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Childhood Cataracts and Other Pediatric Lens Disorders

Childhood Cataracts and Other Pediatric Lens Disorders. Juan G. Santiago, MD Ophthalmology RCM UPR. Structural or Positional Lens Abnormalities. Congenital aphakia Usually associated with ocular malformation. Structural or Positional Lens Abnormalities. Spherophakia

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Childhood Cataracts and Other Pediatric Lens Disorders

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  1. Childhood Cataractsand Other Pediatric Lens Disorders Juan G. Santiago, MD Ophthalmology RCM UPR

  2. Structural or Positional LensAbnormalities • Congenital aphakia • Usually associated with ocular malformation

  3. Structural or Positional LensAbnormalities • Spherophakia • Small, spherical lens that may dislocate • Usually bilateral • Weill-Marchesani

  4. Structural or Positional Lens Abnormalities • Lens coloboma • Notched or flattened area • Abnormal closure of the embryonic fissure • Usually associated with dislocation

  5. Structural or Positional Lens Abnormalities • Lenticonus (lentiglobus) • Central ectasia of lens surface • Anterior or posterior • Alport syndrome: Nerve deafness + nephritis + anterior lenticonus

  6. Lenticonus • Posterior more common • Cataract may have later onset • Important prognostic factor

  7. Dislocated Lenses in Children • Ectopia lentis • Lens is not in normal anatomic position • a.k.a. lens dislocation, luxation, subluxation

  8. Dislocated Lenses in Children • Simple ectopia lentis • No other abnormality • Bilateral, symmetric • Upward temporal • Autosomal dominant • Ectopia lentis et pupillae • Pupil also displaced • Bilateral, asymmetric • Autosomal recessive

  9. Dislocated Lenses in Children • Differential diagnosis • Trauma • Spontaneous (ocular anomaly) • Aniridia • Iris Coloboma • Hereditary ectopic lentis • Congenital glaucoma • Systemic disorders

  10. Dislocated Lenses in Children • Must know: • Marfan • Homocystinuria • Weill-Marchesani • Extra credit: • Hyperlysinemia • Sulfite oxidase deficiency • Syphilis • Ehlers-Danlos

  11. Marfan syndrome • Autosomal dominant • Fibrillin mutation (chromosome 15) • Physical features • Tall, long limbs, arachnodactyly, loose joints, scoliosis, chest deformities • Cardiovascular • Aortic dilation / Dissecting aneurysm • Dislocated lens in 50%-80% • Usually bilateral and upward

  12. Homocystinuria • Autosomal recessive; high homocysteine • Physical features: • Tall, osteoporosis, scoliosis • Life-threatening problem • Thrombotic vascular occlusions; hypertension • Avoid surgery! • Lens dislocation in 90% • Usually bilateral and downward

  13. Weill-Marchesani syndrome • Autosomal recessive • Physical features • Short stature • Short stubby fingers • Microspherophakia • Progressive lens dislocation • Pupillary block common • Consider prophylactic peripheral iridectomy?

  14. Dislocated Lenses: Treatment • Refraction • Try both parts of pupil: • Phakic • High minus, high astigmatism, irregular • The endless retinoscopy • Aphakic • High plus, low astigmatism • Consider chronic mydriasis + bifocals • Glaucoma • Cataract

  15. Pediatric Cataracts: Overview • Prevalence • 1 / 250 newborns • Types • Systemic associations • Clinical evaluation • Treatment • Prognosis

  16. Pediatric Cataracts: Types • Congenital • Bilateral • 1/4 hereditary • 1/4 associated diseases • 1/2 idiopathic • Unilateral • Hereditary • Autosomal dominant most common • Traumatic

  17. Morphologic Cataract Types • Zonular • Nuclear, Lamellar, Sutural, Capsular • Polar • Anterior polar, Posterior lenticonus • Total • PHPV (PFV)

  18. Anterior Polar Cataract • Nonprogressive • Small (<3 mm) • Surgery rarely required • Beware anisometropia! • Anisometropic amblyopia

  19. Lamellar Cataract • Often hereditary • Often acquired and progressive • Opacification peripheral to Y sutures • Clear nucleus • Bilateral

  20. Nuclear Cataract • Congenital • Often autosomal dominant inheritance • Microphthalmia frequent • Dense, axial opacity

  21. Other morphologic types • Posterior lenticonus / lentiglobus • Bulging posterior lens capsule • Acquired; better prognosis • PHPV (PFV) • Microphthalmia • Glaucoma • 90% unilateral • Poor prognosis

  22. Cataracts: Key causes • Genetic / Metabolic • Lowe syndrome • Galactosemia • MANY others • Infectious • TORCH • Syphilis

  23. Cataract Evaluation: General • Family history • Examine family members • If positive, further evaluation may not be needed • But: Beware hereditary syndromes (e.g. Lowe) • Pregnancy • Rash, febrile illness • Physical exam

  24. Cataract Evaluation: Eye Exam • A scan • Corneal diameter • Keratometry if IOL planned • B scan if needed • Rule out mass / stalk / detachment • Intraocular pressure

  25. Cataract evaluation: Lab studies • When to defer laboratory studies: • Most unilateral cases • But rule out subtle bilateral disease • Bilateral cases with a definite hereditary basis

  26. Cataract evaluation: Lab studies • Urine • Reducing substance (after milk feeding) • Amino acids IF Lowe suspected • Blood • Fasting blood sugar • Plasma calcium/phosphorous • RBC transferase / galactokinase • TORCH (toxoplasmosis, rubella, CMV, HSV) • IF maternal rash during pregnancy: • Varicella antibody titers

  27. When to intervene • Dense cataracts • Urgent removal and optical correction • Provide focused image by age 8 weeks • Nystagmus = poor prognosis

  28. When to intervene • Partial cataracts • Judgment call • Party line: 3 mm or greater • My approach: • Retinoscopy • If you can’t refract, then baby can’t see • Direct ophthalmoscopy • If you can’t see, then neither can baby

  29. Treatment approaches • Non-surgical (very mild cases) • Mydriasis • Occlusion of uninvolved eye • Lensectomy / vitrectomy • Intraocular lens implantation • PC or not PC?

  30. Lensectomy • Stab incisions • Limbus or pars plana • Insert vitreous cutter • Aspirate nucleus and cortex • Primary posterior capsulectomy • Anterior vitrectomy

  31. Lensectomy • Postoperative optical rehabilitation • Aphakic spectacles • Contact lenses

  32. Intraocular Lens Implant • Party line: OK for age 2 and up • Some say older; many will go younger • Under 6 months not advisable • Under 3 months strongly discouraged

  33. Intraocular Lens Implantation: Mitigating factors • Unilateral cataract • Earlier threshold for IOL insertion • Ocular anatomy • Anterior chamber size (microphthalmia) • Coexisting ocular disease • Glaucoma • Inflammation • Dislocated lens • Aniridia • ROP

  34. Bilateral cataracts: When to consider IOL • Nystagmus • Contact lenses difficult to fit and position • Dry eye • Unusual circumstances • Dirty or sandy living conditions • Limited access to contact lens care • Compliance concerns

  35. IOL power: Target refraction • Age at cataract surgery • Myopic shift of 7-8 D from age 1-10 yr • The closer to birth, the more marked the undercorrection will need to be • Status of the fellow eye • Monocular cataract or pseudophakia – Minimize the aniseikonia • Both eyes – Larger amount of hyperopia may be acceptable • Visual acuity • Late myopia is acceptable if it helps the child recover vision during the amblyopia treatment years • Expected compliance • Poor compliance – Better to leave less refractive error.

  36. IOL power: Target refraction • Parent’s refractive error (Myopic parents) • Expected to undergo more growth • May be left with more hyperopia • IOL power • The higher the IOL power, the higher early residual refraction • Microphthalmia • The highest available IOL power should be chosen

  37. Postoperative Care:Short term • Aggressive control of inflammation • Topical steroids every 1-2 hours for 2 weeks • Taper slowly for IOL, quickly for contact lens • Maintain antibiotic coverage as well • Cycloplegia • Atropine for first day • Then use judiciously to prevent IOL capture • Prompt fitting of contact lenses

  38. Postoperative Care:Long term • Contact lens management issues • Constant surveillance for amblyopia • Prompt institution of penalization or patching • Strabismus is common • Don’t over-patch! • Constant surveillance for glaucoma • May not show up for 10 years • EUA to check IOP for sudden myopic shift

  39. Summary: Pediatric cataract • Autosomal dominant most common • Several important hereditary conditions • Early treatment is key • Surgical approach differs from adults • Surgery is the easy part • IOL power calculations • Contact lenses or IOL problems • Amblyopia, strabismus, glaucoma

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