Essential neurosurgery

Atlantoaxial dislocation
Dislocations at the atlantoaxial joint can result
from congenital malformations, involving in particular
the congenital fusion of the occiput to the
atlas and fusions of C2 and C3. Multiple congenital
cervical fusions occur in the KlippelFeil syndrome.
These types of fusions will increase the
strain on the ligaments of adjacent vertebrae,
resulting in instability. Atlantoaxial dislocation
may also result from inflammatory conditions
such as rheumatoid arthritis or following trauma
(Fig. 11.8).

Spinal dysraphism
Spinal dysraphism results from incomplete or
faulty closure of the dorsal midline embryological
structures. The major forms of spinal dysraphism
are:
1 Myelomeningocele.
2 Meningocele.
3 Lipomyelomeningocele.
4 Occult spinal dysraphism:
(a) dermoid tumours
(b) diastematomyelia
(c) intraspinal lipoma
(d) hypertrophic filum terminale.
Spina bifida occulta is a bony deficit usually
found in the laminae of the lumbosacral spine
and due to a midline fusion defect. It is an incidental
radiological finding and is present in up to
20% of adults. In the vast majority there is no neurological
involvement and the lesion is asymptomatic
throughout life.
Myelomeningocele
Myelomeningocele is the most common and important
form of spinal dysraphism presenting
during the neonatal period (Fig. 11.10). It is characterized
by protrusion of the neural elements
through a vertebral defect into a meningeal-lined
sac. Typically, the cord at this level is not fused
but is in its flattened embryological state, with
the nerve roots arising from the ventral surface
and the open central canal lying dorsally. The
major disability from this condition results from
the irreversible neurological deficit caused by
this spinal cord abnormality. Depending on the
level of the defect the spinal cord or conus
and cauda equina may be involved. Although
myelomeningocele may occur at any level, it is
most common in the lumbar and lumbosacral
segments.
This disorder occurs in approximately 1 in
1000 live births. There is a greater frequency
among whites than blacks, with a slight female
predominance. There is a familial incidence: if
one member of a family is affected the risk of the
disorder occurring in subsequent offspring is
about 5%. Seasonal outbreaks of myelomeningocele
have been reported and there are ethnic11
differences, with a higher incidence of the condition
in the western United Kingdom, northern
India and Egypt.
Prenatal diagnosis of myelomeningocele and
other open neural tube disorders is possible by
measuring alpha-fetoprotein in the amniotic
fluid at 1416 weeks and by prenatal ultrasound
examination. The widespread use of increasingly
accurate ultrasound examination in the first
trimester of pregnancy has led to detection of
myelomeningocele, and in many centres detection
is followed by parental decision to terminate
the pregnancy. Consequently there has been a
dramatic decrease in the incidence of children
born with this type of anomaly. The ultrasound
findings can be confirmed by MRI.
Myelomeningocele is frequently associated
with other congenital abnormalities, most
commonly the Chiari type II malformation,
aqueduct stenosis (forking) and hydromyelia
(syringomyelia).
The management of myelomeningocele
involves:
1. Penilaian sac dan selubungnya
2. Evaluasi neurologi
3. Pemeriksaan lanjur kondisi berikut : hidrosefalus atau kelainan ekstrakranial
4. Edukasi orangtua
5. Penjelasan prosedur operasi
1 Assessment of the sac and its coverings.
2 Neurological evaluation.
3 Examination for other associated conditions:
(a) within the CNS, e.g. hydrocephalus
(b) extracranial anomalies, e.g. gastrointestinal,
urinary.
4 Counselling and careful discussions with the
parents.
5 Surgical procedures.
Decisions concerning whether a patient should
undergo a surgical procedure with closure of the
sac are among the most difficult in neurosurgical
practice. On the one hand, the child has a right to
life but there is also a right to a life of sufficient
quality to be worth living. The immediate decision
is whether to close the myelomeningocele
sac. In 1959 physicians in Sheffield undertook a
programme of immediate closure in all neonates,
with aggressive treatment of the hydrocephalus
and other malformations. However, when the
series was examined, only 7% had less than
grossly crippling disability and may be considered
to have a quality of life not inconsistent with
self respect, earning capacity, happiness and
even marriage. Consequently, selective criteria
were developed and the children excluded from
treatment were those with:
paralysis at L2 to L3 or above
marked hydrocephalus
kyphosis
other major congenital abnormalities or birth
injuries.
However, although when these criteria are
used, a large number of untreated infants do not
live long, a significant minority do, and there is
great concern about their quality of life.
The initial operation aims to:
preserve all the neural tissue and reduce it into
the intervertebral canal; untether the spinal cord
obtain a watertight closure of dura lining the
sac
cover the defect with muscle, fascia and skin.
It will subsequently be necessary to make decisions
concerning the treatment of hydrocephalus
and other associated malformations.
The continued care of these children requires a
multidisciplinary approach including urologists,
orthopaedic surgeons, physicians, physiotherapists
and social workers. The children frequently
have severe urological problems, which may result
in renal failure as adolescents. The musculoskeletal
disorders, including talipes and hip
dislocation, require careful orthopaedic management
to maximize any residual lower limb
function.
Lipomyelomeningocele
This is a much less common disorder than
myelomeningocele. The mass is covered by skin
and the lipomatous tissue extends intradurally
and is intimately interwoven with the rootlets
of the cauda equina and the conus medullaris,
which is not usually fused. Neurological examination
is usually normal at birth and progressive
neurological deficits occur, resulting from
growth and tethering of the spinal cord. The most
common symptoms include bladder and bowel
disturbance, back pain and progressive paralysis
in the legs with foot deformities and loss of
sensation.
Surgery is delayed until about 4 months. The
lipoma is removed as completely as possible
without endangering the neural tissue; the
primary surgical aim is to untether the spinal
cord.
Meningocele
This is much less common than myelomeningocele
and is characterized by a cystic lesion
containing only meninges and CSF; it does not
contain any neural tissue.
Occult spinal dysraphism
Spina bifida occulta paling sering mengenai regio lumbal dan bisa menyebabkan defisit neurologis, yang disebabkan lilitan
oleh korda spinalis. Lesi yang umum ditemukan adalah :
- Lipoma intraspinal (lipomyelomeningocele)
- Tumor dermoid
- Diastematomyelia
- Seringkali lesi pada kulit berupa hairy patch, sinus dermal, dimple, hemangioma atau lipoma
Gejala neurologis sering dijumpai pada masa kanak-kanak. Gejala tersebut bersifat progresif lambat, mencakup gangguan
miksi, kelemahan angota gerak bawah, nyeri punggung, disabilitas sensorik, skoliosis yang bersifat progresif
Occult spinal dysraphism includes a number of
spinal disorders, the vast majority of which are
situated in the lumbar region and produce
progressive neurological dysfunction, often as a
result of tethering of the spinal cord. The more
common lesions include:
intraspinal lipomas (and lipomyelomeningocele)
dermoid tumours
diastematomyelia.
The underlying intraspinal lesion can often be
suspected from an overlying skin lesion such as a
dimple, sinus tract, fatty mass, haemangioma or
abnormal tuft of hair. The neurological symptoms
are usually first noted in childhood or adolescence.
There is usually a slowly progressive
neurological dysfunction involving:
bowel and bladder disturbance
progressive weakness of the legs and foot
deformities
back pain
sensory disability in the lower limbs
 progressive scoliosis.
Depending on the level of the abnormality, the
neurological examination will show evidence
of either lower and/or upper motor neurone
damage.

Lipomas are the most frequent form of occult

spinal dysraphism. The lumbosacral lipoma presents
at birth as a soft subcutaneous mass, usually
in the midline and covered by skin. In its
most severe formlipomyelomeningocelethe
lipoma will involve neural tissue and will extend
through a spina bifida. Less severe abnormalities
will be associated with a low-lying conus and
with tethering of the cord by an enlarged filum
terminale or fatty tissue.
Diastematomyelia is a condition in which the
spinal cord is bifid and the two hemicords are
separated by a bony spur or dural band. Progressive
neurological dysfunction will occur due to
traction on the cord that is transfixed during
growth periods (Fig. 11.11).
The cord is occasionally tethered by a shortened,
thickened filum terminalehypertrophic
filum terminaleand the only associated abnormalities
are spina bifida occulta and an occasional
hairy patch over the lower spine.
Radiological assessment will utilize plain Xray,
CT scan (with intrathecal contrast) and MRI.
Plain X-rays will show a range of vertebral abnormalities
including spina bifida, hemivertebrae,
abnormal spinal curves, diastematomyelic
spurs and widened interpedicular distances.
The surgical treatment is aimed at:
removing the underlying pathological cause
while preserving neural tissue
untethering the cord.

In summary the clinical features of spinal cord

compression are:
painlocal and radicular
progressive weakness of the limbs
sensory disturbanceoften a sensory level
sphincter disturbance.

There has been considerable controversy over

surgical intervention in patients with spinal cord
injuries. The damage to the spinal cord occurs
principally at the time of the injury and it is not
surprising that there has been no evidence to
show improved neurological function from acute
operative decompression of the spine. The following
are the general indications for surgical
intervention.
1 Progression of neurological deficit is an absolute
indication. It requires emergency intervention
if a compressive lesion is demonstrated by
either MRI or CT scan.
2 Patients who have a partial neurological injury,
with preservation of some distal neurological
function, and who fail to improve should have
further radiological assessment. Surgery should
be considered if this shows persisting extrinsic
compression of the spinal cord within the canal
(particularly from a herniated cervical disc), a depressed
fractured lamina or an osteophytic bar,
although removal of the compression may not
result in any neurological improvement.
3 An open injury from a gunshot or stab wound
should be explored to remove foreign particles,
elevate bone spicules and, if possible, repair the
dura.
4 The most common indication for operative intervention
is to stabilize the spine. This is usually
undertaken:
(a) if there is gross instability, particularly in
the presence of an incomplete neurological
lesion
(b) if it has not been possible to reduce locked
facets by closed reductionposterior reduction
and fusion is appropriate
(c) if operative procedures are needed to stabilize
the unstable cervical spine and so avoid
prolonged bed rest; these are favoured by
many spinal injury units (Fig. 16.6).

Other treatments for spinal cord injury

Numerous pharmacological agents have been
investigated in experimental laboratory models
but as yet there has been no controlled clinical
study to show the usefulness of these agents in
spinal cord injury. The treatments include glucocorticoids,
diuretics, local hypothermia, barbiturates
and endogenous opiate agonists. Although
there is no proven benefit, some spinal units use
glucocorticoids and osmotic diuretics for the
treatment of oedema. The early use of high-dose
dexamethasone may be beneficial, but there is no
conclusive proof.

Careful attention to the general condition and

metabolic state is essential following the injury;
patients frequently develop a negative nitrogen
balance and may become anaemic. Urinary tract
or other infections will aggravate the metabolic
disorders. The patients and their relatives will require
skilled counselling and emotional support
during the treatment and rehabilitation period.
Skin care
Meticulous attention to pressure areas will avoid
the development of pressure sores which would
seriously complicate the recovery and rehabilitation
of the patient.
Gastrointestinal complications
Paralytic ileus occurs in acute spinal cord paralysis
and if this is unrecognized, the patient is at
risk from vomiting and aspirations. Anasogastric
tube should be passed in the initial management
of the patient. Acute gastric dilatation may also
develop and is also treated by nasogastric suction.
In 35% of patients with acute spinal cord
injuries acute peptic ulceration occurs.
Bladder care
Urinary tract problems are a major cause of
potential morbidity and mortality. During the period
of spinal shock the areflexic, flaccid paralysis
below the level of the lesion includes bladder
function. The patient will develop acute retention
with overflow incontinence and a catheter is
required. Reflex activity returns in upper motor
neurone lesions as the phase of spinal shock passes.
The spinal micturitional reflex arc is intact in a
lesion above the conus and an autonomic type of
bladder results. The bladder will empty involuntarily
as it fills with urine. The capacity may be
less than normal, but there is good voiding pressure
capacity. There is no sensation of bladder
fullness. In a lower motor neurone lesion the
spinal micturitional reflex is interrupted and an
autonomous bladder results. Bladder function is
governed by a myogenic stretch reflex inherent in
the detrusor fibres themselves. This type of dysfunction
is characterized by a linear increase in
intravesical pressure, with filling until capacity is
reached. Urine may then flow past the sphincter
by overflow incontinence. In a mixed upper and
lower motor neurone lesion, such as with a conus
medullaris and cauda equina injury, it is possible
to have a flaccid lower motor neurone detrusor
and a spastic sphincter, as well as the reverse.
The principles of management of the bladder
consist of maintaining proper bladder drainage
to prevent dilatation of the upper urinary tract
and renal impairment, and the treatment of urinary
tract infections. Acatheter is passed initially
and bladder training is commenced after the
period of spinal shock. The details of bladder
training and management depend on whether
the neurogenic bladder has resulted from an
upper or a lower motor neurone lesion and on the
results of a cystometrogram showing the intravesical
volume pressure relationship.

Limb care
It is essential that the paralysed limbs should be
put through their full range of movements regularly.
Physiotherapy, in particular, is essential
to prevent contractures as the tone returns and
spasticity develops.