Paediatrics Glaucoma

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    Evaluation and Diagnosis of

    Paediatric Glaucoma

    Dr Ashwin Sainani

    Consultant Paediatric Ophthalmologist

    P.D. Hinduja Hospital

    Mumbai

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    Paediatric Glaucoma

    Paediatric glaucoma represents a rare,complex collection of diversepathophysiological entities. This type of

    glaucoma occurs early in life and usuallyresults from structural mal-development ofthe anterior segment and angle structures.

    Predominantly sporadic

    Inherited in 10%27 %

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    Classification of Paediatric

    glaucomaPrimary congenital glaucoma

    Juvenile glaucoma

    Secondary glaucomas:Angle anomaly :

    Sturge Weber syndrome

    Aniridia

    NF

    Ant segment anomalies:

    Peters anomaly

    Axenfeld Riegers syndrome

    ICE syndrome

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    Secondary glaucomas

    Iris Lens diaphragm Abnormalities

    Marfans syndrome

    Spherophakia

    Ectopia Lentis

    Homocystinuria

    PHPV

    ROP

    Aphakic glaucoma

    Trauma

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    Secondary glaucomas

    Uveitis

    JRA

    Sarcoid

    Idiopathic

    Tumours

    Retinoblastoma

    Juvenile Xanthogranuloma

    Steroid induced

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    Diagnosis

    Presentations:

    ObviousBuphthalmos (

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    Differential Diagnosis of cloudy

    cornea

    Ant segment anomalies

    Peters anomaly

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    D.D. (contd)

    Sclerocornea

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    D.D. (contd)

    Corneal dermoid

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    D.D. (contd)

    Congenital infections

    Rubella

    CMV

    Herpes simplex

    Influenza

    Trauma

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    D.D. (contd)

    Corneal Dystrophies

    PPMD (Post polymorphous dystrophy)

    CHED(Congenital Hereditary Endothelial Dystrophy)

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    D.D. (contd)

    Metabolic Diseases

    Gangliosidosis (GM 1)

    Mucopolysaccharidosis (Hurlers disease)

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    Presentation

    ElusiveEpiphora, photophobia, blepharospasm, subtle

    corneal enlargement. (bilat)

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    Examination

    Clinic (>5 yrs)

    EUA:

    Short inhalational induction with face mask.

    Laryngeal mask

    Anaesthetic agents:

    GasesSevofluraine, halothane

    IVPropofol, ketamine, pentothal Muscle Relaxants:

    Succinylcholine

    Atracurium

    vecuronium

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    Effects of Anaesthetic agents on IOP

    Most anaesthetic agents cause reduction in

    IOP.

    Ketamine and Succinylcholine cause a rise

    in IOP and are thus avoided in an EUA for

    glaucoma.

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    Evaluation

    Corneal Diameter: (Vernier Caliper)

    Newborn9.5 to 10.5mm

    1 yr11mm

    2-3 yrs12mm

    (>12 mm in an infant less than a year is

    abnormal)

    Corneal Haze / Haabs Striae: (Portable Slit

    Lamp)

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    Corneal Measurements

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    Evaluation (contd)

    Ant /Post Segment examination: (Portable slit lamp) Cornea

    Opacification

    Posterior Embryotoxon

    Iris

    Iris hypoplasia

    Iris strands

    Correctopia/polycorrea Ectopia uveae

    AC

    Uveitis

    hyphema

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    Evaluation (contd)

    Lens Cataract

    Subluxation

    Aphakia

    Kerato-lenticular touch

    Vitreous

    Retrolental fibrosis

    Vitritis Retina

    Tumours

    ROP

    Choroidal hemangioma

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    Evaluation (contd)

    IOP: (Perkins, Tonopen, Shiotz)

    Normal IOP in infantsUpper teens

    21mm is still a useful upper limit

    Not the only critical parameter

    Comparison with other eye is useful inunilateral disease.

    Measured as early as possible after anaesthesia

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    Perkins Tonometry

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    Evaluation (contd)

    Cup:Disc ratio

    Rapid increase in cupping

    May show reduction in cupping when IOP is

    controlled.

    Due to reduction in elastic components notreversal of axonal atrophy

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    Evaluation (contd)

    Gonioscopy: (Koeppes lens, Barkans lens)

    Anterior insertion of iris

    Absent scleral spur

    Iris processes

    Membranous structure covering the meshwork

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    Evaluation (contd)

    A scanBuphthalmic eye would have an

    increased axial length.

    B scan / UBM

    Hazy cornea

    Retinoscopy

    Myopic shift

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    Diagnosis

    Diagnosis is based on the entire clinicalpicture and not on any single parameter:

    IOP

    Size of corneaCorneal oedema

    Cupping

    Evidence of trabeculodysgenesisAnterior segment abnormalities

    Evidence of progression is highlysignificant.

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    Follow up

    Frequency depends on the stage ofglaucoma

    Includes regular EUAs to monitor effect oftreatment and progression of disease

    Management of Amblyopia:Cycloplegic refraction

    Patching - The only proof of the presence ofamblyopia is the improvement of vision onpatching.

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