Stridor, Myelomeningocele, and Hydrocephalus in a Child

Abstract

IT WAS Heatley1 in 1939 who first noted the occurrence of laryngeal stridor in a child with myelomeningocele and hydrocephalus. In the years that followed, other reports appeared in otolaryngological2-6 and pediatric7,8 literature of the rare association of disturbed laryngeal function, spinal dysraphic state, malformations of the hind brain, and hydrocephalus. Recently in the neurosurgery clinic of University College Hospital, Ibadan, we encountered a Nigerian child who presented first with lumbar myelomeningocele and later developed hydrocephalus and severe laryngeal stridor, in whom a functioning ventriculo-peritoneal shunt for the rapidly progressing hydrocephalus dramatically relieved the laryngeal stridor which threatened his life. A review of the literature showed that the neurosurgical implications of this syndrome have only re
Fig 1.—Cisternal pneumoencephalography (inverted head position) demonstrating hydrocephalus and elongated, deformed fourth ventricle (arrow). Note presence of Till-Dahl-Wade valve used in shunt operation. ceived passing mention until Kirsch et al9

Full text

271
Stridor,
Myelomeningocele,
and
Hydrocephalus
in
a
Child
Adelola
Adeloye,
MB,
BS,
MRCP,
FRCS;
Sant
P.
Singh,
MB,
BS,
FRCS(Ed);
and
E.
Latunde
Odeku,
MD,
Ibadan,
Nigeria
IT
WAS
Heatley1
in
1939
who
first
noted
the
occurrence
of
laryngeal
stridor
in
a
child
with
myelomeningocele
and
hydrocephalus.
In
the
years
that
followed,
other
reports
appeared
in
otolaryngological2-6
and
pediat-
ric7,8
literature
of
the
rare
association
of
disturbed
laryngeal
function,
spinal
dys-
raphic
state,
malformations
of
the
hind
brain,
and
hydrocephalus.
Recently
in
the
neurosurgery
clinic
of
University
College
Hospital,
Ibadan,
we
encountered
a
Nigeri-
an
child
who
presented
first
with
lumbar
myelomeningocele
and
later
developed
hy-
drocephalus
and
severe
laryngeal
stridor,
in
whom
a
functioning
ventriculo-peritoneal
shunt
for
the
rapidly
progressing
hydroceph-
alus
dramatically
relieved
the
laryngeal
stri-
dor
which
threatened
his
life.
A
review
of
the
literature
showed
that
the
neurosurgical
implications
of
this
syndrome
have
only
re-
Accepted
for
publication
April
17,
1970.
From
the
Neurosurgery
Unit,
Department
of
Sur-
gery
(Drs.
Adeloye
and
Odeku),
and
the
Subdepart-
ment
of
Ear,
Nose,
and
Throat
(Dr.
Singh),
Univer-
sity
of
lbadan,
lbadan,
Nigeria.
Reprint
requests
to
University
College
Hospital,
Department
of
Neurosurgery,
Ibadan,
Nigeria,
West
Africa
(Dr.
Adeloye).
Fig
1.-Cisternal
pneumoencephalography
(inverted
head
position)
demonstrating
hydrocephalus
and
elongated,
deformned
fourth
ventricle
(arrow).
Note
presence
of
Till-Dahl-Wade
valve
used
in
shunt
opera-
tion.
ceived
passing
mention
until
Kirsch
et
a19
in
1968
reported
five
cases
with
detailed
ac-
count
of
their
neurosurgical
management.
In
view
of
the
scanty
literature
on
the
neuro-
surgical
aspects
of
this
syndrome,
we
feel
our
present
case
will
be
of
added
interest.
Report
of
a
Case
A
2-day-old
male
infant,
the
product
of
a
full-
term,
uneventful
pregnancy
and
spontaneous
vertex
delivery,
was
first
seen
at
the
University
College
Hospital,
lbadan,
on
May
24,
1969,
having
been
referred
from
another
hospital
because
he
was
born
with
a
mass
in
his
lumbar
spine.
On
physical
examination,
he
was
of
adequate
nutrition
and
weighed
2.7
kg
(6
lb).
Both
the
anterior
and
posterior
fontanelles
were
opened,
flat,
and
soft.
His
head
and
chest
circumfer-
ences
(at
nipple
level)
were
34
cm
and
33
cm,
respectively.
He
had
an
infected
lumbar
myelo-
meningocele
which
measured
6
cm
x
5
cm
and
bilateral
equinus
deformity
of
the
legs.
There
were
no
other
abnormal
findings.
His
hematocrit
reading
was
32%,
his
geno-
type
was
AA,
and
the
white
blood
cell
count
was
6,050/cu
mm.
Skull
radiographs
showed
the
luckenschuidel
(lacunar)
appearance.
The
ulcerating
myelomeningocele
sac
was
treated
with
antiseptic
(Eusol)
dressings
and
the
child
placed
on
a
regimen
of
systematically
administered
antibiotics.
The
mother
defaulted
from
the
clinic
with
her
child;
when
they
were
both
seen
six
weeks
afterwards,
the
ulcerating
summit
of
the
sac
had
healed
completely,
but
Arch
Neurol-Vol
23,
Sept
1970
Downloaded From: http://archneur.jamanetwork.com/ by a Georgetown University Medical Center User on 05/22/2015
Adelola Adeloye, MB, BS, MRCP, FRCS; Sant P. Singh, MB, BS, FRCS(Ed); E. Latunde Odeku, MD
Arch Neurol. 1970;23(3):271-273. doi:10.1001/archneur.1970.00480270081011

STRIDOR-ADELOYE
ET
AL
Fig
2.-Myodil
ventriculography
showing
elongated,
narrowed
fourth
ventricle
(arrows).
Left,
Lateral
view.
Right,
Anteroposterior
view.
the
child
then
presented
with
hydrocephalus
with
moderately
tense
anterior
fontanelle
and
a
head
circumference
of
42
cm.
Three
weeks
later,
on
Aug
25,
1969,
when
the
child
was
3
months
old,
the
mother
complained
that
the
child
had
suffered
for
two
weeks
with
an
inspiratory
grunt
which
was
mild
at
first,
but
which
gradually
worsened
over
the
days.
On
examination
at
this
time,
the
child
weighed
4.6
kg
(10
lb
3
oz)
and
was
more
hydrocepha-
lic,
with
a
head
circumference
of
46
cm,
a
tense
lumbar
myelomeningocele
with
soundly
epithe-
lialized
sac,
and
marked
inspiratory
stridor.
The
stridor
was
intermittent,
being
absent
when
the
child
breathed
quietly
at
rest
or
when
asleep,
and
aggravated
by
handling
or
feeding
the
baby.
The
chest
was
clinically
and
radiolog-
ically
clear.
On
direct
laryngoscopy,
he
had
bilateral
ab-
ductor
paralysis
of
the
vocal
cords,
with
the
latter
remaining
in
the
midline
during
inspira-
tion.
The
cords
were
otherwise
normal;
there
was
no
paralysis
of
the
palate,
pharynx,
of
the
tongue.
He
was
admitted
into
the
hospital
on
Sept
9,
1969.
Air
ventriculography
showed
marked
hy-
drocephalus.
His
laryngeal
stridor
worsened
the
day
after
the
air
studies,
and
he
obtained
only
little
relief
following
ventricular
tapping
through
the
right
lateral
angle
of
his
anterior
fontanelle.
A
ventriculo-peritoneal
shunt
opera-
tion,
using
a
Till-Dahl-Wade
valve,
was
per-
formed
on
Sept
11,
1969.
The
laryngeal
stridor
was
remarkably
re-
lieved
immediately
after
the
operation.
The
anterior
fontanelle,
and
later
his
myelomenin-
gocele
sac,
became
flat
and
soft.
Direct
laryn-
goscopy
on
the
fourth
postoperative
day
showed
that
the
right
cord
was
mobile
but
not
the
left,
and
the
patient
had
a
slight
stridor
after
this
procedure.
A
week
later,
both
cords
were
demonstrated
to
be
moving
well
on
direct
laryngoscopy,
and
there
was no
postlaryngos-
copy
stridor.
His
myelomeningocele
was
repaired
on
Sept
25,
1969,
at
the
age
of
4
months,
and
he
was
discharged
from
the
hospital
two
weeks
later.
He
has
been
regularly
followed
up
in
the outpa-
tient
since
then
and
has
remained
well.
Cister-
nal
pneumoencephalography
performed
two
months
after
his
discharge
from
the
hospital
showed
that
the
fourth
ventricle
was
elongated
and
deformed
(Fig
1).
Myodil
ventriculog-
raphy
later
confirmed
these
features
(Fig
2),
suggesting
the
presence
of
a
congenital
malfor-
mation
in
the
hindbrain,
probably
an
Arnold-
Chiari
malformation.
Comment
The
presentation
of
our
patient
is
typical
of
this
syndrome.
The
usual
history
is
of
a
child
born
with
a
myelomeningocele,
who
after
a
few
weeks
of
life
develops
progres-
sive
hydrocephalus
concurrently
with
a
lar-
yngeal
stridor.
The
stridor
worsens
as
the
head
enlargement
progresses;
ventricular
tapping
may
give
temporary
relief
of
laryn-
geal
symptoms,
or
not
at
all;
respiratory
obstruction
may
require
tracheostomy
as
an
emergency
procedure,
but
a
functioning
shunt
operation
for
the
hydrocephalus
usu-
ally
affords
definite
relief
of
both
hydro-
cephalic
and
laryngeal
symptoms.
Evidence
of
increased
intracranial
pressure
may
be
radiologically
demonstrable
as
the
lacunar
skull
shown
by
our
patient
and
by
those
of
others,9
or
as
the
eggshell
appearance
of
an
attenuated
calvarium
at
autopsy.4
Arch
Neurol-Vol
23,
Sept
1970
272
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STRIDOR-ADELOYE
ET
AL
The
laryngeal
stridor
in
our
patient
was
etiologically
related
to
the
hydrocephalus,
since
it
worsened
as
the
hydrocephalus
in-
creased.
Smith4
and
Fitzsimmons8
therefore
postulated
that
the
rapidly
progressing
hy-
drocephalus
probably
led
to
"coning"
of
the
medulla
and
hence,
to
selective
impairment
of
vagal
action
sufficiently
enough
to
disrupt
normal
laryngeal
function.
Where
there
was
subjective
or
objective
evidence
of
dysfunc-
tion
of
other
lower
cranial
nerves,
laryngeal
palsy
of
central
origin
may
result
from
brain
stem
involvement.7"10
This
is
rare
in
infancy
and
has
only
been
encountered
in
adults."'
In
other
instances
where
only
isolated
bilat-
eral
vocal
cord
palsy
was
found
in
associa-
tion
with
myelomeningocele,
an
Arnold-
Chiari
malformation
has
been
incriminated
by
most
authors
who
assumed
that
the
caudal
dis-
placement
of
the
medulla
exerted
traction
on
the
vagus
nerve
and
caused
the
stridor.3,5,6,8'12
However,
Morley'3
in
1969,
who
found
lo-
calized
bilateral
medullary
hemorrhages
in
two
cases
of
this
syndrome
with
Arnold-
Chiari
malformation,
made
the
alternative
proposal
that
the
abnormal
anatomical
con-
figuration
of
the
medulla
may
cause
ischemic
infarction
of
its
lateral
parts
and
the
vagal
nuclei
present
therein
and
so
lead
to
laryngeal
stridor.
Although
there
were
few
instances
where
the
laryngeal
stridor
spontaneously
im-
proved
without
any
form
of
surgical
inter-
vention,8
in
most
patients
neurosurgical
op-
erations
have
improved
laryngeal
function.
Suboccipital
and
high
cervical
decompres-
sion
have
been
employed
in
those
with
Ar-
nold-Chiari
malformation,3'8'9
but
the
re-
sults
of
this
operative
procedure
have
been
variable.
Thus,
Kirsch
et
a19
found
it
to
be
of
value,
and
in
one
of
their
patients
whose
laryngeal
dysfunction
persisted
in
spite
of
a
functioning
valve,
suboccipital
decompres-
sion
succeeded
in
restoring
laryngeal
func-
tion
to
normality.
In
spite
of
this,
the
place
of
suboccipital
decompression
as
a
consis-
tently
useful
procedure
is
not
fully
estab-
lished
as
the
operation
is
a
major
one
in
children,3
and
it
is
attended
by
a
lot
of
risks
and
high
mortality.14
Besides,
in
some
pa-
tients
where
the
operation
was
technically
successful,
it
may
still
fail
to
relieve
laryn-
geal
stridor.6
Shunt
procedures,
on
the
other
hand,
have
generally
been
more
successful.
They
are
simpler
to
perform,
and
are
less
hazard-
ous
and
carry
less
mortality.
As
an
initial
procedure
in
those
whose
laryngeal
stridor
worsened
as
their
hydrocephalus
rapidly
progressed,
shunt
procedures
have
better
chances
than
suboccipital
and
high
cervical
decompression
in
improving
laryngeal
func-
tion.6
Summary
A
case
of
laryngeal
stridor
is
associated
with
myelomeningocele
and
hydrocephalus
in
a
Nigerian
male
child.
The
respiratory
distress
was
successfully
and
dramatically
relieved
by
a
functioning
ventriculo-perito-
neal
shunt
procedure
using
Till-Dahl-Wade
valve,
after
which
the
myelomeningocele
was
excised
and
repaired.
The
literature
on
this
rare
syndrome
is
briefly
reviewed.
References
1.
Heatley
CA:
The
larynx
in
infancy:
A
study
of
chronic
stridor.
Arch
Otolaryng
29:90-103,
1939.
2.
Work
WP:
Paralysis
and
paresis
of
the
vocal
cords:
A
statistical
review.
Arch
Otolaryng
34:267-
280,
1941.
3.
Cavanagh
F:
Vocal
palsies
in
children.
J
Lar-
yng
69:399-418,
1955.
4.
Smith
MEN:
The
association
of
laryngeal
stri-
dor
with
meningo-m)ielocele.
J
Laryng
73:188-190,
1959.
5.
Graham
MD:
Bilateral
vocal
cord
paralysis
associated
with
meningomyelocele
and
the
Arnold-
Chiari
malformation.
Laryngoscope
73:85-92,
1963.
6.
Snow
JB
Jr,
Rogers
KA
Jr:
Bilateral
abductor
cord
paralysis
of
the
vocal
cords
secondary
to
the
Amold-Chiari
malformation
and
its
management.
Laryngoscope
75:316-320,
1965.
7.
Apley
J:
The
infant
with
stridor:
A
follow-up
survey
of
80
cases.
Arch
Dis
Child
28:423-435,
1953.
8.
Fitzsimmons
JS:
Laryngeal
stridor
and
res-
piratory
obstruction
associated
with
meningomyelo-
cele.
Arch
Dis
Child
40:687-688,
1965.
9.
Kirsch
WM,
Duncan
BR,
Black
FO,
et
al:
Laryngeal
palsy
in
association
with
myelomeningo-
cele,
hydrocephalus,
and
the
Arnold-Chiari
malfor-
mation.
J
Neurosurg
28:207-214,
1968.
10.
Bigler
JA,
Holinger
PH,
Johnston
KC,
et
al:
Tracheostomy
in
infancy.
Pediatrics
13:476,
1954.
11.
Rullan
A:
Associated
laryngeal
palsy:
Presen-
tation
of
a
case
of
bilateral
abductor
paralysis
in
a
patient
with
Arnold-Chiari
deformity.
Arch
Otolar-
yng
64:207-212,
1956.
12.
Kiger
JR,
Kohlmoos
HVW,
May
JA:
Bilateral
vocal
cord
paralysis
in
infancy.
Amer
J
Dis
Child
108:648-650,
1964.
13.
Morley
AR:
Laryngeal
stridor,
Arnold-Chiari
malformation
and
medullary
haemorrhages.
Develop
Med
Child
Neurol
11:471-474,1969.
14.
List
CF:
Neurologic
syndromes
accompanying
developmental
anomalies
of
occipital
bone,
atlas
and
axis.
Arch
Neurol
Psychiat
45:577-616,
1941.
Arch
Neurol-Vol
23,
Sept
1970
2r73
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