I' - f

of the

examination should be
postoperatively, barring the finding
of an unsuspected myelomeningocele with
adherent neural elements that have been
during dissection. Such a compli
cation should be rare if magnified vision
and nerve stimulation are used, The head
circumference is followed closely to detect
hydrocephalus, which will occur in 10 to 20
per cent of patients. The risk of hydroceph
alus is less with lower-level
meningo
celes.?" The spine develops
normally
without scoliosis or kyphosis. assuming the
vertebral bodies are normal. The incidence
of late deterioration of neurological func
tion is lower than in myelomeningocele,
particularly if care is taken to relieve teth
ering at the original operation.
Genetic
counseling should be arranged
for the
parents.
nor'11181

THE ARNOLD-CHIARI
MALFORMATION
Historical Background
and Definition
The term "Amold-Chiari
malformation' has
been reinforced by 70 years of use, al
though it does ignore the contribution
of
Cleland in 1883 and exaggerates the contri
bution of Arnold in 1894.25,118 Cleland de
scribed an elongation of the vermis of the
TABLE 35-8

and dilation of the

spinal cord. In 1891 scribed
four types of
hydrocephalus
associated
with
fida.lOl.114,llS.76I3n type 1, the
sils of the inferior lobe of each
hemisphere extend into the upper
spinal canal with no involvement
brain stem, In type II. a hypoplastk ]
vermis plus tonsils of the
pocket-shaped elongation of the
tricle and choroid plexus. and the
oblongata are displaced into the upper
vical canal. A dorsal protuberance is
ent at the cervicomedullary
junction ,
cervical spinal cord is shortened. with
I'OOtsclose together. and syringo
lia may be found in the distal spinal cont
Cleland's 1883 description corresponds to
the Chiari type II deformity, The
definitions of types I and II are listed'
Table 35-8, In type III, the entire cerebel
lum is displaced into the cervical
with the fourth ventricle emptying into a
The c
cervical hydroencephalocele.
for type III have been modified in
times to include extension of the fourth
'I,',,'11t1';;: le
into
an occ ipital
encephala
cele .1{:r ,:171 Type IV involves hypoplasia of
rhe cerebellum and is not now considered a
form of dvsraphisrn.
In 1894, Arnold had
described multiple anomalies in a newborn
infant with spina bifida, but devoted most
of the description
to details of the visceral
and lung abnormalities.
The description
and illustration of the cerebellar and hind-

PATHOLOGY OF TYPE I AND TYPE II ARNOLD-CHIARI

Age group
Caudal displacement of cerebellar tonsils
Caudal displacement of inferior vermis and
fourth ventricle
Caudal displacement of medulla oblongata
Dorsal kink of cervicomedullary junction
Course of upper cervical nerve roots
bifida aperta

TYPE II

TYPE I

FEATURE

MALFORMATlON

Adult
Yes
No

Infant
Yes
Yes

No No
Normal
Usually absent
May be present; frequently
nonprogressive
May develop late

Yes
Yes
Usually cephalad
..Always" present
" Always" present:
frequently
May develop early

, P. W., and Markesbery, W. R.: Early descriptions

J. Neurosurg., 37:543-547, 1972.

of the Arnold-Ch"

If
t'on' "I!Iioj&
ian ma arma'
'"'"