Spinal Disorders: Fundamentals of Diagnosis and Treatment Part 83 pdf

Asymptomatic patients with tethered cord should be instructed to avoid the fol-lowing activities because of the risk of a potential sudden neurological deteriora-tion [57]: deep bending

from 16 – 20 weeks However, spina bifida may be missed, particularly in the

L5 – S2 region [24, 44]

Magnetic Resonance Imaging

MRI is the modality of choice for prenatal imaging

Since its advent, MRI has become the imaging modality of choice While

ultraso-nography is an excellent screening procedure, it requires considerable expertise

to interpret, whereas MRI is definitive Prenatal MRI can also be used to

charac-terize the Chiari II and other associated malformations [24] Prenatal imaging

studies help to predict neurological deficits

Postnatal Diagnostic Tests

Imaging Studies

For evaluation of the spinal cord malformations and tethered cord syndrome, the

most helpful diagnostic images are obtained by MRI, which provides excellent

details of anatomy and characterization of soft tissue anomalies [39, 58] Other

imaging studies, including standard radiographs and CT, may also be helpful

Plain radiographs will show vertebral anomalies A CT scan is particularly useful

for the evaluation of bony anomalies and split cord malformations [34, 39]

Magnetic Resonance Imaging

The best demonstration of the entire craniospinal axis is made by MRI and

should be performed after the birth whenever possible The T1- and T2-weighted

MR images in the sagittal and axial planes provide excellent demonstrations of

the anatomopathological characterization of the components of the

malforma-tion, i.e relationship between placode and nerve roots and other associated

sequences (Chiari II, hydrocephalus, hydromyelia) [32]

Investigate the entire neural axis when spinal malfor-mations are suspected

Before the MRI era, it had been assumed that after untethering, there would be

upward migration of the spinal cord, which in fact does not occur in most cases

[19] Postoperative follow-up MRI almost always shows low-lying conus and

should not be confused with a “retethering” [10] The diagnosis of retethering

and decision for untethering requires clinical judgment Attempts to improve

conventional MRI techniques, including the use of prone positioning [10],

upright MRI and dynamic phase MRI, have been investigated but await

valida-tion through further studies [19]

Urodynamic Studies

Urodynamic studies may show low bladder capacity and overflow incontinence,

and may serve as a baseline for postoperative follow-up [15]

Treatment

It is important to recognize tethering of the spinal cord as early as possible Once

the neurological deficits have occurred, many patients will not have recovery of

lost functions

Tethered cord should be treated as soon as possible

Although the underlying causes of tethered cord vary, the signs and symptoms

of tethering are generally the same Individuals with spinal malformations need

both surgical and medical lifelong management which should be provided by a

multidisciplinary team

Asymptomatic patients with tethered cord should be instructed to avoid the

fol-lowing activities because of the risk of a potential sudden neurological deteriora-tion [57]:

) deep bending (touching the toes, high leg kicking) ) holding any weight while standing that causes back and leg pain ) sitting position such as the Buddha pose

) sitting in a slouching position ) horse riding

) skiing at high altitude (might produce spinal hypoxia) ) Valsalva-type maneuvers to prevent spinal venous congestion

In Utero Treatment

Fetal surgery for spinal

dysraphism is feasible

After a diagnosis of fetal spinal dysraphism, there are two choices: either

termi-nation or fetal surgery [24] The period of legal termitermi-nation differs between

countries The first cases of in utero open spinal dysraphism repair were done in

1994 but proved unsatisfactory [3] In 1997, in utero repair by hysterotomy was reported [3, 20] Up to 2004, more than 200 in utero, open spinal dysraphism clo-sures are estimated to have been done [20] Urodynamic and lower extremity function seem to be similar in infants treated in utero and postnatally [20] Com-pared with historical controls, infants treated in utero have a lower incidence of Chiari II and hydrocephalus requiring shunting [3, 20] Delivery via cesarean sec-tion is preferred [28]

Postnatal Surgery

Open spinal dysraphisms must be treated surgically as early as possible (Table 6):

Table 6 General aims of surgery

) untether the spinal cord ) prevent infections ) repair of the dural/cutaneous defect ) restore normal anatomy as far as possible

Closure of the spinal lesions is usually done within 48 – 72 h of birth [20, 28, 58]

If there are signs of hydrocephalus, a shunt is placed at the same time as the lesion

is closed

There are some standard rules for closure of open spinal dysraphism, but in

many cases the surgeon must vary the technique on the basis of individual anat-omy The surgical microscope should assist in defining distorted anatomy and associated pathologies in great detail The interested reader is referred to repre-sentative articles in the literature and textbooks [26, 28, 31, 50, 58]

Open Spinal Dysraphism

After careful and extensive dissection of the sac from the neural placode, neural tissue is repositioned into the dural sac to preserve functional neural tissue There is no proven technique for closure of myelomeningocele at the time of the original surgery that will prevent retethering However, there are some tech-niques that may minimize the amount of retethering that occur: The neural pla-code can be folded over and anatomically made into a tube by suturing the edges

of the open placode together It does not prevent retethering, but it seems to make the surgery for untethering easier Sometimes the use of vascularized flaps may

be necessary

Closed Spinal Dysraphism

In the cases of closed spinal dysraphisms, the associated lesions need careful

dis-section In split cord malformation, after opening the dura, complete excision of

the bony spur or fibrous septum is performed A thickened or fatty filum

termi-nale is cut and also released to detether the cord Sometimes, closure of the dura

is a problem In these cases, it is necessary to use fascia lata or synthetic dura

sub-stitutes to repair the dural deficiency Wound closure is done in multiple layers in

order to prevent liquor leak

Tethered Cord Syndrome

Surgery for tethered cord must be early

In open spinal dysraphism, short- and long-term survival has increased with

improvements in medical and surgical management Surgical intervention for

tethered spinal cord must be as early as possible to prevent progressive neural

tis-sue damage Once neurological function is lost it may never recover The value of

early prophylactic surgical intervention in tethered cord is evident in the

litera-The only effective treatment

is surgical untethering

ture [16, 35, 48] The only effective treatment is surgical untethering of the spinal

cord from the underlying cause The goal of the untethering surgery is to stop any

further neurological deterioration [35, 48] One of the current controversies with

respect to tethered cord management includes the untethering of the spinal cord

in asymptomatic patients The majority of authors recommend prophylactic

sur-gery [16, 48]

The decision about the surgical technique should be made individually on a

case-by-case basis The special details of the various surgical techniques are

beyond the scope of this chapter Several excellent textbooks exist in the field of

spinal malformations–tethered cord surgery Interested readers are referred to

representative articles in the literature and these textbooks and atlases [26, 28, 31,

50, 58]

Untethering is generally a safe surgical procedure in experienced hands [16].

Complications include infection, bleeding, and damage to the functional part of

the spinal cord Although the causes of tethered cord vary, the general principles

of the surgery are similar

The operating microscope and microsurgical technique are necessary for

bet-ter visualization and precise dissection Different instrumentations are used to

perform the dissection including endoscopy, ultrasonic aspirator, and lasers; one

method is not necessarily better than the others, and the surgeon usually has her

or his own preference based upon their experience [8, 10, 48]

Intraoperative neuromoni-toring and the microscope are invaluable intraoperative aids

Various intraoperative monitoring techniques such somatosensory evoked

potentials (SSEPs), lower extremity and anal sphincter EMGs, external anal

sphincter monitoring and nerve root stimulation studies are helpful to identify

functional elements [15, 58] But it remains valid that the most important factor

for a good postoperative result is the experience of the surgeon in handling these

complex anomalies [12] Retethering remains a risk and requires reexploration if

signs of tethered cord syndrome are seen

In secondary tethered cord the untethering procedure usually involves

open-ing and dissectopen-ing the scar from the prior closure

Epidemiology. Neural tube defects are the most

common congenital abnormalities of the central

nervous system

Classification. Spinal cord malformations can be

classified based on the pathomorphological

pre-sentation as presenting with and without a back

mass A secondary discriminator is related to the

coverage with skin in the presence of a back mass.

The vast majority of spinal cord malformations

re-sult in a tethering of the spinal cord We

differenti-ate primary tethered cord as a result of spinal

mal-formations and secondary tethered cord which

re-sults from a surgical intervention

Pathogenesis.Spinal cord malformations ( = spinal

dysraphism) arise from defects occurring in the

em-bryological stages of gastrulation (weeks 2 – 3),

neurulation (weeks 3 – 6) and caudal regression

There is an increased risk of spinal malformations

in pregnant women who are taking certain drugs

An increased risk of spinal malformation is

associat-ed especially with exposure to valproic acid or

carb-amazepine Patients with myelomeningocele and

myelocele almost always have associated Chiari II

malformation Hydromyelia may occur in as many

as 80 % of these patients, and may be localized or

extend through the whole cord It may cause rapid

development of scoliosis if left untreated

Classical-ly tethered cord is defined as having the tip of the

conus below the L2 disc space and a pathologically

elongated spinal cord However, in the medical

lit-erature, there are many publications of tethered

cord syndrome with the conus in a normal position

Clinical presentation. Tethered cord–spinal cord

malformations are usually diagnosed at birth or

early infancy (open spinal dysraphism, closed

spi-nal dysraphisms with back mass) but sometimes

are discovered in older children or adults Tethered

spinal cord should be highly suspected and

consid-ered in the differential diagnosis of patients who

present with cutaneous midline abnormalities,

low back pain, lower extremity and foot

deformi-ties, subtle neurological deficits, and bladder and

sexual dysfunctions Irreversible neuronal damage

can occur when there is sudden stretching of the al-ready chronically tethered conus

Diagnostic work-up The prenatal examination

en-compasses maternal serum [ -fetoprotein examina-tion and ultrasound The advent of diagnostic

mo-dalities such as MRI has increased the number of

tethered spinal cord diagnoses and will require awareness and prompt multidisciplinary manage-ment of the syndrome before neuronal loss ad-vances Since multiple tethering lesions and cere-bral anomalies coexist in a significant number of cases, it is absolutely necessary to investigate these patients with craniospinal MRI to screen the entire neuroaxis

Prenatal treatment. It is important to counsel women of childbearing age about the need to take dietary supplements containing foliate before be-coming pregnant Up to 70 % of spina bifida cases can be prevented by periconceptional folic acid supplementation Intrauterine surgery is possible but superiority over postpartum surgery needs to

be established

Postnatal treatment.Individuals with spinal malfor-mations need both surgical and medical lifelong management which should be provided by a

multi-disciplinary team Open spinal dysraphism requires

immediate surgery (within 2 – 3 days postpartum).

Main goal of surgery is to untether the spinal cord, prevent infections, repair the dural/cutaneous de-fect, and restore normal anatomy as far as possible Mainly the goal of the untethering is to stabilize the progressive neurological deterioration but some authors recommend a prophylactic untethering procedure for asymptomatic patients Early unteth-ering, when minimum or mild symptoms are

detect-ed, is essential for tethered cord syndrome treat-ment Surgical intervention for tethered cord in-volves identification of the tethering lesion, release

of the spinal cord and reconstruction of the normal anatomy as soon as possible The operating micro-scope and microsurgical technique are necessary for better visualization and precise dissection Intra-operative neuromonitoring is useful

Key Articles

Yamada S ( 1996) Tethered cord syndrome The American Association of Neurological

Surgeons, Park Ridge, Illinois

This is a first and excellent textbook on tethered cord syndrome There are 16 chapters on

embryology, pathophysiology, diagnosis, imaging, and therapy that cover all aspects of

the syndrome All chapters are superb didactically not only for neurosurgeons but also

for orthopedic surgeons, neurologists, pediatricians, and urologists.

Pang D ( 1995) Disorders of the pediatric spine Raven Press, New York

This book covers perfectly all aspects of childhood spine, beginning with a section on

embryology and biomechanics, and bridging the philosophies of orthopedic surgeons

and neurosurgeons by including chapters written by these two specialties A large section

is devoted to the many congenital malformations with deeply detailed definitions, nice

photos and drawings of operative techniques.

Tortori-Donati P, Rossi A, Cama A ( 2000) Spinal dysraphism: a review of

neuroradiolog-ical features with embryologneuroradiolog-ical correlations and proposal for a new classification

Neu-roradiology 42(7):471–91

This paper presents the correlation between anatomy, embryology, neuroradiology and

clinical findings of spinal dysraphism and formulates a working classification of these

malformations.

Mitchell LE, Adzick NS, Melchionne J, Pasquariello PS, Sutton LN, Whitehead AS ( 2004)

Spina bifida Lancet 364:1885–1895

This is an excellent review which highlights the key features of spina bifida.

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Cervical Spine Injuries

Michael Heinzelmann, Karim Eid, Norbert Boos

Core Messages

✔Cervical spine injuries account for about

one-third of all spinal injuries and the most

com-monly injured vertebrae are C2, C6 and C7

✔A neurological deficit occurs in about 15 % of

all spinal injuries

✔Atlas burst fractures result from axial

compres-sion in slight extencompres-sion, dens fractures are due

to a combination of horizontal shear and

verti-cal compression, and traumatic

spondylolisthe-sis is caused by an extension-distraction injury

✔The flexed lower cervical spine is susceptible to

ligamentous injuries without fractures on axial

loading, which can result in bilateral facet

sub-luxation or sub-luxation Additional rotation leads

to unilateral dislocations

✔Whiplash associated disorders, which

fre-quently result from rear-end collisions, tend to

become chronic in about half of injured

indi-viduals Late whiplash disorders have strong

similarities with chronic pain syndrome

✔The assessment of vital and neurological

func-tions is a priority in cervical injuries

✔Polytraumatized and head injury patients are at

very high risk of having sustained a cervical

injury

✔Standard radiography is indicated in cervical

injuries according to the Canadian C-Spine Rule

or NEXUS criteria

✔CT is the imaging modality of choice for the

evaluation of cervical fracture/dislocation but

MRI can add important information with regard

to neural compromise and injury to the

disco-ligamentous complex

✔Patients with a cervical sprain/strain or

whip-lash injury should be treated with reassurance

about the absence of serious pathology (after

diagnostic assessment), education about the

prognosis, early return to normal activities and

physical exercises (if needed)

✔Fracture reduction by traction and/or urgent

decompression is recommended in patients with progressive or incomplete SCI and persis-tent spinal cord compression

✔Traction must not be applied before ruling out

atlanto-occipital or discoligamentous dislocation

✔Occipital condyle fractures, atlanto-occipital

dislocation and atlantoaxial instabilities are rel-atively rare after trauma but must not be over-looked

✔Unstable burst (Jefferson) fractures of the atlas

must be treated by rigid external fixation or surgery (C1/2 or Judet screw fixation)

✔Type I and III dens fractures can be treated

non-operatively by rigid external fixation but Type II fractures require a surgical approach because

of the high non-union rate

✔Type II dens fractures are treated by anterior

screw fixation or posterior atlantoaxial instru-mented fusion in cases with delayed union or advanced age

✔Traumatic spondylolisthesis of the axis can be

treated non-operatively in Type I fractures, while Type II and III require anterior or posterior instrumented fusion

✔Lower cervical spine fractures can be classified

into Type A (compression), Type B (distraction) and Type C (rotation) injuries

✔Type A injuries are usually treated

conserva-tively in the absence of severe anterior column involvement and neurological deficits

✔Type B and Type C injuries should be treated

operatively by anterior or posterior instru-mented fusion

✔Most lower cervical spine injuries can be

treated successfully by an anterior approach

✔Facet dislocation injuries require closed or open

reduction and adequate fixation with rigid external or internal fixation

a b c

d

Case Introduction

This 20-year-old male patient had a

mo-tor vehicle accident with a polytrauma.

Extraspinal injuries included a closed

head injury (Glasgow Coma Scale 6) with

shearing injuries and consecutive

intra-cranial pressure monitoring for 2 weeks, a

thorax injury with lung contusions,

bilat-eral hematopneumothorax, manubrium

sterni fracture, and multilevel spinal

inju-ries with fractures of the vertebrae T6, T8,

T10, T12 and L3 The thoracolumbar spinal fractures were treated conservatively In addition, a traumatic spondylolisthe-sis C2 (Type Effendi II) was initially treated conservatively (a) After 6 weeks, the instability of the C2 injury became obvi-ous, as shown in the standard lateral radiographs (b) and the CT scan (c) The small bony fragment indicates a rupture of the disc C2/C3 The fractures of the pedicles C2 are shown in the CT scan (d,e) The ruptured disc C2/C3 was removed and replaced with a tricortical iliac crest bone graft Subsequently, the cervical spine was stabilized with an anterior plate The lateral views demonstrate the radiographs/CT scan taken during the operation (f), postoperatively (g,h), and after

9 months (i) Note that the fractures of the arc/pedicles healed after 9 months.

The most commonly injured vertebrae are C2, C6, and C7

Cervical spine injuries account for one-third

of all spinal injuries

Cervical spine injuries account for about one-third of all spinal injuries

Gold-berg et al [89] prospectively studied 34 069 patients with blunt trauma

undergo-ing cervical spine radiographs at 21 institutions to accurately assess the

preva-lence, spectrum, and distribution of cervical spine injury after blunt trauma Of

these patients, 818 (2.4 %) had a total of 1 496 distinct cervical spine injuries The

second cervical vertebra was the most common (24.0 %) level of injury, one-third

of which were odontoid fractures In the subaxial spine, C6 and C7 were the most

frequently affected levels (40 %) The most frequent fracture site was the

verte-bral body Nearly two-thirds of all injuries (71 %) were considered clinically

sig-nificant

A neurological injury occurs

in about 15 % of spine trauma patients

In order to evaluate the true incidence of spinal column and cord injury, Hu et

al [108] used the database of the Manitoba Health Services Insurance Plan

(1981 – 1984) to identify all patients who had spinal injuries The annual

inci-dence rate of all spinal fractures was 64 per 100 000 A total of 2 063 patients were

identified, 944 of whom were admitted to hospital There were two incidence

peaks, one occurring in young men and the other in elderly women Of the

hospi-talized patients, 182 had cervical injury, 286 had thoracic fracture, and 403 had

injury in the lumbosacral spine Associated injuries occurred in 38 % of

hospital-ized patients Neurological injury occurred in 122 patients (13 %)

A low GCS indicates

a high risk for a concomitant cervical injury

In a retrospective review of 14 577 blunt trauma victims in a tertiary referral

center in Baltimore, 614 (4.2 %) had cervical spine injuries In a series of 14 755

trauma cases in Los Angeles [64], 292 (2 %) patients had cervical spinal injuries

Of these, 86 % had fractures, 10 % had subluxations and 4 % had an isolated

spi-nal cord injury without fracture or obvious ligamentous damage Importantly,

the incidence of cervical injuries increased in patients with a low Glasgow Coma

Scale (GCS) score, indicating that patients with a relevant head injury are at risk

of having concomitant cervical injuries The combination of head injury and

cer-vical spine injury represents a difficult diagnostic problem due to the lack of

con-sciousness in these patients In a consecutive study of 447 patients with head

injuries [106], 24 (5.4 %) patients suffered a cervical spine injury Of these, 14

(58 %) sustained spinal cord injuries Furthermore, patients with a GCS of less

than 9 have an almost 3 times higher risk of sustaining a cervical injury [64]

Sim-ilarly, patients involved in motor vehicle accidents – either as passengers or as

pedestrians – are at high risk of sustaining cervical spine injuries Alker et al

examined 312 victims from traffic accidents and found cervical spine injuries in

24.4 % Of these, 93 % affected the upper cervical spine [15]

A specific entity of cervical injuries (sprains and strains) is related to rear-end

or side impact motor vehicle collisions [184], but can also occur during diving or

other mishaps [201] In the United States, neck strain/sprain is the most

com-mon type of injury to motor vehicle occupants treated in US hospital emergency

departments, with an annual incidence of 328 per 100 000 inhabitants [158] The

impact during the motor vehicle collision may result in bony or soft-tissue

inju-ries (whiplash injury), which in turn may lead to a variety of clinical symptoms

(whiplash-associated disorders, WAD) [184].

Injury mechanism and symptoms after rear-end collision must be differentiated

The unfortunate term “whiplash” was introduced into the literature by Crowe

in 1928 [55] This expression was intended to be a description of a motion, but it

has been accepted by physicians, patients and attorneys as the name of a disease

This misunderstanding has led to its misapplication by many physicians and

oth-ers over the years [55]

The incidence of WAD is substantially increasing Reliable epidemiological data on this type of injury is hampered by the fact

that definitions are largely variable [181] Depending on the definition of