Cranial Deformity and Optic-Nerve 
Atrophy. 
BY 
HARRY FRIEDENWALD, A.8., M.D., 
THE AMERICAN JOURNAL OF THE MEDICAL SCIENCES, 
FROM 
May, 1893. Extracted from The American Journal of the Medical Sciences, May, 1893. 
CRANIAL DEFORMITY AND OPTIC-NERYE ATROPHY. 
By Harry Friedenwald, A.8., M.D., 
OF BALTIMORE^IH. 
There are three classes of congenitally abnormal crania ; 1, The 
enlarged skulls of hydrocephalus; 2, the very small skulls or micro- 
cephali; and 3, the variously deformed skulls. 
The last named, to which I wish to draw attention in this paper, are 
produced by the too early union or synostosis of the bones that make up 
the cranium, and the peculiar deformity depends upon which sutures 
are prematurely closed. If it be the frontal suture, then the deformity 
will consist in a very narrow forehead and very wide, head behind, and 
is known as trigonocephalus. When the synostosis is along the sagittal 
suture it gives rise to the long scaphocephalus or boat-shaped head, with 
the depression across the middle. 
The general rule is that if there is any interference with the early 
growth or extension of the cranial cavity in one part there will be com- 
pensatory development in other parts; the deficiency of one portion 
together with the excessive development of the others constitute the 
peculiar deformities under consideration. It would be impossible for 
me to name all the deformities that have been described. I wish to men- 
tion but one in addition to those named above, the Thurmschaedel or 
steeple-shaped skull, known as oxycephalus, which is due to premature 
“ synostosis of the parietal with the occipital and temporal bones, with 
compensatory development in the region of the bregma.” 
These many varieties of cranial deformity form a subject of great 
interest to the neurologist and alienist on account of their relation to 
epilepsy, idiocy, insanity, and crime. While it is of less importance, it 
is certainly not of less interest to the physician to study their relation 
to optic-nerve disease and to blindness. 
To illustrate this I shall report the following case: 
The patient is an intelligent salesman, aged twenty-six years. There 
was no blood relationship between his parents, who were both healthy, as 
are likewise his brothers and sisters. There is no mental trouble in the 
family. His head, deformed since birth, presents a typical example of 
steeple-shaped skull, as is seen in the sketch of his profile and by the 
appended craniometrical figures.1 At school he found that his sight was 
1 The cranial measurements were made according to Peterson, New York Medical 
Record. 2 
FRIEDENWALD: SKULL DEFORMITY. 
defective, the left eye being the worse. Since his school days his sight 
has not changed appreciably. He suffered from severe headaches until 
his sixteenth or seventeenth year, but since then they have been rare 
and mild. The eyeballs are bulged slightly forward. 
Fig. 1. 
1. Circumference (50.5 cm., from which deduct 6 per cent, for skin and hair, making) 
47.5 cm. (Average in men, 52 cm.) 
'2. Naso-occipital arc (34.5 minus 6 per cent. leaving) 32.4 cm. (Average 32 cm.) 
3. Naso-bregmatic arc, 15 cm. (Average 12.5 cm.) 
4. Bregmato-lambdoid arc, 11 cm. (Average 12.5 cm.) 
5. Binaurieular arc (35.5 cm., from which deduct 6 per cent.) 34.2 cm. (Average 32 
cm.) 
6. Antero-posterior diameter, 16.4 cm. (Average 17.7 cm.) 
7. Greatest transverse diameter, 14.7 cm. (Average 14.6 cm.) 
8. Binaurieular diameter, 11.9 cm. (Average 12.4 cm.) 
9. Distance of bregma from point in median plane midway between meatus, 13 cm. 
(Average 12.4 cm.) 
10. Facial length, 13.3 cm. (Average 12.37 cm.) 
11. Empirical greatest height, 14.5 cm. (Average in men, 13.3 cm.) 
The peculiar deformity is prominently shown when measurements 1 and 11 are 
compared. 
The ophthalmoscopic examination made November 21, 1891, revealed 
atrophic papillse which were grayish-white and not transparent, so that 
the lamina cribrosa was not visible; the papillary edges were slightly 
blurred and irregular. The arteries and veins presented no abnormality. 
(They were not tortuous.) It was evident that this is a case of post- 
neuritic atrophy. 
The vision of the right eye is 8/xxin, 8/xn partly, that of the left eye 
8/xvm. The vision cannot be improved by glasses. The fields of vision 
of both eyes are very much contracted on the nasal sides. (See Fig. 2.) 
The history as well as the cranial and ophthalmoscopic appearances 
of hitherto recorded cases (see table) are in the main like the one just Fig. 2, 
COD 
White 
Blue 
ffed 
Green 
COS 4 
FRIEDENWALD: SKULL DEFORMITY. 
presented, as will be seen by referring to the list classified below, which 
are all that I can find reported since Y. Graefe published the first in 
1866. Of the seventeen cases (including the one above), nine are 
properly classed among the steeple-shaped skulls (Nos. 2, 7, 8, 9, 11, 12, 
13, 14, 15, 17), and three are long heads (Nos. 1, 3, 10). In almost all 
there was post-neuritic atrophy of the optic nerve, or the papillitis itself 
was still visible. The visual power varies from almost full vision to 
total amaurosis; the field of vision wherever mentioned is found to be 
much contracted. The blindness came on unnoticed in early childhood 
in most cases, or was perhaps congenital (?). In case No. 3 of the table, 
the child when examined first was five years old and the neuritis was 
still active. I think it is very probable that in most cases it dates from 
the first few years of life. In none did it come on late in life. The 
intelligence of most of the patients is noted as being normal, though 
two suffered with epileptic convulsions. It is curious that fifteen of the 
seventeen cases are males. 
Three cases of post-mortem examination are on record (Nos. 2, 15, 
16), and in these narrowing of the optic foramen, with general hyper- 
ostosis or thickening of the bones, is noted. Still, most agree with 
Michel, who published one of these three cases, that as constriction of 
the optic foramen is rare, the immediate cause of the optic-nerve affec- 
tion in most cases is to be sought in meningeal changes and in hydro- 
cephalic conditions of the brain. At the same time stenosis of the optic 
foramen must be looked upon as a very important factor. 
Virchow, who examined a number of Hirschberg’s cases, believes that 
the cranial deformity and also the blindness are often to be referred 
to the same cause, namely, an inflammatory affection of the dura mater 
and the bone. Hirschberg concurs in this view. 
I should ascribe case No. 1, in which total blindness came on very 
rapidly and soon disappeared, to hydrocephalus. 
The question, I think, admits of still another explanation, or at least 
of another factor in the causation of the optic neuritis. The cranial 
deformity is due, as was pointed out above, to an enlargement of one 
part of the skull to compensate for the restricted development of another. 
Is it not probable that this compensatory enlargement offers more resist- 
ance to brain growth than does the normal enlargement, and that, 
during the early years of life, when brain growth is most active, there 
are periods when this resistance means such increase in intra-cranial 
pressure as would be produced by the growth of intra-cranial tumors ? 
In other words, is it not possible that in many cases the optic-nerve 
affection is due to temporary increase in intra-cranial pressure? Certain 
it is that the optic-nerve affection, the optic neuritis which is produced, 
is such as would be present if this were the cause. FRIEDENWALD: SKULL DEFORMITY 
5 
Tabulated Cases of Cranial Deformity and Optic-Nerve 
Atrophy. 
Reported by 
Sex. 
Age. 
Deformity. 
Vision. 
Ophthalmo- 
scopic 
appearance. 
Autopsy. 
Remarks. 
1. v. Graefe, 
Arch. f. Oph., 
Bd. xii. No. 
2, p. 133. 
M. 
8 
Bong, narrow 
skull. 
Complete blind- 
ness, which 
came on sud- 
denly. 
Papillo-re- 
tinitis. 
Pupils wide- 
ly dilated ; 
patient 
cured; mo- 
ther epilep- 
tic, father 
drunkard 
and suicide ; 
other chil- 
dren died 
young. 
2. Michel, 
Archiv. d. 
Heilk., xiv. 
p. 39 ; also, 
Handb. d. 
Kinderkr. 
M. 
15 
Steeple-shaped 
skull. 
Neuritis 
followed 
by atrophy 
Hyperostosis 
of skull; 
foramina 
optica nar- 
row; pachy- 
meningeal 
thickening 
over cortex. 
Probably 
hereditary 
syphilis. 
3. Hirschberg, 
Oentralbl. f. 
Augenbeilk, 
Jan., 1883. 
M. 
5-14 
Scaphocepha- 
lus(Yirchow). 
R. = — L. = 0 
cc 
Field of vision 
concentrically 
narrowed to 
about 20° all 
around ; blind- 
ness came on 
gradually and 
unnoticed. 
L. divergent. 
When first 
examined, 
optic neu- 
ritis, later 
atrophy. 
Had convul- 
sions ; eyes 
prominent. 
4. Hirschberg, 
loc. cit. 
F. 
3 
Great bulging 
of temporal 
region espe- 
cially on the 
right side 
(Virchow). 
Slight visual 
power ; reduced 
gradually. 
Neuritic 
atrophy. 
5. Hirschberg, 
loc. cit. 
M. 
15 
Synostosis of 
sagittal suture 
with very 
broad fore- 
head (Yir- 
chow). 
Vision defective 
since early 
youth. R. = — 
CO 
L. almost blind 
and divergent. 
Right field re- 
duced about 10 
or 20°. 
Neuritic 
atrophy. 
Had convul- 
sions ; intel- 
ligence fair. 
6. Hirschberg, 
loc. cit. 
M. 
4% 
Leptooephalus 
(Virchow). 
Gradual blind- 
ness during first 
year; after that 
time complete. 
Neuritic 
atrophy. 
Peculiar cra- 
nial deform- 
ity noticed 
when born. 
7. Hirschberg, 
loc. cit. 
M. 
20 
Skull narrow 
and high. 
15 
R. = ; field 
XX 
of vision nor- 
mal. L. counts 
fingers at 5' (ex- 
centric). ' 
L. divergent. 
Neuritic 
atrophy. 
Deformity 
noticed 
when born; 
intelligence 
good. 
8. Hirschberg, 
loc. cit. 
M. 
7 
Steeple-shaped 
skull. 
15 
R- = — ; field 
XX 
of vision fair. 
L. =0. 
Papilla) 
atrophic 
(light 
greenish- 
white color. 6 
Reported by 
Sex. 1 
Age. 
Deformity. 
Vision. 
Ophthalmo- 
scopic 
appearance, j 
Autopsy. 
Remarks, 
9. Hirschberg, 
loc. cit. 
M. 
9% 1 
1 
Steeple-shaped 
skull. 
15 1 
R. = — ; field 
XX 
of vision very 
narrow, about 
20° in every 
direction. 
L. = 0 diver- 
gent. 
Atrophy. 
Maternal 
grandfather 
had same 
cranial de- 
formity ; 
intelligence 
good. 
10. Yossius, 
Klin. Monat. 
Zehender, 
1884, p. 172. 
M. 
Long skull, 
temples very 
■wide, glabella 
broad, fore- 
head low, and 
rises very 
slantingly. 
R.=0. L. counts 
fingers at 8'. 
Divergence. 
Atrophy, 
blurred 
papillary 
edges (prob- 
ably neu- 
ritic, H.F.) 
Intelligence 
normal. 
11. Stood, 
Zehender’s 
klin. Monat., 
1884. p. 241. 
M. 
33 
Occipital por- 
tion undevel- 
oped ; steeple- 
shaped ? 
(H. F.) 
Frequent 
convulsions 
(epileptic ?) 
intelligence 
normal. 
hood. 
R.= i L.=— 
O 00 
Divergence; 
nystagmus. 
12 Manz, 
Bericht der 
Oph. Gesells , 
Heidelberg, 
1887. 
M. 
50-60 
Steeple-shaped 
skull. 
Had some vision 
as child, but 
gradually lost it 
almost entirely. 
Post-neu- 
ritic atro- 
phy. 
13 Manz, 
loc. cit. 
M. 
40 
Steeple-shaped 
skull. 
Had slight 
amount of vis- 
ion, but lost it 
almost entirely 
after variola. 
R.=0. L. counts 
fingers. 
Post-neu- 
ritic atro- 
phy. 
14. Manz, 
loc. cit. 
M. 
young 
man 
Steeple-shaped 
skull. 
Blind since early 
childhood ; 
slight percep- 
tion of light in 
left eye. 
Post-neu- 
ritic atro- 
phy. 
15. Manz, 
loc. cit. 
M. 
40 
Asymmetrical 
steeple-shaped 
skull. 
R. =0. L. about 
one-half exam- 
ined, due to 
papillitis. Vis- 
ion became nor- 
mal, leaving a 
permanent de- 
fect in the field 
of vision. 
R. old atro- 
phy. 
L. fresh 
papillitis 
(due to in- 
tra-cranial 
tumor). 
Cause of 
death ; os- 
teosarcoma 
on inner sur- 
face of right 
parietal and 
temporal 
bones. Base 
of skull very 
irregular. 
Foramina 
large and 
edges sharp, 
but optic 
foramina 
narrow. 
Died at age 
of 40. The 
deformity 
of the skull 
was old, 
the tumor 
recent. 
Stenosis of 
original not 
accessible, 
quoted by 
Manz. 
skull. 
optic fora- 
mina. 
accessible. 
17. H. Fried- 
enwald. 
M. 
28 
Steeple- shaped 
K. = —-— 
XVIII 
8 
XVIII 
Post-neu- 
ritic atro- 
phy. 
Intelligence 
very good. 
FRIEDENWALD; SKULL DEFORMITY FRIEDENWALD: SKULL DEFORMITY. 
I make this suggestion not simply for its theoretical interest, but 
because it may give us the solution of the treatment of this otherwise 
incurable affection. The last few years have shown that brain and skull 
surgery has great possibilities. In epilepsy and idiocy from impeded 
cranial growth we have reason to hope for many cures, if the cranial 
vault is trephined and weakened, and thus allowed to expand. I believe 
that optic neuritis in children when due to cranial deformity should be 
put down similarly as a possible indication for trephining. Case No. 3 
in the table might thus come under this head. If in such a patient we 
would be able to find any other signs of increased intra cranial pressure, 
I do not think that the operation would be out of place. 
In microcephaly blindness is rare, as we would expect a priori, at least 
if we accept the view of Broca, that commonly this condition is primarily 
due to impaired development of the brain, and not to interference offered 
to its growth by the cranial bones. Still there may be cases where this 
last explanation holds, and in some of these we may expect optic 
neuritis. 
The consideration of the relation of hydrocephalus to blindness is 
beyond the province of this paper. 
Bibliography. 
V. Oraefe: Arch. f. Ophth., xii., No. 2, p. 133. 
Michel: Arch. d. Heilkunde, xiy., p. 89. 
Hirschberg: Centralbl. f. Augenheilk., 1883 (which includes cases published previously by 
himself and Schuller). 
Leber: Graefe and Saemisch, v., p. 805. 
Michel: Handb. d. Klnderkrankh.—Gerhardt, Bd. v. Abth. ii., p. 515. 
Mans: Berichte d. Ophthal. Gesellsch., Heidelberg, 1887, p. 18. 
Gowers: Manual of Ophthalmoscopy. Third Amer. ed., p. 180. 
Stood: Klin. Monats. f. Augenheik., 1884, p. 241. 
Vossius: Klin. Monats. f. Augenheik., 1884, p. 172. 
Ponfick: Bresl. arztl. Zeitschr., No. 21, 1886. 
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