty is rarely indicated.

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CONGENITAL ANTERIOR STAPHYLOMA

Protuberant congenital corneal opacity.Secondary epithelial metaplasia into

keratinized, stratified squamous epithelium occurs.

Cause : Probably secondary to an intrauterine infection or related to developmental abnormality such as Peter’s anomaly.

MANAGEMENT : Visual prognosis is poor. Enucleation or Evisceration

may be considered for cosmesis.

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CONGENITAL HEREDITARY ENDOTHELIAL DYSTROPHY

Rare disease with AD or AR inheritance.AD CHED mapped to pericentromeric

region of chr20.AR CHED also mapped to same gene but

different locus.AD form does not cause congenital corneal

opacification. AR form generally presents as bilateral

corneal clouding at birth

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CHED continued… PATHOGENESIS :

Primary dysfunction and degeneration of endothelium

Increased permeability

Diffuse corneal oedema and clouding

Abnormal and accelerated Descemet’s secretion

Thickening of DM

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CHED continued…DIAGNOSIS : Differentiating CHED from congenital

glaucoma is difficult because measurement of IOP may give unreliable results in presence of stromal oedema.

It is important to distinguish between CHED and PPMD because the oedema in PPMD may show clearing so that PK may not be needed.

MANAGEMENT : Penetrating keratoplasty to avoid

amblyopia.

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PPMD continued…Epithelialization of

endothelial cells. Features range from mild

endothelial defects to severe stromal and epithelial oedema.

MANAGEMENT : PK if cornea does not clear

spontaneously.

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CONGENITAL HEREDITARY STROMAL DYSTROPHY

Central, bilateral and symmetric

Cause : Corneal lamellar irregularities in the anterior stroma

Stationary from birth

MANAGEMENT : PK if opacification is

severe

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DERMOIDDermoids are solid benign congenital tumors

that frequently arise at the inferotemporal corneoscleral junction.

Classified as choristomas because they contain cellular elements not normally present in that location.

Genetically mapped to ChrXq24-qter

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DERMOIDGrade I Grade II Grade III

Most frequent RarestSmall (5mm) LargerSingleInferotemporal limbus

Entire corneal surface

Superficial Variable depth of stromal extension

Entire anteror segment is involved

33% a/w Goldenhar syndrome

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DERMOIDMANAGEMENT : Correction of astigmatism with spectaclesPatching to treat amblyopiaSURGERY : Complete excision flush with corneal surface,

with lamellar graftIf central cornea is involved, penetrating and

lamellar keratoplasty are vision restoring procedures.

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PK for CONGENITAL CORNEAL OPACITIES :

Indications:Bilateral congenital opacitiesControlled glaucoma with opacificationChronic corneal oedema

Relative contraindications:Poor cooperation from parentsAmblyopiaPerforationNormal fellow eye

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PK for CONGENITAL CORNEAL OPACITIES :

Contraindications : Uncontrolled glaucomaInfectionPoor tear secretion

Reference : From Pediatric Ophthalmology and Strabismus  By Kenneth Weston Wright

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CHALLENGES IN MANAGEMENTRecurrent examination, suture removal is

difficult in childrenRisk of anaesthesia for repeated surgeriesStimulus deprivation amblyopiaGlaucoma

TAKE HOME MESSAGEThe prognosis is less than optimum.

Parents need to be counselled properly to avoid psychological impact.

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Thankyou!