1. Glaucoma after Congenital Cataract Surgery
여의도성모병원 안과
조교수 박명희

2. Developmental and childhood glaucoma
Primary glaucoma
Secondary glaucoma
Congenital open angle glaucoma
Autosomal dominant juvenile glaucoma
Glaucoma associated with systemic abnormalities
Axenfeld-Rieger syndrome
Congenital rubella
Chromosomal disorders
Glaucoma associated with ocular
abnormalities
Aniridia
Microcornea syndromes
Peters syndrome
PPMD
Sclerocornea
Traumatic glaucoma
Glaucoma secondary to intraocular neoplasm
Uveitic glaucoma
Lens-induced glaucoma
Steroid induced glaucoma
Neovascular glaucoma
Secondary angle-closure glaucoma
Glaucoma with increased episcleral venous pressure
Glaucoma secondary to intraocular infections
Glaucoma after congenital cataract surgery

3. Developmental and childhood glaucoma
Of all pediatric glaucomas in a tertiary-referral setting, aphakic glaucoma was responsible for 20% of cases, second only to primary congenital glaucoma. (Taylor et al., J AAPOS 1999)
Among 1492 hospitalized pediatric glaucoma patients in Beijing Tongren Hospital from 2002 to 2008, congenital glaucoma was 46.07%, traumatic glaucoma was 12.13%, and aphakic glaucoma was 9.19%. (Qiao et al., Chin Med J 2009)

4. Incidence of glaucoma after pediatric cataract surgery : Approximate frequency of 10-25% commonly cited
Of 392 consecutive childhood cataract aspiration procedures, chronic glaucoma was found in 6.1% of the eyes during mean 5.5 years follow-up. Coexisting ocular anomalies and retained lens cortex increased the risk for this complication. (Chrousos et al., Ophthalmology, 1984)
After mean 9.6 years follow-up of 137 aphakic eyes, 12% of the eyes developed glaucoma. (Magnusun et al., Acta Ophthalmol Scand 2000)
At a median of 15.1 years after surgery, a majority of the subjects had glaucoma or ocular hypertension (ie, 59%; 37/63). 19% (12/63) had glaucoma (5/22 with bilateral cataracts and 7/41 with unilateral cataracts). Approximately half (7/12) had developed glaucoma during the first 5-year observational period and the remainder (5/12) developed it during the following observational period. (Egbert et al., J AAPOS 2006)
At mean follow-up 9.0 years after cataract surgery, glaucoma developed in 118 of 570 patients' eyes (21%). (Rabiah, AJO 2004)
Of 269 aphakic eyes, 62 (23%) eyes were diagnosed with glaucoma(36 of 130 patients, 27.7%) an average of 59 months (Al-Dahmash et al., Eye & contact lens, 2010).
2 out of 20 pediatric aphakic patients (10%) developed glaucoma after months follow-up. (Kim et al., KJO 2008)

5. Approximately 20% (or more
Approximately 20% (or more!) of children who had a cataract extraction will at some point of their lifetime develop glaucoma.

6. Pediatric cataract 1 per every 2000 births Cause Idiopathic (m/c)
Autosomal dominant > autosomal recessive > sex chromosome related
Associated with hereditary, metabolic, systemic disease
Intrauterine infection (TORCHS)
Associated with ocular abnormalities
Aniridia
Anterior segment dysgenesis
Microphthalmia
PHPV
Posterior lenticonus
Traumatic

7. Pediatric cataract surgery
Corneal incision
Side port paracentesis
Limbal incision
Anterior capsulectomy
CCC
Vitreous-cutting instrument
Lens aspiration
Posterior capsulectomy
Anterior vitrectomy with two port system
(±) PC-IOL implantation with optic capture
Corneal suture

8. Etiology of aphakic glaucoma
Lens debirs or uveitic blockade of trabeculum
Pupillary blockade
Pigment dispersion
Peripheral anterior synechiae / Trabecular damage
Chronic open-angle

9. Etiology of aphakic glaucoma

10. Risk factors for developing of chronic open angle glaucoma
Chen et al., JPOS 2006
Early age of cataract surgery (before 4 months)
Postoperative complications
Multiple procedure
Small corneas (<10mm)
Family history of aphakia
Congenital rubella syndrome
No IOL ?
Khan et al., J AAPOS 2008

11. Hypothesis of pathophysiological mechanism of chronic open angle aphakic glaucoma
chronic trabeculitis from postoperative inflammation
blockade from retained lens material
chemical factors from the vitreous (Phelps & Arafat 1993).

12. Hypothesis of pathophysiological mechanism of chronic open angle aphakic glaucoma
A manifestation of a single ocular syndrome with an abnormal anterior segment
The defect responsible for producing the cataract may also affect aqueous outflow in the angle, causing open angle glaucoma (Phelps & Arafat 1993).
Early lensectomy may interfere with maturation of the trabecular meshwork.
Further damage by surgical trauma and exposure to the vitreous and high-dose steroid
Kang et al., KJO 2006

13. Effect of IOL on the development of chronic open-angle glaucoma
Asrani et al. (J AAPOS 1999)
Comparison of the incidence of open-angle glaucoma in the two large groups of pseudophakic and aphakic pediatric patients
a reduced incidence in the pseudophakic eyes.
Patients in the study routinely had IOLs implanted when the corneal diameter was > 10 mm.
1/377 pseudophakic
Eyes during 3.9 years f/u
14/124 aphakic eyes during 7.2 years f/u

16. Effect of IOL on the development of chronic open-angle glaucoma
2 theories for the lower incidence in pseudophakic eyes (Asrani et al. J AAPOS 1999)
Chemical theory
In aphakia, a vitreous chemical component (toxic to the trabecular meshwork) may have access to the trabecular meshwork, resulting in damage.
This is prevented in pseudophakic eyes by the presence of an implant that forms a barrier to the vitreous.
Mechanical theory
The support to trabecular meshwork is lost in aphakia. A disorganization or collapse of the TM results in its diminished function as a filter.
Placement of a PC IOL might minimize this loss of support.

18. No relationship?
Cataract surgery at an early age (< 4.5 months) are risk for the development of glaucoma c/s an IOL implant (Trivedi et al., J AAPOS 2006)

20. How to find a glaucoma? IOP Corneal cloudiness Corneal diameter
Refractional change: less hyperopic
Axial length
Optic nerve

21. Examination of children
Office examination
A complete ocular examination can be performed in the office in children older than 5 years of age and in some children as young as 3 with some training.
Many children after age 5 can undergo kinetic Goldmann VF testing with the assistance of a parent.
By the age of 8-10 years, some children can cooperate for a full quantitative visual field examination.
For an infant, a careful examination can be performed over a bottle or breast milk.
Chloral hydrate (100mg/kg of BW, maximum dose of 3g) can be given if necessary and does not affect IOP readings.

22. Examination of children
Examination under anesthesia (EUA)
General anesthesia is usually required for a thorough examination of children under the age of 5.
Most of general anesthetics lower IOP to variable amounts and at variable times after administration.
IV Ketamine may raise IOP slightly.
Succinylcholine and endotracheal intubation significantly elevate IOP.

23. Examination of children
Examination under anesthesia (EUA)
The sequential component of the EUA
IOP
Corneal thickness and diameters
Goniosocpy
Ophthalmoscopy
Axial length
UBM
Cycloplegic retinoscopy

24. IOP among normal awake children using different tonometers
Age
Pulsair (SD)
Pensiero et al., JPOS 1992
Perkins (SD)
Jaafar et al., JPOS 1993
Pneumatonometer (SD)
Spierer et al., 1994
Premature
(26-37 weeks)
10.2
18.3 -
0-1 yr
10.6 (3.1)
4.6 (0.5)
14.5 (0.5)
1-2 yrs
12.0 (3.2)
4.9 (0.5)
14.6 (0.6)
2-3 yrs
12.6 (1.5)
5.8 (1.0)
15.3 (1.4)
3-4 yrs
13.7 (2.1)
6.4 (1.8)
14.5 (0.9)
4-5 yrs
14.4 (2.0)
7.9 (1.3)
14.8 (2.0)
5-6 yrs
6-7 yrs
14.2 (2.3)
7-8 yrs
14.0 (2.5)
8-9 yrs
14.3 (1.7)
9-10 yrs
14.0 (2.7)
15-16 yrs
15.2 (2.4)
13.2
16.42 (2.2)

25. Corneal measurements: diameter
A good baseline measurement is required for
Initial diagnosis
Detection of subsequent corneal enlargement under age 3
Age
Corneal diameter
(horizontal, in mm)
Normal
Suspicious for Possible Glaucoma
Birth – 6 months
>12
1-2 years
10-12
>12.5
Older child
=<12
>13

26. Corneal measurements: diameter
Corneal diameter measurement
Horizontal (3 to 9 o’clock) and Vertical (6 to 12 o’clock)
From white to white
Co-examination for corneal haziness and tears of Descemet’s membrane (Haab striae) is needed.

27. Axial lengths among normal eyes and eyes suspicious for glaucoma
Age
Axial length
(mm)
Normal
Suspicious for Possible Glaucoma
Newborns
16-17
>20
1 year
20.1
>22.5
2 years
21.3
>23
3 years
22.1
>24
>3 years 23
>25
Sampaolesi et al., Arch Ophtahlmol 1982

28. Corneal measurements: central thickness
CCT
The clinical effect of CCT measures on IOP in children is similar to that seen in adults.
In congenital glaucoma, CCT is thinner in glaucomatous eyes and positively correlated with their larger corneal diameters and longer axial lengths.
CCT in children without glaucoma
Hussein et al., Am J Ophthalmol 2004

29. Effect of IOL implantation on CCT
In the absence of factors known to affect CCT, CCT is similar in eyes with congenital cataract and normal controls and increases after cataract surgery (Muir et al., AJO 2007)

30. Effect of IOL implantation on CCT
CCT in children with cataracts increases after cataract surgery while the fellow eye remains stable. This increase seems to occur early after surgery, likely remaining stable thereafter, though glaucoma can accentuate the increase. (Lim et al., AJO 2011)

31. CCT in various kinds of pediatric glaucoma
Patients with aphakic glaucoma are different from those with congenital glaucoma.
Lopes et al., JPOS 2007
Tai et al., J glaucoma 2006

33. Ophthalmoscopy
Fundus examination is challenging in many aphakic/pseudophakic children because of small pupil, PCO, persistent fetal vasculature, and nystagmus.
But glaucoma should not be diagnosed by single high IOP itself unless extreme.
Direct ophthalmoscopy can be used during EUA.
C/D >0.3 is rare in healthy infants.
With successful control of the IOP, the cup will either remain stable or its size will decrease.
Concentric enlarging of cupping is more common than vertical notching in children.

34. Treatment of pediatric aphakic glaucoma
Medication
Surgery
Goniotomy and/or Trabeculotomy
Variable success rate reported (16~57%)
Multiple procedures may be needed
Trabeculectomy c MMC soaking
Glaucoma Shunt Devices
Trans-scleral diode laser cyclodestruction

35. Pediatric aphakic glaucoma
Second most common type of pediatric glaucoma
Approximately 20% of incidence during lifetime after congenital cataract surgery
Chronic open angle glaucoma is more common than acute angle closure glaucoma.
If any risk factor is accompanied, careful lifetime examination is necessary.
IOL implantation may play a protective role in glaucoma development.
CCT may change after cataract surgery and thus influence on IOP measures.
Elevated IOP by itself, unless extreme, is insufficient to conform the diagnosis of glaucoma.

36. Variables to consider when assessing a child’s IOP
The child’s age
The patient’s level of activity or sedation
Effects of anesthetics
Corneal thickness and health
Diurnal variations
Measuring instrument

37. University of California, San Francisco (UCSF), Beckman Vision Center, Glaucoma Service
Mentor: Robert L Stamper, M.D.
Professor of Ophthalmology
Director of the Glaucoma Clinic
the chairman of the Prevent Blindness Northern California

38. University of California, San Francisco (UCSF), Beckman Vision Center, Glaucoma Service