CRANIOSTENOSIS

PRESENT ADDRESS: (J.M.F.) Neurological Institute, Columbia-Presbyterian Medical Center, 62.2 West 168th Street, New York 32, New York.

57

PEDIATRICS, July 1962

CRANIOSTENOSIS

Review

of

the

Literature

and

Report

of Thirty-four

Cases

John Mark Freeman, M.D., and Shirley Borkowf, M.B., B.Ch., D.C.H.

Department of Pediatrics, Harriet Lane Home, Johne Hopkins Hospital, Baltimore, Maryland

C

RANIOSTENOSIS is an uncommon but

certainly not a rare condition, whose

management has been troublesome to

oph-thalmologists, neurosurgeons, and

pedia-tricians for a long time.

It is the purpose of this study to offer a

long-term follow-up of children with

craniostenosis, both operated and

unoper-ated, in an effort to discover the natural

history of this condition. The study will

show that isolated stenosis of a suture does

not invariably carry with it the ominous

prognosis cited in the literature, and that-at

least in some cases the retardation is

as-sociated, rather than causally related. A

re-cent study by Hemple et al.1 has reached

essentially the same conclusions.

The authors are aware that the numbers involved in this study are too small to be

significant in themselves. However, since

craniostenosis is an uncommon condition,

and since at the present time most of the

patients with craniostenosis are operated

upon, it would be difficult if not impossible

for any one clinic to amass enough cases

to be statistically significant. It is hoped

that these 34 cases will increase the signi-ficance of the 42 cases presented by

Hemple et al., and that this will stimulate others to review their indications for

op-eration.

BACKGROUND

While premature closure of the cranial

sutures had been described by Hippocrates, the first important work on the condition was published by Virchow in 1852.2 He

found that growth of the skull was impaired

in the direction perpendicular to the suture

which had fused and that growth took

place parallel to that suture. This has since

become known as Virchow’s Law.

The first operations for this condition

were performed by Lannelogue3 in 1890

and by Lane4 in 1892. Lane described the

operation on a 9-month-old “decidedly

microcephalic imbecile” whose mother

asked, “Can you unlock my poor child’s

brain and let it grow?” The child died, but

the second microcephalic child on whom

Lane operated had “unequivocal evidence

of mental improvement.” Thus, in 1892, the

concept of “unlocking” the growth potential

of the brain is first seen. This concept has

been carried into recent times by McLaurin

and Matson,5 who feel that early operation

is necessary to release “whatever growth

potential is present.”

Ophthalmologists, who saw these

pa-tients because of the exophthalmos, optic

atrophy, and papilledema, did much of the

early work on this condition and made most

of the contributions to the literature. Hay-ing seen these patients because of the

complications of craniostenosis, they

ob-tamed a biased view of the condition. Even

operations were not entirely satisfactory

then, since the artificially created sutures

remained open for only 4 to 6 months, and

then reoperation was necessary. General

success was not achieved until 1947, when

Simmons and Peyton’ advocated the use

of tantalum foil to line the edges of the

linear craniectomy. This was followed 2

years later by the use of polyethylene by

Ingraham et al.7

It was not until Hemple’s recent paper

58 CRANIOSTENOSIS

natural history of the condition, and to

as-certain the extent of the

deformity-cos-metic, ophthalmologic, and mental-which

is present in unoperated and unselected

cases. Statements appear frequently in the

literature that the brain in this condition is

normal, and that if the children are

oper-ated upon early, before 6 months of age,

they will be intellectually normal. There

are no studies of either operated or

un-operated patients, prior to Hemple’s, with

adequate neurological and psychological

examination, and with a reasonable

fol-low-up. Apart from many scattered case

reports, the only extensive series of cases

are those reported from Boston by

In-graham et al.7 in 1948, McLaurin and

Mat-son5 in 1952, Laitinen and ‘ and

Bertelsen.1#{176}

In the first of these studies, Ingraham

et al. showed that 25% of those with sagittal stenosis, 37% of those with coronal stenosis,

and 83% with two or more sutures closed,

were retarded. This was a total of 40% of

their group of 50 cases. However, they state

that of the 20 patients operated upon

be-fore the age of one year, only one was

re-tarded.

In the paper by McLaurin and Matson,

36 patients operated upon under the age of

6 months are discussed. There were no

cases of mental or visual impairment in

their patients with sagittal stenosis, and

only two of the nine patients with coronal

stenosis were retarded. These two were felt

to be “progressing in mental development”

after operation. However, only 8 of the 36

patients were followed 2 or more years, and

many had no follow-up. Cosmetic

improve-ment is also cited as a reason for operation.

ETIOLOGY

Figure 1 shows the rapid increase in

brain weight during the first 2 years. The

brain doubles in weight by 7% months and

triples in weight by 2% years. This rapid

increase necessitates and is the impetus for

the rapid enlargement of the skull. Almost

all of the growth of the skull takes place at

the suture lines. These sutures are normally

open at birth and by 7% months are

inter-digitated but not fused. Complete fusion

does not take place until the fourth decade.

The etiology of premature closure of the

sutures is not known. The many theories

concerning the etiology have been well

de-612, 13 These theories have varied

from intrauterine meningitis, rickets,

syphi-us,

and birth trauma, to disturbances in the

growth of the sphenoid and defects in the

germ plasm. The latter mechanism13 is the

most generally accepted.

It is known that the bones of the skull

are derived from two different sources.

The bones making up the base are

carti-laginous in origin, and the growth is similar

to that of the long bones, with the cartilage

serving as an epiphysial plate. The bones

of the calvarium are membranous in origin.

According to Anderson and Woodhall14 the

ossification centers arise within the

mem-branous capsule. As the center grows, the

capsule is split into two layers, with the

periosteal layer within and the pericranial

layer without. The capsule remains fused at

the ends like an envelope and presents a

barrier to the meeting of the adjoining

ossi-fication centers. This constitutes the

mesen-chymal sutural gap. It has been shown

ex-perimentally1 that a bony bridge placed

across a suture leads to complete stenosis

of that suture. Thus, if there is a defect in

the germ plasm of the mesenchymal layers, the ossification centers may join, leading to

stenosis of the suture.

The principal ocular complications of

craniostenosis are exophthalmos,

papil-ledema, and optic atrophy, although

strab-ismus, medulated nerve fibers, congenital

cataracts, and congenital ptosis have been

reported. The exophthalmos has been

shown to be due to the shallow orbits which

are secondary to deformity of the anterior

and middle fossa. This may be due to

ste-nosis at the base of the skull and

deformi-ties of the sphenoid which would be an

in-operable condition.

I It 2 2f 3 3$ 4 43 #{182},#{182}$6 6$ 7 7$ 8 b 9 93 0 0$ ‘I 11$2‘2$ 3 3$ 4 4) AGEINYEARS

FIG. 1. Graph showing increase in the weight of the brain. (From Faber and Towne”; reprinted with permission.)

ARTICLES 59

clear. There is no doubt that optic atrophy

may be secondary to increased intracranial

pressure. Blodi16 and Mann17 have pointed

out that in most cases, the optic atrophy is

of the primary type rendering increased

pressure unlikely. Hemple states that there

was increased intracranial pressure in 13 of

his 42 cases. However, there are no

meas-urements of the spinal fluid pressure. The

diagnosis of increased pressure was based

on the presence of optic atrophy,

papil-ledema, or sutural separation. Since the

optic atrophy may be primary, and since

all his criteria are combined, it is difficult

to evaluate evidence of increased pressure.

Other theses for the optic atrophy include (1) small optic foramina which constrict the

optic nerve (Blodi), (2) angulation of the

optic nerve due to deformity of the

ante-nor and middle fossa (Mann), and (3)

de-fective arterial or venus return secondary

to the first two.

CLASSIFICATiON

Craniostenosis has been confused in the

past by the many names that have been

attached or given to changes in the shape

of the skull. These changes depend on

which sutures have closed and in what

or-der. In this publication we shall use a modi-fication of the classffication of Laitinen and

Sulama for simple craniostenosis, which is

divided according to the suture closed.

I. Simple craniostenosis

A. Scaphocephaly (boathead)-fusion of the

sagittal suture.

Case Sex and uace Age . First been Age De-. formity ivotea

Suture Symptoms #{149}

Development

.

Family . History

.

Seizures

.

Basw . Deformity TABLE DETAILS OF 34 CASES

6 da Birth

64 yr 4 mo (?) Birth Birth Birth Birth Birth Birth (?) 9mo 2 mo mo 1 yr 9da l8mo f24yr 1 3 4 5 6 7 8 9 10 11 1 13 14 15 M

w

M N 11 N F w M w M w M N M N F

w

M

w

M

w

M N M N M N M

w

Sagittal Sagittal Sagittal Sagittal Sagittal Sagittal Sagittal Sagittal Sagittal Sagittal Sagittal Sagittal Sagittal Sagittal Sagittal Deformity Deformity,convulsions, retardation Mild deformity Deformity Deformity Deformity Deformity Deformity, feeding problem, slight in-creased tone Retardation, deformity Deformity Deformity Deformity Deformity Deformity incidental Deformity; breath holding Normal

Sat, 9 mo;

walked, 12I yr;

talked, no

Sat, 6 mo;

walked, 11 mo; talked < yr

Sat, 7 mo

Sat, 7 mo; walked, 9 mo

Sat, 6mo; walked, <lyr; talked, 4 mo

Sat, 6 mo;

walked, I yr; talked, yr

Sat, 7 mo; walked, 17 mo;

talked, 18 mo

Sat, 1 yr; walked, yr

Sat, 5 mo; walked, 14 mo; talked, 4 yr

Normal; same

as twin

Walked, 3 yr; talked, 4 yr

Normal

Sat, 7 mo; walked, 13 mo; talked, Q4 mo

Sat, 7 mo; walked, 1 yr

)F CRANIOSTENOSIS

Head .

Circum-ferenee

Per-cen-

_.

tile

. Cephaiw

Index Eyes

.

Operation

Follow-up

(yr)

Mental Status (ommenis

40.6 >97 73 Normal None 104 IQ-118 Normal; no obvious deformities

50 77 Convergent None 0 MA 1-2 yr (at 64) Convulsions from 3 mo;

moder-strabismus; ate deformity

fundi normal

44 50 68

.

5 Normal None 5 Mild retardation Thick tables on x-ray; mild

de-formity

44

.

7 50 65 Normal Parasagittal, 7mo 0

38 5 68 Normal Parasagittal, 8 mo 5 Low normal Little residual deformity at 5 yr;

operative sutures open

5

.

5 > 97 66 Normal Parasagittal, 6 mo 0 “Precocious” at

6 mo

48 75 70 Normal None 10 IQ-100 Top of class; moderately severe

deformity

33

.

8 3 59 Alternating None 5 IQ-102 Marked deformity, but bright

esotropia & and appealing

myopia

45 3 71 Normal None 7 IQ-70 Difficult birth; (?)cortical atrophy

on pneumoencephalogram; 1#{176} ye-tardation;moderate scaphoceph-aly

53

.

5 >97 Normal Parasagittal, 30 mo 3 IQ-80 Severe residual deformity; “im-proved”

65 Normal Parasagittal, 13 mo 11 “A” student- Minimal deformity; inadequate

(1 1yr same as twin operation

post-op)

39

.

5 50 66 Normal None 6 Severely retarded Pierre Robin; no digital marking;

Strabismus spastic at mo; retarded;

mod-erate deformity

4’2 97 64 Normal None 3 Normal Sagittal sutures barely open at

mo; severe deformity, S yr

5

.

5 60 Normal None 0

46 .7 50 61 Normal None 10 Normal Moderate deformity; no

62

TABLE I

CRANIOSTENOSIS

v

Case Sex and

Race

Age

#{149}

First

Seen

Age

De-.

formity

Noted

Suture Symptoms Development

.

iainily

_#{149}

history

.

Seizures

.

Basic

_#{149}

Deformity

16 M 10 yr 7 mo Sagittal Speech difficulty; de- Sat, 6 mo; 0 0 Scaphocephaly

W formity walked, 13 mo

17 M 10 yr (?) Sagittal Deformity incidental Sat, 5 mo; 0 0 Scaphoceplialy

W walked, 12 too

18 M 9 yr 1 mo Sagittal Deformity Sat, 6 mo; 0 0 Scaphocephaly

W Walked, 1 yr;

talked, 2yr

19 M 11 yr Birth Sagittal Strabismus; deform- Sat, 8 mo; 0 0 Scaphocephaly

N ity incidental walked, 11 mo

F 3 mo Birth Coronal Deformity Sat, 5 mo; 0 0 Brachycephaly

W walked, 1 yr;

talked, 14 yr

M 5 mo Birth Coronal Slight retardation; de- 0 0 0 Brachycephaly,

W formity plagiocephaly

F 4 yr Birth Coronal Deformity Sat, 6 mo; 0 0 Brachycephaly

W walked, 18 mo

F 10 mo Birth Coronal Deformity Sat, 8 mo; 0 0 Oxycephaly

W walked, 12 mo

talked, 14 yr

M mo Birth Coronal, Deformity Sat, 9 mo 0 0 Oxycephaly

W sagittal,

lambdoid

M 9mo Birth Coronal, Deformity Sat, 9 mo; 0 0 Oxycephaly

W sagittal, walked, 1 yr

lambdoid talked, 14 yr

F 3 yr Birth Coronal, Retardation; deform- Sat, 6 mo; 0 0 Brachycephaly

W sagittal, ity walked, 1 yr;

lambdoid no speech

F 3.9 yr Birth Coronal, Cleft palate; mild de- Walked, 1 yr 0 0 Brachycephaly

Head

Circum-ference

Per- cen-tile

Cephalw

Index

Eyes Operation

Follow-up

(yr)

Mental Statue Comments

Normal

Normal

None

None

None

None

None

Normal

Normal 8

0

0

0

9

6

14

5 55

49

53.5

53.5

39

41

45

38.3

44.5

>97

50

97

75

75

25

<3

50

<3

<3

<3 63

69

(?)

90

89

87

74

None

Normal

Normal

Fundi normal

Normal

Protuberant; discs blurred

Exophthalmus;

right exotrope

Esophoria;

fundi normal

Normal

13

6 (Continued)

ARTICLES 63

Bilateral

internal

strabisimus

Paracoronal, 6mo

Paracoronal, 10 mo

Sagittal & coronal created; bilateral frontal decompres-sion, mo

Sagittal & coronal; frontal; orbital

ridge, 9 mo

Coronal & sagittal; bitemporaldecom-pression, S yr

None

IQ-95

IQ-100

IQ-117

IQ-88

IQ-89

IQ-Normal

11I “Bright”

IQ-67

IQ-73 at 3 yr

IQ-50 at 3 yr;

45 at 8 yr

Minimal deformity; “much im-proved”; moderate speech

im-pairment

Parietal foramina

Moderate deformity

Mild deformity

No obvious cosmetic deformity; bright and attractive

Right facial asymmetry; mild

retardation; remained

brachy-cephalic; normal

pneumoenceph-alogram

Abnormality of bones of hand; mild retardation; difficult

de-livery & perinatal period

Cleft palate; moderately severe

residual deformity

Still has moderately severe

era-nial deformity but no mental

symptoms

Several operations and revisions; right ambliopia exanopia;

mod-erately severe deformity but

marked improvement; retarda-tion; anosmia; hearing loss;

syn-dactylism

Retarded; moderately severe residual deformity; left internal

strabismus; facial asymmetry

Double cleft palate and lip; club

TABLE I

Case Sex and Race

Age

.

First

Seen

Age

De-.

formity

Noted

Suture Symptoms Development

.

Family

. History

. Seizures

.

Basic

. Deformity

28 M W

3mo Birth Coronal,

lambdoid

Deformity; aperts. (?) Retardation

in siblings

0 Brachycephaly

29 F \v

3yr 18 mo Sagittal,

coronal

Deformity Walked, 10 mo;

talked, 18 mo

0 0 Oxycephaly

30 M

N

Birth 20 mo Coronal, sagittal

Deformity Sat, 10 mo;

walked, 11 mo;

talked, 2 yr

(?) Several

uncles

0 Oxycephaly

31 M

W

19 mo 19 mo Sagittal,

coronal,

lambdoid

Retardation;

deform-ity

Sat, 1 yr;

walked, 2 yr

0 0 Scaphocephaly

32 M N

6yr Birth Sagittal, metopic

Deformity Sat, 6 mo;

walked 9 mo;

talked, 14 yr

0 0 Scaphocephaly

33 M

W

6yr (?) Sagittal,

rt. coronal

Retardation;

deform-ity

Sat, 6 mo;

walked, 14 mo;

talked, yr

Two

retarded

siblings

0 Scaphocephaly,

plagiocephaly

34 M

W

4yr (?) Sagittal, coronal,

lambdoid

Headache; vomiting Sat, 6 mo; walked, 1 year;

talked, I year

0 0 (?)

64 CRANIOSTENOSIS

C. Oxycephaly (tower head)-fusion of the coronal and another suture.

D. Plagiocephaly (asymmetric head)-fusion of a suture on one side.

II. Craniostenosis with added anomalies

A. Crouzon’s disease (dysosteosis

craniofaci-alis)-characterized by exophthalmos, small maxilla, prognathism, beaked nose,

and stenosis of any or all of the cranial

sutures.

B. Apert’s syndrome (achrocephalysyndac-tyly)-craniostenosis of any type associ-ated with syndactyly.

One problem that frequently arises is the

differentiation of craniostenosis with

sec-ondary microcephaly from microcephaly

due to microencephaly, or primary

micro-cephaly. Whereas, in primary microcephaly

the occipitofrontal circumference is always

small, in cases of craniostenosis the circum-ference is usually normal or even increased.

This is not always the case in oxycephaly,

in which much of the increase in cranial

volume is due to increase in the vertical

diameter. Another differentiating point

em-phasized by Greig12 and by Simmons and

Peyton6 is that in primary microcephaly the

sutures are usually open. In craniostenosis,

while the sutures may be open at birth,

they are very narrow and fuse within a few

months. Perhaps the most valuable sign at

birth is the head shape. This was noted to

be abnormal in 32 of 42 cases in Hemple’s

study and in 23 of the 34 cases in this study.

Thus, while there is no one criterion for the

differentiation, when all of the criteria-the

skull circumference, the skull x-ray, and the

head shape-are used, the differentiation

should not be difficult.

PRESENT STUDY

The present study was undertaken to

con-Continued)

head

.

Circum-ference

Per-

cen-.

tile

. Cephalie

Index Eyes

.

Operation

FollOw-up (yr)

Mental Status Comments

47 50 82 Normal None 10 IQ-67 Aperts; Klippel-Feil deformity;

retardation; operation scheduled

but not done; moderate

deform-ity

52

.

5 97 88

.

5 Normal Sagittal & coronal;

sutures 3 yr

6 IQ-Normal Mild residual deformity; CSF

pressure 160

42

.

5 <3 82 Normal None 7 IQ-80 Premature, 1,500 gm;

hyperbili-rubinemia ; moderately severe

deformity 45 .3 <3 71 Myopia, 10 mo;

papilledema,

30 mo

Coronal &sagittal;

19 mo

8 IQ-60, 19 mo; 65, 8 yr

Sagittal suture closed, 19 mo;

others, 24 mo; (?)2#{176}to

‘inderly-ing defect; thick tables; myopia and irritability decreased post-operatively

47 <3 73 Papilledema, 1-2 days;

proptosis

None 7 IQ-91 Vision good; moderately severe

deformity; no residual

papil-ledema

49

.

5 3 74 Normal None 6 IQ-90 Left congenital ptosis; probably

familial retardation; mild

de-formity

50

.

5 50 74 Low grade

papilledema

Sagittal & coronal;

4 yr

6 Normal “Good result”; slightly slower

than siblings; thick tables

ARTICLES 65

dition and to discover whether it is

neces-sary or desirable to operate on children

with the condition.

This study includes all patients under

the age of 13 years with craniostenosis who

were seen at the Harriet Lane Home of the

Johns Hopkins Hospital between the years

1950 and 1960. There were a total of 34

cases, and these were divided into three

groups : sagittal stenosis, 19 cases; coronal

stenosis, 4 cases; and combined stenosis, 11

cases. Follow-up was attempted either by

personal examination or by personal letter.

There was adequate follow-up in all but

seven patients. The average duration of

fol-low-up was 7 years. The results of this

study are presented in Table I.

Sagittal Stenosis

There were 19 cases of stenosis of the

sagittal suture, representing 55% of the total

cases. Of these, 17 were males and 2

fe-males. Eleven were white and eight Negro.

Deformity was noted at birth in 13 of the

15

cases where the information is available.

The family history was unremarkable in

17 cases. One patient had a cousin who was

said to be scaphocephalic but to have

fin-ished college. Another patient had a normal

fraternal twin with no deformity.

The head circumference was greater than

the fiftieth percentile in 15 cases (80%). No

abnormality was found in the eyes or fundi

of 14 patients. Nonparalytic strabismus was

present in four cases. There were no cases

of papilledema or optic atrophy.

Fourteen of the 19 patients were

unoper-ated. Nine of these had normal IQ at

fol-low-up, and four were retarded. Of these

four, two had mild retardation. One of

thes had cortical atrophy indicated by the

66 CRANIOSTENOSIS

TABLE II

SUMMARY OF CASES

Type N

sm-er

Six Race

W ‘

Defect Noted Family history Cephalic Index Eyes

Operalal

M F Age No. Finding No. Index No. Finding No.

Sagittal 19 17 11 8 Birth

<7 mo Not recorded

13

Q

4

Neg. 17 <70

70-77 Not recorded

I

4 3

Normal

Strabismug

15

4 5

Coronal 4 1 3 4 0 Birth 4 Neg. 4 >89

Not recorded

Normal

4lightstrabismus

and proptosis Papilledema

S

I 0

7 4 S

6 Combined ii 8 S 9 Birth

<

Not recorded

6 Neg. 9 >8

70-75

Not recorded 4

5

Normal Papilledema Proptosis

Exophthalmos

thick tables indicated on a skull film and

was not felt to be intellectually inferior to

his siblings. The other two patients were

severely retarded. One had spastic diplegia

diagnosed at 2 months of age. The other

had a normal cephalic index0 and no

evi-dence of increased pressure. There was no

follow-up in one case.

These findings cannot be explained by

the closure of the sagittal suture alone. It

is well known that when one anomaly is

present, there is a higher incidence of other

anomalies. There is thus evidence to

sug-gest that three of the four cases of

retarda-tion may be on the basis of underlying

brain abnormality which is associated with,

rather than the result of, the craniostenosis.

The other may well be familial.

Three of the five patients who were

op-erated on had adequate follow-up. Two

of these had normal intelligence, and one

was mildly retarded at the time of

follow-up. Of the unoperated patients seen at

fol-low-up, four had little or no cosmetic

de-formity, six had moderate deformity, and

two had marked scaphocephaly. Two of

the three operated patients seen at

follow-up had mild residual cosmetic deformity;

the other had marked scaphocephaly.

The cosmetic indications for surgery are

even more difficult to evaluate. The extent

0 Cephalic index is “skull breadth ± skull length

x 100.” Normal is 70 to 80.

of ultimate deformity in children with

sagittal stenosis is quite variable and

un-predictable. In most cases the head assumes

a more normal shape spontaneously, as

was shown by Bertelsen.1#{176} Even patients

operated upon early have variable amounts

of residual deformity. Few patients finally

have extreme scaphocephaly. Since it is

difficult to predict the extent of future

cos-metic deformity during the first 6 months

of life, when operation should give the best

results, it would not seem warranted to

op-erate on all of these patients to prevent

Se-vere deformity in a few. For these few,

cranioplasty and duraplasty can be done at

a later time if necessary, as is advocated by

Mullen.hi

The cases of scaphocephaly are

sum-marized in Tables II and IIA.

Coronal Stenosis

There were four cases in the study of

closure of only the coronal suture. All of

the patients were white, three female and

one male. All had brachycephaly. One also

had slight plagiocephaly due to earlier

cbs-ure of the right coronal suture than the left.

All of the patients had normal fundi; one

had strabismus and slight proptosis. One

patient who was operated upon at the age

of 6 months had an IQ of 88 and remained

brachycephalic. Another, who was operated

on at 10 months of age, had a normal IQ

ARTICLES 67

TABLE Il-A

SUMMARY OF NINETEEN CASES OF SAGITTAL STENOSIS

Cases Total

Number

Mental Status

Symptoms & Defects

Cosine/ic Deformity

Severity Number

Evaluation Number

Operated 5 Superior I

Low normal I

Mild 1

No follow-up 2

Mild 2

Severe I

No follow-up 2

Unoperated 14 Superior I

Normal 8

Mild 4

Moderate 6

Severe 2

No record 2

Mild retardation 2 Thick tables of skull I

Cortical atrophy I

Severe Spastic diplegia I

Convulsions, 3 mo;

normal cephalic in- I

dex

No follow-up 1

deformity. The two unoperated patients was present in four, and exophoria in 2.

had IQ’s of 117 and 89; the former had lit- One patient had amblyopia exanopsia.

As-tie cosmetic deformity at follow-up. One sociated anomalies included syndactylism

patient had deformity of the bones of the (Apert’s syndrome) in three and cleft

pal-hand. The cases are summarized in Tables ate in two. One had Klippel-Feil deformity II and IIB. and anomalies of the cervical vertebrae. The family history was negative for

cranio-Combined Stenosis stenosis in all cases, but there was a history

There were 11 cases of combined stenosis of mental retardation in two families.

involving more than one suture. Of these, Operations were performed on six of

8 were male and 3 female, 9 white and 2 these patients, two of whom were less than

Negro. The deformity was noted at birth in one year of age. Of these six, three had

nor-six cases. Only one of these patients pre- mal IQ’s, one was mildly retarded, and two

sented symptoms of increased intracranial were moderately retarded at the time of

fob-pressure, although four had papilledema. low-up. This was independent of the age

The fundi were normal in seven, proptosis of operation. Of the five unoperated cases,

TABLE Il-B

SUMMARY OF FOUR CASES OF CORONAL STENOSIS

tases Total

Number

Mental Status Cosmetic Deformity

Evaluation Number Severity Number

Operated 2 Normal 1

Slight retardation (88) 1

Moderately severe 1

Mild I

Unoperated 2 Superior (117) 1

Slight retardation (89) 1

Minimal I

68 CRANIOSTENOSIS

TABLE TI-C

SUMMARY OF ELEVEN CASES OF COMBINED STENOSIS

I

‘

ases Total

urn er

ilental Status

--

----Evaluation Number

Symnptommms 4. !)efe(1.s

(‘osmelie Deformity

---Severity Number

Operated 6 Normal 3

Mild (80) 1

-

-Moderate (65 &67) ‘2

---

----Mild

Moderate ‘2

Severe ‘2

‘Fhick tables of skull I

Unoperated 5 Normal I

Mild (80) ‘2

Moderate (67) 1

Severe (50) 1

. .

..

(?)Familial retardation ‘2

Apert’s I

Multiple anomalies 1

Mild 1

Moderate 4

one patient had a normal intelligence, two

were mildly retarded, one was moderately

retarded, and one was severely retarded.

These latter two were not operated upon

because of the retardation at the time they

presented.

Tables II and Il-C summarize these cases.

COMMENT

While premature closure of a suture will

lead to deformity of the skull, one cannot

assume from this that growth of the brain

will necessarily be restricted. No

measure-ments of skull volume have been done in

either normal or abnormal skulls at

dif-ferent ages. It is mathematically impossible

to estimate volume from roentgenograms of

the skull. Thus, unless at some future time

volumetric measurements should prove

otherwise, there is no proof that growth of

the brain is restricted by closure of a

su-ture.

There is, however, evidence to suggest

that in most cases, growth of the brain is

not restricted. If the brain were to grow

more rapidly than the skull, there would be

increased intracranial pressure, and this

should be manifest as papilledema.

Papil-ledema is a rare finding in sagittal stenosis,

uncommon in isolated coronal stenosis, but,

as would be expected, is common when all

of the sutures are closed.

In sagittal stenosis, where growth of the

skull is primarily in the anteroposterior

diameter, the circumference of the skull

offers an appoximation of the change in

volume. Here, the circumference is usually

normal or greater than normal, thus

mdi-cating that the anteroposterior growth has

compensated for lack of lateral growth. In

other forms of craniostenosis, where head

shape is more variable, there is no

measure-ment that can give a comparable

approxi-mation.

Thus, the evidence would suggest that in

isolated stenosis of a suture, as opposed to

combined stenosis, growth of the brain is

not necessarily impaired.

To ascertain the prognosis of

craniosteno-sis, one therefore must not look at this as a

single disease entity but must separately

evaluate closure of individual sutures. The

necessity for this is seen when one analyzes

the three groups in this study.

In considering sagittal stenosis, we have

four retarded patients in 14 unoperated

cases. In each of these there is reason to

suspect either familial retardation or an

associated underlying brain defect not

caused by the craniostenosis. There were no

ophthaimological findings in this group that

would necessitate operation. From the

cos-metic viewpoint, there was residual

de-formity in both the operated and

unoper-ated groups.

coro-ARTICLES

TABLE III

COMBINED RESULTS OF HARRIET LANE CASES AND HEMPLE’S CASES

69

Suture No.

Degres of Retardation’ Papilkdemne tjzophihi:lmo

Normal Mild ifoderale S’erere Unknown

Sagittal HLII eases---unoperatetl Ilemple’s cases----unoperated 14 10 0 0 0 0 9 9 0 0 0 0 1 1

Total 4 0 0 18,’;81% /; 9% 0 /; 9% /y4; 8%

IILII cases--operated Ilemple’s cases-operated 5 S 0 0 0 I 8 1 0 0 0 0 0 0

Total IS 0 1 10/I1;91% I/I1;9% 0 0 /IS;l5%

Coronal

liLt! cases-unoperated

liemples cases-unoperated 7

0 S 0 3 1 1 0 0 0 0 0 0 5 Total 9 S S -;j;:,-;-;--- 1/4;’tS% 0 0 5/9; 55% IILII cases-operated

Hemple’, cases-operated 8

0 0 4 1 7 1 0 0 0 0 0 0 1

Total 10 4 8/9; 88% 1/9; 11% 0 0 1/10; 10%

Combined lILIl eases-unoperated Ilemple’s cases-unoperated 5 S 1 S 0 S 1 1 0 I 0 1 0 0

Total 8 3 3 Q/6; 33% /6; 33% 1/6; 17% 1/6; 17% /8; 5%

IlL!! cases-operated Hemples cases-operated 6 6 3 t 4 3 5 1 1 0 0 0 0 0

Total It S 6 8/1; 66% t/I; 17% /1t; 17% 0 0

S Normal =IQ >90; mild =IQ 70-89; moderate =IQ 50-69; severe =IQ <50.

nal stenosis, there was no detectable

differ-ence in results from the mental and

cos-metic viewpoint between the two operated

and the two unoperated cases.

In combined stenosis, the prognosis is

not as favorable either with or without

op-eration. Some of these patients developed

papilledema and signs of increased

intra-cranial pressure. In these, operation was

certainly necessary.

Although the number of cases of

com-bined stenosis is small, there is no evidence from this series that a prophylatic operation

would achieve either better cosmetic or

mental results than one done at the time of

symptoms. Two of the children operated

on at 3 and 4 years of age have normal

IQ’s, whereas two operated on at 9 and 19

months of age are severely retarded. It is

important that unoperated patients be

fob-bowed closely for signs of increased

intra-cranial pressure.

Table III presents the combined results

of this study and Hemple’s series. It can be

seen that there is little difference between

the operated and the unoperated groups

except in the combined stenosis group, and

here two patients were unoperated because

of retardation.

While there are only three reported

op-erative fatalities in this 1

Ingra-ham and Matson report one case of

post-operative wound infection necessitating

re-operation, and we have a similar case.

Hem-ple had one case which had a cardiac arrest

during induction of anesthesia. One of our

patients had status epilepticus

70 CRANIOSTENOSIS

now shows evidence of cerebral damage.

It would seem that while the risk of

opera-tion is small, the gains from operation are

not sufficient to advocate early operation

solely because of the presence of a closed

suture.

SUMMARY

Thirty-four cases of craniostenosis are

presented. Of the 19 cases of sagittal

ste-nosis, 14 were unoperated. Four of these

patients were retarded. This retardation

was not believed to be caused by the

crani-ostenosis. Of the five operated patients,

three were seen at follow-up, and one was

retarded. There was no difference in

cos-metic results between the operated and

un-operated cases. In the four cases of coronal

stenosis there was no detectable difference

in mental or cosmetic results between the

operated and unoperated cases. In the

com-bined stenosis group of 11 cases, four

per-sons developed papilledema. There was no

difference in mental or cosmetic results

be-tween those operated on before one year of

age and those operated on when symptoms

became evident. There is evidence to

sug-gest that in sagittal and coronal stenosis

growth of the brain is not restricted by

closure of the suture. It would thus seem

that there is little need for prophylactic

op-erations but that instead operations should

be performed only for papilledema or very

severe deformity.

REFERENCES

1. Hemple, D. J., et al. : Craniostenosis involving the sagittal suture only. J. Pediat., 58:342, 1961.

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sch#{228}del-formen. Verh. Phys. Med. Gesellsch. W#{252}rzb., 2:230, 1852.

3. Lannelogue: De la craniectomie dans Ia mi-crocephalie. Compt. Rend. Acad. Sci., 110: 1382, 1890.

4. Lane, L. C.: Pioneer craniectomy for relief of mental imbecility due to premature su-tural closure and microcephalus. J.A.M.A.,

18:49, 1892.

5. McLaurin, R. L., and Matson, D. D. : Im-portance of early surgical treatment of craniostenosis : review of 36 cases treated during the first six months of life.

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11. Faber, H. K., and Towne, E. B.: Early opera-tion in premature cranial synostosis for the prevention of blindness and other sequelae: 5 case reports with follow-up. J. Pediat., 22:286, 1943.

12. Greig, D. M. : Oxycephaly. Edinburgh Med. J., 33:189, 280, 357, 1926.

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Amer. J. Dis. Child., 20:235, 1920.

14. Anderson, B., and Woodhall, B. : Visual loss in

primary skull deformities. Trans. Amer. Acad. Ophth. Otol., 57:497, 1953.

15. Giblin, N., and Alley, A. : Studies in skull growth: coronal fixation. Anat. Rec., 88: 143, 1944.

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Acknowledgment

We should like to express our thanks to Dr.

1962;30;57

Pediatrics

John Mark Freeman and Shirley Borkowf

CRANIOSTENOSIS: Review of the Literature and Report of Thirty-four Cases

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John Mark Freeman and Shirley Borkowf

CRANIOSTENOSIS: Review of the Literature and Report of Thirty-four Cases

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