5. INCIDENCE
The 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.
6. SCLEROCORNEA
Sclerocornea 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
7. SCLEROCORNEA continued…
ISOLATED PERIPHERAL SCLEROCORNEA:
Abrupt change from
scleral-like tissue to
clear cornea without
any other ocular
abnormalities.
8. 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
10. SCLEROCORNEA continued…
HISTOPATHOLOGY :
Precise arrangement of stromal lamellae absent
Diameter of collagen fibrils is increased upto
1500A resembling that of scleral fibrils
Stromal vascularisation present
Normal cornea Abnormal cornea
11. 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
17. 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 failed
Trabeculecomy/ Shunt procedures
18. 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.
19. 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.
20. 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.
21. TEARS IN DM : BIRTH TRAUMA
Cause : 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
22. 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
23. 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
24. BIRTH TRAUMA CONGENITAL GLAUCOMA
Normal IOP High IOP
Normal corneal diameter Large corneal diameter with
buphthalmos
Corneal 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
25. BIRTH TRAUMA continued…
MANAGEMENT :
1st choice of treatment is RGP contact lens and
patching
Refraction for glasses or contact lens should be
performed as soon as possible > prevents amblyopia
secondary to severe astigmatism
Patching is necessary to treat amblyopia
Later in life, if endothelium decompensates,
penetrating keratoplasty to restore vision
26. 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
28. ULCER - HSV
NEONATAL 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 monitor
Ocular manifestations : within 2 days to 2 weeks of
life
30. 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 kits
Acute and convalescent serum titers to confirm
primary infection
31. 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.
32. ULCER – HSV continued…
MANAGEMENT :
Prophylactic :
Neonate : Intravenous acyclovir treats systemic
disease, but therapeutic levels are also achieved in
aqueous and tears.
33. ULCER – HSV continued…
Additional topical therapy :
Trifluorothymidine 5-9 times/day
Vidarabine or Acyclovir ointment (Idoxuridine is not
effective)
Skin lesions : Warm compresses,
Topical acyclovir ointment
Topical antibiotic ointments like
bacitracin, erythromycin
Monitor for CNS or disseminated disease
34. ULCER – RUBELLA
CONGENITAL RUBELLA :
Uncommon cause of congenital corneal opacity
It is acquired by the fetus transplacentally during 1st
trimester of gestation
Diagnosis : History
Typical visceral, radiographic anomalies
Viral cultures of the throat, urine, secretions
Confirmatory test : Rubella specific IgM in the cord
serum
35. ULCER – RUBELLA continued…
MANAGEMENT :
Isolated opacification resolves spontaneously.
If persists, penetrating keratoplasty can be performed.
37. ULCER – BACTERIAL continued
ETIOLOGY IS MULTIFACTORIAL
Exposure to many bacteria in the birth canal
Duration of exposure
Integrity of ocular surface
Adequacy of antibiotic prophylaxis
39. ULCER – BACTERIAL continued
In 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.
40. ULCER – BACTERIAL continued
TETRACYCLINE 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.
41. ULCER – BACTERIAL continued
MANAGEMENT :
Systemic treatment with Penicillin G
Saline irrigation of fornices
Beta lactamase producing N. gonorrhoeae -
Intravenous cefotaxime
Pseudomonas – Fortified gentamicin or
tobramycin drops
Chlamydia – Systemic erythromycin
42. ULCER - NEUROTROPHIC
Deficient corneal innervation
Decreased tearing,
Decreased corneal sensation
Sterile corneal ulceration
Familial dysautonomia : Generalized
dysfunction of the autonomous nervous system
43. ULCER - NEUROTROPHIC
MANAGEMENT :
Topical lubrication with preservative-free artificial
tears, gels, and ointments
Amniotic 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.
44. METABOLIC DISEASES
MUCOPOLYSACCHARIDOSIS :
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
45. METABOLIC DISEASES
MUCOLIPIDOSIS :
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
46. PETER’S ANOMALY
Most common congenital opacity requiring
penetrating keratoplasty
It is a congenital disorder characterised by central
corneal opacity with corresponding defects in
posterior stroma, Descemet’s membrane and
endothelium.
47. 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.
48. PETER’S ANOMALY Classification
Type I Corneal Opacity +
Iridocorneal Adhesions
Type II Corneal Opacity +
Iridocorneal Adhesions + Lens
abnormality
Unilateral involvement Frequently bilateral
Central 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 clear
Lens usually clear May be clear or cataractous
Good visual acuity potential
Systemic abnormalities uncommon Severe ocular and systemic
malformations
49. PETER’S ANOMALY continued..
CAUSES :
Incomplete central migration of the neural crest cells
Teratogenic exposures, like intrauterine infection, and
maternal alcoholism
Incomplete development of angle is common, leading
to glaucoma.
50. 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.
51. POSTERIOR KERATOCONUS
Very uncommon
Local 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.
52. 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.
53. 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
54. 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
55. 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.
57. 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.
58. 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
59. DERMOID
Dermoids 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
60. DERMOID
Grade I Grade II Grade III
Most frequent Rarest
Small (5mm) Larger
Single
Inferotemporal limbus Entire corneal surface
Superficial Variable depth of stromal
extension
Entire anteror segment is
involved
33% a/w Goldenhar
syndrome
61. DERMOID
MANAGEMENT :
Correction of astigmatism with spectacles
Patching to treat amblyopia
SURGERY :
Complete excision flush with corneal surface, with
lamellar graft
If central cornea is involved, penetrating and lamellar
keratoplasty are vision restoring procedures.
62. PK for CONGENITAL CORNEAL
OPACITIES :
Indications:
Bilateral congenital opacities
Controlled glaucoma with opacification
Chronic corneal oedema
Relative contraindications:
Poor cooperation from parents
Amblyopia
Perforation
Normal fellow eye
63. PK for CONGENITAL CORNEAL
OPACITIES :
Contraindications :
Uncontrolled glaucoma
Infection
Poor tear secretion
Reference : From Pediatric Ophthalmology and Strabismus By Kenneth
Weston Wright
64. 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.
65. 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.
66. CHALLENGES IN MANAGEMENT
Recurrent examination, suture removal is difficult
in children
Risk of anaesthesia for repeated surgeries
Stimulus deprivation amblyopia
Glaucoma
TAKE HOME MESSAGE
The prognosis is less than optimum. Parents need
to be counselled properly to avoid psychological
impact.