Manchester Pediatric Ophthalmology Lecture 17 PDF

Lecture 17: Pediatric Ophthalmology Pediatric Ophthalmology Leukocoria Differential Diagnosis of white pupil in infants: 1. Retinoblastoma. 2. Congenital cataract. 3. Retinopathy of prematurity: Failure of vascularization of the peripheral retina in prem...

Lecture 17: Pediatric Ophthalmology Pediatric Ophthalmology Leukocoria Differential Diagnosis of white pupil in infants: 1. Retinoblastoma. 2. Congenital cataract. 3. Retinopathy of prematurity: Failure of vascularization of the peripheral retina in prematures babies under 32 weeks gestation and babies under 1.5 Kg weight exposed to high oxygen concentration in incubators with subsequent fibrosis. 4. Persistent Hyperplastic Primary Vitreous: Congenital anomaly with fibrosis of the anterior vitreous. 5. Coat's disease: Unilateral extensive leakage from the retinal vessels resulting in large masses of subretinal lipids. 6. Retinal dysplasia: Chromosomal defect resulting in development of a disorganized retina. Left Leucocoria  Lecture 17: Pediatric Ophthalmology Retinoblastoma It is the most common primary intraocular malignancy in childhood. It almost always presents prior to the age of 3 years. A positive family history is present in only 6% of cases. The tumor may be bilateral in 20% of the cases. It is thought to be caused by a mutation in a gene on chromosome 13. Clinical features: Retinoblastoma may present as: 1. Leukocoria: White pupillary reflex (Amaurotic cat’s eye), accounts for 65% of cases. 2. Squint: If the tumor affects the macular area (Secondary Strabismus) 3. Enlarged globe due to Secondary glaucoma. 4. Proptosis due to extraocular extension. 5. Accidental discovery on routine examination. Diagnosis: Is based on: 1. Ophthalmoscopy: It is of extreme importance to examine both eyes due to the relatively high incidence of bilaterally. 2. Ocular Ultrasonography. 3. CT Orbit may show tumor calcification. 4. MRI Orbit Stages: I. Quiescent stage (Asymptomatic): Small yellowish-white mass with new vessels on its surface. II. Glaucomatous stage. III. Extra-Ocular extension: Perforation of the globe & proptosis.  Lecture 17: Pediatric Ophthalmology Direct extension along the optic nerve to the brain is the most common mode of spread. IV. Distant metastasis. Treatment: I. Quiescent stage: 1. Laser Photocoagulation: for small posterior tumors confined to the retina. 2. Cryotherapy: for small peripheral tumors. 3. Radiotherapy (brachytherapy): using radioactive plaques sutured to the sclera over the tumor. 4. Trans-pupillary Thermo-Therapy (TTT) for medium tumors. II. Glaucomatous stage: Enucleation with excision of a long stump of the optic nerve. III. Extra-ocular (orbital) extension stage: Orbital exenteration. IV. Distant metastases stage: Systemic chemotherapy. Left Leukocoria with Retinoblastoma on Ophthalmoscopy  Lecture 17: Pediatric Ophthalmology Congenital (Developmental) Cataract Definition: Opacities in the lens discovered at birth or in the early years of life. Etiology: 1. Hereditary. 2. Intrauterine infections especially rubella (German measles). 3. Metabolic e.g. galactosemia. 4. Malnutrition (Iron & calcium deficiency). Morphologic Types: 1. Lamellar (Zonular) Cataract It is the most common type. Lens opacification involving one or more layers of lens. The rest of the layers are clear. 2. Anterior Polar Cataract. 3. Posterior polar cataract It appears as a small disc shaped opacity at the posterior pole. Vision is more affected as the opacity is situated near the nodal point of the eye. 4. Total Cataract Following rubella infection to the mother in the first trimester. It is usually associated with heart anomalies and other congenital anomalies. 5. Coronary Cataract A ring of oval opacities seen in the cortex near the equator. It does not affect the vision. 6. Punctate or Blue-Dot Cataract Multiple small bluish dots. It does not affect the vision. 7. Fusiform Cataract Antro-posterior spindle shaped opacity.  Lecture 17: Pediatric Ophthalmology 8. Discform Cataract 9. Sutural cataract either anterior or posterior Clinical picture: Symptoms: Usually given by the mother who may notice: 1. A white pupil (leukocoria). 2. Defective vision of her baby. 3. Squint or nystagmus. Signs: 1. Opacity of the lens may be seen. On examining red reflex, it appears as black parts against a red background. 2. Fundus should be carefully examined to exclude retinal anomalies. 3. Visual acuity Examination could be done by preferential looking test. 4. Appropriate lab tests should be ordered if a metabolic cause is suspected e.g. galactosemia. 5. Evaluation of the density of the cataract: by using both direct & indirect ophthalmoscope in visualization of the fundus through the cataract. The cataract can be classified into: Direct Ophthalmoscope Indirect Ophthalmoscope Very Dense Cataract Fundus CANNOT be seen Fundus CANNOT be seen Moderately dense Cataract Fundus CANNOT be seen Fundus CAN be seen Insignificant cataract Fundus CAN be seen Fundus CAN be seen  Lecture 17: Pediatric Ophthalmology Complications: 1. Bilateral dense opacities may lead to nystagmus (due to lack of development of fixation reflexes) 2. Unilateral opacity may lead to amblyopia and secondary convergent squint. Treatment: Unilateral cases: Should be operated as early as possible to avoid amblyopia. Amblyopia therapy is needed after surgery Bilateral cases: The decision to operate or not depends upon the density and symmetry of the opacity as well as the visual acuity of the child. Surgical lines of treatment include: a. Irrigation-aspiration: Aspiration of lens matter with simultaneous irrigation with saline after performing an opening in the anterior lens capsule. b. Lensectomy: Removal of the lens cortex and capsules as well as the anterior vitreous by vitrectomy machine. Visual rehabilitation: As early as possible after surgery. There are several methods. 1. Contact lenses. 2. Aphakic spectacles: In bilateral aphakia and contact lens intolerance. 3. IOL implantation.  Lecture 17: Pediatric Ophthalmology Lamellar Cataract Posterior Polar Cataract Total Cataract Coronary Cataract Blue dot Cataract Sutural Cataract  Lecture 17: Pediatric Ophthalmology Primary Congenital Glaucoma (Buphthalmos) Definition: Rise of IOP due to developmental anomalies at the drainage angle leading to enlargement of the globe and stretching of the eye coats (Presence of mesodermal membrane obstructing the angle) Symptoms: Early: Photophobia, lacrimation & blepharospasm. Late: Large eye (ox eye). Signs: 1. Cornea: Increase in diameter more than 12 mm and edema 2. Sclera: Stretched, thin, the uvea shines through it giving blue appearance. 3. Anterior chamber: Deep due to enlargement of the globe. 4. Lens: subluxated or dislocated due stretch of zonules with tremulous iris. 5. Tension: High. 6. Optic nerve: Late, glaucomatous cupping and atrophy. 7. Gonioscopy: Angle anomaly. 8. Increase axial length of the globe → axial myopia. Differential diagnosis: 1. Large cornea: a. Megalocornea: Only the cornea is large in diameter, with clear cornea & normal IOP. b. High myopia: Refraction shows high myopia with clear cornea & normal IOP c. Keratoglobus: Bilateral condition with familial incidence later in life with clear cornea.  Lecture 17: Pediatric Ophthalmology 2. Cloudy cornea at birth: Birth trauma, congenital rubella & metabolic disorders. 3. Secondary infantile glaucoma: Retinoblastoma, uveitis, aniridia, ectopia lentis, Sturge-Weber syndrome,…etc Treatment: Treatment of congenital glaucoma is essentially surgical, using either: 1. Goniotomy (Operation of choice): Principle: an incision is made in the mesodermal membrane obstructing the angle, allowing aqueous to pass directly into the Schlemm’s canal. Indicated in early cases when the cornea is still clear & corneal diameter is < 12 mm. 2. Trabeculotomy: Principle: an incision is made in the canal of Schlemm to allow direct passage of aqueous from AC into the canal. Indicated after failure of Goniotomy or in advanced cases with hazy cornea & corneal diameter > 12 mm. 3. External fistulizing operation (Trabeculectomy): Indicated after failure of the above operations or for advanced cases with hazy cornea & corneal diameter > 12 mm. It has high rate of failure in infant & young patients due to rapid & excessive healing Primary Congenital Glaucoma  Lecture 17: Pediatric Ophthalmology Congenital naso-lacrimal duct obstruction (Congenital or infantile dacryocystitis) It usually occurs due to imperforate Hasner’s valve at the lower end of the naso-lacrimal duct. Clinical picture: 1. Epiphora, usually noticed at 2-3 weeks after birth. 2. Pressure over the lacrimal sac leads to reflux (positive regurge) of clear fluid, mucus, muco-pus or sometimes frank pus. Treatment of congenital dacryocystitis: 1. Conservative:  Antibiotics with hydrostatic massage of the lacrimal sac, the mother is instructed to press on the lacrimal sac in a downward direction. This may help to remove any remnants of epithelium or to open Hasner's valve.  This is tried for a period up to 9-12 months.  90% of the cases resolve by conservative treatment. 2. Probing:  It is done on persistence of Epiphora with conservative treatment at 9-12 months.  Successful if done carefully as the lacrimal passages are still elastic 3. Repeated syringing & irrigation:  Tried on failure of first probing.  The lacrimal drainage system is irrigated with saline trying to open Hasner’s valve.  Lecture 17: Pediatric Ophthalmology 4. Silicone intubation:  May be beneficial if probing fails to cure the obstruction.  The silicon tube should remain in the lacrimal drainage system for 6-12 months (as a stent). 5. Dacryo-Cysto-Rhinostomy (DCR):  Done after failure of silicon intubation to relieve the obstruction.  Is to create a surgical opening between the lacrimal sac and the nasal mucosa, allowing drainage of tears directly into the nose. A B Massage of lacrimal sac A: Probing, B: syringing Silicone Intubation  Lecture 17: Pediatric Ophthalmology Ophthalmia neonatorum (Purulent Conjunctivitis) Definition: Any discharge from the eye of a newborn infant is suspicious since tears are not secreted at this early date. Etiology: Infectious: Ocular contact with contaminated maternal passages or towels. 1. Bacterial: Gonococci, staphylococci, streptococci or pneumococci. 2. Viral: Herpes simplex and adenoviruses. 3. Chlamydia. Diagnosis: 1. Clinical picture of purulent conjunctivitis: a) Lid edema. b) Conjunctival edema (chemosis). c) Conjunctival injection. d) Profuse purulent discharge with Gluing of lashes by the discharge 2. Conjunctival smear: May show bacteria, inclusion bodies in chlamydial and herpes infections. Management: I. Prevention: (most important) 1. Treatment of the mother before labor. 2. Washing of the body of the baby from above downward. 3. Broad spectrum antibiotic eye drops are instilled in the eyes after birth for 1 week.  Lecture 17: Pediatric Ophthalmology II. Treatment: 1. Frequent washing with sterile warm water. 2. Local antibiotic eye drops used frequently e.g. Sulfacetamide 10%, Choloramphenicol, Gentamycin, Tobramycin, Quinolones & Fucidic acid. 3. Antibiotic ointment as Tetracyclin or Tobramycin at night. 4. Systemic antibiotics in severe cases. Ophthalmia Neonatorum