SPINA BIFIDA & TORTICOLLIS

PRESENTER MODERATOR
DR.K.BHANU PRAKASH DR.RATHNA KUMAR
2ND YEAR PG ASSISTANT PROFESSOR
DEPARTMENT OF ORTHOPAEDICS
SPINAL BIFIDA
 DEFINITION:
 Spina bifida (Latin: "split spine") is a developmental
congenital disorder caused by the incomplete closing of
the embryonic neural tube.
 Some vertebrae overlying the spinal cord are not
fully formed and remain unfused and open.
 If the opening is large enough, this allows a portion of
the spinal cord to protrude through the opening in the
bones.
 There may or may not be a fluid-filled sac
surrounding the spinal cord.
 INCIDENCE:
 Spina bifida is one ofmost common birth
defects, with an average worldwide incidence of one to
two cases per 1000 births, but certain populations have a
significantly greater risk.
 Myelomeningocele is the most significant and common
form, and this leads to disability in most affected
individuals.
 This condition is more likely to appear in females; the
cause for this is unknown.
 CAUSES:
 Maternal diabetes
 Family history
 Obesity
 Increased body temperature from fever
or external
• sources such as hot tubs and electric
blankets may increase the chances of
delivery of a baby with a spina bifida.
 Medications such as some

anticonvulsants.
 Pregnant women taking Valproic acid have
 EMBRYOLOGY:
 Spina bifida is caused by the failure of the neural tube
to close during the first month of
embryonic development (often before the mother knows
she is pregnant).
 Under normal circumstances, the closure of the neural
tube occurs around the 23rd (rostral closure) and 27th
(caudal closure) day after fertilization.
TYPES:

 Spina bifida malformations fall into three categories:

 spina bifida occulta
 spina bifida cystica with meningocele
 spina bifida cystica with myelomeningocele.
(The most common location of the malformations is the
lumbar and sacral areas)
SPINA BIFIDA OCCULTA:
 Occulta is Latin for "hidden". This is the
mildest form of spina bifida.
 In occulta, the outer part of some of the vertebrae is not
completely closed.
 The splits in the vertebrae are so small that
the spinal cord does not protrude.
 The skin at the site of the lesion may be normal, or it
may have some hair growing from it; there may be a
dimple in the skin, or a birthmark.
 The incidence of spina bifida occulta is
approximately 10% of the population, and most people
are diagnosed incidentally from spinal X- rays
MENINGOCELE:

 The least common form of spina bifida is a posterior

meningocele (or meningeal cyst). In this form, the
vertebrae develop normally, but the meninges are forced
into the gaps between the vertebrae.
MYELOMENINGOCELE:

 This type of spina bifida often results in the most severe

complications.
 In individuals with myelomeningocele, the unfused
portion of the spinal column allows the spinal cord to
protrude through an opening.
 The meningeal membranes that cover the spinal cord form
a sac enclosing the spinal elements.
 Spina bifida with myeloschisis is the most severe form of
myelomeningocele. In this type, the involved area is
represented by a flattened, plate-like mass of nervous tissue
with no overlying membrane.
 The exposure of these nerves and tissues make the baby
more prone to life-threatening infections such as
meningitis.
 CONTD…
 The protruded portion of the spinal cord and the
nerves that originate at that level of the cord are
damaged or not properly developed.
 As a result, there is usually some degree
of paralysis and loss of sensation below the level of the
spinal cord defect.
 CLINICAL MANIFESTATIONS:

Physical Signs:
 Orthopedic abnormalities (i.e., club foot, hip
dislocation)
 Bladder and bowel control problems, including
incontinence, urinary tract infections, and poor renal
function.
 Pressure sores and skin irritations

 Abnormal eye movement

 68% of children with spina bifida have

an allergy to latex
 Paralysis
 CONTD…
 Scoliosis
 Back pain
 Partial or complete lack of sensation
 Weakness of the hips, legs, or feet of a newborn
 Other symptoms may include:
 Hair at the back part of the pelvis called the sacral area
 Dimpling of the sacral area
 Difficulty swallowing, which can lead to choking.
 Hoarseness.
 Breath-holding and problems breathing during sleep.
 Below-average intelligence.
NEUROLOGICAL COMPLICATIONS:

 Many individuals with spina bifida have an

associated abnormality of the cerebellum, called the
Arnold Chiari II malformation. In affected
individuals, the back portion of the brain is displaced
from the back of the skull down into the upper neck.
EXECUTIVE FUNCTION:

 Specific areas of difficulty in some individuals include

planning, organizing, initiating, and working memory.
Problem-solving, abstraction, and visual planning may
also be impaired.
 Children with spina bifida and shunted hydrocephalus
have higher rates of ADHD.
ACADEMIC SKILLS:

academically, especiallyof mathematicsand reading. In

one study, 60% of

children with spina bifida were diagnosed with a

learning disability.
SOCIAL COMPLICATIONS:

 Compared to typically developing children, youths with

spina bifida may have fewer friends and spend less time
with peers.
 DIAGNOSTIC EVALUATION:
 Pregnancy screening:
 Neural tube defects can usually be detected during
pregnancy by testing the mother's blood (AFP
screening) or a detailed fetal ultrasound.
 Increased levels of maternal serum alpha-
fetoprotein (MSAFP) should be followed up by two
tests - an ultrasound of the fetal spine
and amniocentesis of the mother's amniotic fluid (to test
for alpha-fetoprotein and acetylcholinesterase).
 PREVENTION:
 Dietary supplementation with folic acid has been
shown to be helpful in reducing the incidence of spina
bifida. Sources of folic acid include whole grains,
fortified breakfast cereals, dried beans, leaf vegetables
and fruits.
 It is recommended that any woman considering
becoming pregnant take 0.4 mg of folic acid a day.
Pregnant women need 1 mg per day.
 TREATMENT:
 There is no known cure for nerve damage caused by
spina bifida.
 The spinal cord and its nerve roots are put back
inside the spine and covered with meninges.
 In addition, a shunt may be surgically installed to
provide a continuous drain for the excess
cerebrospinal fluid produced in the brain, as happens
with hydrocephalus.
 Shunts most commonly drain into the abdomen or
chest wall.
 CONTD…
 Monitor growth and development of bones,
muscles, and joints.
 Treat and evaluate nervous system issues, such as
seizure disorders.
 Physical therapy

 Speech therapy
 IMMEDIATE CARE:
 Place the child in prone position.
 Cover the affected area with sterile gauze piece
dipped in normal saline.
 Maintain hydration.

 Monitor for associated defects.

 LIFE LONG TREATMENT:
 Catheters
 Braces

 High fiber diet

 Antibiotics may be used to treat or prevent

infections such as meningitis or urinary tract
infections.
 COMPLICATIONS:
 Difficult delivery with problems resulting from a
traumatic birth, including cerebral palsy and
decreased oxygen to the brain
 Frequent urinary tract infections

 Hydrocephalus

 Loss of bowel or bladder control

 Meningitis

 Permanent weakness or paralysis of legs

THANK YOU
 CONTENTS
• INTRODUCTION
• CLASSIFICATION
• ETIOLOGY
• CLINICAL FEATURES
• DIAGNOSIS
• MANAGEMENT
 INTRODUCTION
• Wry neck( Latin meaning “twisted neck”)
• Deformity of the neck –
head is tilted to affected side
the chin is twisted to the opposite shoulder(cock-robin
position)
• The sternocleidomastoid muscle is shortened and may feel
tight and hard
 CLASSIFICATION
• CONGENITAL TORTICOLLIS:-
• Congenital muscular torticollis(m/c)
• Vertebral anomalies:-
Klippel-Feil syndrome
• Failure of formation:-
Congenital hemiatlas
• Ocular torticollis
 ACQUIRED TORTICOLLIS
PAINFUL PAINLESS
• Traumatic:- • Paroxysmal torticollis of
Atlantoaxial sublaxation infancy
• Tumors of CNS
• Inflammatory torticollis:- Acoustic neuroma
Grisel syndrome
• Tumors:-
Osteoid osteoma
Osteosarcoma
 CONGENITAL MUSCULAR
TORTICOLLIS
• The most common form of congenital painless
torticollis
• Fibromatosis of sternocleidomastoid muscle
• a)Temporary:-
Disappears within 1 year(80%)
• b)Permanent:-
Fails to disappear, the muscle becomes
permanently fibrotic and contracted
 ETIOLOGY
• Unknown
• intra uterine compartment syndrome of
sternocleidomastoid muscle
• Malposition of the fetus in utero,
• Birth trauma,
• Infection,
• Vascular injury.
• History of difficult labour ( breech delivery)
PATHOPHYSIOLOGY
 CLINICAL FEATURES
• Deformity of the neck,
• A palpable nodule is typically present in the affected
sternocleidomastoid muscle at birth
• Facial asymmetry (hemihypoplasia)
• Plagiocephaly
 ASSOSIATED CONDITIONS
• Metatarsus adductus
• Congenital dysplasia of hip
 ASSOSIATED SYNDROMES
•Sprengel deformity

•Klippel-feil syndrome

•Sandifer syndrome

•Grisel’s syndrome
 DIAGNOSIS
• Clinical examination
• Birth history
• Ultrasonography
• Cervical spine X-ray - no palpable mass
• CT scan for atlanto-axial rotary displacement
 DIFFERENTIAL DIAGNOSIS
• 1.Postural torticollis -intrauterine malposture.
This can be corrected by manipulation
• 2. Torticollis - contractures of scelenus
anterior and omohyoid
• 3. congenital cervical spine anomalies
Hemivertebrae,
Unilateralatlantooccipital fusion,
The Klippel-Feil syndrome
• 4. Traumatic fracture—
Traumatic fracture or rotary subluxation,
of C-1 and C-2 may
• 5. Inflammatory conditions
lymphadenitis
 MANAGEMENT
• Non-surgical management:-
1ST treatment of choice
Excellent results in 90% cases
passive stretching and manipulation of the
neck to the opposite side
PASSIVE STRECHING EXCERCISES
 MANAGEMENT OF
PLAGIOCEPHALY
• (1) repositioning of the neonate head
• (2) external orthotic treatment with molding
helmet therapy.
 SURGICAL MANAGEMENT
• Indications:-
• not respond to conservative(10%)
• Significant restriction of neck
movements ,30degrees
• Facial asymmetry persists after one year of
age
• Timing:- just before school age , 6 years
• Unipolar release:-
For mild deformity
Release of SCM muscle distally
• Bipolar release:-
For moderate to severe torticollis
Release of SCM muscle distally and proximally
• Endoscopic release
UNIPOLAR RELEASE
 POSTOPERATIVE CARE.
• At 1 week postoperatively manual stretching
of the neck
• three times daily for 3 to 6 months
BIPOLAR RELEASE
 POSTOPERATIVE CARE

• stretching, muscle strengthening, and active

range-ofmotion exercises, early postoperative
period.
• Head-halter traction or a cervical collar also
can be used during the first 6 to 12 weeks
after surgery
 COMPLICATIONS
• Hematoma
• Infection
• Neurovascular injury
• Hypertrophic scar
• Recurrent contracture with severe fibrosis
 Klippel-Feil Syndrome
• A second form of painless congenital torticollis
Congenital osseous fusions (synostosis)
Failure of segmentation of the cervical spine
 CLINICAL FEATURES
• Classic triad
Low posterior hairline,
Webbed neck
Limited motion with vertebral
fusions
• No SCM contracture
 ASSOSIATED CONDITIONS
• Genitourinary anomalies
• congenital heart disease
• deafness
• Synkinesis
• Congenital limb anomalies
• Scoliosis
• Rib anomalies
 INVESTIGATIONS
• X-ray C-spine-vertebral fusion
• Renal ultrasonography- genitourinary
anomalies
• MRI-spinal cord compression
• CT -scan
 MANAGEMENT
• Non-surgical:-
a)Halo fixation:-
Simultaneous correction of maintenance of
the deformity
 SURGICAL MANAGEMENT
• Indications:-
Increased rigidity
Inability to correct the head tilt by halo
fixation
Age -5 to 8 years
• Procedure:-
a)Anterior fusion
b)Posterior fusion
DEWAR TECHNIQUE
GALLIE AND BROOKS TECHNIQUE
MAGERL TECHNIQUE
 ATLANTO-AXIAL ROTATORY
DISPLACEMENT
• Most common cause of acquired painful torticollis
• Traumatic
• Inflammatory:-
Grisel syndrome-
Retropharyngeal abscess
• Upper respiratory tract infections
• Tonsillectomy/adenotonsillectomy
• Otitis media
• (ENT) infections/surgery
 PATHOPHYSIOLOGY OF GRISEL
SYNDROME
• Direct connection between the periodontoidal
venous plexus and the pharyngovertebral
veins of the posterosuperior pharynx
• Hematogenous transport of bacteria to the
upper cervical spine region
• Ligamentous laxity at the atlanto-axial
articulation
 CLINICAL FEATURES
• Fixed dislocation of the atlantoaxial joint is
rare
• SCM muscle on the contralateral side become
prominent
• Range of motion is painful ,restricted
 INVESTIGATIONS
• CT-scan:- • X-RAY :-
Investigation of choice True lateral view of the
skull
• Type 1:- A simple rotatory displacement
without anterior shifting of C1(M/C)
• Type 2:- Rotatory displacement and an
anterior shift of 5 mm or less
• Type 3:- Rotatory displacement with an
anterior shift of more than 5 mm
• Type 4:- Rotatory displacement with a
posterior shift.
FIELDING AND HAWKINS CLASSIFICATION
 MANAGEMENT
• < 1 week’s :-
• Immobilization in a soft collar,
• Rest, and analgesics
• If spontaneous resolution does not occur:-
• halter traction,
• muscle relaxants, and analgesics
• >1 week’s duration:-
Need hospital admission
Cervical traction
Reduction should be
confirmed by CT
Maintained by a halo vest for
up to 3 months
• >1 months duration:-
closed reduction under anesthesia
• Recurrent subluxation:-
posterior C1-2 arthrodesis
 NEUROGENIC TORTICOLLIS-
ETIOLOGY
• Tumors of the CNS (posterior fossa,brain stem)
• Arnold-chiari malformation
• Syringomyelia
• Paroxysmal torticollis of infancy
 CLINICAL FEATURES
• Painful and restricted movements of neck
• Extraocular muscle paralysis, nystagmus,
• Papilledema
• Motor deficit,
• Cranial nerve lesions
 DIAGNOSIS
• MRI:-
Investigation of choice
 SANDIFER ‘S SYNDROME

• Commonly seen in childrens

• Paroxysmal spasms of head, neck, and back
muscles
• Gastroesophageal reflux that produces
abnormal posture of head
 CLINICAL FEATURES
• Vomiting,
• Failure to thrive
• Irritable
• Recurrent respiratory
disease
• Range of motion of the neck
• No of SCM muscle
contracture
 DIFFERENTIAL DIAGNOSIS
• Congenital muscular torticollis
• Epileptic syndromes
• Congenital anomalies of the cervical spine
• Infectious conditions of the neck and head
 DIAGNOSIS
• contrast studies
• pH studies of gastric contents
MANAGEMENT
• a)Non-pharmacologic
• b)Pharmacologic
 OCULAR TORTICOLLIS
• Acquired torticollis
• Paralysis of the extraocular
muscles
Superior oblique—produces
strabismus and diplopia
• the patient rotates the head to
the uninvolved side to correct
the diplopia
 TAKEHOME MESSAGE
• Congenital muscular torticollis most common
type
• Strernocleidomastoid muscle involved
• Etiology-unknown
• Most of the cases are selflimiting
• Passive stretching exercises
 REFERENCE
• Campbell’s Operative Orthopaedics 14th
edition
• Kulkarni-Textbook of Orthopaedics and
Trauma 3rd edition
• Tachdjian Pediatric Orthopaedics 5th edition
• Apley&Solomon’s System of Orthopaedics and
Trauma 10th edition