✍️ typically categorized by age of onset
☝️ Congenital (occurs in infants < 3 months old)
• primary
• secondary
• syndromic
☝️ Infantile (between 3 months and 3 years of age)
☝️ Juvenile (between 3 and 35 years of age)
Primary congenital glaucoma
✍️ Etiology
☝️ mapped to chromosomes
• Ch1p36 (GLC3B)
• Ch2p21-p22 (GLC3A, CYP1B1)
☝️ a mutation in the CYP1B1 gene accounts for 85% of congenital glaucoma
☝️ AR in 10%
☝️ affected parents has 5% chance of having a child with infantile glaucoma
✍️ Epidemiology
☝️ occurs in 1 of 12,500 births
☝️ 40% present at birth
☝️86% present during first year of life
☝️70% bilateral
☝️70% males
✍️ Symptoms
☝️tearing
☝️photophobia
☝️blepharospasm
☝️eye rubbing.
☝️If younger than age 3 months
• corneal clouding
• tearing
☝️older than 3 years of age
• usually asymptomatic
• progressive myopia
• insidious VF loss
✍️ signs
☝️IOP > 21 mm Hg
☝️C/D ratio > 0.3 (2.6% of normal infants)
☝️cupping ( central , steep and pink NRR) is reversible in childhood
☝️buphthalmos (bull’s-eye)
☝️horizontal corneal diameter > 13 mm
☝️limbal ectasia
☝️stretching of zonules can lead to lens subluxation (irreversible)
☝️corneal clouding or edema
☝️Haab’s striae (circumferential or horizontal Descemet’s ruptures )
☝️myopia
✍️ ocular associations
☝️ microcornea
☝️ cornea plana
☝️sclerocornea
☝️Axenfeld’s
☝️Reiger’s
☝️Peter’s anomaly (50%)
☝️aniridia (50–75% )
☝️microspherophakia
☝️nanophthalmos
☝️PHPV
☝️ROP
☝️tumors(RB and JXG)
☝️medulloepithelioma
✍️ syndromic associations
☝️Lowe’s syndrome (50%)
☝️ Reiger’s syndrome
☝️Sturge- Weber syndrome (50%)
• especially if nevus flammeus involves upper lid
• primary defect in angle
• increased episcleral venous pressure
☝️ neurofibromatosis (25% )
• if plexiform neurofibroma involves upper lid
• have hamartomatous infiltration of angle
☝️ congenital rubella (2%– 15%)
☝️Marfan’s syndrome
☝️homocystinuria
☝️Weill- Marchesani syndrom
☝️nevus of Ota
☝️trisomy 13 (Patau’s)
☝️Stickler’s syndrome
☝️mucopolysaccharidoses (Hurler’s and Hunter’s)
✅Childhood glaucoma Diagnosis
✍️ Examination under anesthesia (EUA)
☝️usually required for complete evaluation
☝️ketamine and succinylcholine raise IOP
☝️general anesthesia lowers IOP.
• halothane
• thiopental
• tranquilizers
• barbituates
☝️ Best time for IOP measurement is just as patient goes under and is not too deep
✍️ corneal diameter
☝️ horizontal diameter measures marginally more than vertical ( 0.5 mm).
☝️ 9 to 10.5 mm as normal at birth
☝️ > 12 mm in either meridian at 1 year is abnormal and one should look for other evidence of glaucoma
✍️ Gonioscopy
☝️landmarks are often poorly recognized due to Barkan’s membrane covering TM (but no histologic evidence of such a structure)
☝️Open-angle with anterior iris insertion above scleral spur
☝️ Thickening of TM
☝️Peripheral iris stromal hypoplasia.
Differential Diagnosis of Corneal Clouding (STUMPED)
☝️Sclerocornea
☝️Traumatic rupture
☝️Metabolic
☝️Peter’s anomaly
☝️Posterior polymorphous dystrophy of cornea
☝️Endothelial dystrophy
☝️Dermoid.
DD of buphthalmos
☝️ Congenital myopia
☝️Megalocornea
☝️Anterior megalophthalmos
☝️Keratoglobus
☝️Secondary glaucomas.
DD of watery eye
☝️ CNLDO
☝️ corneal abrasion ( trauma)
✅ Childhood glaucoma Treatment
✍️ definitive treatment is surgical
✍️ medication is a temporary measure
✍️ Goniotomy
☝️ perform in child < 1.5 years of age
☝️ requires clear cornea
☝️ corneal diameter < 14 mm
☝️ 77% success rate
✍️ Trabeculotomy (ab externo)
☝️if cornea hazy
☝️ corneal diameter < 14 mm
☝️ age > 1.5 years old
☝️ if goniotomy fails twice
☝️ 77% success rate
✍️ trabeculectomy with mitomycin C
☝️ If goniotomy and trabeculotomy fail
☝️ corneal diameter > 14 mm
✍️ drainage implant
☝️ if trabeculectomy failed
✍️ cycloablation of CB
☝️ in refractory glaucoma
✍️ Visual rehabilitation in advanced cases
🛑 The following parameters must be assessed postoperatively
☝️ Corneal clarity
☝️ Transient shallowing may occur in the first 3 to 4 days after the surgery
☝️Relief of photophobia
☝️Lowering of IOP in the low teens is preferable
☝️ Bleb: diffuse, pale blebs are seen after using MMC
☝️ Reversal or nonprogression of disk cupping
☝️ Reversal or nonprogression of myopia
☝️ stable axial length of the globe
☝️ Visual outcome: Good visual recovery, which may be difficult to document in children.
☝️ Tonometry may be possible under topical anesthesia while the infant is being fed with the bottle.
☝️ Use of topical or systemic steroids must not be prolonged beyond 6 weeks
