Disorder of the

Skull and Vertebral

Column
dr. Ravanno Fanizza Harahap

Neurosurgery Department
Faculty of Medicine – Padjajaran University
Bandung 2022
 Disorder of the Skull and Vertebral
Column
• Disorders of the skull and vertebral column range from the mundane and minimally significant, such
as spina bifida occulta, to the horrific, such as disfiguring craniosynostosis syndromes.
• Type of disorders that might happen :

Congenital and developmental skeletal disorders

Trauma Trauma and skull fractures

Tumors

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Developmental anomalies
Primary Spinal Anomalies Primary Intracranial Anomalies

• Spinal Arachnoid Cysts

• Arachnoid cysts, intracranial
• Spinal Dysraphisms (spina bifida)
• Craniofacial development 
• Myelomeningocele
craniosynostosis
• Lipomyeloschisis
• Encephalocele
• Dermal sinus
• Dandy Walker malformation
• Klippel-Feil syndrome
• Aqueductal stenosis
• Tethered cord syndrome
• Agenesis of the corpus callosum
• Split cord malformation
• Intracranial lipomas, etc
• Lumbosacral nerve root anomalies

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Congenital and developmental skeletal
disorders
Cranial Dysraphism Spinal Dysraphism

Congenital developmental cranial disorder composed of wide variety group of disorders. These
malformation including :
• Defects of Brain Induction: Hesx1 and Septo-optic Dysplasia
• Defects of Cranial Neurulation: Anencephaly
• Defects of Isthmic Organizer Activity: Brainstem and Cerebellar Malformations (Including
Dandy Walker Malformation)
• Cranial Dysraphism / cephalocele
• Craniosynostosis Syndrome

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Cranial dysraphims

• The cranial dysraphisms can be conceptualized along a spectrum of severity, comprising cranial
dermal sinus tracts at one end, encephaloceles at the other, and meningoceles between the two

Encephaloceles

Cranial Dermal Sinus Tract

ETC

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Craniosynostosis (CSO)
• Craniosynostosis : the premature ossification of a cranial suture.
• Incidence: ≈ 0.6/1000 live births.
• May occur in isolation, but may also be syndromic or secondary

Primary
Prenatal deformity

Secondary Etiologies of secondary CSO include: metabolic (rickets,

hyperthyroidism), toxic, hematologic, structural (lack of brain
growth)

Treatment :
• Treatment is usually surgical.
• In most instances, the indication for surgery is for cosmesis and to prevent the severe psychological
effects of having a disfiguring deformity.

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 DIAGNOSIS OF CSO
• Palpation of a bony prominence over the suspected synostotic suture (exception:
lambdoidal synostosis may produce a trough)
• Gentle firm pressure with the thumbs fails to cause relative movement of the bones on
either side of the suture
• Plain skull x-rays
• Ct scan
• Technetium bone scan
• MRI
• Measurements, such as occipito-frontal-circumference may not be abnormal even in the
face of a deformed skull shape

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 CSO
types
• 4 types of single suture craniosynostosis and 1 type of positional deformity.
• Red arrows identify the involved suture which is depicted as a white line.
• Blue arrowheads depict the direction of the deformities.
• Postural plagiocephaly is a positional deformity and is not a craniosynostosis.
• Broken lines illustrate the concept that posterior plagiocephaly produces a
trapezoidal-shaped deformity (note the ipsilateral ear is displaced posteriorly),
whereas postural plagiocephaly bears more resemblance to a parallelogram
(with the ipsilateral ear shifted anteriorly).

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Surgical treatment of cso
• Skin incision may be longitudinal or transverse. A linear “strip” craniectomy is performed,
excising the sagittal suture from the coronal to the lambdoid suture, preferably within the first 3–6
months of life.
• The width of the strip should be at least 3 cm; no proof exists that interposing artificial substances
(e.g. silastic sheeting over the exposed edges of the parietal bone) retards the recurrence of
synostosis.
• Great care is taken to avoid dural laceration with potential injury to the underlying superior
sagittal sinus.
• The child is followed and reoperated if fusion recurs before 6 months of age. After ≈ 1 yr of age,
more extensive cranial remodelling is usually required.

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Craniofacial dysmorphic syndromes

Greenberg, Mark S. (2019). Handbook of Neurosurgery. Thieme, New York. Ch. 13, 243 – 252
 Spinal dysraphism
• Spinal dysraphism encompasses congenital problems that result in an abnormal bony formation of
the spine and/or the spinal cord  caused by the maldevelopment of the ectodermal, mesodermal,
and neuroectodermal tissues.
• Two major types :

Aperta (open) Occulta (Closed)

• Common manifestations are meningocele, myelomeningocele, lipomeningocele,

lipomyelomenigocele, myeloschisis, and rachischisis.

Greenberg, Mark S. (2019). Handbook of Neurosurgery. Thieme, New York. Ch. 13, 243 – 252
 Etiology and Risk Factors
Etiology :
• Nutritional deficiencies  folic acid
• Antiepileptics and Other Risk Factors 
Carbamazepine and valproic acid 
teratogenic, with an associated 1% risk of
having a child with spina bifida
• Associated Chromosomal Abnormalities
 trisomy 13, trisomy 18

Youmans and Winns – Neurological Surgery 7th Edition

 Spinal dysraphism
• Etiology : multifactorial  low perinatal folic acid intake, genetic inheritance and racial
backgrounds
• Epidemiology : The incidence of all forms of spinal dysraphism (open and closed) is 0.5 to 8
cases per 1,000 live births (wideworld)

Pathophysiology :
• Spinal dysraphism results from abnormal cell migration and differentiation of the
neural tube during the first trimester of pregnancy.
• The pathophysiology of myelomeningocele is a failed closure of neural tube during
primary neurulation producing a cystic mass of neuronal elements including dural,
arachnoid, spinal cord, nerve roots, cerebral spinal fluid through the bone and skin
defect

Greenberg, Mark S. (2019). Handbook of Neurosurgery. Thieme, New York. Ch. 13, 243 – 252
 DIAGNOSIS

MATERNAL High-Resolution AMNIOCENTESI

SERUM Α- Fetal
Ultrasonography
MRI S
fetoporotein
accurately screens for second-line imaging Levels of amniotic AFP and
Measurement between NTDs almost 100% of technique (when US not acetylcholinesterase (ache) as
16-18 weeks the time available) confirmatory test

Youmans and Winns – Neurological Surgery 7th Edition

 Prognosis and Prenatal Counseling
• With the successful hydrocephalus treatment  More than 95% of infants born with
myelomeningocele survive to 2 years of age.
• Cognitive development : if hydrocephalus and infections are managed aggressively, the
majority of children (75%- 80%) can have normal intelligence
• Motor Function : lesion with the upper border above T12 generally results in flaccid
paraplegia.
• Urinary and Fecal Continence Most children with spina bifida are incontinent of urine, with
normal urinary continence seen in only 6% to 17% of children.
• Risk in siblings : myelomeningocele  Autosomal dominant, autosomal recessive, and X-
linked inheritance patterns

Youmans and Winns – Neurological Surgery 7th Edition

 MANAGEMENT :
Perinatal Management Surgery

Perinatal Management

• In Utero Repair : prenatal surgical repair  greater improvement but increasing risk of
preterm delivery and uterine dehiscence at delivery
• Delivery : elective cesarian sections found a 2.2-fold increase in risk of neurological injury if
labor occurred.
• Neurosurgical Evaluation : maintain normothermia and euvolemia, gauze dressing covering
neural placode, Prophylactic antibiotics
• Plastic Surgery  planning of skin closure with minimal complication

Youmans and Winns – Neurological Surgery 7th Edition

 MANAGEMENT :
Perinatal Management Surgery

Surgery

• Timing : Myelomeningocele repair should be performed within the first 72 hours after birth. If
delayed  increase morbidity and mortality.
• Preparation and Positioning : ABC  thus avoid hypothermia, hypovolemia, and
hypoglycemia  intubation
• Hydrocephalus : Children with severe hydrocephalus at birth may require immediate CSF
diversion prior to, or in conjunction with, myelomeningocele repair in order to minimize
pressure on the dural closure.

Youmans and Winns – Neurological Surgery 7th Edition

 SKULL FRACTURES :
ETIOPATHOPHYSIOLOGY

• Direct impact to skull → inbending or outbending

of skull beyond its elastic tolerance.
• Skull fracture  indicator of severe blow to head
• Skull fracture does not necessarily mean that brain
is injured (but in many cases it is).

Greenberg, Mark S. (2019). Handbook of Neurosurgery. Thieme, New York. Ch. 13, 243 – 252
 Types of Skull Fractures
Communication with outside : Underlying cerebral substance damage:

Closed, and open fractures No injury, Compression (by depressed fracture),

contusion, laceration (by depressed fractures)
Location :

Vault & Basilar

Fracture Form
Linear (incl. sutural diastasis
Diastatic fractures extend into and
separate sutures. More common
in young children) Depressed,
and Comminuted Open Fracture

Greenberg, Mark S. (2019). Handbook of Neurosurgery. Thieme, New York. Ch. 13, 243 – 252
 Figures of Skull Trauma

Greenberg, Mark S. (2019). Handbook of Neurosurgery. Thieme, New York. Ch. 13, 243 – 252
 SPINAL TRAUMA

• Spine injuries
• Occipitoatlantoaxial injuries
• Subaxial injuries / fractures
• Thoracic, lumbar, sacral spine fractures
• Penetrating spine injuries
 SPINAL CORD INJURY
A spinal cord injury (SCI) is damage to any part of the spinal cord or nerves extending from the
spinal cord that often results in permanent changes in motor and/or sensory abilities and other body
functions below the point of the injury.

Epidemiology

SCI most commonly affects young, otherwise healthy and productive individuals. Males > females,
with ratio 4:1.

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 PHASES OF SPINAL CORD INJURY

Primary injury Secondary injury

Damage that occurs at the time of the

Delayed, progressive damage that
initial trauma and that causes
continues after cessation of primary
immediate severing of axons and death
injury and that can last for weeks or
of spinal cord cells
even years

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Main Types Of Incomplete
Injury Syndrome

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Main Types Of Incomplete Injury Syndrome
Central Cord Syndrome

Damaged usually in the center part of the spinal cord, and

would usually experience more profound weakness and a lack
of function in the upper limbs compared with the involvement
of the lower limbs  Good recovery and further
T2 weighted sagittal MR image shows a C5-6
improvement. Most people with central cord syndrome will disc rupture and diffuse hyperintensity cord
lesion at C5-C6 level
have an ASIA scale classification of C or D.

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Main Types Of Incomplete Injury Syndrome
Anterior Cord Syndrome

Damage to the front part of the spinal cord will usually result
in partial or complete loss of movement as well as pain,
temperature, and touch sensations below the level of injury.
Some pressure sensation and position sense may be retained.
Most people with anterior cord syndrome will have an ASIA
The owl’s eyes sign
scale classification of B.

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Main Types Of Incomplete Injury Syndrome

Posterior Cord Syndrome

Damage to the back of the spinal cord may leave good muscle power, pain and temperature

sensation, but create difficulties in movement coordination. This is very rare.

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Main Types Of Incomplete Injury Syndrome
Brown Sequard Syndrome

The damage is mainly on one side of the cord. On the injured

side, muscle power may be reduced or absent, and pressure
and position sense are disordered. The other side experiences
loss of, or reduced sensations of pain and temperature but
movement, pressure and position sense tend to remain. ASIA
One and a half brown Sequard syndrome
scale classification of C or D.

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Main Types Of Incomplete Injury Syndrome

Cauda Equina Lesion

Also known as horses tail of nerves that spread out from the
base of the spinal cord. An injured cauda equina may result in
patchy loss of power and sensation in the lower limbs. The
bladder and bowel are usually severely affected. Functional
recovery can happen over 12-18 months if the roots of the
nerves are not permanently damaged.

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 SECONDARY INSULTS AND THE CURRENT CLINICAL
APPROACH TO ACUTE SPINAL CORD INJURY

Clinical management of acute SCI  largely aimed at preventing further injury and optimizing
the provision of nutrients to the injured tissue

Secondary Insults and the Importance of Avoiding Them

Secondary insults are distinct from secondary injury  occur at the level of the organism and lead
to deficient provision of nutrients to injured CNS tissue. Two secondary insults commonly seen
after SCI  Hypotension and hypoxia  worsen neurological injury. priority in managing acute
SCI is assessment and stabilization of vital signs, ATLS management  in order ABC.

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Clinical Approach To Acute Spinal Cord Injury

Neurogenic Shock and Spinal

Shock
Hypotension  secondary insult that is critical to avoid after SCI. Causes : neurogenic
shock, and spinal shock.

Winn. Youmans Neurological Surgery 7th edition. Philadelphia, PA: Saunders, 2011.

 Tumors of Cranial, Spinal, and Peripheral
Nerves

1. Vestibular schwannoma
2. Perineurioma (WHO grade I-III)
3. Malignant peripheral nerve sheath tumors
(MPNST) (no WHO grade)

Greenberg, Mark S. (2019). Handbook of Neurosurgery. Thieme, New York. Ch. 13, 243 – 252
Greenberg, Mark S. (2019). Handbook of Neurosurgery. Thieme, New York. Ch. 13, 243 – 252
Thank
YOu