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Congenital Glaucoma - Latin America - American Academy of Ophthalmology PDF
Congenital Glaucoma - Latin America - American Academy of Ophthalmology PDF
Academy Resource
NOV 2015
EPIDEMIOLOGY
Global Information
Congenital glaucoma is a heterogeneous group of diseases with the following classifications based
on age:
Congenital glaucoma (~40% of cases) is existent or becomes evident at birth.
Infantile glaucoma (~50% of cases) becomes evident during early childhood (<3 years old).
Juvenile glaucoma (~10% cases) becomes apparent in later childhood (>3 years old).
The incidence of primary congenital glaucoma (PCG) is about 1 in 10,000–18,000 live births and
depends heavily on ethnic origin.
PCG is highly prevalent in inbred populations and consanguinity is strongly associated with the
disease (Elder MJ, 1993).
Glaucoma-induced blindness in children is responsible for 18% of children in blind institutions and
5% of pediatric blindness worldwide (Gilbert et al, 1994).
In general, males are more affected than females (Ellong et al, 2007; Das et al, 2001).
In 75% of cases, both eyes are affected.
Ninety percent of cases occur sporadically, and only 10% of cases have an increased frequency in
their family, mostly with an autosomal recessive pattern of inheritance.
Penetrance varies in the range 40%–100%.
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One study including 3 ophthalmologic centers in Mexico City, found that 10 patients at 3 centers
(1.15%) had congenital glaucoma (Gilbert-Lucido et al, 2010).
In schools for the blind, congenital glaucoma was a prevalent cause of blindness.
Table 1. Prevalence of Congenital Glaucoma as a Cause of Vision Impairment in South America
DIFFERENTIAL DIAGNOSIS
For the classic triad of epiphora, photophobia, and blepharospasm:
Nasolacrimal duct obstruction
Conjunctivitis
Uveitis
Aniridia
For buphthalmos
High axial myopia
Megalocornea
PATHOPHYSIOLOGY/DEFINITION
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Abnormalities in anterior chamber development lead to the obstruction of the outflow of aqueous
humor. There are several theories of pathogenesis :
Anomalous trabecular meshwork development (neural crestopathy)
Barkan membrane covering the trabecular meshwork
Anterior insertion of the ciliary body (due to arrest in normal migration of the uvea) may
contribute to narrowing or collapse of Schlemm canal
Absent Schlemm canal
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Secondary Associated with congenital disease:
Iridotrabeculodysgenesis: aniridia
Phakomatoses:
Iridocorneal trabeculodysgenesis:
Congenital rubella
Herpes simplex iridocyclitis
Maternal use of topiramate
Fetal alcohol syndrome
SIGNS/SYMPTOMS
Signs
Enlarged optic cupping (cup to disc ration >0.2) and/or asymmetric cupping (Figure 3)
Newborn IOP >10–12 mmHg
Anterior insertion of the iris and Barkan membrane
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Haab-striae (cracks in Descemet membrane, Figure 4)
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Increased horizontal corneal diameter (above 11mm in first year of life, above 13 mm thereafter)
Symptoms
Triad
Epiphora
Photophobia
Blepharospasm
Note: Normal gonioscopy differences in all infants include trabecular meshwork more lightly pigmented,
Schwalbe line less distinct, and uveal meshwork translucence (Figure 9).
MANAGEMENT
The treatment of congenital glaucoma needs a team approach to normalize IOP, correct existing errors of
refraction and anterior segment architecture and to enable physiologic maturation of the visual pathway to
achieve as much vision as possible in the affected cases. If treated promptly and aggressively, useful vision
may develop, which in most cases is far from physiological but much improved compared to decades
before when PCG lead to blindness in nearly all cases.
Generally, the preferred mode of treatment for congenital glaucoma is surgical not medical.
Topical medication is rarely effective.
Compliance cannot be expected to be very high in a life-long disease that may lead to
blindness.
Surgical Treatment
First-line treatment:
Goniotomy and trabeculotomy (collectively, “angle surgery”) is the preferred treatment: ~90%
success rate
Goniotomy is performed when the cornea is clear and there is a clear view of angle structures.
Trabeculotomy (Figure 10) is performed when the cornea is cloudy.
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50%–95% success rate; difficult because of higher risk for infection, flat anterior
chamber, and hypotony (eye-rubbing)
Trabeculectomy with mitomycin C: 67%–87% success at 1 year, but 58%–59% success at 2 years
Combination trabeculotomy/trabeculectomy
Molteno, Baerveldt, and Ahmed device implantation (glaucoma drainage devices, Figure 11): 50%–
85% success with IOP-lowering drops
Treatments for challenging cases that have failed multiple, more conservative treatments, and cases with
limited visual potential:
Cyclodestructive procedures
Cyclocryotherapy: 33% success with high complications, suitable for cases with altered anterior
segment architecture due to repeated glaucoma surgery
Transscleral diode laser cyclophotocoagulation; 50% success in refractory glaucomas (Figure
12 and Figure 13)
Endolaser cyclophotocoagulation: 50% success (Figure 14)
Cycloablation/YAG for resistant cases
Medical Management
Medical management is preferred only in selected cases and as temporary treatment:
Oral carbonic anhydrase inhibitors (eg, acetazolamide and methazolamide) are most effective.
Topical carbonic anhydrase inhibitors (eg, dorzolamide 2%, brinzolamide 1%) are less effective at
lowering IOP but have fewer systemic side effects.
Topical beta-blockers (timolol 0.25%, levobunolol 0.25%, betaxolol) have been used for 30+ years.
Can cause respiratory distress, caused by apnea or bronchospasm, and bradycardia
Additional Treatment
Spectacles for refractive error
Treat amblyopia and strabismus
Tinted (or transition) lenses (may help with photophobia)
Frequent cycloplegic refraction (every 4–6 months) often necessary with rapid IOP changes
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CASE STUDY
History of Present Illness
Baby Jones was born at full term with no complications during his gestation. At 1 month after delivery, the
mother noticed that his eyes were watering, and he appeared to not like bright lights. She noticed that at
times it seemed as if he would squeeze his eyes shut. The baby’s pediatrician noticed that his corneas
appeared cloudy, and he was referred to an ophthalmologist.
Examination
The ophthalmologist found an elevated intraocular pressure, an increased corneal diameter of 15mm, and
microcystic edema with Haab striae. On dilated fundus exam, the child’s optic nerve heads appeared
normal.
Treatment
He was started on a topical prostaglandin analog. IOP was reduced below 12mmHg in both eyes. He was
then referred to an ophthalmological center with experience in congenital glaucoma treatment. After a
thorough examination under anesthesia, goniotomy was performed in the right eye and at a later point in
time in the left eye. On further examinations, though IOP was normal, a mild amblyopia was detected in the
left eye, probably due to an anisometropic refractive error. Patching was carried out and the refractive error
was treated with glasses. Baby Jones is now 11 years old and has a visual acuity of 20/80 in his right eye
and 20/200 in his left eye. He attends a special school.
IMAGE LIBRARY
Pathophysiology/Definition
Figure 1. Typical appearance of the anterior chamber angle of an infant with congenital glaucoma.
(© 2015 American Academy of Ophthalmology, www.aao.org. Courtesy of Ken K. Nischal, MD.)
Signs/Symptoms
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Figure 2. Sturge-Weber syndrome. A. Sturge-Weber syndrome with increased tortuosity of limbal blood
vessels due to elevated venous pressure leading also to an increase in intraocular pressure. B. Sturge-
Weber syndrome classically comprises the triad of port-wine facial telangectasias (nevus flammeus) in the
distribution of the trigeminal nerve that respects the vertical midline, ipsilateral glaucoma, and intracranial
angiomata. (© 2015 American Academy of Ophthalmology, www.aao.org).
Figure 3. Asymmetry of optic nerve cupping. Note the generalized enlargement of the cup in the right eye
(A) as compared with the left eye (B). Asymmetry of the cup–disc ratio of more than 0.2 occurs in less than
1% of individuals without glaucoma. (© 2015 American Academy of Ophthalmology, www.aao.org.)
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Figure 4. Haab striae. A. Haab striae in Descemet membrane. B. Better seen with retroillumination. (©
2015 American Academy of Ophthalmology, www.aao.org.)
Figure 5. Corneal clouding. Right eye demonstrates cloudy and enlarged cornea in this infant with
congenital glaucoma. (© 2015 American Academy of Ophthalmology, www.aao.org.)
Figure 6. Buphthalmos. A. Buphthalmos in a child with a history of congenital glaucoma. B. Haab striae
with corneal edema and opacification. (© 2015 American Academy of Ophthalmology, www.aao.org.)
Figure
S H A R E S 7. Aniridia in an infant. (© 2015 American Academy of Ophthalmology, www.aao.org.)
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Figure 8. Ectopia lentis. Anterior displacement of the lens into the anterior chamber resulting in iris bombe
and shallow anterior chamber. (© 2015 American Academy of Ophthalmology, www.aao.org.)
Figure 9. The anterior chamber angle of a normal infant’s eye, as seen by direct gonioscopy with a Koeppe
lens. (© 2015 American Academy of Ophthalmology, www.aao.org. Courtesy of Ken K. Nischal, MD.)
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Figure 10. Example of handpiece (Trabectome) used in microsurgical management of adult and infantile
glaucoma. (Image courtesy of NeoMedix Corp.)
Figure 11. Examples of glaucoma drainage devices (aqueous shunts). A. Single-plate (left) and double-
plate (center and right) Molteno implants. B. Baerveldt glaucoma implants: 250 mm2 (left), 350 mm2
(center), and 425 mm2 (right). C. Krupin eye valve with disk, D. Ahmed glaucoma valve. (Reproduced, with
permission, from Sidoti P. “Aqueous Shunting Procedures.” Focal Points: Clinical Modules for
Ophthalmologists. American Academy of Ophthalmology, No. 3, 2002.)
MANAGEMENT
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Figure 12. Cyclophotocoagulation. Probe handpiece attachment, which facilitates orientation parallel to
visual axis. (Courtesy of Iridex.)
Figure 13. Transscleral diode laser cyclophotocoagulation being performed in a child with congenital
glaucoma refractive to other treatments. Transillumination probe being utilized to aid in the correct
localization of ciliary body. (Reproduced from Morales J, et al. Current surgical options for the
management of pediatric glaucoma. Journal of Ophthalmology. Hidawi Publishing: 2013.)
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Figure 14. Endoscopic cyclophotocoagulation in a case where a tube shunt may be failing . (Reproduced
from video. Subspecialty Day 2012: Pediatric Ophthalmology, David A. Plager, Advances in Glaucoma
Care: New Technologies in Pediatric Glaucoma.)
REFERENCES
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in childhood glaucoma in Saudi Arabia. Acta Ophthalmol. 2012;92(1):65–70.
Aponte EP, Diehl N, Mohney BG. Incidence and clinical characteristics of childhood glaucoma: a
population-based study. Arch Ophthalmol. 2010;128(4):478–82.
Banchio C, Castro RI. Ceguera infantil en la provincia de Neuquén. Médico Oftalmól. 2008;21(5):38–40.
Ben-Zion I, Tomkins O, Moore DB, Helveston EM. Surgical results in the management of advanced primary
congenital glaucoma in a rural pediatric population. Ophthalmology. 2011;118(2):231–5.
Bhattacharjee H, Das K, Borah RR, et al. Causes of childhood blindness in the northeastern states of
India. Indian J Ophthalmol. 2008;56(6):495–9.
Das J, Bhomaj S, Chaudhuri Z, Sharma P, Negi A, Dasgupta A. Profile of glaucoma in a major eye hospital
in North India. Indian J Ophthalmol. 2001;49(1):25–30.
Dorairaj SK, Bandrakalli P, Shetty C, RV, Misquith D, Ritch R. Childhood blindness in a rural population of
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Ellong A, Ebana Mvogo C, Nyouma Moune E, Bella-Hiag A. Juvenile glaucoma in Cameroon. Bull Soc
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Friedberg M, Rapuano C. Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye
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EPIDEMIOLOGY
Global Information
DIFFERENTIAL DIAGNOSIS
PATHOPHYSIOLOGY/DEFINITION
SIGNS/SYMPTOMS
Signs
Symptoms
MANAGEMENT
Surgical Treatment
Medical Management
Additional Treatment
CASE STUDY
Examination
Treatment
IMAGE LIBRARY
Pathophysiology/Definition
Signs/Symptoms
REFERENCES
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