On Angiomas of Retina, Brain, and Skin Louis I. Sobel, Jeffrey G. Odel and Alan H.

Friedman Pediatrics 1981;67;753

The online version of this article, along with updated information and services, is located on the World Wide Web at:
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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. PEDIATRICS is owned, published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright 1981 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.

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the

first

variable

in

that

the

control

group

received

nurs-

ery care
niques.

before nurses The authors

were point

aware of the treatment out that parents may experimental program nursery
this not to nurse or the this knew whether authors was their experimental enriched. could If this have obtained, child was would matter

techhave famin the

introduced some variation in that the lies, having observed the intervention nursery, after
enced practical

Scale while their infants were receiving intermediate convalescent care in the nursery and administration the Bayley Scales of Infant Development, growth surements, and physical examinations at six months expected date of confinement.
The prethat and postassessments were studies using administered

and of

meapast by a

may the
short-term

have

modeled observed
results. in that

their
Actually it would

subsequent

behavior and
have

caretaking
importance

in the

influlittle

licensed
We uators would agree

child
who are be highly

clinical
subsequent unaware desirable. of the Scales

psychologist,
of experimental However, author Scales

who

was

not

blind.
evalstatus follow-

independent vs control we offer the

whether

improved
parental
tential

development
observation variable not of

was
the mentioned

due
by

intervention, both. A concerns but not po-

intervention,

ing. the uate and ity,

of

informed
not whether receiving knew parents their

consent.
control special infants expectations

They

indicate parents

At the time Brazelton

study, the and Bayley

who administered was a PhD gradnewborn subjectiv-

care were

or

parents were altered so,

student with experience infant assessment measures.

in administering To minimize

being and

perceptions

their
influenced

relationship
the

with
outcome

their
in

infants
the

and importance

this
of

might
the

have
results.

a reliability check was obtained by two observers, one whom was a licensed clinical psychologist with experience with the Bayley Scales, and the other, a graduate

direction

This would became

would probably

have
not

more
persist

practical
after the

because
program

it

enrichment

student Scales. in the

University was

with training In addition, administration

of Kansas checked

in the administration the author was trained

of Infant by T. Medical was independent the Berry Brazelton Laboratory. Brazelton,

of the Bayley to reliability

Scales Her MD, at reliability at the the

standard.
greatest importance is the necessity for absolute

Of
The

again

objectivity
Bayley

in measuring
motor six authors, experimental of assignment infant it is doubtful could remain and months and

outcome
developmental following since subjects, in has that objective, each control

for behavioral
testing nursery subjects this case. author The

research.
was discharge were would Bayley tested have test cominvested of intendone by

Childrens Hospital more, a 93% agreement and psychologists

Center, Boston. Furtherfound between pediatricians ratings of the infants

approximately
one of the the aware a 6-month-old and months

before
been

current level of development. help clarify the issues raised D.

GARY BENFIELD, LEIB, PHD SUSAN

We hope these by Dr Altemeier. MD Medicine

Center

comments

for
ponent, ten

a definite anyone

subjective who had

irrespective

Department Childrens
281 Locust Akron, OH

of Pediatric
Hospital St
44308

Medical

of Akron

tions
blind,

or
and

intellectual
if not,

honesty.
subsequent

Hopefully
studies of

the
this

tester
type

was
should

utilize
status.

data

collectors

unaware
WILLIAM A.

of experimental
ALTEMEIER

vs control

III, MD
Center

Department Vanderbilt Nashville, REFERENCE

1. Leib SA,

of Pediatrics University Medical TN 37232

On Angiomas
of early Pediatrics interven66:83, To the Editor.-

of Retina,

Brain,

and

Skin

tion and 1980

In Reply.-

Benfield stimulation

G, Guidubaldi J: Effects on the preterm infant.

Recently Russell et al (Pediatrics 66:306, scribed a child with a hemangioma of the right forehead and right upper eyelid, an arteriovenous
Dr
to respond

1980)

de-

side

of the
malfor-

We
and

appreciate
like

Altemeiers
to the

interest
issues he

in our
has raised.

paper, of the given

proce-

would

As control

stated group

in the study,
receiving that time. written

paper, standard
the Parents consent

during intensive
prescribed of the for

the nursery care

time care unit.

enrichment control

period was There

infants

mation of the an ipsilateral isolated growth the of vascular

basal ganglia and suprasellar angiomatous ocular lesion, hormone deficiency. They were syndrome. consistent It is our
vascular

region, and who had an concluded that with a variant that opinion

to were
gave

malformations

all infants
no dures their infants at

in the

neonatal

the

Sturge-Weber

their fundus and clinical description

sent a case

cerebral
of the

angiogram
cutaneous

illustrations
nevi

and

the

repre-

informed

their

infants

to

partic-

of Wyburn-Mason

syndnome.24

ipate

in

a
and

research
development.

project gave which

of

to
Parents

measure
of the

their
infants

infants
in the

The
aneurysms
lateral

Wyburn-Mason
of the retina, and

syndrome

includes

arteriovenous

growth
experimental
their

group
to

informed
in a special

written

consent

for
pro-

brain, racemose angiomas of the ipsiassociated cutaneous vascular nevi.

infants

participate

enrichment

gram nurses.

in the Both
use of the

nursery, groups
following Brazelton the

was
parents

to be administered
were informed measures: Behavior

by the
that administraAssessment we

The photograph of the although rotated 90 degrees the signal fmding of the
racemose angioma of the

right fundus in their article, counterclockwise, represents Wyburn-Mason syndrome, a

retina. This lesion shows an

would
tion

evaluative Neonatal

increased

number

and

size

of seemingly

fully

developed

LETTERS

TO THE

EDITOR

753

Downloaded from pediatrics.aappublications.org at Indonesia:AAP Sponsored on March 19, 2013

tortuous riovenous

and widened communications.

retinal

vessels

with

probable

ate-

Dietary
To the

Chloride
Editor.-

Deficiency

syndrome demonstrates choroid without retinovascular involvement ception of mildly dilated otherwise normal
The angiogram illustrated in their

In contrast the Sturge-Weber cavernous hemangiomas of the

with the exretinal veins.5 article shows a cere-

With regard ride Deficiency

to the article Syndrome

entitled
(Pediatrics

The

Dietary
66:366,

Chlo1980), if

probably unilateral arteriovenous aneurysm of the bral vessels, consistent with the Wyburn-Mason drome. Intracranially the Sturge-Weber syndrome
cludes occipital leptomenigeal lobe and plane angiomatosis x-ray deep nevi are shows to the seen usually covering

the infants mentioned who believe in early dietary addition other chloride to foods their and lived

had been cared for by pediatricians feeding of infants, the problem would the
be unlikely

of In

syninthe

deficiency formula would through

not infants
to

have would

occurred. be getting
such

develop

of cortical
tion of the Cutaneous

calcifications
meninges.6 vascular

a gyriform pattern vascular malformain both the Sturge-

deficiency.
Having mula, the me that feeding, the problems of diarrhea due to forin to

oversteriization
seizures formula, when solid and and

and breaking in infants due now chloride the food ensures

down of sugars in the to pynidoxine deficiency deficiency, off it still seems

Weber
like

and the to emphasize

Wyburn-Mason syndromes. We that the association of cutaneous

would vas-

cular nevi, ocular vascular abnormalities, and cerebral vascular malformations are seen in clinical syndromes other than Sturge-Weber. These syndromes include Wyburn-Mason,2 Von Hippel-Landau,7 Gass,8 and Osler-Weber-Rendu.9 and
of

child is finishing can be introduced. caloric that intake, the helps child

about 4 oz per This assures the

child
the

of adequate night,

him will

sleep through
get an adequate as a result Furthermore, of

Careful

attention findings permit The variable associated

to the

clinical

presentation differentiation therapy, and man-

diet in spite manufacturing

of any goof deficiencies

that might occur in the formula.

fundoscopic these entities. counseling

accurate prognosis, these

genetic

with
LOUIS JEFFREY

syndromes MS

date

accurate

differentiation. I.
SOBEL,

when the infant is young, it is easier to approach mom to put daddy on a decent diet too. Having fought milk anemia for 30 years, it is really disturbing for an old-timer like me to hear those youngsters mula in pediatrics insist the for six months. I think feeding of other foods.
BLACKMAN, PATRICIA

MD ALAN H. FRIEDMAN, MD Department of Ophthalmology Mount Sinai Medical Center 100th St and 5th Ave
ODEL,

G.

babies must have only here is a good argument

forfor

early

MD
and Child of Health Health

Division of Maternal Texas Department 1100 W 49th St

New REFERENCES
1. Russell JD, Wise PH, Rischbieth

York,

NY

10029

Austin,
HG: Vascular malforma-

TX

78756

In Reply.We want Blackmans of solid

tion of hypothalmus: deficiency. Pediatrics

2. Wyburn-Mason R:

A cause

of isolated
aneurysm

growth

hormone

66:306, 1980 Arteriovenous

and

retinal,

facial

naemi

and

mental

changes.

of mid-brain Brain 66:163,

1943 3. Walsh FB, Baltimore, 4. Lecuire J,

dietary
ride-deficient

first is that the early introduction foods would have prevented the occurrence of chloride deficiency in the infants ingesting chloNeo-Mull-Soy. The Table shows the age at

to respond letter. The

to two

of the

issues

raised

in Dr

Neuro-ophthalmology, ed 3. Co, 1969, pp 1693-1694 P: Bonnet-Dechaume-Blanc syndrome, in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology: The Phakomatoses. New York, American Elsevier Publishing Co, 1972, vol 14, pp 260-266 5. Susac JO, Smith JL, Scelfo RJ: The tomato catsup fundus in Sturge Weber syndrome. Arch Ophthalmol 92:69, 1974 6. De Recondo J, Haguenau M: Neuropathologic survey of the phacomatoses and allied disorders, in Vinken PJ, Bruyn GW (eds): Handbook ofClinicalNeurology: The Phakomatoses.

Hoyt WF: Clinical Williams & Wilkins Dechaume JP, Bret

which
the introduced

solid
12 infants

foods
into

were
the

introduced
in our of seven In eight

into
paper. of

the
Solid

diet
foods solid

of

1 1 of

described diets of age.

were infants foods

1 1 of the

prior TABLE.
of Solid Patient
No.

to

5 months

instances

Dietary
Foods
Soy

Chloride Deficiency to Infant Diet Age Solids

Introduced (mo)

and

Introduction

Age Neo-MullStarted (mo)

Age at Diagnosis
Metabolic (mo) 7 Alkalosis

of

New York, American Elsevier Publishing Co, 1972, vol 14, pp 63-65 7. Grossman M, Melmon KL: Von Hippel-Landau disease, in Vinken PJ, Bruyn GW (eds): Handbook ofClinical Neurol.
ogy : The Phakomatoses. New York, American Elsevier Pub-

1
2

1#{189}
#{189}

<6
1#{189}

1#{188}

lishing Co, vol 14, pp 241-259 8. Gaas JD: Cavernous hemangioma

9. oculo-cutaneous Lieboldt GP, syndrome. Am Leiber B: Cutaneous

3 of the
J Ophthalmol dysplasias

1#{189}
2 #{189}

5
2 6

5#{189}
6 6

retina, a neuro71:799, 1971

associated with

4 5

6
7 8

2
2 2
3/4

neurological disorders synopsis and differential diagnosis, in Vinken PJ, Bruyn GW (eds): Handbook ofClinical Neurology: The Phakomatoses. lishing Co, 1972, vol New 14, p 120 York, American Elsevier Pub-

4 1#{189}
4-5

5 6
8

9 10 11 12

3 3
#{189}

3#{189} 5#{189} 3 Unkno wn

6 6#{190}
8#{189}

754

PEDIATRICS

Vol.

67

No.

5 May

1981

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On Angiomas of Retina, Brain, and Skin Louis I. Sobel, Jeffrey G. Odel and Alan H. Friedman Pediatrics 1981;67;753
Updated Information & Services Permissions & Licensing including high resolution figures, can be found at: http://pediatrics.aappublications.org/content/67/5/753.2 Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://pediatrics.aappublications.org/site/misc/Permissions.xhtml Information about ordering reprints can be found online: http://pediatrics.aappublications.org/site/misc/reprints.xhtml

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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. PEDIATRICS is owned, published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright 1981 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.

Downloaded from pediatrics.aappublications.org at Indonesia:AAP Sponsored on March 19, 2013