danielle fiarito

7/26/2019 Danielle Fiarito

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Danielle Fiarito, 16, was diagnosed with glaucoma at age 4 Read more

Download our free booklet (PDF)--Childhood Glaucoma: Facts, nswers,

!i"s, and #esources for $hildren with %laucoma and their Families

Childhood glaucoma also referred to as congenital glaucoma,pediatric, or infantile glaucoma occurs in babies and youngchildren. It is usually diagnosed within the first year of life.

This is a rare condition that may be inherited, caused by incorrect development of the

eyes drainage system before birth. This leads to increased intraocular pressure, which

in turn damages the optic nerve.

Symptoms of childhood glaucoma include enlarged eyes, cloudiness of the cornea, andphotosensitivity (sensitivity to light).

How is it Treated?

n an uncomplicated case, surgery can often correct such structural defects. !oth

medication and surgery are re"uired in some cases.

#edical treatments may involve the use of topical eye drops and oral medications.

These treatments help to either increase the e$it of fluid from the eye or decrease the

production of fluid inside the eye. %ach results in lower eye pressure.

There are two main types of surgical treatments& filtering surgery and laser surgery.

'iltering surgery (also nown as micro surgery) involves the use of small surgical tools to

create a drainage canal in the eye. n contrast, laser surgery uses a small but powerful

beam of light to mae a small opening in the eye tissue.

hat to %$pect

Thousands of children with glaucoma can live full lives. This is the ultimate goal of

glaucoma management. *lthough lost vision cannot be restored, it is possible to

optimi+e each childs remaining vision. %"ually important is to encourage your childsindependence and participation in his or her own selfcare.

Signs of -hildhood laucoma

&nusuall' large e'es

cessi*e tearing

$loud' e'es

+ight sensiti*it'
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CHILDHOOD GL!CO"-hildhood glaucoma is an unusual eye disease and significant cause of childhood blindness.

t is caused by disease related abnormal increase in intraocular pressure. The multiple

potential causes fall into one of two categories and may be primary or secondary to some

other disease process. /rimary congenital glaucoma results from abnormal development ofthe ocular drainage system. t occurs in about 0 out of 01,111 births in the 2nited States and

is the most common form of glaucoma in infants. Secondary glaucomas result from

disorders of the body or eye and may or may not be genetic. !oth types may be associated

with other medical diseases.

Ten percent of primary congenital glaucomas are present at birth, and 31 percent are

diagnosed during the first year of life. The pediatrician or family first notice eye signs of

glaucoma including clouding and4or enlargement of the cornea. The elevated intraocular

pressure (5/) can cause the eyeball itself to enlarge and in6ury to the cornea. mportant

early symptoms of glaucoma in infants and children are poor vision, light sensitivity, tearing,

and blining.

/ediatric glaucoma is treated differently than adult glaucoma. #ost patients re"uire surgery

and this is typically performed early. The aim of pediatric glaucoma surgery is to reduce 5/

either by increasing the outflow of fluid from the eye or decrease the production of fluid

within the eye. 5ne operation for pediatric glaucoma is goniotomy. ts rate of success is

associated with the age of the child at the time of diagnosis, the type and severity of the

glaucoma, and the surgery techni"ue. 5ther surgical options are trabeculectomy and

glaucoma drainage tubes.

*ppro$imately 3171 percent of babies who receive prompt surgical treatment, longterm

care, and monitoring of their visual development will do well, and may have normal or nearly

normal vision for their lifetime. Sadly, primary congenital glaucoma results in blindness in 8

to 09 percent of childhood patients. hen childhood glaucoma is not recogni+ed and treated

promptly more permanent visual loss will result.

htt"s:wwwglaucomafoundationorgchildhood.glaucomahtm
https://www.glaucomafoundation.org/childhood_glaucoma.htmhttps://www.glaucomafoundation.org/childhood_glaucoma.htm


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Pediatric Glaucoma: A Review of the Basics

Despite similarities to glaucoma in adults, the clinical findings and surgical

management of pediatric glaucoma vastly differ.

Wendy uang, !D, "ew #or$ %ity

&'('()*&

Pediatric glaucoma is associated with a wide variety of pathology. +everal

classification systems have een developed to organi-e and categori-e

childhood glaucomas. he ma/ority of systems are ased on etiology and

descrie two main groups: primary and secondary glaucoma. %ongenital and

developmental glaucomas associated with syndromes and systemic

anormalities fall under the umrella of primary glaucomas. %ausative

pathologies ranging from uveitis to congenital cataract surgery fall under

secondary glaucoma.* he incidence of childhood glaucoma is estimated to e

(.(0 per *)),))) patients younger than () years old ased on a defined 1.+.

Population study in 2lmstead %ounty.( Primary congenital glaucoma is the

most common form of childhood glaucoma, with a reported prevalence of (.34

cases per *)),))) irths.5 As in adults, pediatric glaucoma is associated with

elevated intraocular pressure and progressive optic nerve damage6 however, the

clinical findings and surgical management are vastly different.

%linical 7indings

!anifestations of elevated 82P in children can vary depending on age of onset

and rate of pressure elevation. Gradually increasing pressure can result in little

to no corneal clouding. Presentation with uphthalmos and'or symptoms of

tearing, lepharospasm and photophoia are more common 9+ee 7igure *. 8n

contrast, those children with acute pressure elevations present with corneal

clouding. his finding can also e seen at irth 9+ee 7igure (. 7irm tactile

pressure in these cases can e apparent and helpful in differentiating other

causes of corneal opacification. he presence of a poor red refle; can elucidate

sutle corneal clouding, although asence of a red refle; can e related to other

pathology as well. aa


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"eurofiromatosis ype * has an autosomal=dominant inheritance. 8t carries a

spectrum of findings including cafE=au=lait spots, frec$ling of the a;ial'inguinal

area, sphenoid dysplasia, +=shaped ple;iform neurofiromas of the lids, optic

nerve gliomas, isch nodules and choroidal hamartomas. Fyes with an

associated ple;iform neurofiroma have a 4) percent ris$ of glaucoma.*5

reatment

!edical. !edical therapy in pediatric glaucoma is often supplementary to

surgical management. 8t is often used for preoperative treatment to facilitate

clearing of corneal edema. 8n addition, it can play a role in treating patients who

are too unstale to undergo anesthesia. imolol is often used as a first=line agent

and has een shown to effectively lower 82P in the pediatric population. here

is an increased ris$ for ronchospasm, apnea and radycardia. he use of

eta;olol 9* selective antagonist, timolol ).(4H gel, and timolol ).*H can

help to avoid these side effects. 2verall, however, timolol drops are generally

well=tolerated.*& atanoprost has een shown to have 82P=lowering effects,

particularly in older children, ut the non=response rate has een shown to e

higher than in adults. +ide effects are minimal, although dar$ening of the irides

can occur, as in adults.*4 opical caronic anhydrase inhiitors are also

effective in lowering 82P. hey are generally well=tolerated with minimal side

effects. 2ral aceta-olamide has een shown to e more effective in lowering

82P and can e used in children with glaucoma at doses of 4 mg'$g'day to *4

mg'$g'day. 2ral treatment carries a ris$ of systemic side effects, such as

metaolic acidosis. Brimonidine has the most well=estalished side effect

profile in children, causing radycardia, hypotension, hypothermia, hypotonia

and apnea in infants and severe lethargy in toddlers.*@ Because of these side

effects, its use is limited in the pediatric population.

+urgical. Angle surgery is considered the mainstay of treatment for primary

congenital glaucoma, with a reported ) to 0) percent success rate after one to

two procedures in patients treated after 5 months of age and efore * to ( years

of age. his success rate significantly diminishes in patients presenting outside


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of this age range and those who fall in the spectrum of developmental

glaucoma.*

7igure 5. aa


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is a difference in success when comparing traeculotomy alone vs. comined

traeculectomy'traeculotomy.(&

A?ueous shunt implantation has shown significantly greater success when

compared to traeculectomy.(4 ow endophthalmitis rates have een reported.

8t does appear, as in adults, that implants ecome less effective over time and

re?uire reoperation.(@ 8mplants availale for use include: Ahmed valve 9"ew

World !edical6 Baerveldt implant 9Pharmacia6 and !olteno implant 92P 8nc.

Ahmed valves and Baerveldt implants are the most commonly used and have

oth een reported to e effective.(@=(3

%yclodestruction procedures are an option in difficult=to=treat cases.

%yclocryotherapy has een replaced y laser cyclophotocoagulation. A

transscleral techni?ue is most commonly used. Fndoscopic

cyclophotocoagulation has een reported to e effective as well.(0

Prognosis

Reports of visual outcomes vary. %ases resulting in visual acuity sufficient to

?ualify for a motor vehicle driving license range from (0 to &@.@ percent of

patients. Cision at the time of diagnosis, type of glaucoma and amlyopia

appear to e the largest factors in visual outcomes. %hildren with primary

congenital glaucoma have the est prognosis. 8n the setting of well=controlled

intraocular pressure, amlyopia is a $ey factor in vision loss. As in pediatric

patients with congenital cataracts, unilateral cases often have poorer visual

outcomes secondary to amlyopia.5),5*

%ounseling patients and their families with regard to potential future vision loss

can e challenging. %onnecting them to resources for the visually impaired

early is of utmost importance. he following are a few organi-ations with

resources for the visually impaired and lind: ighthouse 8nternational


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@. +ampaolesi R. %ongenital glaucoma. he importance of echometry in its

diagnosis, treatment and functional outcome. 8n %ennamo G, Rosa " 9eds:

1ltrasonography in 2phthalmology ondon, Fngland: >luwer Academic

Pulishers, *00:*=&.

. +haran +, +wamy B, aranath DA, Jamieso R, et al. Port wine vascular

malformation and glaucoma ris$ in +turge=Weer +yndrome. J AAP2+

())06*59&:5&=3.

3. +ullivan J, %lar$e !P, !orin JD. he ocular manifestations of the +turge=

Weer syndrome. J Pediatr 2phthalmol +traismus *00(6 (09@:5&0=4@.

0. Walton D+, >atsavounidou G, owe %1. Glaucoma with the

oculocererorenal syndrome of owe. J Glaucoma ())46*&:*3*=4.

*). Alward W!. A;enfeld=Rieger syndrome in the age of molecular genetics.

Am J 2phthalmol ()))6*5):*)=**4.

**. %hang %, +ummers %G, +chimmenti A, Gra/ews$i A. A;enfeld=Rieger

syndrome: "ew perspectives. Br J 2phthalmol ()*(60@:5*3=((.

*(. ingorani !, anson 8, van eyningen C. Aniridia. Fur J um Genet ()*(

2ct6()9*):*)**=. doi: *).*)53'e/hg.()*(.*)). Fpu ()*( Jun *5. Review.

*5. +ippel >%. 2cular findings in neurofiromatosis type *. 8nt 2phthalmol

%lin ())*6&*9*:(4=&). Review.

*&. Plager DA, Whitson J, "etland PA, Ci/aya , et al, BF2P8% + Pediatric

+tudy Group. Beta;olol hydrochloride ophthalmic suspension ).(4H and

timolol gel=forming solution ).(4H and ).4H in pediatric glaucoma: A

randomi-ed clinical trial. J AAP2+ ())06*59&:53&=0).

*4. Blac$ A%, Jones +, #anovitch , Fnyedi B, +tinnett ++, 7reedman +7.atanoprost in pediatric glaucoma==pediatric e;posure over a decade. J AAP2+

())06*59@:443=@(.

*@. %arlsen J2, Karis$ie "A, >won #, et al. Apparent central nervous

system depression in infants after the use of topical rimonidine. Am J

2phthalmol *0006*(3:(44=(4@.

*. Russell=Fggitt 8!, Rice "+%, Jay B, et al. Relapse following goniotomy for

congenital glaucoma due to traecular dysgenesis. Fye *00(6@:*0=()).


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*3. Joos >!, Alward W!, 7olerg R. F;perimental endoscopic goniotomy: A

potential treatment for primary infantile glaucoma. 2phthalmology

*0056*)):*)@@=*)).

*0. "eely DF. 7alse passage: A complication of 5@) degrees suturetraeculotomy. J AAP2+ ())460:50@=.

(). Cerner=%ole FA, 2rti- +, Bell "P, 7eldman R!. +uretinal suture

misdirection during 5@) degrees suture traeculotomy. Am J 2phthalmol

())@6*&*:50*=(.

(*. Gir$in %A, Rhodes , !cGwin G, !archase ", %ogen !+. Goniotomy

versus circumferential traeculotomy with an illuminated microcatheter in

congenital glaucoma. J AAP2+ ()*(6*@94:&(&=.

((. Bec$ AD. Diagnosis and management of pediatric glaucoma. 2phthalmol

%lin "orth Am ())*6*&95:4)*=*(.

(5. +idoti PA, Belmonte +J, iemann J!, Ritch R. raeculectomy with

mitomycin=% in the treatment of pediatric glaucomas. 2phthalmology

()))6*):&((=0.

(&. !ullaney PB, +ellec$ %, Al=Awad A, Al=!esfer +, Kwaan J. %omined

traeculotomy and traeculectomy as an initial procedure in uncomplicated

congenital glaucoma. Arch 2phthalmol *0006**:&4=@).

(4. Bec$ AD, 7reedman +, >ammer J, Jin J. A?ueous shunt devices compared

with traeculectomy with mitomycin % for children in the first two years of life.

Am J 2phthalmol ())56*5@:00&=*))).

(@. 2

device surgery in refractory pediatric glaucomas: 8. ong=term outcomes. J

AAP2+ ())3 7e6*(9*:55=0. Fpu ()) 2ct *.

(. !orad #, Donaldson %F, >im #!, Adolell !, evin AC. he Ahmed

drainage implant in the treatment of pediatric glaucoma. Am J 2phthalmol

())56*54:3(*=0.

(3. Buden- D, Gedde +, Brandt J, >ira D, et al. Baerveldt glaucoma implant in

the management of refractory childhood glaucomas. 2phthalmology

())&6***:(()&=*).


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(0. "eely DF, Plager DA. Fndocyclophotocoagulation for management of

difficult pediatric glaucomas. J AAP2+ ())*649&:((*=0.

5). >hitri !, !ills !, #ing G, Davidson +, et al Cisual acuity outcomes in

pediatric glaucomas. J AAP2+ ()*( Aug6*@9&:5@=3*.

5*. >argi +, >oc 7, Biglan AW, Davis J+. Cisual acuity in children with

glaucoma. 2phthalmology ())@6**5:((0=53. Fpu ())@ Jan *).

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