External Hydrocephalus

in Two Cats
External hydrocephalus describes an accumulation of cerebrospinal fluid (CSF) between the
cerebral hemispheres and the overlying arachnoid membrane, rather than within the lateral ven-
tricles. Two young cats with encephalopathic signs were diagnosed with external hydrocephalus,
one via magnetic resonance imaging and one via computed tomography. Both cats had abnor-
mally large, broad heads, with no evidence of open fontanelles. A surgical shunt was placed in
each cat to divert the accumulated CSF within the cranial cavity to the peritoneal space. Both
cats improved dramatically soon after surgical shunting was performed, and they continue to do
well clinically, approximately 42 months and 8 months postoperatively, respectively.
J Am Anim Hosp Assoc 2003;39:567-572.

Dallas, Texas 75287.

Curtis W. Dewey, DVM, MS,
Diplomate ACVIM
(Neurology),
Diplomate
ACVS
Joan R. Coates, DVM, MS,
Diplomate ACVIM (Neurology)
Julie M. Ducoté, DVM,
Diplomate ACVIM (Neurology)
Joseph D. Stefanacci, VMD,
Diplomate
ACVR
Michael A. Walker, DVM,
Diplomate ACVR
(Radiology, Radiation Oncology)
Dominic J. Marino, DVM,
Diplomate
ACVS

From Long Island Veterinary Specialists

(Dewey, Stefanacci, Marino),
163 South Service Road,
Plainview, New York 11803
and the Departments of Small Animal
Medicine and Surgery (Coates, Ducoté)
and Large Animal Medicine
and Surgery (Walker),
College of Veterinary Medicine,
Texas A&M
University,
College Station, Texas 77843-4474.

Doctor Ducoté’s current address is

Dallas Veterinary Surgical Center,
4444 Trinity Mills Road, Suite 203,

Introduction
Hydrocephalus is an abnormal
accumulation of cerebrospinal
fluid (CSF) within the ventricular
system of the brain.1-3 There are
numerous classi- fication schemes
for hydrocephalus in dogs and
cats, the majority of which are
based either on etiology (e.g.,
congenital versus acquired) or
suspected location of CSF flow
interruption (e.g., obstructive
versus nonobstructive).1-3 The
location of CSF accumulation
with respect to the lateral
ventricles is typically not
specified. Internal hydrocephalus
refers to excessive CSF within the
lateral ventricles of the brain; with
the excep- tion of one dog,4 all
reported cases of canine and feline
hydrocephalus have fallen into
this category. External
JOURNAL of the American Animal Hospital Association
hydrocephalus is a condition in which the accumulated CSF is primarily
in an extra-axial location, rather than within the lateral ventricles.4-7
This report describes the clinical aspects and imaging findings of two
young cats with external hydrocephalus. An analogous disorder of
human infants, usually found in combination with abnormally large
cranium size (i.e., macrocephaly), is also discussed.
Case Reports
Case No. 1
This case was briefly mentioned in a previously published textbook
chapter.8 An 8-month-old, male castrated, Flame Point Siamese cat pre-
sented to the Texas A&M University Veterinary Medical Teaching
Hospital (TAMU-VMTH) with an 18-week history of intermittent
lethargy, abnormal behavior, pelvic limb weakness, and apparent head
pain. The owners described what they suspected was a short seizure
episode at the onset of the cat’s difficulties. At 6 months of age, the cat
had experienced a very prolonged anesthetic recovery following castra-
tion and declaw procedures. During the period of the patient’s illness
prior to referral to the TAMU-VMTH, he had been treated on several
occasions with varying dose regimens of corticosteroids and antibiotics,
with little to no apparent effect. Approximately 1 week before referral,
the cat had been started on oral prednisone (0.5 mg/kg body weight, q
48 hours) by the referring veterinarian.
567
 56 JOURNAL of the American Animal Hospital Association November/December 2003, Vol. 39

Upon presentation to the TAMU-VMTH, the cat was

alert and responsive. The only notable physical abnormality
was a large, broad skull, which seemed disproportionate to
the patient’s body size [Figure 1]. No open fontanelles were
palpable. Neurological abnormalities included intention
tremors of the head, decreased menace responses bilaterally
with associated visual deficits, bilateral positional
ventrolat- eral strabismus, a hypermetric gait, decreased
propriocep- tive placing responses in the right pelvic limb,
and cervical spinal hyperesthesia evident on palpation of
the neck. The neuroanatomical localization was
multifocal/diffuse encephalopathy, primarily involving the
forebrain (i.e., cerebrum/diencephalon) and cerebellum. A
complete blood count (CBC) and serum biochemical panel
were within ref- erence ranges. Serology was negative for
feline leukemia virus (FeLV), feline immunodeficiency
virus (FIV), and feline infectious peritonitis virus (FIPV).
The patient was anesthetized, and a computed tomography
(CT) scan of the brain was performed. The cerebral
hemispheres were shift- ed axially by extra-axial fluid
accumulation, and the cere- bellum was displaced caudally
by accumulated fluid [Figure 2A]. The lateral ventricles
appeared moderately enlarged. On some of the CT images,
it was apparent that a section of the left occipital cerebral
region was absent, at which point the CSF of the left lateral
ventricle was confluent with that of the overlying
subarachnoid space [Figure 2B]. The CT findings
supported a diagnosis of external hydrocephalus. A CSF
sample obtained from the cerebellomedullary cistern was
visibly xanthochromic, consistent with prior hemor- rhage.
Cytopathological evaluation of the CSF was consis- tent
with chronic hemorrhage with a suspected secondary
mixed-cell inflammatory response. The red blood cell
(RBC) count was 16  103 cells/µL. The white blood cell
(WBC) count was 256 cells/µL (reference range, 0 to 5
cells/µL), with 60% macrophages, 30% nondegenerate neu-
trophils, and 10% lymphocytes. The CSF protein level was
1,487 mg/dL (reference range, <30 mg/dL). A CSF corona-
virus antibody titer was negative (<1:25). Since the previ-

uted tomography (CT) scan of the cat from Figure 1, demonstrating external hydrocephalus. On the transverse (2A) and dorsal (2B) images, both cere- bral
Figure 1—Photograph of an 8-month-old, Flame Point
Siamese cat (case no. 1) with external hydrocephalus;
note the domed-shaped calvarium and macrocephaly.
ously prescribed prednisone had not resulted in any
obvious clinical improvement, it was discontinued.
During the ensuing 3-week period, the cat experienced
several more episodes of lethargy and apparent head pain. At
this point, it was decided to surgically place a shunting
devicea to divert excessive CSF fluid from the cranial cavity
into the peritoneal space. The rostral end of the shunting
device was placed in the expanded subarachnoid space later-
al to the left cerebral hemisphere; the caudal end was placed
in the peritoneal cavity [Figure 3]. At the time of surgery, a
CSF sample was obtained from the dilated subarachnoid
space in the cranial vault and submitted for evaluation. This
ent in the cat from Figures 1 and 2. The cra- nial end (arrow) of the shunt is in the left side of the calvari- um, and the caudal end extends into the peritonea

CSF sample was also xanthochromic. The RBC count for

this second CSF sample was 1.29  103 cells/µL. The WBC
count was 6 cells/µL, with a distribution of 65%
macrophages, 19% neutrophils, 13% lymphocytes, and 3%
eosinophils. The CSF protein level was 1,065 mg/dL. The cat
recovered unevent- fully from surgery. Forty-eight hours
gure 4—Computed
aftertomographic
surgery, thescan 6 weeks
patient’s after
rectal shunt placement
temperature in the cat from Figures 1 through 3. Note that the fluid accumulation is still present. T
was elevated
(104.2˚F), although he was active, had a normal appetite, and
his surgical inci- sions were healing well. A CBC performed
examination was normal. However, he had been experienc-
at this time was also within reference ranges. Oral
amoxicillin/clavulanic acid (22 mg/kg body weight, q 12 ing generalized seizures with increasing frequency during
hours for 14 days) was institut- ed. By day 5 following the 4 months prior to readmission. At the time of readmis-
surgery, the cat’s rectal temperature was near normal limits sion, the cat was having approximately two seizures a day.
(102.7˚F), and he was sent home. The serum phenobarbital level at this time was 12.1 µg/mL.
At a recheck examination approximately 6 weeks after The cat’s phenobarbital dose was increased to 3.2 mg/kg
surgery, the owner reported that the cat had been acting body weight, q 12 hours. A serum phenobarbital level 2
nor- mally since discharge, with no further episodes of weeks later was 35.3 µg/mL. No further seizure activity
lethargy or apparent head pain. Also, according to the was observed following the increase in phenobarbital dose.
owner, the cat had been very playful and had an increased The owner was contacted via telephone approximately
appetite. The cat had gained 0.73 kg since hospital 42 months after initial presentation. The cat was still doing
discharge. The only neu- rological abnormality at this time well at this time and had not experienced any further
was decreased proprio- ceptive placing reactions in both seizure activity.
pelvic limbs. The patient was anesthetized, and a repeat CT
scan of the brain was per- formed. Correct positioning of Case No. 2
the rostral end of the shunt- ing device was confirmed; A 14-month-old, female spayed, domestic shorthaired cat
however, there was no obvious difference in the extent of presented to Long Island Veterinary Specialists (LIVS) on
subarachnoid CSF accumulation [Figure 4]. an emergency basis after having several short focal seizures
Approximately 19 months later, the cat was readmitted (i.e., facial muscle twitching) and one prolonged (approxi-
to the TAMU-VMTH for having experienced three mately 30 minutes) generalized seizure. The patient was
generalized seizures during the 2 weeks preceding actively seizuring at the time of admission; the seizure was
readmission. Other than the seizure episodes, the patient halted after bolus administration of diazepam (0.5 mg/kg
was still doing well neurologically. Oral phenobarbital was body weight, intravenously). Upon physical examination, it
instituted at a dose regimen of 1.6 mg/kg body weight, q 12 was noted that the cat had a large, broad cranium, which
hours. A serum phenobarbital level measured 2 weeks later appeared disproportionately large in relation to her body
was 8.2 µg/mL (therapeutic range, 15 to 45 µg/mL). size. No open fontanelles were palpable. Abnormal
Despite the low serum phenobarbital level, the cat had not findings on neurological examination included obtunded
experienced any further seizure activity. The dose regimen mental sta- tus, absent menace responses bilaterally, and
was not changed at this time. apparent hyper- esthesia upon manipulation of the
The patient returned for evaluation of worsening gener- calvarium. Some of the patient’s neurological abnormalities
alized seizure activity approximately 7 months later, 29 were suspected to be either postictal signs or related to
months after initial presentation. The cat’s neurological recent diazepam adminis-
tration. The only abnormal blood-work finding was a blood
The antibiotics to which both organisms were sensitive
glucose (BG) level of 318 mg/dL (reference range, 70 to
included gentamicin, amikacin, carbenicillin, piperacillin,
150 mg/dL). Because this BG result was based on blood
ceftazidime, cefotaxime, ciprofloxacin, orbifloxacin, and
pulled during a seizure episode, a repeat BG was measured
imipenem.
6 hours later; at that time the BG level was 70 mg/dL. The
The patient recovered well from surgery and was eating
cat’s vac- cination status was current, and serological
voluntarily and walking around her cage approximately 36
samples submit- ted previously by the referring veterinarian
hours after the shunting procedure. The cat was discharged
for FeLV and FIV were negative. Approximately 8 hours
from the hospital 72 hours after surgery. The clindamycin
following the cat’s seizure episode, a repeat neurological
was prescribed for a 3-week period. After the culture/sensi-
examination was performed. Neurological abnormalities
tivity results from the second CSF sample were available,
identified at this time included obtunded mental status,
oral enrofloxacin (2.9 mg/kg body weight, q 12 hours for
bilateral horizontal nystag- mus, positional ventrolateral
14 days) was added to the clindamycin therapy. The cat
strabismus on the left side, and circling to the left side. The
was presented again to LIVS, 72 hours after discharge,
neuroanatomical localization was multifocal
after experiencing four short episodes that were believed to
encephalopathy, involving both the forebrain (i.e.,
be focal seizures. The episodes consisted of transient ataxia
cerebrum/diencephalon) and the cerebellomedullary region
and hypersalivation. The cat was hospitalized, and the oral
of the hindbrain.
phenobarbital dose was increased (2.5 mg/kg body weight,
The cat was anesthetized, and a magnetic resonance
q 12 hours). Over the next 24 hours, the cat experienced
image (MRI) of her brain was obtained. A large amount of
four, short (<30 seconds) generalized seizures.
extra-axial fluid was evident, displacing the cerebrum axial-
Approximately 24 hours later, the patient was released
ly, and the lateral ventricles were moderately enlarged
from the hospital.
[Figures 5A, 5B]. Meningeal enhancement was evident on
Ten days following hospital discharge, the cat was
the T1-weighted images with contrast (i.e., gadolinium)
rechecked at LIVS. The owners reported that the cat was
[Figure 5C]. An apparent fluid line was evident on trans-
doing well, with no further seizure activity. The only neuro-
verse images, suggesting a difference in signal intensity
logical deficit identified was absent menace responses
between dorsal and ventral regions of the accumulated fluid
bilat- erally. The cat appeared to have some visual ability,
[Figure 5C]. The imaging diagnosis was external hydro-
however. The surgical incisions were healed at this time.
cephalus. A CSF sample was obtained from the cerebel-
lomedullary cistern for both cytopathological analysis and Six weeks later, the patient was readmitted to the hospi-
bacterial culture. The CSF analysis was supportive of a tal after having two short (<1 minute) seizures the evening
mild, mixed-cell pleocytosis. The WBC count was 6 prior to presentation. Other than an absent menace response
cells/µL (range, 0 to 5 cells/µL), with a distribution of 47% on the right eye, the cat’s neurological examination was
monocytoid cells, 29% neutrophils, and 24% lymphocytes. within normal limits. A serum phenobarbital level was
The RBC count was normal (2 cells/µL). The CSF protein measured and found to be 23.9 µg/mL (therapeutic range,
level was within reference ranges. The CSF culture failed to 15 to 45 µg/mL). The phenobarbital dose was increased to
yield any growth after 5 days. 3.2 mg/kg, q 12 hours. The cat had no more seizures over
the next 24 hours and was sent home.
Pending CSF culture results, the cat was started on oral
Approximately 8 months following initial presentation,
clindamycin (14 mg/kg body weight, q 12 hours) and oral
the owners were contacted by telephone. The cat was still
phenobarbital (1.8 mg/kg body weight, q 12 hours). The
doing well neurologically. She had experienced two short
patient was alert when handled at this time, but was gener-
(<20 seconds) generalized seizures approximately 4 and 6
ally somnolent, preferring to lay in lateral recumbency. The
months after initial presentation.
cat had to be hand-fed. Approximately 72 hours after
admis- sion to LIVS, the cat was anesthetized again, and a Discussion
shunting devicea was surgically placed to divert excess CSF
Hydrocephalus is not commonly encountered in cats. All
from the cranial vault to the peritoneal cavity. At the time
reported cases of feline hydrocephalus have been internal
of surgery, a second CSF sample and a meningeal biopsy
hydrocephalus, in which the CSF accumulation is primarily
were obtained. The CSF sample was red-tinged and
opaque, sug- gestive of recent hemorrhage. The RBC count within the lateral ventricles.1-3,8 The two cases of this
report represent an unusual manifestation of feline
of this CSF sample was 4.83  106/µL. The WBC count
hydrocephalus. External hydrocephalus is occasionally
was 4.6  103/µL, with a distribution similar to that of reported in humans, most commonly in infants. In these
peripheral blood. Macrophages engulfing erythrocytes were cases, external hydrocephalus is usually associated with an
also appreciated on CSF cytopathology. The CSF protein abnormally large cranium. This latter malformation is
level was 8,000 mg/dL (reference range, <48 mg/dL). The called macro- cephaly and is often the sole indication of
inter- pretation of these CSF findings was subacute to
a neurological abnormality.5-7,9-11 This combination of
chronic intra-arachnoid hemorrhage. The meningeal biopsy
macrocephaly and external hydrocephalus is typically mild
was interpreted as moderate, chronic-active, fibrosing
clinically and usu- ally resolves spontaneously by 2 to 3
meningi- tis. Two organisms, Escherichia coli and
years of age. In a small percentage of human cases,
Pseudomonas aeruginosa, were cultured from the second
however, the condition pro-
CSF sample.
Figures 5A-5C—Magnetic resonance image (MRI) appear-
ance of external hydrocephalus in a 14-month-old domestic
shorthaired cat (case no. 2). The transverse T1-weighted
MRI scan (5A) demonstrated hypointense cerebrospinal
fluid (CSF). A transverse T2-weighted MRI (5B) depicts an
increase in signal intensity indicative of fluid, most likely
CSF. A contrast-enhanced, transverse T1-weighted MRI
scan (5C) revealed meningeal enhancement (arrow) and a
difference in signal intensity between dorsal and ventral
regions of CSF accumulation.
gresses to the point at which surgical shunting is required. 5- An alternate theory suggests that external hydrocephalus is
7,10,11 Macrocephaly/external hydrocephalus is believed to
a sequela to severe internal hydrocephalus; in this theory,
be a heritable congenital syndrome. 10 External hydro- CSF accumulation within the lateral ventricle eventually
cephalus has also been reported in adults, following sub- leads to rupture of a region of the surrounding cerebral
arachnoid hemorrhage.6,12 The pathogenesis of external parenchyma. The fluid subsequently surrounds the
hydrocephalus is unknown. Most theories propose either a cerebrum, shifting it axially. There exists a rat model of
congenital (e.g., developmental abnormality) or acquired hydrocephalus in which this second scenario has been
(e.g., hemorrhage, inflammation) deficiency of the arach- documented. In these rats, severe internal hydrocephalus
noid villi in their ability to absorb CSF as the reason for leads to rupture of the caudal occipital pole of the
intracranial CSF accumulation. A lower resistance to CSF cerebrum; external hydrocephalus develops following this
flow within the extra-axial subarachnoid space in compari- rupture.14 An area of possible cere- bral parenchymal
son with the ventricular system is the suspected reason for rupture in the caudal occipital region was identified on the
preferential subarachnoid CSF accumulation. One view of CT images of case no. 1.
the pathogenesis of external hydrocephalus is that it repre- The pathogenesis of external hydrocephalus in the two
sents an early, clinically mild stage of hydrocephalus that cats of this report is unknown. The abnormally large head
may eventually advance to become internal hydrocephalus.13 size of both cats would suggest they were affected by a
congenital syndrome similar to macrocephaly/external
hydrocephalus of humans. However, there are a number of excessive CSF allowed for functional improvement that was
features of the feline disorder that are very dissimilar to the not likely to be structurally apparent on CT or MRI scans.
human syndrome. In neither case could the disorder be con- Long-term follow-up CT or MRI in these patients would be
sidered mild or self-limiting, as is the rule for human macro- valuable in determining whether or not obvious structural
cephaly/external hydrocephalus. It is possible that both cats changes are to be expected following surgical shunting.
of this report suffered from a severe form of macro- The persistence of seizure activity in both cats following
cephaly/external hydrocephalus. If the feline condition is surgery could be due to several factors. Since clinical signs of
similar to that in humans, it is likely that the majority of neurological dysfunction other than seizures dramatical- ly
cases would not be clinically apparent. Pet owners and vet- improved or resolved in both cats, shunt malfunction as a
erinarians are unlikely to seek CT or MRI of a cat’s brains cause of continued seizure activity is not likely. Permanent
based solely on the presence of large cranium size. The CSF brain damage from the CSF accumulation, leading to a
analysis was abnormal in both cats, suggestive of subacute “seizure focus,” is a more likely explanation. The seizures
to chronic hemorrhage. Cerebrospinal fluid is typically nor- were eventually well controlled in both cats with phenobar-
mal in cases of human macrocephaly/external hydro- bital therapy.
cephalus.10 The subarachnoid hemorrhage in these cats
could represent either a cause of hydrocephalus (i.e.,
obstruction of arachnoid villi) or a consequence of rapidly
developing hydrocephalus (i.e., tearing of stretched
meningeal and cerebral blood vessels). In one cat (case no.
2), repeat CSF culture results were positive for Escherichia
coli and Pseudomonas aeruginosa. Since the initial CSF
culture was negative, and the cat did not exhibit “classic”
signs (e.g., pyrexia, neck pain) of bacterial meningoen-
cephalitis, the possibility exists that these organisms repre-
sent contaminants. However, in a recent study of bacterial
meningoencephalomyelitis of dogs, it was found that “clas-
sic” signs of bacterial central nervous system infection are
often absent.15 It is possible that there truly was an infec-
tious component to the cat’s condition. Whether this sus-
pected infection was causative or contributory is unknown.
A puppy with external hydrocephalus was recently
described, in which a bacterial meningoencephalitis was
suspected as the etiology for the fluid accumulation.4
Unfortunately, CSF culture was not pursued for case no. 1.
The neurological status of both cats improved dramati- cally
shortly after surgical placement of a shunting device. A 6-
week recheck CT scan of case no. 1 verified correct shunt
placement within the cranial vault, although there was no
appreciable difference in the amount of extra-axial fluid
accumulation. It is possible that expecting an obvious dif-
ference in appearance after 6 weeks was premature. Another
possibility is that providing a continuous conduit for the
Addendum
Shortly after submission of this manuscript, case no. 2
re- presented to LIVS on an emergency basis. The cat
had expe- rienced two, short, generalized seizures within
a 24-hour period. Although the cat recovered from the
seizures, the owner elected for euthanasia because of
frustration over continued seizure activity and financial
limitations. A necropsy was not permitted.

a Accura shunt systems; Phoenix Biomedical Corp., Valley Forge, PA

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