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Develop. Med. Child Neurol. 1982, 24, 380-385
Case Report
Spinal Arteriovenous Malformations in an Infant: Unusual Symptomology and Pathology
B. Binder G. D. Eng T. H. Milhorat I;. Galioto
Introduction Congenital spinal arteriovenous ma]-
formations are rarely diagnosed in infancy and childhood, either because they do not cause symptoms or because the symptoms are misinterpreted. Complaints typical of spinal lesions in older children and adults, such as radicular pain, sensory loss and bladder and bowel problems, usually are not evident in infants and small children.
We report the case of a 19-month-old girl who presented with spastic diplegia associated with developmental delay and marked failure to thrive, secondary to a giant intraspinal arterial aneurysm and arteriovenous malformation in the thoracic spine. To our knowledge, there has been no previous report of such a large pathological lesion, nor of such a clinical presentation.
Case report V.S., a 19-month-old Indian girl, was brought
simultaneously to the attention of the departments of Cardiology, Physical Medicine and Rehabil- itation and Neurology because of a heart murmur, failure to thrive and episodes suggestive of breath- holding spells.
~ ~~~~~ ~
Correspondence to Helga Binder, M.D., Depa r tmen t of Physical Medicine a n d Rehabilitation, Children's Hospital National Medical Centre, 11 1 Michigan Avenue, N.W., Washington, D.C. 20010.
38C
Her gestational and birth histories were unremarkable, but she had been irritable, with poor appetite and many upper respiratory tract infections since birth. She had been admitted to hospital twice, at 2% and 4% months, and both times had had a lumbar puncture performed during'investigations for sepsis. The first lumbar puncture was described as 'possibly traumatic', but there were no other CSF abnormalities.
The parents felt that the child's motor development had come to a standstill at the age of nine months, when she was able to crawl and pull to standing, although she had never moved her legs as well as her arms. On physical examination she was seen to be very small, and her head circumference, height and weight were all below the 5th percentile for her age. She had a high- pitched, continuous murmur (I/IV) over the left hemithorax, which did not originate from the heart and was felt to beduetoasmall arteriovenous malformation, the exact location of which was undetermined.
She was hypotonic, in general, with normo- active reflexes in the arms and superimposed spasticity in the legs. Plantar signs were extensor bilaterally. Her abdominal reflexes were not tested. Anal sphincter tone was normal. Sensation to pin-prick appeared to be intact. Muscle strength was generally weak, especially so in the left leg. N o muscle atrophy or coiitractures were noted, except for a tight heel-cord in the left foot. Balance and protective responses were appropriate for age. She slightly dragged the left foot when she crawled, and pulled to standing in a crouched position. Dexterity was age-appropriate, and she strongly preferred the right hand. She exhibited no expressive language skills, but was reported to speak less than 10 words, including the names of family members. She seemed to
CASE REPORT
understand simple, one-step, verbal commands. She was able to feed herself using her fingers, and to hold her bottle. Formal developmental testing was impossible because of the child's clinging, unco-operative behavior.
X-rays of the chest and hips, EEG, CT scan and amino acid screen were within normal limits. The impression was of a child with spastic diplegia, with more involvement of the left limbs, associated with developmental delay and failure to thrive. She was also thought to have a smallarteriovenous malformation in the left thorax.
Re-evaluation at the age of 25 months showed increased spasticity in the legs, but better truncal tone and she could cruise independently. She was still failing to thrive, with constant upper respiratory infections. Her over-all development, aside from gross motor, appeared to beat the 12 to 18 months level, and she had acquired no further skills since the first visit. Her behavior was so manipulative that mother and child were referred to an early education specialist for behavior modification therapy.
On cardiac re-evaluationat the age of26 months it was felt that the murmur, audible left-lateral to the origins of the 5th and 6th ribs, could actually represent a bruit originating in the spinal canal. This led t o a myelogram, which showeda complete block at TS and multiple serpiginous large structures below the block(Fig. 1). An angiogram, with selective injection of the intercostal arteries from T6 to T12, showed a huge aneurysm in the
mid-thoracic area, fed by two arteries originating from the 10th and 1 Ith intercostal arteries o n the left (Fig. 2). Multiple large veins wereseen to drain the aneurysm inferiorly. On re-examining the chest X-ray, interpedicular widening of the midthoracic spine was also noted (Fig. 3).
Neurosurgery revealed a n arterial aneurysm measuring 5 x 4 x 4cm and extending from T I 0 to T12, overlying the dorsal cord. The major feeding vessel, measuring 8mm in diameter, appeared to arise from the artery of Adamkiewicz. Additional feeders were arteries coming through the T I 0 intervertebral foramina bilaterally. All the feeding arteries and draining veins were divided and the aneurysm and malformation were removed in toro. The spinal cord appeared t o be completely intact, but compressed to a diameter of 5mm. Little expansion was observed following the surgery.
Pathological examination of the specimen showed an arterial aneurysm of the thin-wall type. Elastic fibers and muscularis coat marked it as a true aneurysm, associated with an arteriovenous malformation of the infantile (type 111) form.
Following the surgery, the child lost all muscle function in the legs, but regained it partially over the course of one year, since when her status has remained static. Her left-leg and abdominal muscles are profoundly weak. The left leg is smaller and Icm shorter than the right and the left hip is beginning to sublux. Her legs remain very spastic and she has a spastic neurogenic bladder. She also has a mild thoracolumbar scoliosis. Sensation to touch and pin-prick are intact below the lesion, but proprioception appears t o be impaired.
However, her general health has dramatically improved. She is a happy, thriving child who has made steady gains in her development. At the age of 35 months. formal assessment showed her fine- motor and cognitive skills t o be at the 30-month level, and her self-help and language skills a t the 24 and 21 month levels, respectively. At the age of 41 months she functioned at age-level in all areas, except for gross-motor and dressing skills, the latter being impaired because of her poor balance. She walks independently, with a left long-leg brace and right hip-abduction brace, using canes.
Discussion To our knowledge, the size of the
aneurysm in this child is unique in both the adult and pediatric literature. We also think this is the first case of intraspinal bruit to be described in an infant: the youngest reported patients were 11 and 16 Fig. 1
381
DEVELOPMENTAL M E D I C I N E A N D C H I L D NEUROLOGY. 1982, 24
years old (Rand and Rand 1960, Ommaya et a/. 1969). No child has been described with failure to thrive and psychomotor developmental delay secondary to an arteriovenous shunt in the spinal canal.
Spinal-cord arteriovenous malform- ations (AVM) are rare, constituting only between 3 .3 and 11 per cent of spinal-cord tumors according to various published series (Ommaya et a/. 1969). They are even more rare in children. Only 13 per cent of all cases reported were under 20 years of age (Scarff and Riegel 1979). However, the majority of cases with AVM (80 per cent according to Aminoff and Logue 1974~) present with insidiously progressive or fluctuating clinical symptoms, which often precede the diagnosis by decades. These are types I and I1 AVMS, as classified by Ommaya e t a / . (1969) and Milhorat (1978). Their small size, slow blood-flow through the lesion, and their tendency to spontaneous thrombosis or calcification,
lead to a long silent period before symptoms occur, and often they are an accidental finding at autopsy. They are less voluminous than juvenile type 111, which morphologically more resembles cerebral AVM, with rapid flowthrough the lesionand often complete block of the spinal canal, as in our patient.
Of the five children reported to date who were two years of age or younger, clinical and radiological details are available for four (Odom et a/. 1957, Bkraud and Meloche 1964, Kaplan et a/. 1976, Ouaknine et al. 1979) (see Table I). All appear to have presented with juvenile (type 111) spinal AVM. In three cases the clinical picture was strongly suggestive of an intraspinal lesion: in the fourth (Odom et a/. 1957), a high cervical-cord AVM, an intracranial lesion could have been suspected.
Apart from the case of Odom and colleagues, the lesions were in the
Fig. 2
382 Fig. 3
TA
BL
E I
Det
ails
of p
revi
ous c
ases
~
A ge
Cl
inic
al
Rad
iola
gira
l FO
IIOW
-UP
Au
rho
n
(mrh
s)
her
fm
dmEs
fin
ding
s Tr
eatm
ent
findi
ngs
Odo
m c
r 01
. (1
957)
Btr
dud
and
Mel
oche
(I 9
64)
W
00
W
Hou
dart
er
al.
(196
8)
Kap
lan
er a
l. (1
976)
Oua
knin
e el
al.
(I 97
9)
24
12
21
16
20
Gen
eral
ized
con
vuls
ion,
loss
of
cons
ciou
snes
s fo
llow
ed b
y se
vere
fr
onta
l hea
dach
es; C
SF
gros
sly
bloo
dy. S
eque
lae
of r
ight
hem
i- pa
resi
s as
soci
ated
with
slig
ht
wea
knes
s an
d sp
astic
ity in
lef
t lim
bs.
Slow
ly d
evel
opin
g, f
lacc
id p
ara-
pa
resi
s w
ithou
t m
uscl
e at
roph
y;
disl
ocat
ed le
ft hi
p. C
avus
feet
. A
bsen
t ank
le je
rks.
Int
act a
nal
sphi
ncte
r.
No
clin
ical
inf
orm
atio
n
Con
geni
tal c
ut. h
eman
giom
as;
sudd
en s
ubar
achn
oid
hem
orrh
age,
Fl
acci
d pa
rapl
egia
on
4th
day.
Hai
ry p
atch
at
T7;
lack
of
spin
ous
proc
esse
s at
T7-
T9. K
ypho
- sc
olio
sis
and
slow
ly p
rogr
essi
ve
spas
tic p
arap
ares
is.
Sym
met
rica
l ca
lf-m
uscl
e at
roph
y. D
ecre
ased
kn
ee, 2
+ an
kle
ierk
s. B
ilate
ral
Bab
insk
i sig
ns,
norm
al s
phin
cter
s,
norm
al s
ensa
tion.
Mye
logr
aphy
/ang
iogr
aphy
: lar
ge
tort
uous
ves
sels
fro
m C
3-C
7, w
ith
roun
d ce
ntra
l def
ect a
t C
7.
Poss
ible
Typ
e 11
1.
Mye
logr
aphy
/ang
iogr
am:
mul
ti-
ple
vari
cose
vei
ns, f
rom
T8-
TI2
w
ith p
arti
al b
lock
at
TI2
.
My e
logr
aphy
/ang
iogr
aphy
; w
iden
ing
of p
edic
les
and
thin
ning
of
cor
tices
at
L2-3
. M
ultip
le
serp
igin
ous
vess
els,
with
com
plet
e bl
ock
at T
12-L
S. C
ystic
ally
dis
- te
nded
con
us m
edul
lari
s. T
ype
111.
Hyp
opla
stic
ver
tebr
al b
odie
s T6
- T8
. Hyp
opla
stic
ped
icle
at
TIO
, w
iden
ing
of s
pina
l can
al T9-1 I
. La
rge.
rou
nd ti
lling
del
ect Ty-I 1.
A
rach
noid
cys
t ov
erly
ing
AV
m
alfo
rmat
ion
with
spl
ittin
g of
co
rd s
imul
atin
g di
aste
mat
omye
lia.
Prob
able
Typ
e 11
1.
No
trea
tmen
t
Coa
gula
tion
of
I la
rge
vess
el.
Liga
tion
of a
rter
ial
feed
ers.
Rem
oval
of
arac
hnoi
d cy
st a
t T9-
10.
Prob
able
lig
atio
n of
fee
ders
.
At
11 ye
ars,
aft
eron
emor
esub
arac
h-
noid
hem
orrh
age,
clin
ical
st
abili
zatio
n, a
ppar
entl
y w
itho
ut
furt
her d
efic
its.
At
6 ye
ars,
inde
pend
ent a
mbu
lati
on
wit
h va
rus
defo
rmity
of
righ
t fo
ot.
Slig
htly
lax
sph
inct
ers.
At 2
4 m
onth
s fl
acci
d pa
rapa
resi
s.
Am
bula
tes
with
sup
port
and
bra
ce
on r
ight
. D
ecre
ased
sen
satio
n in
lo
wer
ext
rem
ities
. Neu
roge
nic
blad
der.
At 2
5 m
onth
s, s
light
ly d
ecre
ased
m
uscl
e str
engt
h in
legs
, nor
mal
tone
, br
isk
deep
tend
on re
flexe
s. I
nde-
pe
nden
t am
bula
tion
.
DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY. 1982, 24
thoracolumbar spine, which is the most frequent location in all reported cases. Erosive bony changes in the form of pedicle widening and thinning of the cortices were noted in two of the four cases in this age-group and in 11 per cent of the pediatric series of Scarff and Riegel(1979), compared with 3 per cent of the adult series of Aminoff and Logue (1974a, b).
Clinical symptoms such as pain, sensory loss in the lower extremities and bladder and bowel dysfunction, prominent among older patients with spinal-cord lesions, have not been described in infants, and of course would be very hard to diagnose. The two children with acute subarachnoid hemorrhage reported by Odom et al. (1957) and Kaplan et al. (1976) apparently were completely asymptomatic prior to that episode. The other two children with slowly progressive paraparesis (Btraud and Meloche 1964, Ouaknine et al. 1979) had severe musculoskeletal anomalies, in addition to neurological deficits involving the spine and legs. One patient had suggestive stigmata in the form of a hairy patch and lack of spinous processes in the ’area of the lesion.
Failure to thrive and developmental delay have not been described previously in the symptomatology, and we feel that in our case the size of the shunt and the resulting marked oxygen deprivation of
vital tissues were to blame for the child’s poor health. The rapid gains in her development following surgery support this feeling.
Unless there are acute neurological findings or congenital anomalies indicative of intraspinal pathology, the diagnosis of spinal AVM in infants will remain very difficult and often will be delayed (by an average of 4 .9 years according to Scarff and Riegel 1979), until irreversible neurological damage has occurred. Residual disabilities are already consider- able in the four young infants reported previously, and in our patient, and will no doubt increase as the children grow and their skeletal deformities, incontinence and impotence become of added concern.
Although an intraspinal AVM as a cause of paraparesis in infants is rare, it should be kept in mind, since it is one of the few intraspinal lesions which it is possible to cure surgically, and severe lifelong disability can be prevented.
A UTH 0 RS’ APPOINTMENTS H. Binder, Department of Physical Medicine and Rehabilitation; G. D. Eng, Department of Physical Medicine and Rehabilitation; T. H. Milhorat, Department of Neurosurgery; G. Galioto, Department of Cardiology; Children’s Hospital National Medical Center, 11 1 Michigan Avenue, N.W., Washington, D.C. 20010.
SUMMARY A 19-month-old Indian girl presented with a giant arterial aneurysm, associated with a
spinal arteriovenous malformation, secondary bruit, failure to thrive and psychomotor developmental delay. The case is compared with the clinical and radiological details available from four previously reported cases aged two years or younger. Although early diagnosis in this age-group remains difficult, it appears to be of utmost importance, since permanent disability is marked and modern diagnostic and therapeutic measures allow surgical cure in many cases.
RESUMI? Malformations artkrioveineuses mkdullaires durant la premiPre enfance:
prksentation dun cas a symptomatologie inhabituelle
384 Une fillette Indienne de 19 mois a prtsentt un antvrisme arttriel gtant associit B une
CASE REPORT
malformation arttrioveineuse mtdullaire, un souffle secondaire, un retard de croissance et de dkvelopement psychomoteur. Ce cas est compark aux donnkes cliniques et radiologiques disponibles 2i partir des quatre cas rapportts anttrieurement chez des enfants de deux ans ou moins. Bien que le diagnostic prtcoce dans ce groupe d’%ge demeure difficile, il apparait de la plus grande importance puisque l’invaliditt dtfinitive est marqute et que les mesures diagnostiques et thtrapeutiques modernes permettent un traitement chirurgical dans de nombreux cas.
ZUSAMMENFASSUNG Spinale arteriovenose MiJ3bildung im Kindesalter:
Fallbericht mit ungewohnlicher Symptomatologie und Pathologie Ein 19 Monate altes indianisches Madchen hatte ein arterielles Riesenaneurysma
verbunden mit einer spinalen arteriovenosen MiSbildung, einem sekundaren Gerausch, einer Gedeihstorung und einer psychomotorischen Entwicklungsstorung. Dieser Fall wird mit den klinischen und radiologischen Einzelheiten, die von vier bereits veroffentlichten Fallen vorliegen, dei zwei Jahre oder junger waren, verglichen. Obwohl eine fruhzeitige Diagnose in dieser Altersgruppe schwierig bleibt, erscheint sie von groBter Wichtigkeit, da die bleibende Behinderung sehr ausgepragt ist und die modernen diagnostischen und therapeutischen Moglichkeiten in vielen Fallen eine chirurgische Behandlung ermoglichen.
RESUMEN Malformaciones arteriovenosas espinales en el lactante:
presentacidn de un caso con sintomatologia y patologia no corrientes Una niiia india de 19 meses de edad presentaba un aneurisma gigante asociado a
malformaciones arteriovenosas espinales, ruido secundario, fall0 en el desarrollo y retraso en el desarrollo psicomotor. El caso se compara con 10s detalles asequibles clinicos y radiol6gicos de otros cuatro casos anteriores de dos aiios de edad o mas jovenes. Aunque el diagn6stico precoz en esta edad es dificil, de hecho tiene la mayor importacia, ya que la invalidez marcada es importante y las medidas diagnbsticas y terapkuticas modernas permiten una curaci6n quirdrgica en muchos casos.
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Houdart, R., Djindjian, R., Hurth, M. (1968) ‘Les angiomes de la moelle. Etude clinique du mechanisme
Kaplan, P., Hollenberg, R. D., Fraser, F. C. (1976) ‘A spinal artriovenous malformation with hereditary
Milhorat, T. H. (1978) Pediarric Neurosurgery. Philadelphia: F. A. Davies. Odom, G. L., Woodhall, B., Margolis, G. (1957) ‘Spontaneous hematomyelia and angiomas of the spinal
Ommaya, A. K., di Chiro, G., Doppman, J. L. (1969) ‘Ligation of arterial supply in the treatment of spinal
Ouaknine, G. E., Godath, N., Matz, S., Shalit, M. (1979) ‘Congenital vascular malformation of the spinal
Rand, R. W., Rand, C. W. (1960) Intraspinal Tumors of Childhood. Springfield, Ill.: C. C. Thomas. Scarff, T. B., Riegel, D. H. (1979) ‘Arteriovenous malformations of the spinal cord in children.’ Child‘s
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