Management

SYMPOSIUM: RETROLENTAL FIBROPLASIA

183

induced in mice by matemal anoxia: with particular reference to the problem of retrolental fibroplasia in man, Am. J. Ophth., 35:311-329 (March) 1952. 12. Pätz, Arnall: Oxygen studies in retrolental fibroplasia: I V . Clinical and experimental observations, Am. J. Ophth., 38 :291-308 (Sept.) 1954. 13. : Factors influencing the role of oxygen in retrolental fibroplasia. To be published. 14. Pätz, Arnall, Eastham, Ann, Higgenbotham, Don H., and Kleh, Thomas: Oxygen studies in retro­ lental fibroplasia: II. The production of the microscopic changes of retrolental fibroplasia in experimental animals, Am. J. Ophth., 36 :1511-1522 (Nov.) 1953. 15. Pätz, Arnall: Unpublished data. 16. Reese, Algernon B., and Blodi, Frederick C.: Retrolental fibroplasia, Am. J. Ophth., 34:1-24 (Jan.) 1951. 17. Terry, T. L . ; Retrolental fibroplasia in the premature infant: V. Further studies on fibroplastic overgrowth of the persistent tunica vasculosa lentis, Tr. Am. Ophth. Soc, 42:383-396 ( M a y ) 1944.

M A N A G E M E N T

FREDERICK C . BLODI, M . D . Iowa City, Iowa

fibroplasia

tiva and lids in very small babies. T h e dilata­

is still only a palliative one. H o w e v e r , pedia­

T h e management o f retrolental

tion o f the pupils is adequate after an hour

tricians and ophthalmologists should be ac­

even if the iris is heavily pigmented. O n l y

quainted with the various problems that may

in a very f e w aflfected children is dilatation

arise during the course o f this condition.

unsatisfactory after these mydriatics. A t r o ­

T h e y may not always be able to cope with

pine should be avoided in small children.

them, but they may frequently alleviate them.

F o r the examination itself, the baby is

T h e y should be able to reassure the parents,

taken out o f the incubator, put on a table,

to counsel them as to prognosis and

final

and immobilized. This is easily achieved b y

outcome o f the disease, and to advise them

wrapping the child in a blanket.

as to further educational aid and social ad­

moscopic examination is possible with

justment

infant in an incubator, but is thus done only

in the case o f severe visual im­

pairment o f the child.

T h e examiner stands at the baby's head and opens the lids with his fingers or instructs

In order to detect active cases o f retro­ fibroplasia,

the

when the condition o f the baby is precarious.

THE ACTIVE PHASE lental

Ophthal­

premature children have

a nurse to pull d o w n the lower lid as he raises the upper lid. T h e nurse stands at the

to be examined as early in life as possible.

infant's

It is usually feasible to examine a baby dur­

this w a y a satisfactory examination in the

feet and grips the head firmly. In

ing the first week o f life. In order to detect

large majority o f smaller infants is possible.

the exact onset o f the disease and follow its

Only larger or restless

development

pacified with a moistened, sterile sugar teat.*

closely the

infant

should be

seen once a week.

babies need to be

T h e sugar teat is generally effective in placat­

Good dilatation o f the pupils is absolutely

ing the baby up to the age o f four months,

necessary f o r a satisfactory examination o f

after which he is less tractable and it becomes

the fundi. O n e drop of a two-percent homa­

necessary to use a general anesthetic in indi­

tropine hydrobromide solution and one drop

cated cases.

of

a

rine®)

10-percent

phenylephrine

hydrochloride

suspension

(Neosynare

T h e fundus

o f a small premature child

in­

stilled into each eye. T h e phenylephrine o c ­ casionally causes blanching o f the conjunc­

* About one-third of a teaspoon of cane sugar is put in the middle of a sterile two by two inch sponge and then tied with a piece of thread.

F R E D E R I C K C. B L O D I

184

may

in the

beginning be obscured by a

vitreous haze or clouding. Only some o f the

T H E CICATRICIAL PHASE W h e n the active phase is over and

the

vessels may be faintly visible. T h e disc is

disease has run its course, the management

often grayish or whitish in color. It may be

of the case will depend upon the degree o f

best visible with a high myopic lens. A s the

severity into which the disease has developed.

eye matures, or in larger premature children,

The prognosis for the milder cases is e x ­

the vitreous clears and only remnants o f the

cellent and w e can only assure the parents

hyaloid system remain present. These also

that the condition will usually remain sta­

disappear

tionary.

quickly. T h e extreme

periphery

of the fundus has a characteristic, geneous gray color and appears

homo­

Severe cases should be followed at regular

elevated.

intervals. This should be every six months

This grayish peripheral zone persists for a

the first t w o years and then yearly. These

considerable period o f time and should not

later examinations serve mainly as a check

be confused with the peripheral changes in

on the development o f possible complica­

early retrolental

tions. T h e y also give the parents an o p p o r ­

fibroplasia.

The ophthalmoscopic examination should

tunity to discuss with

the

physician

any

not be confined to the posterior segment, but

problems and difficulties which have come

should include the periphery, where the first

up during the preceding year.

pathologic changes may appear. It is usually

Medical treatment o f these severe cicatri­

possible to rotate the child's eye in any di­

cial stages is o f no avail. Surgery, irradia­

rection desired by exerting gentle pressure

tion, and

with the fingers holding the lids.

been

Infants with normal fundi are not

fol­

lowed after the age o f three months. W h e n

other

tried,

ophthalmoscopic

retrolental

made

all

these

methods

have

attempts

have

The remaining vision will depend o n the severity

are

therapeutic

proven unsuccessful.

the patient is discharged before that age, examinations

but

of

the

case. E y e s affected

fibroplasia

of

with

Grade I V o r

V

every two weeks in a follow-up clinic, the

will have only light perception. In Grade I I I

same technique being employed.

vision is 3 / 2 0 0 to 1 0 / 2 0 0 when the fold is

If

retrolental

is suspected or

on the temporal side. It may be better when

detected during one o f these routine exami­

the fold does not cross the macular area. In

nations, the baby should be followed more

these severe cases fixation will develop later

closely. It may be necessary to examine the

than normally and a roaming nystagmus may

child twice o r three times a week in order

persist f o r a long period o f time.

to appreciate

fibroplasia

various progressions o r

re­

gressions during the course o f the disease. Only when the disease has progressed to a more severe stage should the parents be in­ formed and instructed. It means unnecessary anxiety and w o r r y for the whole family if they are informed about the child's condi­ tion at an earlier stage o f the disease when the chances of a spontaneous regression are so great. In the severe stages o f the active phase the pupil should be intermittently or con­ tinuously echias.

dilated

to avoid posterior

syn­

COMPLICATIONS The most frequent complication o f retro­ lental fibroplasia is secondary glaucoma. It develops in a fourth to a third o f all severe cases. It is probably caused by an obstruc­ tion to the outflow o f aqueous when the an­ terior chamber becomes increasingly shallow. Frequently the rise in intraocular pressure is brief and does not cause much discomfort to the child. Occasionally, however, the rise in pressure may be acute and cause severe distress to the infant. Glaucoma usually arises in eyes which are

185

SYMPOSIUM: RETROLENTAL FIBROPLASIA

Fig. 3 (Blodi). Deep-set eyes in severe retrolental fibroplasia.

coma is usually o f a transitory nature. Shallowing o f the anterior chamber during Fig. 1 (Blodi). Peripheral corneal opacities caused by anterior synechias.

blind, and therefore

no treatment is indi-

cated except when pain and other general symptoms

occur. This

type

of

glaucoma

may be controlled by miotics and the pressure will be normal after

a few days or

weeks. In a few cases it may be necessary to perform an operation to relieve the glaucoma if medical treatment alone does not siÆce. H o w e v e r , the opening o f an

eye-

ball in such a patient may be followed b y disastrous complications.

a

period o f increased intraocular pressure may be followed by a deepening o f the anterior chamber when the eye becomes atrophic. Another frequent complication o f severe retrolental fibroplasia is a corneal opacification. It is caused by anterior synechias and may present a cosmetic problem (figs. 1 and 2). Another cosmetic problem is the smallness of the eye, deep set in the orbits, as enophthalmos occurs often

in severely

affected

eyes (figs. 3 and 4 ) . If the eye is completely blind it can be managed

like any

other

atrophic eye.

F e w o f the glaucomatous eyes in

these

children become buphthalmic as the

glau-

Fig. 2 (Blodi). Central corneal opacities caused by anterior synechias.

Fig. 4 (Blodi). Lateral view of deep-set eyes in severe retrolental fibroplasia.

186

A L G E R N O N Β, R E E S E

A retinal fold (partial retinal detachment) that is seen in the cicatricial phase o f the disease usually does not progress. Such an eye may deviate, thus enabling the patient to use an attached part o f the retina for fixa­ tion. Esotropia o f the more amblyopic eye is a common complication. It can be surgically corrected for cosmetic reasons. This should not be done when the deviation brings a func­ tioning area o f the retina into the line o f fixa­ tion. Cataract develops only in about three percent o f eyes affected with Stage I V or V . It is therefore an uncommon complication o f this disease. EDUCATIONAL A N D SOCIAL ADJUSTMENT

A s long as no effective treatment for retrolental fibroplasia is available these pa­ tients will present an educational and social

problem more than a medical problem. T h e parents have to be guided in their endeavor to bring up a visually handicapped child. Many agencies and trained social workers will be able to give help to them, but the first responsibility lies with the physician who makes the diagnosis and informs the parents about the poor prognosis. W h i l e the physician has to discuss with the parents many medical problems o f retro­ lental fibroplasia, he will have to refer them to the proper agency for further counseling. T h e panel has prepared a pamphlet written for parents o f children with retrolental fibro­ plasia. This booklet is distributed by the physician and it should bridge the gap between the physician and the social worker. It will enlighten the parents about many problems of the disease. University

Hospitals.

CONCLUSIONS A L G E R N O N B . REESE, M . D . Neiv York, New

All theories concerned with the etiology of retrolental fibroplasia excepting that con­ cerned with oxygen have failed o f confirma­ tion. W e feel that we can recommend with confidence a program entailing a more ra­ tional use o f oxygen in the management o f the premature infant and that w e may antici­ pate a significant drop in the incidence o f this disease, which' has become the greatest cause o f blindness in children. While great advances have been made in the better understanding o f the etiology o f retrolental fibroplasia, it is probable that even with judicious use o f oxygen there will be occasional cases of retrolental fibroplasia. It is hoped that future investigations will re­ veal other etiologic factors. It must be remembered, however, that use o f oxygen is sometimes a life-saving measure, and it will be necessary to con­

York

tinue its use for infants with cyanosis and definite signs o f respiratory distress. Pediatricians throughout the country are taking the facts into consideration in the man­ agement o f newly born prematures. T h e indications for o x y g e n administration are now considered to be fewer than they have been in the recent past. Cyanosis and respira­ tory distress are held to be bona fide symp­ toms requiring o x y g e n therapy. H o w e v e r , there is a growing conviction that irregu­ lar or periodic respirations unaccompanied by other signs o f distress do not require o x y ­ gen administration. W h e n oxygen is indi­ cated, every effort is made to keep the c o n ­ centration in the incubator below 40 percent and to discontinue treatment as soon as res­ piratory distress is relieved. W e feel that there is insufficient evidence available to support the idea that retrolental