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5 May 2000 V
Syringomyelia and
FOCAL POINT Hydromyelia in
★Intraspinal cord abnormalities
caused by cyst formation are an
increasingly recognized cause of
Dogs and Cats
spinal cord disease in dogs and
cats. Washington State University
Rodney S. Bagley, DVM Patrick R. Gavin, DVM, PhD
Gena M. Silver, DVM, MS Michael P. Moore, DVM, MS
KEY FACTS Hege Kippenes, DVM Rebecca Connors, LVT
■ Syringomyelia refers to abnormal
cavities filled with fluid in the
ABSTRACT: Spinal cord abnormalities are common causes of limb dysfunction in dogs and
spinal cord. cats. With the advent of advanced imaging techniques, intraspinal diseases are more frequent-
ly being diagnosed antemortem. Many intraspinal abnormalities are associated with fluid ac-
■ Hydromyelia refers to a cumulation and cyst formation. Spinal cord fluid accumulation may primarily involve the
pathologic condition that is parenchyma (syringomyelia) or the central canal (hydromyelia). Advanced imaging studies,
characterized by accumulation such as magnetic resonance imaging, are often necessary for diagnosis. An increased aware-
of fluid within an enlarged ness of these diseases is important to identify animals with spinal disease.
central canal of the spinal cord.
S
■ Possible pathogenic mechanisms pinal cord abnormalities are frequent causes of limb dysfunction in dogs
of syringo-/hydromyelia and cats. Numerous diseases have been associated with the spinal cord,1 in-
formation include changes in cluding intervertebral disk abnormalities, vertebral fracture, tumors,
cerebrospinal fluid pressure diskospondylitis, myelitis, and congenital malformations. From a diagnostic
relationships within the spinal standpoint, survey spinal radiographs are often helpful in diagnosing vertebral
cord, loss or abnormal fractures, vertebral tumors, and diskospondylitis. Myelography is useful in de-
development of spinal tecting diseases that compress or expand the spinal cord. Diseases that primarily
parenchyma, stenosis of the or exclusively affect the spinal cord parenchyma without associated vertebral
central canal, and obstruction bony change, however, have been elusive to diagnose with survey radiography
of cerebrospinal fluid flow. and myelography. Such advanced imaging modalities as magnetic resonance
imaging (MRI) and computed tomography (CT) are being used more frequently
■ Concurrent abnormalities of the to diagnose spinal disease. With the advent of these newer spinal imaging
cerebellum, caudal brain stem, modalities, the clinicians’ ability to “see” the spinal cord has vastly improved.
and foramen magnum may be the Consequently, spinal cord diseases previously thought to be uncommon are be-
cause of syringo-/hydromyelia. ing diagnosed regularly.
Syringomyelia and hydromyelia are examples of diseases that are recognized
more frequently in the small animal hospital at Washington State University,
where MRI is used as the primary spinal diagnostic imaging modality. Although
previously described in dogs and cats, these diseases were often found only at the
time of necropsy.1–7 Because antemortem diagnosis is now possible, clinical and
diagnostic features of these diseases become important for appropriate manage-
Small Animal/Exotics Compendium May 2000
ment of affected animals. An increased understanding though originating as hydromyelia, may be lined by
of the disease process also offers the potential for devel- glial tissue consistent with a syrinx.8
opment of newer treatments.
CAUSES
TERMINOLOGY Pathologic Mechanisms
Syringomyelia and hydromyelia are cystic abnormalities No single pathologic mechanism adequately explains
of the spinal cord. Syringomyelia refers to abnormal cavi- all instances of syringomyelia and hydromyelia.8 Possible
ties filled with fluid in the substance of the spinal cord causes of cyst formation include changes in CSF pres-
(Figure 1).8 A syrinx refers to one of these cavities. Hy- sure relationships within the spinal cord (as occurs with
dromyelia refers to a pathologic condition characterized hydrocephalus or foramen magnum abnormalities), loss
by accumulation of fluid
within an enlarged central
canal of the spinal cord (Fig-
ure 1). In both conditions,
the fluid that accumulates is
similar, if not identical, to
cerebrospinal fluid (CSF).
Some authors refer to hy-
dromyelia as a communicat-
ing syringomyelia and use the
term syringomyelia to de-
scribe all intraspinal abnor- Figure 1A Figure 1B
mal fluid accumulations.8
DIFFERENTIATING
BETWEEN
SYRINGOMYELIA
AND HYDROMYELIA
Because it is often diffi-
cult clinically and diagnos-
tically to differentiate be-
tween syringomyelia and
hydromyelia, the term sy-
ringo-/hydromyelia is used. Figure 1C Figure 1D
The diagnosis is ultimately
made based on histology. In
hydromyelia, the fluid cavi- H S
ty is lined by ependymal
cells characteristic of the
central canal, whereas a sy-
ringomyelic cavity is usual- H
ly within the spinal cord S
external to the central canal
and is lined by glial cells. In
some instances, differenti-
Figure 1E Figure 1F
ating between the two is
more difficult because an in- Figure 1—(A) Sagittal and (B) transverse T2-weighted magnetic resonance images (MRIs) of the
creasing hydromyelia may thoracolumbar spine of a cat. There is significant increased signal (hyperintensity; whiter area)
destroy or disrupt the epen- consistent with excessive fluid accumulation involving diffuse areas within the spinal cord (ar-
dymal layer with fluid rup- rows). (C) A sagittal T2-weighted MRI of a normal feline spinal cord is shown for comparison.
turing into the surrounding (D) Pathologic specimens from the cat in Figures 1A and 1B at different spinal cord levels show-
spinal cord. These abnor- ing hydromyelia and (E) a combination of hydromyelia (H) and syringomyelia (S). (F) Histolog-
mal fluid-filled areas, al- ic section of the spinal cord in Figure 1E showing hydromyelia (H) and syringomyelia (S).
Figure 5A
Figure 5—(A) Sagittal T2-weighted magnetic
resonance image (MRI) from the spinal cord
of a normal dog showing truncation artifact
(represented by the linear signal simulating the
central canal; arrows). (B) No similar abnor-
mality of the central canal is seen on the trans-
verse T2-weighted MRIs from same area.
Figure 5B
quent finding in dogs undergo- from the incised cavity. A biopsy may
ing spinal MRI in our hospital. be obtained from the cyst wall if the
T2-weighted scanning sequences diagnosis is in doubt. The overlying
are helpful in screening animals dura is usually not closed in dogs.
for these types of abnormalities. Posterior fossa decompression is
Increased T 2 signal intensity performed via a suboccipital craniec-
within the spinal cord, however, tomy in dogs and also often requires
is not pathognomonic for sy- a partial laminectomy of C1. If oc-
ringo-/hydromyelia because oth- cipital dysplasia is present, there is a
er lesions (e.g., edema, some fibrous covering in the caudal occipi-
stages of hemorrhage, malacia) Figure 7A tal bone area. This tissue is incised
may have a similar appearance. with a scalpel blade or microscissors.
Dilation of the central canal may If the occipital bone is intact, a high-
occur with compressive myelopa- speed nitrogen-powered drill is use-
thies and other diseases that dam- ful in removing the occipital bone.
age or disrupt spinal cord paren- Similar to performance of a laminec-
chyma (Figure 6). 48 In these tomy, the bone is removed with the
instances, the cystic abnormali- drill to a thin (egg-shell) layer of in-
ties may reverse with appropriate ner cortical bone. The remaining
treatment of the primary disease. bone is removed with rongeurs. The
Sometimes, however, clinically more fibrous dura is then incised
significant syrinxes may develop with a scalpel or scissors. CSF will
Figure 7B
at a later time. flow freely from the area of the dura
Figure 7—(A) Postoperative sagittal and (B)
incision.
transverse T 2-weighted magnetic resonance
TREATMENT images from the dog in Figure 2A with hydro- Before closure, plugging of the cen-
In humans, the treatment of cephalus and syringo-/hydromyelia. A fourth- tral canal at the obex has been recom-
cranial cervical syringomyelia ventricle-to-peritoneal shunt has been placed; mended in humans in an attempt to
with or without caudal fossa ab- the shunt tip is visible in the fourth ventricle prevent flow of CSF from the fourth
normalities remains controver- (black arrows). Air (white arrows) in Figure 7A ventricle into the cervical spinal cord.
sial. In instances where the le- is present in the fourth ventricle and dorsal to Based on more recent pathologic stud-
sions are subclinical or clinical the cerebellum primarily because of surgery. ies, however, this practice is being
signs are mild and nonprogres- questioned and often not performed.19
4,8
sive, no definitive treatment may be needed. If clini- Whether marsupialization of the dura in the foramen
cal signs are progressive, definitive treatment should be magnum is needed is also unclear. Aseptic meningitis is a
considered. Surgical approaches include decompression potential complication, presumably a result of chemical
of the syrinx via myelotomy, posterior fossa decompres- irritation of the nervous tissues and meninges from
sion via a suboccipital craniectomy and associated cer- hemoglobin breakdown products.52 Too few dogs have
vical vertebral laminectomy, and syringosubarachnoid been treated surgically to establish objective criteria for
shunting.14,19–21,49–52 In one report comparing the latter surgical treatment of these abnormalities.23
two procedures, no difference was found between pa- Treatment responses are influenced by the type of
tient groups, with both procedures being equally effec- pathology present. Humans with hydromyelia in asso-
tive in causing syrinx collapse.49 Clouding the issue fur- ciation with hydrocephalus may likely benefit from
ther, some syrinxes have regressed spontaneously, ventriculoperitoneal shunting, whereas those with hy-
resulting in some authors questioning the role of dromyelia that does not communicate with the fourth
surgery as treatment for this problem.50 In addition, di- ventricle may not benefit.8 Extracanalicular syrinxes
rect syrinx drainage without shunting may also be help- that do not communicate with the fourth ventricle may
ful in some cases. require direct shunting. In addition, because this type
Myelotomy and cyst decompression may help relieve of syrinx often results in irreversible damage to spinal
pressure within the syrinx cavity.7,27 Laminectomy over tracts, overall treatment responses may not be as favor-
the affected area is required to access the spinal cord. able as with hydromyelia. Surgical treatment of sy-
Cautious incision into the spinal cord may be per- ringomyelia associated with arachnoid scarring may not
formed with either a scalpel (No. 11 Bard Parker blade) be consistently helpful.51
or microdissecting instruments. CSF will flow freely Fourth ventricular shunting has been performed in
humans and dogs to treat some abnormalities of CSF myelia. J Neurosurg 52:812–817, 1980.
dynamics of the posterior fossa (Figure 7). 23,53 The 17. Watson AG, deLahunta A, Evans HE: Dorsal notch of the
foramen magnum due to incomplete ossification of the su-
shunt is placed from the fourth ventricle into the peri- praoccipital bone in dogs. J Small Anim Pract 30:666–673,
toneal cavity. Technically, placement of the proximal 1989.
catheter tip into the fourth ventricle may result in ia- 18. Adams RD, Victor M, Ropper AH: Developmental diseases
trogenic trauma to the brain stem. Morbidity as high as of the nervous system, in Adams RD, Victor M, Ropper AH
42% has been associated with placement of the rostral (eds): Principles of Neurology, ed 6. New York, McGraw-Hill,
catheter tip in humans.53 Clinical signs include cranial 1997, pp 1007–1008.
19. Vaquero J, Martínez R, Arias A: Syringomyelia-Chiari com-
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Treatment responses depend on appropriate diagnosis 20. Batzdorf U: Chiari I malformation with syringomyelia. J
and understanding of the pathophysiologic events that Neurosurg 68:726–730, 1988.
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treatment of syringomyelia and the Chiari malformation. J
With improved diagnostic capabilities, these diseases Neurosurg 57:24–31, 1982.
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Knowledge of these spinal cord diseases should lead to of Chiari malformation: A morphometric study of the poste-
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Neuroanatomy of Computed Tomography and Magnetic Reso- About the Authors
nance Imaging. Philadelphia, Lea & Febiger, 1984, pp 13–29.
Drs. Bagley, Gavin, Silver, Moore, and Kippenes and Ms.
46. Haughton VM, Daniels DL, Czervionke LF, et al: Cervical
spine, in Stark DD, Bradley Jr WG (eds): Magnetic Reso- Connors are affiliated with the Department of Clinical Sci-
nance Imaging, ed 3. Philadelphia, Mosby, 1999, pp 1833– ences, College of Veterinary Medicine, Washington State
1850. University, Pullman, Washington. Drs. Bagley and Moore
47. Kjos BO, Brant-Zawadzki M, Kucharczyk W, et al: Cystic
are Diplomates of the American College of Veterinary In-
intracranial lesions: Magnetic resonance imaging. Radiology
155:363–369, 1985. ternal Medicine, and Dr. Gavin is a Diplomate of the
48. Kirberger RM, Wrigley RH: Myelography in the dog: Re- American College of Veterinary Radiology.
view of patients with contrast medium in the central canal.