Moderators

Dr A Suri
Dr Deepak Gupta

Presented by
Dr A Bisht
 A distinct group of congenital anomalies characterized
by a failure of midline structures of ecto- and
mesodermal origin to fuse

 Nicolas Tulp (1651) spina bifida

 Virchov spina bifida occulta
 Lichtenstein spinal dysraphism
 Von Recklinghausen (1866) classification
 Ruysch 1691 “with respect to cure little or nothing can
be done”
 Puncture of the sac
 Ligation of the sac suture, enterotome (Rizzoli)
 Sclerosants
 Bayer Flap closure(1892)
 Marcy Surgical closure (1895)
 Hautin closure at 18 M if IQ normal(43)
 Lorber, Matson, Sharrad closure in 1st week
 Neural tissue development in 3rd week of
gestation by induction by anteriormost
part of the primitive streak (Hensens
node) during gastrulation
 Neural groove formation and folding to
form the neural tube
 Neurulation develops faster than
embryonal axis and neural tube closes
before axis extension is complete (26 days
in humans)
 The remnants of the primitive streak in
the caudal pole form the caudal cell mass
which forms a lumen communicating
with the lumen of the neural tube and
gives rise to neural tissue distal to S2

MARK S. DIAS, MICHAEL PARTINGTON: Embryology of

myelomeningocele and anencephaly. Neurosurg Focus 16 (2):
1-16
 The first part of the tube to close
is the cervical part
 At least 5 waves of closure in
mammals
 Posterior neuropore closes at 25-
27 days
 Posterior neuropore located at S2
level

MARK S. DIAS, MICHAEL PARTINGTON: Embryology of

myelomeningocele and anencephaly. Neurosurg Focus 16 (2):
1-16
 Prolonged exposure of neural tissue to amniotic fluid
leads to destruction of neural tissue
 Open neural tube- loss of CSF- low pressure in 4th
ventricle- low attachment of tentorium. When
cerebellar primordium grows, insufficient place to
accommodate it and it herniates to the cervical canal

McLone DG, Knepper PA (1989) The cause of Chiari II

malformation: a unified theory. Pediatr Neurosurg 15:1-12
CLASSIFICATION (Tortori -Donati)
OPEN SPINAL DYSRAPHISM (95%)
Myelocele
Meningocele

CLOSED SPINAL DYSRAPHISM (5%)

With subcutaneous mass
Lipomeningocele
Lipomyeloschisis
Myelocystocele (cervical, terminal)
Meningocele , cervical myelomeningocele
without subcutaneous mass
Simple dysraphic states
Posterior spina bifida
Lipoma ( intradural, intramedullary, filum terminale)
Tight filum, abnormally long cord
Persistant terminal ventricle
Complex dysraphic states
Dorsal enteric fistula/ neurentric cysts
SCM
Dermal sinus
Caudal regression Tortori-Donati P, Rossi A, Cama A (2000) Spinal
dysraphism: a review of neuroradiological features
Segmental spinal dysgenesis with embryological correlations and proposal for a
new classification. Neuroradiol 42:471-491
 Wide variation 0.3-3.8/ 1000 live births
 Lowest in Asians
 Slight female preponderance
 Genetic
 Trisomy 13,18
 Family history of NTD 3-4% (triples with each affected sibling)
 Syndromic (acrocallosal, CHILD, Waardenberg, Fraser, Meckel
- Gruber)
 MTHFR, Folate synthetase, Pax 1,3
 Folate defeciency
 Obesity
 Diabetes with hyperinsulinemia 1% risk
 AED ( valproate, carbamazepine) 1-2% risk
 Febrile illness early in pregnancy
 Pesticide , solvents, radiation exposure
 Tobacco use
 MSAFP
 16-18 weeks
 2.5-3 multiples of median warrant a second USG
 USG
 High resolution USG 100% prediction of level
 Banana sign Chiari (93% of MMC)
 Lemon sign hydrocephalus (80% of MMC)
 Demonstration of defect in posterior elements
 Amniocentesis
 Amniotic fluid acetylcholinesterase
 Amniotic fluid AFP
 Rise also seen with Turners and omphalocele
 MRI
 Foetal MRI in the second trimester might be a clinically valuable
adjunct to ultrasound for the evaluation of CNS anomalies, especially
when ultrasound is inconclusive due to maternal obesity

Acta Obstet Gynecol Scand. 2010 Dec;89(12):1571-81

 Ideally in the ante natal period
 It should not come as a surprise
 Parents should know what to expect
 The course of action already discussed and charted
 If detected post birth for the first time
 Realistic expectations from therapy
 The parental hope a determinant in health related quality of life
 Screening in subsequent pregnancies
 Termination of pregnancy
 Legal up to 24 weeks in India
 Right of fetus Vs maternal right

 To treat or not to treat (? historical )

 Quality of life?
 10-15% will die <6 Years despite therapy
 20-25% have subnormal IQ
 38% require bowel programme for continence
 Social isolation, low self esteem 80% require psychiatric
counseling
 10-15% require custodian care
 < 10% are economically independent
 Reduced Amniotic fluid exposure of exposed neural
tissue
 Birth trauma to exposed neural tissue
 Reduced shunt requirement
 Reduced incidence of Chiari
 No maternal mortality
 Perinatal foetal mortality 4%
 VACTERL ( vertebral, anal, cardiac,
tracheoesophageal, renal, limb)
 OEIS (omphalocele, extrophy, imperforate anus, spinal
defects)
 Curriano triad (partial sacral agenesis, sacrococcygeal
teratoma, anorectal malformation)
 CTEV
 Renal (90% have neurogenic bladder)
 ? LSCS for all (after lung maturity before labour)
 20-30% have latex allergy (latex precautions )
 Nurse lateral/ prone
 Avoid entral feeding
 Closure 24-72 Hours (before colonization)
 85-90% have HCP
 Most have Chiari malformation
 Imaging
 Renal, GIT, cardiac
 Spinal MRI (with fat suppressed sequences )
 Cranial CT, USG
 Only essential imaging to assess fitness for surgery and extent
of surgery
 Hb, glucose
 Counseling parents
 Neurological assessment
 2/3 have myelopthic features due to incomplete
functional cord lesion
 Stimulus applied from distal to proximal till the infant
grimaces
 Kyphoscoliosis , limb asymmetry may be due to SCM
 Obstructive apnoea, stridor, opisthotonus due to
Chiari
 Hypothermia, hypoglycemia, hypocalcaemia
 Hb, coagulation
 Analgesia
 Infection avoidance
 Frequent dressing
 Antibiotics

 Nurse prone with head low

 Avoids pressure on wound
 CSF drainage (reduces leak)
 Reduced tethering
 CSF collection/ Leak
 Poor dural / wound closure
 4-8 days
 Wound breakdown / infection
 Catabolic state
 Poor feeding
 Tension in closure/ kyphosis
 Meningitis
 Pneumothorax
 Ileus / NEC
 Neurological deterioration (10%)
 Retethering
 Meningocele, meningomyelocele, hemimeningocele,
hemimyelomeningocele
 Posterior bony defect with prolapse of meninges and
the cord
 85% caudal thoraco lumbar spine, 10 % in the thorax
and the rest cervical
 Multisegmental in 1%
 80 90% associated with Hydrocephalus requiring
shunt
 Most have Chiari II malformation
 Delay in repair increases infection
 If delayed confirm negative cultures from placode
 15 % require shunt in the same sitting
 Avoid betadine contact with the placode
 Any tandem lesions dermoid ,lipoma , SCM,
neurentric cysts, meningocele manque should be
addressed
 Aim
 Protect functional neuronal tissue
 Prevent CSF loss
 Reduce risk of meningitis by reconstruction of cord and
coverings
 Technique
 Isolation and tubulation of placode
 Watertight dural closure
 Closure of lumbodorsal fascia
 Subcutaneous Undermining and tensionless skin closure
 Plastic closure with flaps may be required
 Congenital spinal defects covered by intact skin
 lesions seen
Lipomeningocele
Lipomyeloschisis
Myelocystocele (cervical, terminal)
Meningocele , cervical myelomeningocele
Spina bifida
Lipoma ( intradural, intramedullary, filum terminale)
Tight filum, abnormally long cord
Persistent terminal ventricle
Dorsal enteric fistula/ neurentric cysts/ Dermal sinus
SCM
Caudal regression
Segmental spinal dysgenesis
Cutaneous stigmata Orthopedic deformities Urologic problems
Asymmetric gluteal cleft Foot or leg deformities Neurogenic bladder
Capillary hemangioma Scoliosis UTIs
Subcutaneous lipomas Sacral agenesis Incontinence
Hypertrichosis Delay in toilet training
Dermal sinus tract
Cutis aplasia
Infants Toddler Older children Young adults
Decreased Delayed walking Asymmetric Back pain
spontaneous leg motor/ sensory
movements development
Absent reflexes Abnormal gait Back/leg pain Leg
cramping/pain
Leg atrophy UMN signs Spasticity
Foot asymmetry Painless ulceration Hyperreflexia

Decreased urinary Bowel/bladder

stream incontinence.
Structure Findings
Lamina Fusion defects,midline
defects,abnormal
spinous processes
Vertebral bodies Hemivertebrae,Butterfl
y vertebrae,Block
vertebrae,Midline cleft
defects, canal stenosis
Disk space Congenital narrowing
Pedicles Flattening, thinning
Widening of spinal Interpedicular
canal widening, scalloping of
posterior border,
Midline bony spur.
Failure of development Reduced number of
vertebral bodies,
Absence of parts of
vertebrae, sacral
dysjunction
Spinal curvature Scoliosis, kyphosis,
lordosis.
 Lipoma with lipoma cord interface
posteriorly distracted out of the spinal
canal
 Congenital lumbosacral lipomas the entire
spectrum
 Dysjunction in timing of closure between
the surface ectoderm and neural tube
allowing mesenchyme to migrate/
differentiation of caudal mass cells into
lipoma
 Symptoms due to tethering/ compression
 1:4000 births F>M
 Defecits start by 1-2 years
  Type I dorsal
 Attached to posterior cord
 Distinct cord lipoma interface
 cord and coverings distally normal
 Lipomeningocele/ lipo MMC

 Type II transitional
 Interface continues beyond myeloschisis
 No normal cord distally
 Roots enmeshed in lipoma

Pang D et al .Long-term outcome of total and near-total resection of

spinal cord lipomas and radical reconstruction of the neural placode:
part I—surgical technique. Neurosurgery 65:511–529, 2009
  Type III caudal
 Arise from conus/ filum
 Largely/ completely intradural
 Interface diffuse

 Chaotic (Pang)
 Caudal portion ventral to placode
 Fusion lines blurred distally
 Location of DREZ and roots
unpredictable

Pang D et al .Long-term outcome of total and near-total resection of

spinal cord lipomas and radical reconstruction of the neural
placode: part I—surgical technique. Neurosurgery 65:511–529, 2009
 Indications
 SC lipoma in infants > 2M with cord lipoma attachment
 Fresh / progression of defecits
 Undergoing scoliosis correction
 Severe dysesthetic pain

 Goals
 Detach cord tethering structures
 Debulk as much lipoma as possible
 Close myeloschisis and reconstruct tubular cord
 Technique
 Expose 1-2 vertebral level above and 1-2 cm below
 Wide laminectomy
 Detach dura from lipoma
 Identify and separate fusion line (medial to DREZ and roots)
 Sharp dissection rostro caudally in the white plane
 Neural tissue after 3 sacral roots may be sacrificed
 Pia – pia sutures with non absorbable sutures
 Expansile duroplasty
 50% Associated with sacral/ gluteal cleft dimples
 Leg weakness, numbness, sphincter weakness
 MRI conus below L2 and filum > 2 mm
 Fibrous / fatty
 VACTERL, arthrogryphosis
 TCS Symptoms may be mimicked by non functional shunt
 Surgery
 All symptomatic
 ? Asymptomatic
 Identify filum/ ensure no roots on it ( nerve stimulator)
 Cauterize and divide filum
 Commoner in females 3:1
 Average presentation 4-6.5
years
 Cutaneous lesions in 71%
 Associated with MMC 6%
 Location of split
 Lumbar 47%
 Thoracolumbar 27%
 Thoracic 23%
 Cervical / sacral 1.5%
 Formation of accessory
neurentric canal with
communication between
yolk sac and amnion
 Type I
 2 Cords in separate dural sleeves
separated by bony/ cartilaginous
septum

 Type II
 2 cords In single dural sleeve
separated by fibrous septum

Mahapatra AK, Gupta DK: Split cord malformations: a clinical study of 254 patients
and a proposal for a new clinical-imaging classification. J Neurosurg 103 (6
Suppl):531–536, 2005
  Management
 Detethering of the cord
 Excision of septum/ fibrous
septae
 Look for and divide ventral
tethering elements
 Conversion into single dural
tube
 Associated defects addressed

Mahapatra AK, Gupta DK: Split cord malformations: a clinical study of 254 patients
and a proposal for a new clinical-imaging classification. J Neurosurg 103 (6
Suppl):531–536, 2005
 Occult spinal dysraphism with a localized, cystic
dilation of the central canal of the spinal cord that is
herniated through a posterior spina bifida
 4-8 % of LS occult dysraphism
 Terminal / non terminal
 No familial , sex preponderance
 Associated with OEIS
 Cystic, skin covered LS mass, in
gluteal cleft
 Defecits due to associated lesions
 Theories
 Steinboch and Cochrane :LDM in a
hydromyelic cord
 Mc lone and Naidich : dilatation of
terminal ventricle due to closure of
terminal neouropore
 MRI trumpet like flaring of distal
central canal
Closure of the dural layer (2) and
 Surgery for all reinforcement of the dural closure (3) with
mobilized pedicled muscle on a
lumbosacral terminal lipomyelocystocele.
The terminal end of the dilated cystocele (1)
is left open to communicate with the
subarachnoid space distally.

Mahapatra AK, Gupta DK: Terminal myelocystoceles: a series of 17 cases.

Neurosurg (pediatrics 4) 103: 344-352, 2005
 Can occur along the entire
neural axis
 Male predominance
 Complicated lesions present
later in life
 Pain myelopathy, meningitis
acute neurological
deterioration
 MRI high protein content
without haemorrhage

Santos R et al. Neurentric Cysts in The Spina bifida Management and

outcome. M Memmet Ozek ed. Springer Velarg. 475-485
 Complete excision is the goal
 Approach anterior Vs posterior
 Aspiration reduces the cyst size and eases dissection
 Dense adhesions if previous meningitis
 Post op complication rate 23%
 Recurrence rate 11%
 Tracts reaching from the skin to varying structures
 10% subcutaneous
 20% between fascia and dura
 10% subdural
 60% subarachnoid space

 Location
 90% lumbosacral
 ≈ 1% cervical
 ≈10% thoracic

 The tract may traverse several vertebral levels before

penetrating dura
 Theories
 Abnormal separation of cutaneous and neuroectoderm
 Splitting of notochord and persistence of mesenchymo
cutaneous fistula
 Presentation
 Majority detected by 5 years on cutaneous stigmata
 TCS
 Infectious ,Neurological, urological, orthopedic

 Management
 Surgery for all
 If infected after infection subsides
 To relieve mass effect in presence of infection
 Prolapse of meninges and CSF through a defect
 Posterior, anterior, lateral
 Absence of Chiari, Hydrocephalus, Limb anomalies Vs
MMC
 Concomitant neurocutaneous lesions which may cause
tethering (posterior)
 Elective surgery at 4-6 months
 Commoner in females
 LS location commonly presacral
 Curriano’s triad
 Anterior approach to close dural defect and detether