Evaluation and Diagnosis of Paediatric Glaucoma

Dr Ashwin Sainani Consultant Paediatric Ophthalmologist P.D. Hinduja Hospital Mumbai

Paediatric Glaucoma
Paediatric glaucoma represents a rare,

complex collection of diverse pathophysiological entities. This type of glaucoma occurs early in life and usually results from structural mal-development of the anterior segment and angle structures.
Predominantly sporadic
Inherited in 10% 27 %

Classification of Paediatric glaucoma

Primary congenital glaucoma

Juvenile glaucoma
Secondary glaucomas: Angle anomaly :
Sturge Weber syndrome Aniridia NF Ant segment anomalies:
Peters anomaly Axenfeld Riegers syndrome ICE syndrome

Secondary glaucomas
Iris Lens diaphragm Abnormalities Marfans syndrome Spherophakia Ectopia Lentis Homocystinuria PHPV ROP Aphakic glaucoma Trauma

Secondary glaucomas
Uveitis JRA Sarcoid Idiopathic Tumours Retinoblastoma Juvenile Xanthogranuloma Steroid induced

Diagnosis
Presentations: Obvious Buphthalmos (<2-3 yrs), hazy cornea, conjunctival injection.

Differential Diagnosis of cloudy cornea

Ant segment anomalies

Peters anomaly

D.D. (contd)
Sclerocornea

D.D. (contd)
Corneal dermoid

D.D. (contd)
Congenital infections Rubella
CMV Herpes simplex

Influenza

Trauma

D.D. (contd)
Corneal Dystrophies PPMD (Post polymorphous dystrophy)
CHED(Congenital Hereditary Endothelial Dystrophy)

D.D. (contd)
Metabolic Diseases Gangliosidosis (GM 1)
Mucopolysaccharidosis (Hurlers disease)

Presentation
Elusive Epiphora, photophobia, blepharospasm, subtle corneal enlargement. (bilat)

Examination
Clinic (>5 yrs)

EUA: Short inhalational induction with face mask. Laryngeal mask Anaesthetic agents: Gases Sevofluraine, halothane IV Propofol, ketamine, pentothal Muscle Relaxants: Succinylcholine Atracurium vecuronium

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.

Evaluation
Corneal Diameter: (Vernier Caliper) Newborn 9.5 to 10.5mm 1 yr 11mm 2-3 yrs 12mm

(>12 mm in an infant less than a year is abnormal) Corneal Haze / Haabs Striae: (Portable Slit Lamp)

Corneal Measurements

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

Evaluation (contd)
Lens Cataract Subluxation Aphakia Kerato-lenticular touch

Vitreous Retrolental fibrosis Vitritis Retina Tumours ROP Choroidal hemangioma

Evaluation (contd)
IOP: (Perkins, Tonopen, Shiotz) Normal IOP in infants Upper teens
21mm is still a useful upper limit Not the only critical parameter

Comparison with other eye is useful in unilateral disease. Measured as early as possible after anaesthesia

Perkins Tonometry

Evaluation (contd)
Cup:Disc ratio Rapid increase in cupping
May show reduction in cupping when IOP is controlled. Due to reduction in elastic components not reversal of axonal atrophy

Evaluation (contd)
Gonioscopy: (Koeppes lens, Barkans lens) Anterior insertion of iris
Absent scleral spur Iris processes Membranous structure covering the meshwork

Evaluation (contd)
A scan Buphthalmic eye would have an

increased axial length.

B scan / UBM Hazy cornea

Retinoscopy Myopic shift

Diagnosis
Diagnosis is based on the entire clinical

picture and not on any single parameter:

IOP Size of cornea Corneal oedema Cupping Evidence of trabeculodysgenesis Anterior segment abnormalities

Evidence of progression is highly

significant.

Follow up
Frequency depends on the stage of

glaucoma
Includes regular EUAs to monitor effect of

treatment and progression of disease

Management of Amblyopia: Cycloplegic refraction Patching - The only proof of the presence of amblyopia is the improvement of vision on patching.