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My Clouding Cornea
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CONGENITAL CLOUDING OF CORNEA
Causes and Management
Presenter : Dr. RujutaModerator : Dr. Monica, Dr. Sangit
DEFINITIONThe term “congenital” refers to any condition
that is present in the newborn.“Clouding” refers to loss of transparency.
“STUMPED” ClassificationS – SclerocorneaT – Tears in descemet’s membrane
Congenital GlaucomaBirth trauma
U – UlcerHerpes simplex virusBacterialNeurotrophic
M – Metabolic (rarely present at birth)MucopolysaccharidosesMucolipidosesTyrosinosis
Classification continued…P – Posterior corneal defect
Peter’s anomalyPosterior keratoconusStaphyloma
E – Endothelial dystrophyCongenital hereditary endothelial dystrophyStromal : Congenital hereditary stromal dystrophy
D- Dermoid
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.
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
SCLEROCORNEA continued…ISOLATED PERIPHERAL
SCLEROCORNEA:Abrupt change from scleral-like tissue to clear cornea without any other ocular abnormalities.
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
SCLEROCORNEA continued…TOTAL SCLEROCORNEA:
Most common form causing congenital corneal opacity.
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
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
TEARS IN DM : CONGENITAL GLAUCOMA
CONGENITAL GLAUCOMA continued…
Cause :Sporadic
ORMutation in CYP1B1 gene on Chr 2p21
CONGENITAL GLAUCOMA continued…
SIGNS : Raised IOP Corneal enlargement and clouding Corneal diameter >10-10.5mm Optic nerve cupping Increased axial length Gonioscopic abnormalities
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
CONGENITAL GLAUCOMA continued…
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
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.
CONGENITAL GLAUCOMA continued…
OUTCOME OF SURGERY :Primary combined trabeculotomy-
trabeculectomy is safe and effective for developmental glaucoma when performed within 6 months of birth. It leads to excellent IOP control and good visual outcome.*
*Mandal AK, Bhatia PG, Bhaskar A, Nutheti R. Long-term surgical and visual outcomes in Indian children with developmental glaucoma operated on within 6 months of birth. Ophthalmology. 2004 Feb;111(2):283-90.
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.
TEARS IN DM : BIRTH TRAUMACause : Placement of forceps blade across
the globe and orbit during delivery leading to rupture of Descemet’s membrane
Associated soft tissue injury: Unilateral periorbital oedema and ecchymoses
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
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
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
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
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
ULCERSHerpes simplex virusRubellaBacterialNeurotrophic
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
ULCER – HSV continued…
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
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.
ULCER – HSV continued…MANAGEMENT : Prophylactic : Neonate : Intravenous acyclovir treats
systemic disease, but therapeutic levels are also achieved in aqueous and tears.
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
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
ULCER – RUBELLA continued…MANAGEMENT : Isolated opacification resolves spontaneously.If persists, penetrating keratoplasty can be
performed.
ULCER – BACTERIAL continuedETIOLOGY IS MULTIFACTORIALExposure to many bacteria in the birth canalDuration of exposureIntegrity of ocular surfaceAdequacy of antibiotic prophylaxis
ULCER – BACTERIAL continuedGONORRHOEAL OPHTHALMIA
NEONATORUM :
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.
ULCER – BACTERIAL continuedTETRACYCLINE AND POVIDONE IODINE : Tetracycline ointment 1% was found to be
marginally more effective against infective ON than povidone iodine solution 2.5%. For these reasons, tetracycline, rather than povidone iodine, is recommended for prevention of ON.*
* David M, Rumelt S, Weintraub Z. Efficacy comparison between povidone iodine 2.5% and tetracycline 1% in prevention ofophthalmia neonatorum. Ophthalmology. 2011 Jul;118(7):1454-8. Epub 2011 Mar 25.
ULCER – BACTERIAL continuedMANAGEMENT :Systemic treatment with Penicillin GSaline irrigation of fornicesBeta lactamase producing N. gonorrhoeae
- Intravenous cefotaximePseudomonas – Fortified gentamicin or
tobramycin dropsChlamydia – Systemic erythromycin
ULCER - NEUROTROPHIC
Deficient corneal innervation
Decreased tearing, Decreased corneal sensation
Sterile corneal ulceration
Familial dysautonomia : Generalized dysfunction of the autonomous nervous system
ULCER - NEUROTROPHICMANAGEMENT : Topical lubrication with preservative-free
artificial tears, gels, and ointmentsAmniotic membrane grafting :
The success rate of AMG in the patients with neurotrophic ulcer was found to be 93.3%*
* Park JH, Jeoung JW, Wee WR, Lee JH, Kim MK, Lee JL. Clinical efficacy of amniotic membrane transplantation in the treatment of various ocular surface diseases. Cont Lens Anterior Eye. 2008 Apr;31(2):73-80. Epub 2008 Jan 30.
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
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
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.
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.
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
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.
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.
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.
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.
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
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
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.
POSTERIOR POLYMORPHOUS DYSTROPHY
Similar to CHED with AD inheritance.
Cause- Primary dysfunction of corneal endothelium.
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.
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
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
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
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.
PK for CONGENITAL CORNEAL OPACITIES :
Indications:Bilateral congenital opacitiesControlled glaucoma with opacificationChronic corneal oedema
Relative contraindications:Poor cooperation from parentsAmblyopiaPerforationNormal fellow eye
PK for CONGENITAL CORNEAL OPACITIES :
Contraindications : Uncontrolled glaucomaInfectionPoor tear secretion
Reference : From Pediatric Ophthalmology and Strabismus By Kenneth Weston Wright
PK for CONGENITAL CORNEAL OPACITIES :
OUTCOME OF SURGERY IN INFANTS :First graft survival at 12 months was 61% Peter's anomaly, lensectomy, and repeat
penetrating keratoplasty were factors most highly associated with poor graft survival and a low final visual acuity.*
*Comer RM, Daya SM, O'Keefe M. Penetrating keratoplasty in infants.J AAPOS. 2001 Oct;5(5):285-90.
PK for CONGENITAL CORNEAL OPACITIES :
OUTCOME OF SURGERY IN INFANTS :Surgery must be performed early to avoid
amblyopia. Prompt postoperative optical rehabilitation, combined with occlusion therapy when appropriate, is an important determinant of success.*
*Reidy JJ. Penetrating keratoplasty in infancy and early childhood. Curr Opin Ophthalmol. 2001 Aug;12(4):258-61.
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.
Thankyou!