2735 Pediatric Cataract Manual - Compressed

ORBIS International
Cataracts in
Childhood
Cataracts in Childhood
Daniel E. Neely MD, M. Edward Wilson MD,

David A. Plager MD and Itay Ben Zion, MD
This manual is part of a series of specialized manuals produced

by ORBIS Telemedicine, Cyber-Sight.
Edited by:
Eugene M. Helveston, MD
Lynda M. Smallwood
Illustrations by:
Sharon Teal
Dedicated to
All the children
and
their families
November 2011
TABLE OF CONTENTS
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
Classification of Pediatric Cataracts by Age of Onset
. . . . . . . . . . . . . . . . . . . . . . . . . . . 11
Congenital . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
Infantile . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
Juvenile . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
Morphology of Common Pediatric Cataracts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22

Anterior Polar Cataract . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Anterior Subcapsular Cataract . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Lamellar Cataract . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Nuclear Cataract . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Posterior Subcapsular Cataract . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Posterior Lentiglobus (Posterior Lenticonus) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Diffuse or Total Cataract . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Persistent Fetal Vasculature (PFV) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Etiology of Pediatric Cataracts
22
22
22
33
44
44
55
55
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
Idiopathic . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
Hereditary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
Ocular Syndromes with Associated Cataracts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
Systemic Syndromes with Associated Cataracts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88
Metabolic Cataracts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99
10
Traumatic Cataracts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
Step-by-Step Approach for Pediatric Cataract Surgery
. . . . . . . . . . . . . . . . . . . . . . . . . . 11
11
Preoperative Routine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
11
11
Preoperative Laboratory Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
12
Preoperative Drops . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
12
Examination of Both Eyes Under Anesthesia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
13
Intraocular Lens Calculations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
14
Cataract Surgery Techniques . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14
15
Cataract Surgery Without IOL in Children 4 Years of Age and Younger: Vitrector Technique . . 15
Cataract Surgery With IOL and Primary Posterior Capsulotomy Via Anterior Approach in
23
Children 4 Years of Age and Younger: Vitrector Technique . . . . . . . . . . . . . . . . . . . . . . . . 23
Cataract Surgery With IOL and Primary Posterior Capsulotomy Via Pars Plana Approach in
Children 4 Years of Age and Younger: Vitrector Technique . . . . . . . . . . . . . . . . . . . . . . . . . 36
36
Cataract Surgery With IOL in Children 5 Years of Age and Older: Irrigation/Aspiration
Technique (and no posterior capsulotomy or anterior vitrectomy) . . . . . . . . . . . . . . . . . . . . 50
50
Postoperative Management of Pediatric Cataract Patients . . . . . . . . . . . . . . . . . . . . . . . . 50

50
Postoperative Medications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52
52
Postoperative Follow-Up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52
52
Refractive Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53
53
Amblyopia Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
54
Secondary Opacification of the Visual Axis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55
55
Secondary IOL Implantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55
55
Long-Term Follow-Up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56
56
Anesthesia for Pediatric Cataract Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 57

57
Summary
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59
59
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60
60
INTRODUCTION
Appropriate management of the pediatric cataract patient presents the ophthalmic surgeon with
a unique set of challenges. The entire process, from diagnosis and evaluation to surgical
intervention and postoperative care, is significantly different from that of the typical adult
cataract patient. This is highlighted by the need for close follow up and support, both by the
family and the ophthalmologist, to help the child develop vision and avoid amblyopia by
employing appropriate optical correction and regular visual stimulation. Therefore, while an
ophthalmologist may be extremely skilled in performing adult cataract surgery, this does not
ensure similar success in children. The purpose of this monograph is to describe the unique
characteristics of congenital and childhood cataracts emphasizing the differences in treatment
techniques compared with the adult cataract patient. The reader is offered a basic,
standardized technique for approaching pediatric cataracts in early childhood. The techniques
presented are not intended to be the only way to perform pediatric cataract surgery but are
generally accepted and considered to be safe, effective, and relatively easy to master. From
this basic technique, the reader is free to modify as needed to fit personal preferences and
adapt to equipment and supplies available. Considering all of us have something to learn, we
welcome your input. If you have a particular surgical tip or tool that you feel would benefit
other pediatric cataract surgeons, please feel free to contact us with your suggestion. Also
remember that the cataract removal is only the first step in the process. Refraction, amblyopia
treatment, and frequent follow up visits monitoring the reaction of the eye, are essential steps
leading to successful surgery.
CLASSIFICATION
OF
PEDIATRIC CATARACTS
Congenital: Present at birth
Infantile: Develop within the first 2 years of life
w Juvenile:
BY
AGE
OF
ONSET1,2
Develop before 10 years of age
MORPHOLOGY OF COMMON PEDIATRIC CATARACTS

u
Anterior Polar Cataract
Small (usually less than 1-2 mm) white opacity of the central anterior lens capsule
i Usually do not increase in size over time
Commonly bilateral
Can be hereditary and present at birth
Favorable visual prognosis
i Usually do not require surgery

i Patients may be found to have significant astigmatic refractive errors
monitor refractive error and correct as needed
v Anterior Subcapsular Cataract
Irregular, refractile or crystalline partial opacities usually seen just beneath the central
anterior capsule
May be secondary to radiation, uveitis, trauma or atopic skin disease
i May require surgery if vision is reduced
w Lamellar Cataract
Partial or complete opacity of the cortical layers surrounding the central lens nucleus
i Peripheral lens cortex may be clear

i Commonly have an associated posterior capsule plaque opacity
2
Usually acquired and bilateral but may be asymmetric
Opacity may initially be mild but tends to progressively worsen over time
i Frequently has a more favorable visual prognosis compared to dense fetal nuclear
cataracts that are more opaque at birth
Lamellar opacities of cortex have a tendency to push forward out of the capsular bag
during aspiration and irrigation of lens material
Usually normal sized eyes
Nuclear Cataract
Central white opacity of the lens nucleus (area within the Y sutures; the Y is anterior
and the posterior)
i Peripheral cortex is usually clear but may have irregular opacification adjacent to
the nucleus and this tends to worsen over time
i Commonly have an associated posterior capsule plaque opacity
Probably the most common type of congenital cataract
Usually associated with mild microphthalmia or microcornea
Severe impact on visual development unless removed at a very early age
i Unilateral cataract ideally treated and optically corrected before 6 weeks of age
i Bilateral cataract ideally treated and optically corrected before 8 weeks of age
i Later treatment or delayed optical correction may result in irreversible amblyopia
and nystagmus
Surgery should be performed (if possible) before appearance of nystagmus
Note that even in late presentation and with nystagmus, surgery usually has good
results with favorable prognosis including attaining functional vision
Posterior Subcapsular Cataract
Irregular, refractile or crystalline, partial opacities usually seen just beneath the central
posterior capsule
May be idiopathic but are most commonly seen secondary to radiation, trauma or
chronic steroid use
i While a less common cause, posterior subcapsular cataracts are frequently found
in children with a diagnosis of Neurofibromatosis Type 2. Therefore, evaluation for
this diagnosis should be considered if the etiology of a posterior subcapsular
cataract is unexplained.
May require a posterior capsulotomy (surgical or YAG-laser) to completely clear the

visual axis
Posterior Lentiglobus (Posterior Lenticonus)
Bulging of the posterior capsule that is progressive over time and results in opacification
of the overlying cortical lens material as the posterior capsule weakens
i May result in a sudden total cataract if the posterior capsule ruptures
Typically unilateral
Not associated with microphthalmia or microcornea
i Corneal diameter is normal and symmetric
Delayed onset usually results in a more favorable visual prognosis
Hydrodissection should be avoided during surgery as it may result in a large rupture of

the thin and weakened posterior capsule. Even without hydrodissection, the central
posterior capsule will frequently rupture during aspiration of lens material. The resulting
defect tends to be round and centrally located. Therefore, it typically does not prevent
placement of an intraocular lens if desired.
During aspiration of the lens material, try to avoid collapse of the anterior chamber as
this may push vitreous forward into the bag through the weakened posterior capsule. If
this occurs, try to push the vitreous posterior with injection of viscoelastic to avoid
engaging vitreous during aspiration of the remaining lens material.
Diffuse or Total Cataract
Complete opacification of lens substance (nucleus and cortex)
Usually the end-stage of partial cataracts (lamellar, nuclear, or posterior lentiglobus) that
progress over time
May also be secondary to trauma
Lens may show resorption (in unilateral cases)
i Liquification and dissipation of lens material leaves a thin, white membrane of fused
anterior and posterior capsule.
If no view of the posterior segment is possible, a B-scan ultrasound of the posterior

segment is recommended to rule-out retinal detachment, intraocular mass or foreign
body.
Peeling of a calcified fibrotic plaque, if present, may result in capsule break but can
usually be limited to the size of the plaque.
Persistent Fetal Vasculature (PFV)

Formerly called Persistent Hyperplastic Primary Vitreous (PHPV)
Dense white vascular membrane at the posterior capsule and anterior vitreous, usually
with attachments to the ciliary processes
i Ciliary processes may be stretched
Presentation is variable but there is usually a persistent hyaloid artery or stalk
i This may extend all the way back to the optic nerve or may be a partial remnant
See full description of PFV detailed later

A minimal expression of persistent
fetal vasculature is the Mittendorf dot
which can be considered a variation
of normal.
ETIOLOGY OF PEDIATRIC CATARACTS3

u
Idiopathic: The majority (60%) of bilateral congenital/infantile cataracts are of unknown

cause.
Hereditary: Familial cataracts that are passed from one generation to the next
Approximately 30% of bilateral congenital/infantile cataracts are hereditary
Usually autosomal dominant transmission
In these cases, cataracts are an isolated ocular finding with no other associated
systemic pathology of other organ systems.
w Ocular Syndromes with Associated Cataracts: Some cataracts presenting primarily

as ocular abnormalities may be associated with abnormalities elsewhere in the body. A few
important examples include, but are not limited to, the following:1,3,4,5
Aniridia
i Bilateral absence of most or all iris tissue

i Associated with cataract, glaucoma, corneal neovascularization, corneal scarring,
foveal hypoplasia and nystagmus
i Usually hereditary and autosomal dominant (2/3 of cases)
The remaining 1/3 of cases are nonhereditary, sporadic, and are frequently
associated with a deletion of chromosome 11 (mutation of PAX 6 gene).
Because the gene for Wilms tumor (WTI) is also nearby on chromosome 11,
these children have a significant risk for developing Wilms tumor during the
first several years of childhood.
If genetic testing is available, sporadic cases of aniridia should undergo this

genetic testing to assess their relative risk of developing Wilms tumor.
Otherwise, serial abdominal ultrasounds and a careful evaluation by a
pediatrician are recommended.
v Age 0 5 years, every 3 months
v Age 6 10 years, every 6 months
v Age 11 16, once yearly
i While cataract formation is common in all cases of aniridia, it should be kept in
mind that these patients usually have significant vision impairment from other
factors such as the foveal hypoplasia and secondary nystagmus. Therefore, most
mild to moderate cataracts in these patients do not require surgical intervention.
Peters anomaly
i Congenital central corneal opacity with underlying defects of the posterior corneal
stroma, Descemets membrane and endothelium
E
E
E
P
Usually bilateral but asymmetrical

Usually associated with secondary glaucoma
May have adherent iris with keratolenticular contact and cataract
v Lens may be in place or it may be displaced anteriorly
Microphthalmos
i Small, malformed eye that may be unilateral or bilateral, sporadic or hereditary,
isolated or sometimes associated with a systemic disorder
i May have abnormalities of:
E
E
E
Anterior segment (small corneal diameter, iris coloboma)

Lens (cataract, coloboma)
Posterior segment (persistent fetal vasculature, retinal dysplasia)
i Vision may range from normal to no light perception

i May be associated with CHARGE syndrome (Charge Association):
E
E
E
E
E
E
P
Colobomatous microphthalmia
Heart defects
Atresia choanae
Retarded growth and development
Genital hypoplasia
Ear malformation
Persistent fetal vasculature (PFV) - formerly called persistent hyperplastic primary

vitreous (PHPV) 1,6
i Variable malformation of the eye caused by an abnormal persistence of the fetal
vasculature. May include some or all of the following clinical features:
E
E
E
E
E
E
E
E
E
E
E
Persistent pupillary membrane

Stretching of the ciliary processes
Iridohyaloid blood vessels
Persistence of the posterior fibrovascular sheath of the lens
Persistent hyaloid artery
Bergmeister papilla
Congenital tent-shaped retinal detachment
Macular abnormalities
Optic nerve abnormalities
Microphthalmos
Mittendorf dot
7
i Usually a sporadic, isolated abnormality of one eye only
E
E
E
Most common cause of unilateral cataract in a smaller than normal eye

Less than 10% are bilateral
Only rarely associated with other systemic abnormalities
i Management requires release of traction on the ciliary body and retina
Surgery is best performed by use of vitrectomy instrumentation to remove

cataract, retrolental mass and the anterior portion of the persistent hyaloid
stalk, either by an anterior (limbal) or posterior (pars plana) approach.
Because these eyes tend to be significantly smaller and may have extensive
capsular involvement, they frequently are not good candidates for placement of
an intraocular lens.
Systemic Syndromes with Associated Cataracts: These cataracts are associated

with some other primary systemic pathology or abnormality of another organ system. A
few important examples include, but are not limited to, the following: 7,8,9
Lowe syndrome (oculocerebralrenal syndrome)
i X-linked recessive
Patients mothers are typically carriers and may have asymptomatic, punctate
or spoke-like lens opacities
i Renal tubular dysfunction leads to metabolic acidosis, hyperparathyroidism and
hypophosphatemia
i Clinical manifestations:
Cataract, in almost 100% of patients, frequently found with glaucoma and

miotic pupils
E
E
Mental retardation
Bone demineralization and weakening (rickets)
i Diagnosis is confirmed by identifying amino acids in the urine. Molecular genetic

testing is also available.
i Beyond treating the cataracts and glaucoma, management options are limited and
patients have a significantly shortened lifespan.
Marfan syndrome
i Connective tissue disorder that is frequently autosomal dominant

i Clinical manifestations:
Lens subluxation is very common, approximately 80%

v Usually displaced superiorly
v Induces secondary lenticular myopia and astigmatism
E Cardiovascular problems are frequent and may be severe
v Aortic root dilation leads to aortic valve regurgitation and ascending aortic
aneurysm
u Cardiology evaluation and echocardiogram are recommended
8
Skeletal abnormalities
v Arachnodactyly
v Joint laxity
i Ocular management:
E
E
Optical correction through either the phakic or aphakic portions of the pupil
Surgical lensectomy if the lens edge bisects the pupil or if anterior
displacement of the lens produces pupillary block glaucoma
v High risk for vitreous loss and retinal detachment
u Surgical risk is diminished by performing lensectomy with vitrectomy
instruments.
v Patients are usually left aphakic (treated with glasses) but iris or scleral
fixated IOLs may be a reasonable option for some patients.
Down syndrome (Trisomy 21)
i Small lens opacities are very common (50%) but the incidence of visually
significant cataract requiring surgery is probably less than 5%.4,5,6
Cataracts may be present at birth or may develop during the first decade of
life.
Opacities may be punctate, sutural, zonular or complete.
y Metabolic Cataracts: These are typically early onset cataracts (congenital, infantile or
juvenile) associated with an underlying metabolic disorder. Early diagnosis and intervention are
critical to preventing permanent systemic damage, particularly to the central nervous system.
Important examples include, but are not limited to, the following:
P
Galactosemia
i Deficiency of galactose metabolism; patients are unable to convert ingested

galactose to glucose.
i Milk products containing lactose are the main source of the problematic galactose.
E
E
Management involves a galactose restricted diet

Early diagnosis and treatment prevents clinical manifestations of the disease
i Autosomal recessive disorder of one of three enzymes:
E
E
E
galactose-1-phosphate uridyltransferase (GALT)

galactokinase (GALK)
uridine diphosphate galactose-4 epimerase (GALE)
i Clinical manifestations include:
Cataract
v Typically bilateral oil droplet nuclear changes but may be cortical, lamellar
or punctuate opacities.
v Opacity is secondary to accumulation of galactitol (dulcitol) in the lens
Liver dysfunction
v Jaundice
v Hepatomegally
v Elevated liver enzymes
E Renal tubular dysfunction
v Metabolic acidosis
v Galactosuria
v Glycosuria
v Albuminuria
v Amino aciduria
i Prenatal screening for all three enzyme defects is available.
i Preoperative metabolic screening for galactosemia in children with isolated
congenital cataracts is not routinely indicated as long as the child appears to be
otherwise in good health.
Homocystinuria
i Deficiency of cystathionine beta-synthase which interferes with methionine

metabolism
i Autosomal recessive inheritance
i Clinical manifestations include:
Ectopia lentis
v Bilateral, progressive lens dislocation
v Commonly displaced inferiorly or nasally
v May develop pupillary block glaucoma
E Osteoporosis
E
E
Mental retardation in 50%
Thromboembolism
v Patients are at an increased risk of morbidity with cataract surgery.
i Early diagnosis and treatment with low methionine, cystine-supplemented diet is
important to prevent or diminish clinical manifestations of the disease. Specific
precautions should be undertaken to reduce the risk of thromboembolic
phenomena at surgery including preoperative aspirin, vitamin B6 maintaining blood
volume and compression stockings for the legs.
Traumatic Cataracts: These can occur in children demonstrating a wide range of clinical
appearance while having in common a history of trauma. Surgery must be appropriate for the
condition of the eye.
10
STEP-BY-STEP APPROACH
u
PEDIATRIC CATARACT SURGERY
Preoperative Routine
FOR
Informed surgical consent stressing the need for postoperative follow-up care including
parental (caregiver) involvement, need for appropriate optical correction, and a realistic
discussion of any limitations of visual potential specific to each patient and type of
cataract
Preoperative Laboratory Evaluation
Most pediatric cataract patients do not require an extensive laboratory evaluation.
i Unilateral cataracts are most often secondary to an isolated ocular abnormality.

i An otherwise apparently normal child with a family history of hereditary cataracts
(frequently autosomal dominant) also does not require special laboratory
evaluation.
Children with bilateral, unexplained cataracts should preferably undergo a few basic
laboratory studies:
i TORCH titers:
E
E
E
E
Toxoplasmosis
Rubella
Cytomegalovirus (CMV)
Herpes
i Syphilis screening
More extensive metabolic, genetic or infectious disease workups should be reserved for
children with other, usually obvious, systemic abnormalities and should be done in
conjunction with a careful evaluation by a pediatrician.
Congenital rubella syndrome (CRS)
i It is particularly important to identify those children who may have cataract

secondary to congenital rubella infection.
E
E
E
E
E
E
E
E
Cataracts/congenital glaucoma
Congenital heart disease
Hearing impairment
Pigmentary retinopathy
Purpura
Hepatosplenomegaly
Jaundice
Microcephaly
11
E
E
E
Developmental delay
Meningoencephalitis
Radiolucent bone disease
i The cataractous lens (usually a nuclear type cataract) may serve as an infectious
reservoir of live virus. Pregnant health care workers should not be allowed to care
for children undergoing surgery for cataract secondary to suspected or confirmed
CRS.
i Because of the associated systemic disease, children with CRS may be at an
increased risk for general anesthesia.
i Children with CRS have a high risk of postoperative complications, including:
E
E
E
E
E
Corneal edema and corneal opacification

Secondary glaucoma
Intense inflammation, uveitis
May require prolonged postoperative steroids
Probably best to avoid primary implantation of IOLs
w Preoperative Drops
The pupil must be well-dilated at least one hour prior to surgery using locally available
mydriatic or cycloplegic agents. Combinations of cyclopentolate, tropicamide, atropine
and phenylephrine in various strengths are all used with success.
i Phenylephrine 10% should be avoided in younger/smaller children.
Preoperative topical antibiotic drops may be administered if desired. The same bottle
may then be given to the patient for postoperative use.
Examination of Both Eyes Under Anesthesia
12
Intraocular pressure should be measured (if possible) during the early induction phase
of anesthesia and then again after the airway is secured. This should be done with
either the Tono-pen, Shiotz or Perkins applanation devices.
Keratometry measurement if not already
performed in clinic
Retinoscopy if possible
Horizontal corneal diameter

measurement (mm) using calipers
i The average corneal diameter of a
full-term infant is 9.9 mm at birth and
11.1 mm at 12 months of postnatal
age.12
Evaluation of anterior chamber structure and depth
Evaluation of lens and ciliary body:

i Document location and characteristics of lens opacity
i Inspect anterior and posterior capsules for plaques that may interfere with
capsulotomies or abnormally thin areas that may easily rupture during surgery
(posterior lentiglobus or other posterior polar opacities)
i Look for small lens size or stretching of ciliary processes that would suggest
persistent fetal vasculature in smaller eyes (usually unilateral)
Evaluation of retina and optic nerve using indirect ophthalmoscopy if possible
i Ultrasound B-scan evaluation of posterior segment in clinic or operating room is

recommended (if available) when the cataract prevents visualization of the retina.
Intraocular Lens Calculations
Performed preoperatively in clinic in older, cooperative children
Performed intraoperatively during examination under anesthesia in younger or

uncooperative children
Keratometry: measurement of corneal curvature

i Older, cooperative children should have manual or automated keratometry
performed preoperatively in the clinic setting.
i Younger, uncooperative children should preferably have measurements performed
under anesthesia with a hand-held keratometer at the time of surgery. Several
measurements should be taken and averaged as they tend to be quite variable.
The examiner should attempt to carefully align the hand-held keratometer with the
patients visual axis.
i If it is not possible to obtain keratometry measurements in either the clinic or
operating room setting, the operating physician may elect to use previously
published normative values as a last resort.13
Age Group (months)
0-3
3-6
6 12
12 18
18 30
30 42
Mean K (D)
47.9
45.9
45.1
44.9
43.6
44.0
A-scan biometry: measurement of axial length

i Immersion technique (preferred)
i Contact technique if immersion technique not available or if eye is too small for
immersion bath shell
13
Selection of appropriate intraocular lenses for placement within the posterior capsular
bag (preferred) or in the ciliary sulcus
i Most common intraocular lens calculation formulas seem to be relatively equal for
most children, including SRK II, SRK-T, Hoffer-Q and Holladay-I.10,11
The Hoffer-Q may be slightly more accurate in the shorter eyes of children.11
i A-scan at this stage serves also as a base line measurement (with IOP) as routine
follow up measures are needed to rule out or treat aphakic/pseudophakic
glaucoma.
Cataract Surgery Techniques
Make sure your operating microscope is functional; preferably have a coaxial light.
A superior rectus traction suture can be helpful.

i Suggest a 4-0 silk on a noncutting (taper or round point
needle)
i Large toothed forceps to grasp
superior rectus
i Superior rectus traction suture
is especially important if
performing a scleral tunnel
A solid blade speculum suitable for

the eye size is used.
The choice of incision, capsulotomy technique, lens removal technique,

management of the posterior capsule, and use of an IOL depend upon the
age of the child as well as other factors. For this reason different
procedures will be presented - including a technique for children aged 4
years and younger with a posterior capsulotomy (with and without an IOL)
and another, more adult-like technique, for children aged 5 years and
older where a primary posterior capsulotomy is not done. A technique for
inserting a rigid lens with a scleral tunnel will also be shown.
14
Cataract Surgery Without IOL in Children 4 Years of Age and Younger:

Vitrector Technique
Step 1
A 20-gauge MVR blade is used to

create a temporal corneal incision
just anterior to the limbus beyond
the terminal loops of the limbal
blood vessels. (Figure 1)
Step 2
Insert a 20-gauge anterior chamber

maintainer (Figure 2) or a short,
blunt-tipped, infusion cannula that
is connected to the saline infusion
bottle either via the vitrectomy
machine or as a free-hanging
infusion bottle.
i Suggest a BD Visitec 20gauge self-retaining anterior
chamber maintainer (Lewicky)
[BD Ophthalmics product
number 585061]
Figure 1
Right eye, surgeons view
Alternatively, may simply

use a 23-gauge butterfly
needle as an infusion line
first cut the butterfly
wings off and insert the
needle through clear
cornea with minimal or no
prior incision. Must ensure
that the sharp tip of the
needle avoids contact with
the anterior capsule of the
lens or the endothelium of
the cornea.
Figure 2
15
i A bimanual vitrectomy instrument

technique is preferred by some
and if chosen it proceeds as
follows:
Using two incisions, 10 and 2

oclock, the vitrectomy
handpiece is used in the
surgeons dominant hand and
the irrigation handpiece is
used for infusion and is held
in the non-dominant hand.
(Figure 2a)
Figure 2a
Open the infusion line to deepen the anterior chamber.

i We prefer the Alcon Accurus machine with an active fluid pump. It is set at 30
mmHg for infusion pressure and provides constant anterior chamber depth in
contrast to gravity infusion systems where anterior chamber depth can be variable.
i Or, use the anterior chamber maintainer with a gravity infusion to stabilize the
anterior chamber.
Step 3
A second, superiorly located

incision is created. (Figure 3)
i The anterior chamber is
entered at the near-clear
cornea, just anterior to the
arcade of limbal vessels
using the 20-gauge MVR
blade either as a straight
stab incision or as a biplanar
incision using a dimpledown technique.
Figure 3
16
Dimple-down technique: the tip of the 20-gauge MVR blade is initially used to
press down perpendicular to the corneal surface; (Figure 3a) before the tip
enters the anterior chamber, about half thickness, the MVR blade is flattened
downward approximately 45 degrees so that the entry is completed parallel to
the plane of the iris, creating a biplanar incision. (Figure 3b)
45 degrees
Figure 3a
Figure 3b
Step 4
The vitrectomy handpiece (Figure 4)

is then inserted and placed open
port-down, just touching the central
anterior capsule to create a
vitrectorhexis type anterior
capsulotomy. You should become
familiar with the behavior of your
vitrector as characteristics tend to
vary. It is advisable to have prior
experience with the vitrector before
it is used for infant cataract surgery.
i Suggested vitrector settings
are*: cut rate 250 cuts/ minute
(typical range, 150 to 300);
Figure 4
aspiration 250 mmHg
maximum.
i At this stage the surgeon selects low aspiration to avoid a radial tear. In some
vitrectors used commonly in the developing world, the aspiration is done manually
using a syringe. In that case, make sure you start with "minimal" aspiration and
increase accordingly if needed.
*These recommended settings are for the commonly used Alcon Accurus. Surgeons may need to make
adjustments for other machines.
17
This vitrectorhexis technique makes it possible to create a uniform anterior

capsulotomy opening in younger children who tend to have an elastic anterior capsule
prone to radial tears, especially in the presence of a greater positive vitreous pressure
contributing to anterior chamber collapse during surgery. The vitrectorhexis technique
is particularly helpful in children 2 years of age and younger but may be utilized
effectively up to 5 years of age. More experienced surgeons, particularly those
comfortable with small-incision capsulotomy forceps, may prefer to perform a
traditional continuous curvilinear tear capsulorhexis in all children 3 years of age and
older.
Step 5
18
Using the minimum amount of

aspiration and just enough
downward pressure to engage the
anterior capsule, use the vitrector
in the cutter-on mode and slowly
take bites out of the central
anterior capsule. Try to avoid
engaging the anterior lens cortex.
(Figure 5)
i Slowly circle the handpiece,
gradually enlarging the
anterior capsular opening until
it is circular and of the
intended size.
Figure 5
Since the patient is to be left aphakic and will possibly receive a secondary
IOL, the anterior capsulotomy should be approximately 5 mm in diameter.
Keep in mind that the cutting action occurs slightly behind the tip of the
instrument where the port opening is located, not at the very end.
Step 6
Turn the cutter function off and use the aspiration-only setting to begin aspirating the
lens material. (Figure 6)
If the vitrector does not have a suction only capacity, remove the vitrector and perform
a manual cortex aspiration using a Simcoe cannula or similar instrument.
i It may be helpful to begin

aspiration at 12-oclock or just
below the corneal incision first.
(Figure 6)
The large amount of lens

material present at this stage
helps to prevent engagement
of the posterior capsule
within the vitrector port.
(Figure 7)
i Gently engage the cortical
material adherent to the capsule
and strip or pull it into the center
of the eye, being careful not to
grab the irregular, scalloped
edges of the anterior
capsulotomy.
Figure 6
i If sticky fragments of lens occlude
the aspiration port, turn the port
so that it is facing up, away from the posterior capsule, and briefly turn the cutter
function back on until the port is clear, then go back to aspiration-only. (Figure 8)
Figure 7
Figure 8
19
Step 7
Perform posterior capsulotomy and anterior vitrectomy via anterior approach.

i The vitrectomy handpiece is now placed with the port facing down and the machine
settings are changed to a higher cut rate and lower aspiration level which is
appropriate for working with vitreous rather than lens material. (Figure 9)
Suggested settings for the Alcon Accurus vitrector are a minimum cut rate of 400500 cuts/minute and low levels of aspiration with a maximum vacuum of 100
mmHg. The infusion/ irrigation pressure is also kept low at 30 mmHg.
i The posterior capsule is engaged centrally and the aspiration is increased just
enough to engage the capsule in the cutter and create the initial opening. (Figure 9)
Continuing to use a minimum amount of aspiration with a high cut rate, the central
opening is enlarged until it is round and at least 4 mm in diameter, usually just
slightly smaller than the anterior capsulotomy diameter of 5 mm. (Figure 10)
Alternatively, after meticulous cortex removal the MVR blade can be used to
create a central rent (opening) in the posterior capsule. After this, the vitrector
can be used with minimal aspiration and high cutting to perform the anterior
vitrectomy.
Figure 9
Figure 10
posterior capsulotomy not yet complete
20
Step 8
Once the posterior capsulotomy is of the desired size and shape, a limited anterior
vitrectomy is performed using the same machine settings. The purpose is to remove the
anterior vitreous elements that may serve as a surface allowing retained lens epithelial
cells to grow across the visual axis. This may be just as amblyopiogenic as the original
cataract.
i The vitrectomy handpiece is slowly moved around, just posterior to the posterior
capsulotomy, until no movement of vitreous to the port is noted. (Figure 11)
The end point of the vitrectomy is difficult to define but there should be no
evidence of vitreous strands in the pupillary plane or anterior chamber.
The vitrectomy handpiece should not be inserted very far into the vitreous and
certainly should never be used where the port is not easily seen.
i When the vitrector handpiece is withdrawn, the superior incision is checked to be

certain that it is free of vitreous strands that tend to follow the instrument out of the
eye. If unsure, the wound should be swept with a cyclodialysis spatula or iris
repositor. (Figure 11a)
i If desired, nonpreserved or washed Kenalog steroid (triamcinolone) may be
injected into the anterior chamber to stain strands of vitreous that may be extending
to the wounds. The suspended Kenalog solution is white and binds to the
transparent vitreous strands, making them easier to identify.
Figure 11
Figure 11a
21
Step 9
The superior wound is now sutured closed using 10-0 or 9-0 nylon or absorbable suture
such as Vicryl (polyglactin 910, Ethicon, Sommerville, New Jersey, USA) or Biosorb
(polyglycolic acid, Alcon Laboratories, Inc., Fort Worth, Texas, USA). This suture is
placed in a figure 8 fashion as shown. (Figure 12)
Step 10
The infusion cannula is removed and

this incision is also sutured closed.
(Figure 13)
i The eye is repressurized with
saline solution and the suture
knots are buried.
i The procedure is now finished
and the eye is patched. (Figure
14)
i Note that it is advisable in
children to suture each
paracentesis! Their low corneoscleral rigidity and high activity
level may contribute to wound
leaks.
Figure 13
22
Figure 12
Figure 14
Cataract Surgery With IOL and Primary Posterior Capsulotomy Via Anterior
Approach in Children 4 Years of Age and Younger: Vitrector Technique
Step 1

Step 2
Insert a 20-gauge anterior

chamber maintainer (Figure 16) or
a short, blunt-tipped, infusion
cannula that is connected to the
saline infusion bottle either via the
vitrectomy machine or as a freehanging infusion bottle.
chamber maintainer
[Lewicky] BD Ophthalmics
product number 585061
Figure 15
Alternatively, may use a

23-gauge butterfly
needle as an infusion line
first cut the butterfly
wings off and insert the
needle through clear
cornea with minimal or no
prior incision. Must
ensure that the sharp tip
of the needle avoids
contact with the anterior
capsule of the lens or the
endothelium of the
cornea.
Figure 16
23

technique is preferred by some and
if chosen it proceeds as follows:

the irrigation handpiece is
used for infusion and is held in
the non-dominant hand.
(Figure 16a)
Figure 16a

Step 3
A second, superiorly located incision

is created. (Figure 17)
i If a clear corneal incision is to
be used, with a small-incision
foldable/injectable IOL, the
anterior chamber is entered at
the near-clear cornea, just
anterior to the arcade of limbal
vessels using the 20-gauge
MVR blade either as a straight
incision using a dimple-down
technique.
Figure 17
24
enters the anterior chamber, about half thickness, the MVR blade is flattened
downward approximately 45 degrees so that the entry is completed parallel to the
plane of the iris, creating a biplanar incision. (Figure 17b)
45 degrees
Figure 17a
Figure 17b
i If a scleral tunnel incision is to be used, create this 1 to 2 mm from the superior

limbus and tunnel forward at half-thickness into clear cornea. Use the cresent knife
to create the tunnel 1mm into clear cornea. Enlarge width to the size necessary for
IOL insertion but do not enter anterior chamber. Enter the anterior chamber later
through this wound, keeping the entry small, using a small keratome or 20-gauge
MVR blade. Create a shelved edge entry into the anterior chamber, making sure
your tunnel and entry are on a single surgical plane.
Open conjunctiva at or
4 mm from the limbus
exposing bare sclera.
(Figure 18)
v 8 mm wide
v Light cautery, if
needed
After the conjunctiva is

incised, exposing bare
sclera, a 5.5 mm wide
track for the scleral
incision is measured 1
mm behind the limbus
and the endpoints of
the wound are marked
by indenting the sclera
using surgical calipers.
(Figure 18a)
Figure 18
Figure 18a
25
Using a #15 or similar

blade, a straight,
approximately 1/2
thickness scleral groove
is made with two
backward extensions at
each end. (Figure 19)
v The two backward
cuts of 1 - 1.5 mm
are made from each
end of the wound at
a 45 degree angle
away from the
limbus.
A scleral tunnel is then

constructed using an
angled crescent blade.
(Figure 20)
v The incision extends
Figure 19
approximately 2 - 2.5
mm into the cornea.
The dissection is carried out towards the limbus on both sides to create a funnelshaped pocket.
v The crescent blade is then angled to cut backwards to incorporate the
backward cuts into the pocket.
v A frown biplanar incision is constructed but note the anterior chamber is not
entered. (Figure 21)
Figure 20
Figure 21
26
The anterior chamber is then entered with a needle knife, and a chamber
maintainer is inserted. (Figure 22)
A needle knife is inserted in the middle of the tunnel and enters the anterior
chamber making a hole just large enough to admit the vitrector or other aspiration
device. (Figure 23)
i Once the entry is made into the anterior chamber at the center of the scleral tunnel,
management of the anterior lens capsule and lens are carried out in a similar
manner to that used with a smaller clear corneal entry.
Figure 22
Figure 23
Step 4
The vitrectomy handpiece (Figure 24) is then inserted into the opening made in the
anterior chamber either through clear cornea or in the middle of the tunnel and placed
open port-down just touching the central anterior capsule to create a vitrectorhexis
type anterior capsulotomy. You
should become familiar with the
behavior of your vitrector as
characteristics tend to vary. It is
advisable to have prior experience
with the vitrector before it is used in
infant cataract surgery.
aspiration 250 mmHg
maximum.
*These recommended settings are for the

commonly used Alcon Accurus. Surgeons may
need to make adjustments for other machines.
Figure 24
27
This vitrectorhexis technique makes it possible to create a uniform anterior capsulotomy

opening in younger children who tend to have an elastic anterior capsule prone to radial
tears, especially in the presence of a greater positive vitreous pressure contributing to
anterior chamber collapse during surgery. The vitrectorhexis technique is particularly
helpful in children 2 years of age and younger but may be utilized effectively up to 5
years of age. More experienced surgeons, particularly those comfortable with smallincision capsulotomy forceps, may prefer to perform a traditional continuous curvilinear
tear capsulorhexis in all children 3 years of age and older.
Step 5

anterior capsule, use the vitrector in
the cutter-on mode and slowly
take bites out of the central anterior
capsule. Try to avoid engaging the
anterior lens cortex. (Figure 25)
gradually enlarging the anterior
capsular opening until it is
circular and of the intended
size.
Since the patient is to be left aphakic and will possibly receive a secondary IOL
later, the anterior capsulotomy should be approximately 5 mm in diameter.
Keep in mind that the

cutting action occurs
slightly behind the tip of
the instrument where the
port opening is located, not
at the very end.
Note: A continuous curvilinear

capsulorhexis (a) or a can opener
technique using a bent tip small needle
(b) can also be used at any age.
(Figure 26)
28
Figure 25
b
Figure 26
Step 6
Turn the cutter function off and use the aspiration-only setting to begin aspirating all
lens material.
i It may be helpful to begin aspiration at 12-oclock or just below the corneal incision
first. (Figure 27)
The large amount of lens material present at this stage helps to prevent
engagement of the posterior capsule within the vitrector port.
i Gently engage the cortical material adherent to the capsule and strip or pull it into
the center of the eye, being careful not to engage the irregular, scalloped edges of
the anterior capsulotomy. (Figure 28)
Figure 27
Figure 28

the aspiration port, turn the port so
that it is facing up, away from the
posterior capsule and briefly turn
the cutter function back on until
the port is clear, then go back to
aspiration-only. (Figure 29)
Figure 29
29
Step 7
30
Perform primary posterior capsulotomy

and anterior vitrectomy via anterior
approach
i The vitrectomy handpiece is now
placed with the port facing down
and the machine settings are
changed to a higher cut rate and
lower aspiration level as
appropriate for working with
vitreous rather than lens material.
i Suggested settings for the Alcon
Accurus vitrector are a minimum
cut rate of 400-500 cuts/minute
and low levels of aspiration with a
maximum vacuum of 100 mmHg.
The infusion/ irrigation pressure is
also kept low at <30 mmHg.
The posterior capsule is engaged

centrally and the aspiration is increased
just enough to engage the capsule in
the cutter and create the initial opening.
(Figure 30) Continuing to use a
minimum amount of aspiration with a
high cut rate, the central opening is
enlarged until it is round and at least 4
mm in diameter, usually just slightly
smaller than the anterior capsulotomy
diameter of 5 mm. (Figure 31)
Once the posterior capsulotomy is of

the desired size and shape, a limited
anterior vitrectomy is performed using
the same machine settings. The
purpose is to remove the anterior
vitreous elements that may serve as a
surface allowing retained lens epithelial
cells to grow across the visual axis.
This may be just as amblyopiogenic as
the original cataract.
i Caution: If placing a primary IOL
after the posterior capsulotomy,
making the posterior capsulotomy
too large may make it more
difficult to place the IOL securely
within the capsular bag.
Figure 30
Figure 31
Posterior capsulotomy not yet complete
The vitrectomy handpiece is slowly moved around, just posterior to the posterior
capsulotomy, until no movement of vitreous to the port is noted. (Figure 32)
i The end point of the vitrectomy is difficult to define but there should be no evidence
of vitreous strands in the pupillary plane or anterior chamber.
i The vitrectomy handpiece should not be inserted very far into the vitreous and
certainly should never be used where the port is not easily seen.
i If desired, nonpreserved or washed Kenalog steroid (triamcinolone) may be
injected into the anterior chamber to stain strands of vitreous that may be
extending to the wounds. The suspended Kenalog solution is white and binds to
the transparent vitreous strands, making them easier to identify.
At this point, the vitrector handpiece is withdrawn, the superior incision is checked to be
certain that it is free of vitreous strands that tend to follow the instrument out of the eye.
If unsure, the wound should be swept with a cyclodialysis spatula or iris repositor.
(Figure 32a)
Figure 32
Figure 32a
31
The anterior chamber and the capsular bag are now filled with a high-viscosity,
cohesive viscoelastic agent. (Figure 33)
i The benefit of a highly

cohesive viscoelastic agent is
that it is better able to maintain
the volume and shape of the
pediatric eye which has low
scleral rigidity. It is also easier
to remove completely at the
end of the procedure.
However, some viscoelastic
agents may be so viscous that
they tend to clog the infusion
cannula or vitrectomy hand
piece. If this occurs, squeezing
the infusion line or hitting
reflux on the vitrector will
usually clear the obstruction.
The superior anterior chamber incision is then enlarged to a sufficient width with a
keratome to allow placement of the foldable IOL. (Figure 34)
Figure 34
32
Figure 33
The IOL is inserted or injected and dialed into position with an IOL hook as needed.
(Figure 35, 36, 37)
Figure 35
Figure 36
Figure 37
If you inject the foldable IOL, remember to inject slowly in the bag and avoid dialing
through both openings into the vitreous. To reduce the chance of this, have a cushion
of visco-elastic. Some surgeons find it easier to inject the lens into the anterior
chamber and then use an IOL hook to dial the IOL into the bag. Alternatively, forceps
can be used to place the foldable IOL by aiming the front haptic into the bag then using
the hook to dial the near haptic into the bag.
33
When a rigid IOL is used, the superior

anterior chamber scleral tunnel incision
is then enlarged to a sufficient width
with a keratome to allow placement of
the foldable IOL. (Figure 38)
The rigid lens is inserted and dialed in

the bag with the leading haptic placed
first and the trailing haptic placed behind
the anterior lens capsule and then the
lens is dialed in. (Figure 39, 40)
Remember: If operating in a setting with

limited resources, it is better to secure
solid sulcus placement rather than
dealing with complications of having one
haptic in the bag and one in the sulcus,
IOL dislocation, etc.
Figure 39
34
Figure 38
Figure 40
It is essential to have a good operating microscope providing a good view of both

anterior and posterior capsular openings, as this method is more prone to IOL
dislocation if visibility is not optimal.
The vitrectomy handpiece is used to aspirate residual viscoelastic from the eye which is
then re-formed using balanced saline solution.
The anterior chamber and superior incision should be checked very carefully to ensure
that no vitreous strands have adhered to the lens insertion instruments and followed
them back to the wound. Careful sweeping of any vitreous strands with a cyclodialysis
spatula, weckcell, or iris repositor is recommended.
The superior corneal incision used for insertion of a foldable lens is then closed using
9-0 or 10-0 nylon or absorbable suture such as Vicryl (polyglactin 910, Ethicon,
Sommerville, New Jersey, USA) or Biosorb (polyglycolic acid, Alcon Laboratories, Inc.,
Fort Worth, Texas, USA).
Remove the anterior chamber infusion cannula (anterior chamber maintainer) and close
the corneal incision using 9-0 or 10-0 nylon or absorbable suture such as Vicryl or
Biosorb. (Figure 41)
The corneal incision used for insertion of a rigid lens is closed first and then the
conjunctiva is closed in a separate layer. (Figure 42)
Figure 41
clear corneal incision
It is optional to administer topical antibiotic

and subconjunctival steroid injections.
Examples include:
i Cefazolin (cefazoline or cephazolin)
50 mg
i Dexamethasone 2 mg
Cover the operated eye with a patch and

protective shield. (Figure 43)
NOTE: Another option for anterior vitrectomy

is first to place the IOL in the bag, then
placing viscoelastic into the capsular bag
gently lift up slightly the IOL optic and using a
MVR or stiletto blade create a tear in the
central posterior capsule followed by anterior
vitrectomy through the corneal incision.
Figure 42
scleral tunnel incision
Figure 43
35
Cataract Surgery With IOL and Primary Posterior Capsulotomy Via Pars Plana
Approach in Children 4 Years of Age and Younger: Vitrector Technique
Step 1

Step 2
Insert a 20-gauge anterior

chamber maintainer (Figure 45)
or a short, blunt-tipped, infusion
cannula that is connected to the
saline infusion bottle either via
the vitrectomy machine or as a
free-hanging infusion bottle.
Figure 44

chamber maintainer
[Lewicky]. BD Ophthalmics
product number 585061
36
Alternatively, may simply

use a 23-gauge
butterfly needle as an
infusion line first cut the
butterfly wings off and
insert the needle through
clear cornea with minimal
or no prior incision. Must
ensure that the sharp tip
of the needle avoids
contact with the anterior
capsule of the lens or the
endothelium of the
cornea.
Figure 45

technique is preferred by some and
if chosen it proceeds as follows:

the irrigation handpiece is used
for infusion and is held in the
non-dominant hand.
(Figure 45a)
Figure 45a

Step 3
A second, superiorly located

incision is created.
i If a clear corneal incision is to
be used, with either no IOL
implant or with a small-incision
foldable/injectable IOL, the
anterior chamber is entered at
the near-clear cornea, just
anterior to the arcade of limbal
vessels using the 20-gauge
MVR blade either as a straight
incision using a dimple-down
technique. (Figure 46)
Figure 46
37
enters the anterior chamber about half thickness, the MVR blade is angled 45
degrees downward so that the entry is completed parallel to the plane of the
iris, creating a biplanar incision. (Figure 46b)
45 degrees
Figure 46a
Figure 46b
i If a scleral tunnel incision is to be used, create this 1 to 2 mm from the superior

limbus and tunnel forward at half-thickness into clear cornea. Enlarge width to the
size necessary for IOL insertion but do not enter anterior chamber. Enter the
anterior chamber later through this wound, keeping the entry small, using a small
keratome or 20-gauge MVR blade.
E
E
Open conjunctiva at or 4 mm from the limbus exposing bare sclera. (Figure 47)
v 8 mm wide
v Light cautery, if needed
After the conjunctiva is incised, exposing bare sclera, a 5.5 mm wide track for
the scleral incision is measured 1 mm behind the limbus and the endpoints of
the wound are marked by indenting the sclera using surgical calipers.
(Figure 47a)
Figure 47
Figure 47a
38
Using a #15 or similar blade, a straight, approximately 1/2 thickness, scleral

groove is made with two backward extensions at each end. (Figure 48)
v The two backward cuts of 1 - 1.5 mm are made from each end of the
wound at a 45 degree angle away from the limbus.
Figure 48
A scleral tunnel is then constructed using an angled crescent blade. (Figure 49)
v The incision extends approximately 2 - 2.5 mm into the cornea.
Figure 49
39
The dissection is carried out towards the limbus on both sides to create a
funnel-shaped pocket.
v The crescent blade is then angled to cut backwards to incorporate the
backward cuts into the pocket.
v A frown biplanar incision is constructed but note the anterior chamber is
not entered. (Figure 50)
After the anterior chamber is entered with a needle knife, a chamber

maintainer is inserted. (Figure 51)
A needle knife is inserted in the middle of the tunnel and enters the anterior
chamber making a hole just large enough to admit the vitrector or other
aspiration device. (Figure 52)
Figure 50
Figure 51
Figure 52
i Once the entry is made into the anterior chamber at the center of the scleral tunnel,
management of the anterior capsule and lens material is carried out in a similar
manner to that used with a smaller clear corneal entry.
40
Step 4
The vitrectomy handpiece (Figure 53) is then inserted into the opening made in the
anterior chamber either through clear cornea or in the middle of the scleral tunnel and
placed open port-down just touching the central anterior capsule to create a
vitrectorhexis type anterior
capsulotomy. You should become
familiar with the behavior of your
vitrector as characteristics tend to
vary. It is advisable to have prior
experience with the vitrector before
it is used in infant cataract surgery.
aspiration 250 mmHg
maximum.
*These recommended settings are again for the

commonly used Alcon Accurus. Surgeons may
need to make adjustments for other machines.
Figure 53
This vitrectorhexis technique makes it possible to create a uniform anterior capsulotomy

opening in younger children who tend to have an elastic anterior capsule prone to radial
tears, especially in the presence of a greater positive vitreous pressure contributing to
anterior chamber collapse during surgery. The vitrectorhexis technique is particularly
helpful in children 2 years of age and younger but may be utilized effectively up to 5
years of age. More experienced surgeons, particularly those comfortable with smallincision capsulotomy forceps, may prefer to perform a traditional continuous curvilinear
tear capsulorhexis in all children 3 years of age and older.
Step 5

anterior capsule, use the vitrector
in the cutter-on mode and slowly
take bites out of the central anterior
capsule. Try to avoid entering the
anterior lens cortex. (Figure 54)
gradually engaging the anterior
capsular opening until it is
circular and of the intended
size.
Figure 54
41
The anterior capsulotomy should be approximately 5 mm, or just slightly

smaller than the diameter of the optic of the IOL chosen for implantation.
i Keep in mind that the cutting action occurs slightly behind the tip of the instrument
where the port opening is located, not at the very end.
Step 6
Turn the cutter function off and use the aspiration-only setting to begin aspirating lens
material.
i It may be helpful to begin aspirating at 12-oclock or just below the corneal incision
first. (Figure 55)
The large amount of lens material present at this stage helps to prevent
engagement of the posterior capsule within the vitrector port.
i Gently engage the cortical material adherent to the capsule and strip or pull it into
the center of the eye, being careful not to grab the irregular, scalloped edges of the
anterior capsulotomy. (Figure 56)
Figure 55
Figure 56

the aspiration port, turn the port so
that it is facing up, away from the
posterior capsule and briefly turn
the cutter function back on until the
port is clear, then go back to
aspiration-only. (Figure 57)
Figure 57
42
Once the capsular bag is completely

empty of lens material, the surgeon
should now re-form the anterior
chamber and fill the capsular bag with a
high-viscosity, cohesive viscoelastic
agent (examples include Healon and
Healon GV from manufacturer AMO,
Santa Ana, California, USA). (Figure 58)
i The benefit of a highly cohesive
viscoelastic agent is that it helps to
maintain the volume and shape of
pediatric eyes which have low
scleral rigidity. It is also easier to
remove completely at the end of
the procedure. However, some
Figure 58
viscoelastic agents may be so
viscous that they tend to clog the
infusion cannula or vitrectomy hand piece. Squeezing the infusion line or hitting
reflux on the vitrector will usually clear the obstruction if this happens.
Step 7
The superior, anterior chamber incision is then enlarged sufficiently with a keratome to
allow placement of the IOL; smaller and through clear cornea if a foldable IOL is used
(Figure 59) or larger through the scleral tunnel if a rigid lens is used (Figure 60)
Figure 59
wound enlargement if a
clear corneal incision is utilized
Figure 60
wound enlargement if a
scleral tunnel is utilized
43
The foldable IOL is inserted or injected and dialed into position with an IOL hook as
needed. (Figure 61, 62, 63)
Figure 61
Figure 62
Figure 63
The rigid lens is inserted and dialed in the bag with the leading haptic placed first and
the trailing haptic placed behind the anterior lens capsule and then the lens is dialed in.
(Figure 64, 65)
Figure 64
44
Figure 65
The vitrectomy handpiece is used to aspirate residual viscoelastic from the eye which is
then re-formed using balanced saline solution.
The superior clear corneal incision is then closed using 9-0 or 10-0 nylon or absorbable
suture such as Vicryl (polyglactin 910, Ethicon, Sommerville, New Jersey, USA) or
Biosorb (polyglycolic acid, Alcon Laboratories, Inc., Fort Worth, TX, USA). (Figure 66)
The scleral tunnel is closed in layers, scleral first and then conjunctiva. (Figure 67)
Figure 66
Figure 67
Step 8
Perform posterior capsulotomy and anterior vitrectomy using pars plana approach.
Conjunctiva and Tenons are incised

to access sclera several millimeters
behind the limbus in the superiortemporal or superior-nasal quadrant
using scissors to expose the bare
sclera overlying the pars plana.
(Figure 68)
i Avoid cutting down directly onto
the superior rectus muscle.
i Hemostasis is achieved with
light cautery if needed.
Figure 68
45
Calipers are used to measure back an appropriate distance* before entering the eye
with the MVR blade. (Figure 69, 70)
< 1 year of age:

1 4 years of age:
> 4 years of age:
enter 1.5 - 2.0 mm from limbus

enter 2.5 mm from limbus
enter 3.0 mm from limbus
Figure 69
Figure 70
The MVR blade is directed toward the center of the vitreous cavity while entering the
eye and the tip of the blade should be clearly visualized behind the IOL prior to inserting
the vitrectomy hand piece. (Figure 71)
Figure 71
46
The vitrectomy handpiece is

inserted and should be visualized:
then make initial cuts but only
after visualizing the cutter tip. An
initial cut is made in the central
posterior capsule with the vitrector
port facing upward. This will allow
the infusion to more easily
circulate from the anterior
chamber to the posterior chamber.
(Figure 72)
i Be certain that the vitrector is
behind the posterior capsule
and has not inadvertently
been passed into the capsular
bag.
Figure 72
i Suggested settings for the
Alcon Accurus vitrector are a
minimum cut rate of 400-500
cuts/minute and low levels of aspiration with a maximum vacuum of 100 mmHg.
The infusion/irrigation pressure is also kept low at < 30 mmHg.
Gradually enlarge the posterior capsule opening to be at least 4 mm in diameter or just

slightly smaller than the 5 mm anterior capsulotomy (and smaller than the IOL optic
diameter), ensuring that an adequate rim of support is left in place to support the IOL.
(Figure 73, 74)
Figure 73
Figure 74
47
Perform a limited vitrectomy of the anterior vitreous just posterior to the IOL and
capsular bag. (Figure 75)
i The vitrectomy handpiece is slowly moved around just posterior to the posterior
capsulotomy until no movement of vitreous to the port is noted.
i The vitrectomy handpiece
should not be inserted very
far into the vitreous and
certainly should never be
used where the port is not
easily visualized.
Lower the infusion pressure to

less than 20 mmHg before
removing the vitrector to help
reduce the likelihood of vitreous
prolapse when the handpiece is
removed. Clean up any extruded
vitreous at the entry incision
using the vitrectomy cutter or
scissors.
Figure 75
Suture the sclerotomy closed once it is free of vitreous. (Figure 76)

i The suture most commonly used is 9-0 nylon or 8-0 Vicryl.
i Some surgeons prefer to pre-place the scleral suture before performing posterior
capsulotomy and vitrectomy.
Figure 76
48
Close the conjunctival incision

using an absorbable suture (can
use the same 8.0 Vicryl used to
close the sclerotomy). (Figure 77)
Remove the anterior chamber

infusion cannula (anterior
chamber maintainer) and close
the corneal incision using 9-0 or
10-0 nylon or absorbable suture
such as Vicryl or Biosorb.
(Figure 78)
Administer topical antibiotic and

steroid eye drops or a
combination antibiotic/steroid
ointment.
Subconjunctival antibiotic and

steroid injections are optional.
Examples include:
i Cefazolin (cefazoline or
cephazolin) 50 mg
i Dexamethasone 2 mg
Cover the operated eye with a

patch and protective shield.
(Figure 79)
NOTE: This pars plana approach for

posterior capsulotomy anterior
vitrectomy seems to be the most
controlled but is advisable only with
the use of an automated high cut rate
vitrector.
Figure 77
Figure 78
Figure 79
49
Cataract Surgery With IOL in Children 5 Years of Age and Older: Irrigation/
Aspiration Technique (and no posterior capsulotomy or anterior vitrectomy)
Create a superiorly located incision.

i If a scleral tunnel incision is to be used, create this 2-3 mm from the superior
limbus and tunnel forward at half-thickness into clear cornea. Enlarge width to the
size necessary for IOL insertion but do not enter anterior chamber.
Enter the anterior chamber through this wound using a small knife blade
(example, 2.5 mm keratome).
i If a clear corneal incision is to be used with a foldable or injectable IOL, enter the
near-clear cornea, just anterior to the arcade of limbal vessels using a small knife
blade (example, 2.5 mm keratome), either as a straight stab incision or as a
biplanar incision using a dimple-down technique.
50
press down perpendicular to the corneal surface; before the tip enters the
anterior chamber about half thickness, the MVR blade is angled 45 degrees
downward so that the entry is completed parallel to the plane of the iris,
creating a biplanar incision.
Fill the anterior chamber with a viscoelastic agent.

i High-viscosity, cohesive viscoelastic agents such as Healon and Healon GV
help to better maintain the volume and shape of pediatric eyes with low scleral
rigidity and are easier to remove completely at the end of the procedure.
Perform an anterior capsulotomy measuring 5 mm or greater in diameter using either a

bent-tip needle cystotome or capsulorhexis forceps.
i If the patient is to be left aphakic with a possible secondary IOL later, the anterior
capsulotomy should be approximately 5 mm in diameter.
i If the patient is to have a primary IOL placed at the time of cataract surgery, the
anterior capsulotomy should be slightly smaller than the diameter of the optic of the
IOL chosen for implantation.
i Keep in mind that pediatric anterior capsules tend to be more elastic and tend to
extend outward, particularly in younger children. This can be minimized by:
Using a high-viscosity, cohesive viscoelastic agent to keep the anterior

chamber full and the anterior capsule flat.
Reinjecting additional viscoelastic as needed during the performance of the

anterior capsulotomy, particularly if the tear begins to extend outward.
E
E
Regrasping the capsulorhexis edge frequently to maintain control.
In young children, the direction of the force should be toward the center of the
pupil to keep the capsulorhexis from extending peripherally.
Staining the anterior capsule with trypan blue under air bubble if the cataract is
mature.
Perform aspiration of the lens substance.

i Use an automated irrigation/aspiration handpiece or manual Simcoe syringe to
remove both lens cortex and nucleus.
E
E
Pediatric cataracts are soft; phacoemulsification power is not needed.
Multiquadrant hydrodissection may be performed prior to lens aspiration to

facilitate the removal of lens material.
v DO NOT HYDRODISSECT IF THE POSTERIOR CAPSULE IS
ABNORMAL OR QUESTIONABLE.
u traumatic cataract
u posterior polar cataract
u posterior lentiglobus cataract
After removing a small amount of the anterior, central lens material, engage the
peripheral lens cortex and strip it into the center of the pupillary space for
aspiration using a technique similar to that of cortical clean-up in an adult
cataract patient.
v It is generally safer to remove the subincisional (12o'clock) cortex early in
the procedure while there is still a large amount of central and posterior
lens substance in place to keep the posterior capsule away from the
aspiration port.
It is helpful to have a larger aspiration port size such as a 0.5 mm diameter

opening.
After removal of all lens material, re-form the anterior chamber and posterior capsular
bag using viscoelastic.
Enlarge the anterior chamber incision with a keratome to allow placement of the IOL.
The IOL is inserted or injected and dialed into position with an IOL hook as needed.
Use the irrigation/aspiration handpiece to remove the viscoelastic from the anterior
chamber and around the IOL.
The corneal/scleral incisions are then closed using 9-0 or 10-0 nylon or absorbable
suture such as Vicryl or Biosorb.
Administer topical antibiotic and steroid eye drops or a combination antibiotic/steroid

ointment.
i Subconjunctival antibiotic and steroid injections are optional. Examples include:
E Cefazolin (cefazoline or cephazolin) 50 mg
E
P
Dexamethasone 2 mg
Cover the operated eye with a patch and protective shield.
51
POSTOPERATIVE MANAGEMENT
u
OF
PEDIATRIC CATARACT PATIENTS
Postoperative Medications
Topical steroid
i Prednisolone acetate 1% or similar, 4 to 6 times daily for 1 to 2 months
i If evidence of significant inflammation persists (anterior chamber flare, cell, fibrin or
deposits on the IOL), the topical steroid may be continued longer, up to 3 to 6
months with monitoring of intraocular pressure.
Topical antibiotic
i Drop or ointment applied 4 times daily for 1 to 2 weeks
Atropine
i Aphakic and pseudophakic eyes: apply atropine 1% once daily for 2 to 4 weeks
E If an IOL is implanted, the use of postoperative cycloplegic is optional. Some
surgeons use it routinely in all cases while others use it only if inflammatory
adhesions begin to develop. If the IOL or anterior capsulotomy are unstable, it
may be wise to defer any pupil dilation until the wounds have become
watertight and the anterior chamber is stable.
i In very small or young infants (less than 1 year of age), consider using more
dilute atropine 0.5% if available.
v Postoperative Follow-Up
P
Postoperative examination schedule:

i 1 day
i 1 week
i 1 month (3-5 weeks after surgery)
i 3 months (every 3 months after surgery for the first year)
i At least twice yearly after the first postoperative year for younger children
E The younger the patient, the more careful and frequent the follow-up should
be, particularly in younger children at risk for amblyopia (less than 8 years of
age).
E
P
52
Consider taking out the corneal sutures (unless absorbable suture material
was used) at the 3 month post op visit.
Postoperative examination elements:

i Assessment of visual acuity with comparison between the two eyes
i Ocular motility and alignment
i Intraocular pressure (particularly if using topical steroids)
E Tonometry (preferred) or palpation
i Anterior segment examination
Slit-lamp (preferred) or hand-held light source

v Penlight, muscle light, flashlight/torch held obliquely at an angle from the
side to illuminate the anterior chamber
v May magnify the view of the anterior segment structures by directly
viewing through a 20 to 30 diopter indirect ophthalmoscope lens used
without the ophthalmoscope
Assess the adequacy of the pupil size and centration of the IOL if one has
been implanted
i Red reflex
E Ensure clarity of the visual axis by assessing the quality (brightness) of the red
reflex using either:
v Direct ophthalmoscope set on either a slit-beam or small, bright field of
illumination
v Retinoscope set to a thin beam of light
i Refraction
E Hand-held retinoscope with loose lenses, refraction bar, or phoropter*
i Ophthalmoscopy
E Indirect (preferred) or direct ophthalmoscope
Perform at least once or twice yearly and anytime there is change in quality
of the vision or red reflex, or if there is a significant refractive shift
Refractive Management
Refraction should be performed as early as is possible in the postoperative period.
Aphakic children and younger pseudophakic children should ideally receive optical
correction within the first 1 to 2 weeks following cataract surgery.
Older pseudophakic children may have residual refractive error corrected with glasses
after the wound and refraction have stabilized 4 to 6 weeks postoperatively.
Correction of postoperative refractive error

i Bifocals versus single-vision correction
E Young children, particularly those less than 2-3 years of age, do not generally
require a bifocal and do well with single-vision correction using either glasses
or contact lens(es) targeted to overcorrect, producing a mild amount of myopia
(for better near vision).
*See ORBIS Vision and Refraction manual
53
The eye is overcorrected by 2.00 to 3.00 diopters so that the far point is 50 to
33 cm.
Aphakic eye example:
Aphakic refraction**
= +23.00 D
Prescription given
= +25.00 D
**If an aphakic contact lens is being fitted, the prescription must also be adjusted
according to the vertex distance of the loose lens or phoropter being used for the
refraction.
i Older children (> 2-3 years of age) have greater visual demands and benefit from
a traditional bifocal.
E Upper segment: distance correction for emmetropia
54
Lower segment: near correction with a +2.50 to +3.00 add

v Flat-topped lower segments (D-shaped or executive style) are easier
for children to use and have larger optical zones with minimal
distortion.
Amblyopia Management
Appropriate and effective amblyopia management is vital to the visual result obtained by
younger children. Parents or caregivers should have this concept explained to them with
sufficient care and detail to ensure successful administration.
While occlusion (patch) therapy is most productive in younger children, even children
older than 9 years of age may show some benefit to a therapeutic trial of patching.
Bilateral cataract patients require occlusion therapy only if there is a measurable visual
acuity or fixation difference between the two eyes.
It is crucial to develop a team with the parents and child to ensure good patching
compliance. Explain to parents the importance of amblyopia treatment even before the
operation. In young children even the "perfect" surgery without prompt amblyopia
treatment will not yield a favorable outcome. The parents are your partners on the
journey to best visual outcome.
Unilateral cataract patients almost always require more intensive occlusion therapy than
bilateral cataract patients unless the child was older and therefore less sensitive to
amblyopia when the cataract developed.
i Infants with unilateral cataract should have a patch on the unoperated eye 1
awake hour per day per each month of age, up to 8 months of age (maximum 8
hours/day).
Example: a 6-month-old infant would patch 6 hours each day
i After 8 months of age, children with unilateral cataracts should have the
unoperated eye patched 50% of all awake hours each day.
E This amount of patching may be adjusted downward as the child becomes
older and becomes less sensitive to amblyopia or if accurate visual acuity
testing shows no significant difference between the two eyes.
Secondary Opacification of the Visual Axis
Because secondary opacification of the posterior capsule and anterior vitreous face will
occur in virtually all children less than 5 years of age, it is recommended that a primary
surgical posterior capsulotomy and anterior vitrectomy be performed at the time of initial
cataract surgery.
i This step is optional in children 5-8 years of age and a reasonable alternative in
this group would be a primary surgical capsulotomy alone, without anterior
vitrectomy.
i Children older than 8 years of age can generally be managed as adults with the
posterior capsule left intact at the time of cataract surgery.
If the posterior capsule is left intact in children and a secondary YAG laser capsulotomy
is required, surgeons should be cautioned that the opacification of the posterior capsule
and anterior vitreous face can be quite dense and difficult to open.
i Generally, slightly increased amounts of laser energy and a greater number of
laser bursts are required compared to that used for adult capsulotomies.
i Reopacification of the visual axis following YAG laser capsulotomy is frequent and
repeat treatments may be required.
i Some opacifications are so dense that the only method to adequately clear the
visual axis is surgery with a pars plana vitrectomy approach.
E Incising the membrane with an MVR blade may assist in creating an edge
that the vitrector can grab and cut.
E Vertical or horizontal cutting intraocular scissors are also quite helpful for
removing dense membranes occluding the visual axis.
Secondary IOL Implantation
Children requiring a secondary IOL implantation should have the lens placed either into
the ciliary sulcus or within the capsular bag remnants if possible.
Implantation within the ciliary sulcus is generally the easiest and most convenient site
for secondary IOL implantation.
i Posterior synechia should be broken by blunt dissection using an iris spatula. This
may be facilitated by visco-dissection using a cannula with a cohesive viscoelastic
to expand the ciliary sulcus.
i Some Soemmering rings can be so thick and bulky that they need to be debulked
before an IOL can be implanted into the ciliary sulcus.
E After incising the inner portion of the Soemmering ring with an MVR blade,
the vitrectomy handpiece can be used to aspirate the bulk of the retained
lens material, thereby decompressing the Soemmering ring.
v This usually requires multiple approaches from several different clear
corneal incisions.
55
i If the anterior and posterior capsular halves are easily incised and separated, it
may be possible to place the IOL entirely within the capsular remnants after the
Soemmering ring of retained lens material is aspirated. (Figure 80)
E If this is not possible, the IOL should be placed securely within the ciliary
sulcus. (Figure 81)
Secondary IOLs placed within the ciliary sulcus should have an optic diameter of 6 - 7
mm and an overall haptic diameter of 13 - 14 mm.
Secondary IOLs placed within the capsular bag may be smaller with an optic diameter
of 5 - 6 mm and an overall haptic diameter of 12 - 13 mm.
Figure 80
Figure 81
Long-Term Follow-Up
Even after the first postoperative year is complete, children should be seen at least
once or twice yearly to monitor for postoperative complications. This is particularly
important in children less than 8 years of age at risk for amblyopia.
Long-term postoperative conditions to monitor include:

i Amblyopia
i Significant shifts of refractive error
E Unpredictable growth patterns
E
56
May be secondary to glaucoma
i Opacification of the visual axis
E
E
E
Posterior capsule opacification

Secondary pupillary membranes
Opacification of the anterior vitreous

i Decentration of IOL, particularly if implanted in the ciliary sulcus
i Retinal complications
E Retinal detachment
Cystoid macular edema (less common in children)

i Glaucoma
E Secondary glaucoma is frequent in both aphakic and pseudophakic
children.
E Children undergoing surgery at a very young age and those with small
corneas are at particularly high risk for glaucoma.
E Abnormalities of the angle structures are more frequent in children with
congenital cataracts.
v Trabeculotomy and goniotomy may be effective surgical treatment
options if angle abnormalities exist.
E In addition to elevated intraocular pressure, signs of glaucoma in younger
children include:
v Myopic refractive shifts
v Increased corneal diameter
v Decreased corneal clarity
v Increased axial length measurements
v Optic nerve cupping (may be reversible)
ANESTHESIA FOR PEDIATRIC CATARACT SURGERY

Competent anesthesia care is vital for the conduct of safe and effective cataract surgery in
children. While local anesthesia may be possible in older, cooperative children, most young
children will require some form of general anesthesia. Infants less than one year of age are
particularly challenging to the anesthetist, while at the same time the pediatric patient
population are most in need of timely cataract surgery to prevent profound vision loss from
amblyopia and nystagmus.
General anesthesia for children is best administered by a physician with particular training or
experience in pediatric anesthesia care. However, this may be an unrealistic expectation for
many areas of the world. If other medical officers or nurse anesthetists are utilized, they should
be adequately skilled and comfortable in pediatric anesthesia care to ensure a safe and
comfortable procedure. If adequate anesthesia skills and equipment are not available, it is
better to not perform surgery at all rather than placing the general health and safety of the child
at unacceptable risk. Local physicians and medical teams are best suited to make this
decision.
57
Recommended Basic Equipment List for Pediatric Anesthesia

Airway
t
t
t
t
t
t
t
t
t
Drugs:
t
t
t
t
t
t
t
t
Management:
Facemasks - age range 0-16 yr
Oropharyngeal airways - size 0 to 4
LMAs (regular and flexible)- size 1 - 4
ETT (regular, RAE type) - sizes 3-8
Bougie and stylets
Laryngoscopes - range of blades
(Mackintosh and Miller) and sizes
Suction
Tape or ties to secure airway
NG tubes (range of sizes - decompress
stomachs in small children)
Premedication - midazolam
Induction - propofol, thiopentone
Muscle relaxants - suxamethonium, atracurium, rocuronium
Reversal agent - glycopyrrolate/neostigmine
Anti-emetics - ondansetron, dexamethasone, metoclopramide, cyclizine
Analgesics - paracetamol (PO/PR/IV), NSAIDs
(PO/PR/IV), opiates (fentanyl, alfentanil, morphine)
Emergency - atropine, glycopyrrolate. adrenaline
IV fluids - isotonics (saline or Ringers)
Breathing:
t Anesthetic circuits - peds T piece, circle
t Anesthetic machine - oxygen/air/nitrous oxide + volatile agents isoflurane/
sevoflurane
t Ventilator - ventilatory modes suitable for children
t Scavenging of gases from theatre
Circulation:
t Range of cannulas
t IV
Monitoring:
t Full monitoring - HR, BP, ECG, SpO2, end tidal CO2, gas analysis, temperature
58
When available, general anesthesia with inhalational and intravenous agents administered with
a secure airway (endotracheal tube or laryngeal mask) provides an adequate depth of
anesthesia. This may be supplemented by depolarizing muscle relaxants to ensure akinesia
and reduced positive vitreous pressure. These benefits must be measured against potential
drawbacks such as the difficulty of obtaining intravenous access in small children, need for
adequate oxygen delivery, need for monitoring oxygen saturation (pulse oximetry) as well as
the possible side effects of postoperative nausea and vomiting. General anesthesia with
ketamine, administered by either intravenous or intramuscular routes, is also possible and
requires a less sophisticated health care system for safe use but it may be more difficult to use
during long procedures and has the disadvantages of inducing eye movement and increased
positive vitreous pressure. These features make it less ideal than inhalation agents and require
the use of a supplemental retro- or peribulbar anesthesia.
In older, more cooperative children, local or
regional anesthesia becomes a more viable
alternative to general anesthesia. We have
found that children as young as 7 years of
age may be safely and adequately
operated on using a peribulbar injection
technique.14 One clinical method to
identify good potential candidates for such
local anesthesia surgery is the lid
speculum test. In this assessment,
children are given a topical anesthetic and
then have an eyelid speculum placed while
in the clinic. If the child tolerates this
process without significant resistance, they
will usually be cooperative enough to
tolerate cataract surgery under local
anesthesia that can be supplemented with
intravenous sedation as needed.
SUMMARY
We have attempted to present a concise summary of the fundamental techniques and
management issues that are specific to pediatric cataracts. The reader will notice that we have
given particular emphasis to dealing with younger children, particularly those less than five
years of age, as this is the group whose needs differ most from typical adult cataract patients.
These younger patients have eyes that are smaller, more elastic and present with a diversity of
cataract types that may be accompanied by other ocular abnormalities. Additionally, these
younger patients are particularly sensitive to vision loss from amblyopia, large refractive shifts
and secondary glaucoma. All of these factors serve to make the pediatric cataract patient more
complicated to manage and emphasize the need for specialized surgical techniques and the
maintenance of long term follow-up care. We urge the reader to expand their knowledge of
these subjects. Online resources and consultation opportunities with other pediatric cataract
surgeons are available at www.cybersight.org.
59
REFERENCES
1. Wilson ME, Trivedi RH, Pandey SK. Pediatric cataract surgery techniques, complications,
and management. Lippincott Williams & Wilkins; 2005.
2. Lambert SR, Drack AV. Infantile cataracts. Survey Ophthalmology 1996;40:427-458.
3. Wilson KW, Spiegel PH. Pediatric ophthalmology and strabismus. Springer-Verlag; 2003.
4. Roy, FH. Ocular syndromes and systemic diseases. Lippincott Williams & Wilkins; 2002.
5. Nelson LB, Olitsky SE. Harleys pediatric ophthalmology. Lippincott Williams & Wilkins;
2005.
6. Goldberg MF. Persistent fetal vasculature (PFV): an integrated interpretation of signs and
symptoms associated with persistent hyperplastic primary vitreous (PHPV) LIV Edward
Jackson Memorial Lecture. American Journal Ophthalmology 1997;124:587-626.
7. Jones, KL. Smiths recognizable patterns of human malformation. W.B. Saunders
Company; 1988:10.
8. Bardelli AM, Lasorella G, Vanni M. Congenital and developmental cataracts and
multiformation syndromes. Ophthalmic Pediatrics and Genetics 1989;10:293-298.
9. Berk AT, Saatci AO, Ercal MD, Tunc M, Ergin M. Ocular findings in 55 patients with Downs
syndrome. Ophthalmic Genetics 1996;17:15-19.
10. Neely DE, Plager DA, Borger, SM, Golub RL. Accuracy of intraocular lens calculations in
infants and children undergoing cataract surgery. Journal of AAPOS 2005;9:160-165.
11. Nihalani BR, VanderVeen DK. Comparison of intraocular lens power calculation formulae in
pediatric eyes. Ophthalmology 2010; August; 117:1493-1499.
12. Isenberg SJ. The Eye in Infancy. Mosby; 1994.
13. Capozzi P, Morini C, Piga S, et. al. Corneal curvature and axial length values in children
with congenital/infantile cataract in the first 42 months of life. Investigative Ophthalmology
& Visual Science 2008; 49:11:4774-4778.
14. Tomkins O, Ben-Zion I, Garzozi HJ, Moore DB. Comparison of anesthesia options for
pediatric cataract surgery in a rural setting. Journal of AAPOS 2011; 15:e30-31.
60
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ORBIS International

Daniel E. Neely MD, M. Edward Wilson MD,

This manual is part of a series of specialized manuals produced

Morphology of Common Pediatric Cataracts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22

Etiology of Pediatric Cataracts

Step-by-Step Approach for Pediatric Cataract Surgery

Postoperative Management of Pediatric Cataract Patients . . . . . . . . . . . . . . . . . . . . . . . . 50

Anesthesia for Pediatric Cataract Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 57

Congenital: Present at birth

Infantile: Develop within the first 2 years of life

Develop before 10 years of age

MORPHOLOGY OF COMMON PEDIATRIC CATARACTS

Anterior Polar Cataract

i Usually do not increase in size over time

Can be hereditary and present at birth

Favorable visual prognosis

i Usually do not require surgery

monitor refractive error and correct as needed

v Anterior Subcapsular Cataract

May be secondary to radiation, uveitis, trauma or atopic skin disease

i May require surgery if vision is reduced

i Peripheral lens cortex may be clear

Usually acquired and bilateral but may be asymmetric

Usually normal sized eyes

Probably the most common type of congenital cataract

Usually associated with mild microphthalmia or microcornea

Severe impact on visual development unless removed at a very early age

Surgery should be performed (if possible) before appearance of nystagmus

Posterior Subcapsular Cataract

May require a posterior capsulotomy (surgical or YAG-laser) to completely clear the

Posterior Lentiglobus (Posterior Lenticonus)

Not associated with microphthalmia or microcornea

i Corneal diameter is normal and symmetric

Delayed onset usually results in a more favorable visual prognosis

Hydrodissection should be avoided during surgery as it may result in a large rupture of

Diffuse or Total Cataract

Complete opacification of lens substance (nucleus and cortex)

May also be secondary to trauma

Lens may show resorption (in unilateral cases)

If no view of the posterior segment is possible, a B-scan ultrasound of the posterior

Persistent Fetal Vasculature (PFV)

Presentation is variable but there is usually a persistent hyaloid artery or stalk

See full description of PFV detailed later

ETIOLOGY OF PEDIATRIC CATARACTS3

Idiopathic: The majority (60%) of bilateral congenital/infantile cataracts are of unknown

Approximately 30% of bilateral congenital/infantile cataracts are hereditary

Usually autosomal dominant transmission

w Ocular Syndromes with Associated Cataracts: Some cataracts presenting primarily

i Bilateral absence of most or all iris tissue

If genetic testing is available, sporadic cases of aniridia should undergo this

Usually bilateral but asymmetrical

Anterior segment (small corneal diameter, iris coloboma)

i Vision may range from normal to no light perception

Persistent fetal vasculature (PFV) - formerly called persistent hyperplastic primary

Persistent pupillary membrane

i Usually a sporadic, isolated abnormality of one eye only

Most common cause of unilateral cataract in a smaller than normal eye

i Management requires release of traction on the ciliary body and retina

Surgery is best performed by use of vitrectomy instrumentation to remove

Systemic Syndromes with Associated Cataracts: These cataracts are associated