CC CALIMOSO, KAI B.

TOPIC: CONGENITAL CATARACT

Patient Background

This is a case of patient K., an 8-month-old male who came in due to photosensitivity. Patient
was born to a G1 (1001) mother, who had regular prenatal check-up with no infection or
complications as claimed. Patient was delivered full term and was discharged without
complications. At birth, folks claimed that there was note of lens opacity but no consult, nor
meds were done. Interim, persistence of lens opacity. 1 week prior to consultation, folk noted
photosensitivity and persistence of lens opacity. consult was done and was diagnosed to have
congenital cataract and was advised ultrasound thus consult.

Pediatric Cataracts

Pediatric cataracts are one of the leading causes of treatable childhood blindness. If left
untreated, it can have a significant impact socially, economically, and emotionally on the child,
family, and society at large. This remains a challenging entity in ophthalmological practice
because of the need to identify, diagnose and manage the condition as early as possible in
order to prevent amblyopia.
Routine screening and awareness among parents about leukocoria and strabismus lead to
early diagnosis and management. A good pre-op evaluation, intraocular lens (IOL) power
calculation, meticulous surgery, and equally efficient postoperative care and visual
rehabilitation are important for a favorable outcome. An interdepartmental effort involving
pediatrics, anesthesia, ophthalmology, and optometry help in the proper and effective
management of pediatric cataract.

Etiology
Causes of pediatric cataracts are diverse and can range from being idiopathic to being
associated with systemic disorders. They can be unilateral or bilateral based on etiology. A
majority of unilateral and some bilateral cases are idiopathic.

Bilateral Cataract
§ Idiopathic
§ Hereditary cataracts (autosomal dominant most common, also autosomal recessive or X-linked)
§ Genetic and metabolic diseases
→ Down syndrome
→ Hallermann-Streiff syndrome
→ Lowe syndrome
→ Galactosemia
→ Marfan syndrome
 → Trisomy 13-15
→ Hypoglycemia
→ Alport syndrome
→ Myotonic dystrophy
→ Fabry disease
→ Hypoparathyroidism
→ Conradi syndrome
§ Maternal infection
→ Rubella
→ Cytomegalovirus
→ Varicella
→ Syphilis
→ Toxoplasmosis
§ Ocular anomalies
→ Aniridia
→ Anterior segment dysgenesis syndrome
§ Toxic
→ Corticosteroids
→ Radiation (may also be unilateral)
Unilateral Catracts
§ Idiopathic
§ Ocular anomalies
→ Persistent fetal vasculature (PFV)
→ Anterior segment dysgenesis
→ Posterior lenticonus
→ Posterior pole tumors
§ Traumatic (rule out child abuse)
§ Rubella
§ Masked bilateral cataract

Evaluation
Laboratory Investigations

1. Blood and Routine Investigations

o Complete hemogram
o Blood sugar
o Urine routine microscopy
2. Other Tests (case to case basis depending upon the history and examination)
o Serum calcium for hyper or hypoparathyroidism.
o VDRL for syphilis
o Antibody titer for TORCH.
o Red cell galactokinase or uridyl transferase for galactosemia
o Urine protein for Alport syndrome
o Urine amino acid for Lowe syndrome
o Urine sodium nitroprusside/ plasma homocysteine for homocystinuria
o Urine/serum copper for Wilson disease
o Karyotyping for a genetic defect
Ocular Investigations

1. Ultrasound B scan: B scan ultrasonography has to be performed to rule out other

posterior segment pathologies that mimic congenital cataract (other causes of
leukocoria include retinoblastoma, persistent hyperplastic primary vitreous, coats
disease, ROP with retrolental fibroplasia, organized vitreous hemorrhage, congenital
falciform fold, ocular toxocariasis, and retinal hamartomas.
2. Ultrasound biometry can also help to obtain optical parameters such as axial length
(AL), anterior chamber depth (ACD), and lens thickness (LT), which is crucial for IOL
power calculation.
3. Keratometry: Using handheld keratometers. A child’s cooperation is the key to
accurate keratometry readings. In the case of an uncooperative child, standard K
values of 43.00 D can be used.

Treatment/Management

Managing pediatric cataracts requires a team effort consisting of ophthalmologists,

pediatricians, anesthesiologists, counselors, and parents/family members. Before surgery, the
family members are counseled regarding the visual prognosis, the different available
treatment options, and the need for regular follow-up, post-operative visual rehabilitation, and
compliance to the amblyopia therapy, if required.

Treatment Options
Mydriasis: Use 2.5% phenylephrine as we need only mydriatic action. This method is used
when the partial or nonamblyogenic cataract is present to permit vision through the non
opacified area.
Optical iridectomy: no longer used. The same purpose as mydriatics; that is, it permits vision
through the non-opacified area.
Surgery

1. Indications:
o Presence of any visually significant opacity
o Cataract with vision ≤ 20/60 or disc not visible with an indirect ophthalmoscope
o Central cataract ≥ 3 mm
o Posterior subcapsular cataract
o Nuclear cataract
o Bilateral cataract
o Cataract associated with strabismus, nystagmus
2. Anesthesia: general anesthesia, along with constant monitoring of vital parameters, is
preferred for cataract surgery. A trained anesthesiologist team and interdepartmental
support are required in dealing with children.
3. When to operate: surgery should be performed as soon as possible for visually
significant cataracts, ideally within a few weeks after birth to prevent amblyopia. For
nonamblyogenic cataract, surgery is better planned after 4years of age as ocular
development will be complete and post-operative complications are less, but close
follow-up and visual monitoring is required to prevent amblyopia and treat as indicated.
The consensus is to operate a unilateral cataract as early as 4-6 weeks after birth.
Bilateral cataracts should be operated on by 6-8 weeks, and the second eye should
be operated on within 2 weeks of the first surgery.
4. Type of surgery and the surgical challenges: there has been a paradigm shift and
advancement in cataract surgeries. Discission has been replaced by modern-day
phacoaspiration and IOL implantation. Choyce and Binkhorst first performed the
implantation of a monocular intraocular lens in a child's eye in 1959. Pediatric cataract
surgery is different from adult cataract surgery and poses various intraoperative
challenges: low scleral rigidity causes difficulty in incision construction and wound
closure, the smaller size of the eyeball, shallow anterior chamber depth and small pupil
size causing decreased maneuverability, elastic capsule, high positive intravitreal
pressure and risk of vitreous loss and expulsion of intraocular contents. Advantages of
primary IOL implantation are immediate post-operative refractive correction, minimal
or no optical aberration, full visual field, less chance of development and progression
of amblyopia, strabismus, nystagmus, and minimal dependence on patients'
compliance. IOL implantation in below 2 years of age is still a controversial topic. Lack
of long-term data to predict the success rate, associated other ocular abnormalities
along with cataract, frequent occurrence of deprivational amblyopia, and increased
postoperative complications are the main reason for limited uses of IOL implantation
in children below 2 years of age. Infant Aphakia Treatment Study (IATS) showed higher
intra-op and post-op complications and the need for additional surgeries in children
with IOL implantation in less than 1 yr of age. A consensus regarding surgery
performed, and IOL implantation is as follows:
1. Less than 18 months to 2 years of age - Lens aspiration without IOL with
posterior curvilinear capsulorhexis (PCCC) with limited anterior vitrectomy
(LAV), leaving the child aphakic and giving post-op aphakic correction followed
by secondary IOL implantation at a later age.
2. 2 years to 5 years- Lens aspiration with IOL implantation with PCCC with LAV.
 3. More than 8 years - Lens aspiration with IOL implantation with PCCC without
LAV.
4. More than 8 years - Phacoaspiration with IOL implantation like an adult cataract
surgery.
5. PCCC (in < 8 years) and LAV (in < 5 years) are important in younger children
because of the higher risk of VAO post-surgery.
5. IOL POWER CALCULATION IN CHILDREN: Accurate IOL power calculation is
essential for post-surgical refractive outcomes in children. It is challenging both in
terms of calculating it and adjusting it for the age of the child and/or axial length of the
eye. Biometry and keratometry measurements differ according to the type of
instruments and method used to measure them. Studies have shown that SRK/T and
Holladay 2 formulae had the least predictive error (PE) among the various IOL formulas
available. Newer IOL formulas are being investigated to optimize the variability of IOL
power in children. Emmetropisation is the change of three major variables, namely
axial length, corneal curvature, and lenticular power from its birth values to its adult
values as the child grows. As the size of the eyeball grows, axial length increases, and
there is a myopic shift. Thus, the initial desired refractive outcome after IOL
implantation is hypermetropia. Various studies have shown that under correction of the
required IOL power depending upon the child's age improves the refractive outcome
in these children post-surgery. Dahan and Drusedau suggested an under correction of
20% in <2 years; 10% between 2 and 8 years. A consensus is to have residual
refraction of +6D (1 to 2 years), +5 (2 to 4 years), +4 (4 to 5 years), +3 (5 to 6 years),
+2 (6 to 7 years), and plano for >14 years.

Prognosis

Numerous factors affect the final visual outcome in a child with a pediatric cataract. Visually
significant cataract produces blurred images on the retina and thus affect the development of
visual pathways and connections in the occipital cortex. Today, with a better understanding of
the consequences of cataracts in the early age group and the advancement of the surgical
techniques to deal with the same, it is recommended to remove a visually significant cataract
at the earliest to prevent sensory deprivation amblyopia. Unilateral cataracts are by far more
amblyogenic than bilateral cataracts and thus to be operated on within the first few weeks to
months.
The time of diagnosis of a pediatric cataract also plays a crucial role in prognosticating the
final visual outcome—the earlier the diagnosis, the earlier the treatment, the better the
prognosis, and vice versa. Associated ocular diseases include microcornea, corneal opacities,
glaucoma, intraocular inflammation, posterior segment abnormalities, and ocular movement
disorders such as unsteady fixation, strabismus, nystagmus, or nystagmoid movements
denote poorer prognosis post-surgery.

References:
1. American Academy of Ophthalmology . Eye Health Statistics at a
Glance. url: https://web.archive.org/web/20150317004848/http://www.aao.org/newsro
om/upload/Eye-Health-Statistics-April-2011.pdf Accessed: February 24, 2017.

2. Gupta P, Patel BC. Pediatric Cataract. [Updated 2023 May 29]. In: StatPearls
[Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from:
https://www.ncbi.nlm.nih.gov/books/NBK572080/