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My Clouding Cornea

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My Clouding Cornea

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My Clouding Cornea

  1. 1. CONGENITAL CLOUDING OF CORNEA Causes and Management Presenter : Dr. Rujuta Moderator : Dr. Monica, Dr. Sangit
  2. 2. DEFINITION  The term “congenital” refers to any condition that is present in the newborn.  “Clouding” refers to loss of transparency.
  3. 3. “STUMPED” Classification  S – Sclerocornea  T – Tears in descemet’s membrane Congenital Glaucoma Birth trauma  U – Ulcer Herpes simplex virus Bacterial Neurotrophic  M – Metabolic (rarely present at birth) Mucopolysaccharidoses Mucolipidoses Tyrosinosis
  4. 4. Classification continued…  P – Posterior corneal defect Peter’s anomaly Posterior keratoconus Staphyloma  E – Endothelial dystrophy Congenital hereditary endothelial dystrophy Stromal : Congenital hereditary stromal dystrophy  D- Dermoid
  5. 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. 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. 7. SCLEROCORNEA continued…  ISOLATED PERIPHERAL SCLEROCORNEA: Abrupt change from scleral-like tissue to clear cornea without any other ocular abnormalities.
  8. 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
  9. 9. SCLEROCORNEA continued…  TOTAL SCLEROCORNEA: Most common form causing congenital corneal opacity.
  10. 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. 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
  12. 12. TEARS IN DM : CONGENITAL GLAUCOMA
  13. 13. CONGENITAL GLAUCOMA continued… Cause :  Sporadic OR  Mutation in CYP1B1 gene on Chr 2p21
  14. 14. CONGENITAL GLAUCOMA continued…  SIGNS : Raised IOP Corneal enlargement and clouding Corneal diameter >10-10.5mm Optic nerve cupping Increased axial length Gonioscopic abnormalities
  15. 15. 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
  16. 16. CONGENITAL GLAUCOMA continued…
  17. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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
  27. 27. ULCERS  Herpes simplex virus  Rubella  Bacterial  Neurotrophic
  28. 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
  29. 29. ULCER – HSV continued…
  30. 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. 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. 32. ULCER – HSV continued…  MANAGEMENT :  Prophylactic :  Neonate : Intravenous acyclovir treats systemic disease, but therapeutic levels are also achieved in aqueous and tears.
  33. 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. 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. 35. ULCER – RUBELLA continued…  MANAGEMENT :  Isolated opacification resolves spontaneously.  If persists, penetrating keratoplasty can be performed.
  36. 36. ULCER - BACTERIAL
  37. 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
  38. 38. ULCER – BACTERIAL continued  GONORRHOEAL OPHTHALMIA NEONATORUM :
  39. 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. 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. 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. 42. ULCER - NEUROTROPHIC Deficient corneal innervation Decreased tearing, Decreased corneal sensation Sterile corneal ulceration  Familial dysautonomia : Generalized dysfunction of the autonomous nervous system
  43. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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.
  56. 56. POSTERIOR POLYMORPHOUS DYSTROPHY  Similar to CHED with AD inheritance.  Cause- Primary dysfunction of corneal endothelium.
  57. 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. 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. 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. 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. 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. 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. 63. PK for CONGENITAL CORNEAL OPACITIES :  Contraindications : Uncontrolled glaucoma Infection Poor tear secretion Reference : From Pediatric Ophthalmology and Strabismus By Kenneth Weston Wright
  64. 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. 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. 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.
  67. 67. Thankyou!

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