Childhood glaucoma

☝️ 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