Childhood glaucoma

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Childhood glaucoma

  1. 1. CHILDHOOD GLAUCOMA
  2. 2. Definition of Terms Primary congenital/ infantile glaucoma-  present at birth or 1st few years of life  Anterior chamber angle abnormalities  No systemic anomalies  Juvenile- > 3 y/o Secondary infantile glaucoma  Associated with inflammatory, neoplastic, hamartomatous, metabolic or other congenital abnormalities
  3. 3. Epidemiology and Genetics Primary Congenital Glaucoma  Rare  1- 10,000  50- 70 % of congenital glaucomas 60%- diagnosed by 6 months 80 %- 1st year of life M > F (65 %) Bilateral > unilateral – ( 70 %)
  4. 4.  Inheritance:  AD  AR with variable penetrance GLC3A/B/C Ch 2, Ch 1, Ch 14 CYP1B1 gene- congenital glaucoma gene at the GLCA3 locus
  5. 5. PATHOPHYSIOLOGY Exact mechanism- unknown1. cellular or membranous abnormality in the TM  Impermeable TM or a BARKAN membrane2. abnormal insertion of the ciliary muscle Developmental arrest in the late embryonic period
  6. 6. CLINICAL FEATURES Triad: 1. Epiphora 2. Photophobia 3. Blepharospasm Buphthalmos Corneal enlargement ( > 12 mm) Corneal edema Haab striae
  7. 7.  Decreased Visual Acuity:  Optic atrophy  Corneal clouding  Astigmatism  Amblyopia  Cataract  Lens dislocation  Retinal detachment
  8. 8.  Examination under GA Most GA agents and sedatives lower IOP, except KETAMINE Dehydration- lowers IOP Normal IOP – 10- 15
  9. 9.  Gonioscopy  High and flat iris insertion  Absence of angle recess  Peripheral iris hypoplasia  Tenting of peripheral iris pigment epithelium  Thickened uveal TM  Open angle
  10. 10. OPTIC DISC Direct and indirect ophthalmoscopy Optic nerve photography Glaucomatous cupping:  Superior and inferior  Cup enlargement  Reversible  Indicates control of IOP
  11. 11. ULTRASONOGRAPHY Axial length Minimally reversible Corneal enlargement- irreversible
  12. 12. DIFFERENTIAL DIAGNOSIS Excessive tearing  NLDO  Corneal epithelial defect/abrasion  Conjunctivitis
  13. 13.  Corneal Enlargement  X- linked megalocornea  Exophthalmos  Shallow orbits
  14. 14. Corneal Clouding Birth trauma  Metabolic disorders Inflammatory corneal  Some skin disorders disease  Choristomas CHED  Intrauterine Corneal malformations inflammation Keratomalacia
  15. 15. OPTIC NERVE ABNORMALITIES Optic nerve pit Optic nerve coloboma Optic nerve hypoplasia Optic nerve malformation Physiologic cupping
  16. 16. PROGNOSIS and FOLLOW- UP Surgery- preferred treatment Goniotomy Trabeculotomy Trabeculectomy, aqueous shunts- Cyclophotocoagulation
  17. 17. MEDICAL  temporizing  Control IOP, clear the cornea B- blockers  Apnea  Hypotension  Cough CAI  Acidosis  hypoK A- adrenergic agonist  CNS adverse effect  Should not be used in patients < 3 y/o  With caution- < 10 y/o
  18. 18.  Better prognosis- asymptomatic at birth, symptomatic before 24 months old Guarded- symptomatic at birth, and diagnosed after 2 y/o COMPLICATIONS: Amblyopia, corneal scarring, strabismus, anisometropia, cataract, lens subluxation, recurrent glaucoma
  19. 19. Secondary Developmental Glaucoma
  20. 20. Associated ocular anomalies Microphthalmos Corneal anomalies Anterior segment dysgenesis Aniridia Lens anomalies PFV Congenital ectropian uvea syndrome
  21. 21. Axenfeld- Rieger Syndrome Abnormal development of tissues derived from the neural crest Bilateral AC angle , iris and TM AD, sporadic 50% associated wit Glaucoma
  22. 22. Axenfeld Anomaly RIEGER SYNDROME Posterior emobryotoxon  Rieger anomaly withmultiple adherent peripheral iris stands  Defects of teeth bones  RedundantRieger Anomaly periumbilical skin Axenfeld anomaly  Pituitary abnormalities Iris hypoplasia  hypoplasia Corectopia
  23. 23. OCULAR FINDINGS Posterior embryotoxon Cornea – NORMAL Iris – normal- atrophic Corectopia Hole formation Ectropion uvea
  24. 24. PETERS ANOMALY Central corneal opacity Iridocorneal adhesion Bilateral- 80% Sporadic- AD, AR 50%- associated with glaucoma
  25. 25.  Annular- central leukoma Defect in endothelium, descemet, stroma w/ or w/o adhering iris strands +/- corneloneticular adhesions Microcornea, angle anomalies, systemic abnormalities ( heart, GUS, MS, ear, palate, spine)
  26. 26. ANIRIDIA Bilateral Iris hypoplasia- absent iris Limbal stem cel abnormalities pannus( peripheral to central) Cataract Foveal hypoplasia pendular nystagmus, reduced vision
  27. 27.  AD, sporadic 20% of sporadic cases- inc risk of Wilms tumor PAX6 gene, ch 11 50- 75%- develop glaucoma ( 2nd decade of life) 85%- not associated with systemic illness  1.WAGR- 13 %  2.Gillespie- cerebellar ataxia, MR- 2%
  28. 28. STURGE WEBER SYNDROME Ancephalotrigeminal angiomatosis Unilateral Ipsilateral cavernous hemangioma/ facial cutaneous hemangioma/ leptomeningeal angioma 30- 70%- Glaucoma Elevated episcleral venous presssure CNS symptoms
  29. 29. NEUROFIBROMATOSIS Most common phakomatosisI. NF 1- von Recklinghausen or peripheral NF  Most common  1: 3000- 5000  AD, ch 17  Ectropion uvea  Lisch nodules  Optic nerve glioma  Eyelid neurofibroma  Café au lait  Axillary/inguinal freckling  Cutaneous neurofibromas
  30. 30. II. NF2 Central NF Chromosome 22 Posterior subcapsular cataract in adolescence Not associated with glaucoma Bilateral acoustic neuroma Meningioma, schwannoma, ependymoma
  31. 31. Weil – Marchesani Syndrome Short Short fingers and limbs Microspherophakia Lens dislocation  pupillary block glaucoma
  32. 32. SYSTEMIC CONGENITAL ANOMALIESASSOCIATED WITH CHILDHOOD GLAUCOMA Trisomy 21 Trisomy 13 Trisomy 18 Turner Syndrome
  33. 33. SYSTEMIC CONGENITAL ANOMALIES ASSOCIATED WITH CHILDHOOD GLAUCOMA Lowe syndrome Stickler Zellweger Hallermann- Streiff Rubinstein- Taybi Oculodentodigital dysplasia Prader willi Cockayne Syndrome Fetal Alcohol Syndrome
  34. 34. THANK YOU!

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