Journal Reading

Cataract extraction in patients with primary congenital

glaucoma
Nugraha Wirawan – 30101507529
Advisor :
dr. Hj. Christina Indrajati, Sp.M
KEPANITERAAN KLINIK ILMU PENYAKIT MATA
RSI SULTAN AGUNG SEMARANG
FAKULTAS KEDOKTERAN
UNIVERSITAS ISLAM SULTAN AGUNG SEMARANG
Journal Identity
ABSTRACT ABSTR
 INTRODUCTION

Primary congenital glaucoma (PCG)

→ The most common form of glaucoma in childhood, and a significant cause of vision
loss in children.

Diagnosed by two of the following criteria:

• intraocular pressure (IOP) higher than 21mmHg,
• suspicious optic disk cupping,
• increase in the corneal diameter or Haab Striae,
• increase in axial length or myopia,
• and altered visual field.

Classification :
• neonatal (less than 1month),
• infant (between 1 and 24 months),
• or late (more than 24months)
 INTRODUCTION

• Recessive autosomal pattern. Mutation of the gene CYP1B1

• They are considered as risk factors for a worse prognosis of a younger age at diagnosis
and CYP1B1 gene mutations.

• PCG is a consequence of the poor embryonic development of the angular structures,

and is therefore different from adult glaucoma. PCG is characterized by its poor
response to medical treatment. This requires the patient to undergo antiglaucoma
surgery, frequently more than once; however, these surgical interventions do not
guarantee good control of IOP.
 INTRODUCTION

• common for patients with PCG to require other surgical interventions to treat
abnormalities of the eye surface, cataracts, refraction defects and complications
involving the retina, and so on.

• Many of these abnormalities are products of PCG itself, but can be caused by
antiglaucoma surgery, such as a greater incidence of cataract surgery after glaucoma
surgery.
 INTRODUCTION

The aim of this study is to determine the incidence of, and risk
factors for, the need for CE in patients with PCG, as well as to
describe the results and complications of this surgery.
 METHODS

• Retrospective Cohort Study

• Single Medical Centre (Glaucoma department at the
Hospital Clínico San Carlos (HCSC, Madrid, Spain))
• Data collected from 1970 – 2016
• Total 198 eyes from 108 patients
 METHODS

Exclusion Criteria:
Patients with other types of early onset childhood glaucoma (inflammatory,
aniridia, dysgenesis, or iridocorneal endothelial syndromes…) were excluded, as
were those with coexisting congenital cataract(s) or cataract(s) at the time of
diagnosis of PCG.

Inclusion Criteria:
Patients were only included if all surgeries had been performed at the HCSC. All
patients were monitored for longer than 5years.
 METHODS

Data Collections:
• The principal variables were the requirement for CE, and the patient age when this
surgery was performed.

• CE was performed when visual acuity (VA) was reduced due to the presence on
biomicroscopic inspection of dense cataract (nuclear opacity, cortical opacity, or
posterior subcapsular opacity involving the center of the lens).
 METHODS

The following therapeutic algorithm was the surgical strategy followed with each
patient:
• Goniotomy is preferred as the primary surgical procedure as long as the cornea is
sufficiently transparent.
• TBC is chosen in the first instance only in cases where corneal edema or the
presence of leukoma at diagnosis prevents visualization of the angle structures.
• Ahmed Valve Implantation is considered when the other interventions fail.
RESULTS
 RESULTS

• 198 eyes of 108 patients with PCG were included,

• 55 males (51.0%) and 53 females (49.0%): 18 eyes were unilateral cases (9.09%) and
180 eyes (90 patients) were bilateral cases (90.9%).

• The median follow-up period was 8 years, (inter quartile range (IQR) 6.75–15.25; range
5–53)
RESULTS
 RESULTS

Incidence of need for CE

• A total of 32 eyes (16.16% of the eye sample) of 24 patients (22.22% of the patients)
eventually required CE
Analysis of predisponent factors
• There was no significant difference between incidence of cataract surgery in
unilateral versus bilateral PCG (p=0.068). The HR for cataract surgery in those with
bilateral PCG versus unilateral was HR 0.60 (95% CI 0.18–1.99), p=0.42 (Table 2

Surgical Complication
• 14 eyes of the total of the sample (all patients with PCG) (7 left and 7 right eyes)
developed phthisis resulting from endophthalmitis after TBC (n= 1), hypotony related
to glaucoma surgeries (n=4), retinal detachment (n=5), traumatism (n= 3), and after
penetrant keratoplasty (n=1).
 RESULTS

CYP1B1 gene mutation

• Were detected in 40 of 107 patients (37.4%; one patient declined to participate in this
part of the study). Some 28.2% was homozygous and 71.8% was heterozygous.
• No significant differences were found in terms of the presence of CYP1B1 mutations
between those eyes that eventually required CE and those that did not (Table 1).
Predictor need for CE
• The main risk : previous antiglaucoma surgeries performed (HR 1.48 (95% CI 1.29–
1.71); p<0.001) and the use of GDD (HR 2.78 (95% CI 1.76–4.40); p=0.001).
 RESULTS

Outcomes of CE
• Improvement in VA in 66.67% of the patients, which went from 0.12 (95% CI 0.04–
0.16) to 0.20 (95% CI 0.07–0.28) (Table 3).
• Reduction in the refractive sphere in 66.67% of the children. It was possible to implant
intraocular lens (IOL) in 30/32 eyes, the majority of them (24/30) in bag (Table 4), the
mean IOLs potency was +14.17 dioptres for a mean axial length of 26.75 mm. All the
implanted IOLs were monofocal.

Regarding complications :
• the most frequent was the loss of control of IOP (13 eyes)
- controlled with topical medication (9/13)
- the rest required a new hypotensive surgery
• capsular fibrosis (7/32),
• corneal decompensation (4/32),
• IOL subluxation (4/32),
• endophthalmitis (1/32)
RESULTS
RESULTS
RESULTS
 DISCUSSIONS

• This study shows the impact of these interventions on cataract development and the
factors that increase the risk that CE will become necessary.

• No antiglaucoma surgery is free of the risk of complications.

- Glaucoma filtering surgery accelerates cataract formation.
- TBC mainly increases the risk of needing CE over 5years after it is performed.
- GDD implantation increases the risk of the need for CE.

• The appearance of a cataract soon after antiglaucoma surgery is well known and may
be the result of the manipulations performed during surgery, such as contact with the
lens during goniotomy.
 DISCUSSIONS

• Other factors that might be considered as risk factors for the need for CE include
patient age at diagnosis of PCG, the seriousness of the patient’s condition, and the
first type of antiglaucoma surgery performed.

• CYP1B1 gene mutations were identified in 34.7% of the present Spanish patients with
PCG (Table 1). Mutations in CYP1B1 gene have been associated with more severe
glaucoma, a greater need for antiglaucoma surgery, and a poorer prognosis.
 CONCLUSSIONS

There is a high incidence of cataract surgery in patients with PCG

but generally outside of pediatric age.

We must consider a higher risk in those patients with greater

number of antiglaucomatous surgeries and especially in those
who underwent a valvular implant.
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