Discuss the radiologic investigations and features in a 2 month old child with lumbar myelomeningocele

By
Dr. Ekwama E. B.
Dept of Radiology,
FMCKeffi
 Outline
• Introduction
• Epidemiology
• Pathology
• Associations
• Clinical features
• Radiological features
• Conclusion
• References
 Introduction
• Myelomeningocele also known as spina bifida cystica,
spina bifida aperta, open spinal dysraphism and Chiari II
malformation complex is a complex congenital spinal
anomaly characterized by the failure of closure of the
neural tube usually (but not only) at the lumbo-sacral
level
• This results in a sac covered by leptomeninges containing
CSF and variable amount of neural tissues herniated
through a defect in the posterior or anterior elements of
the spine
• It is typically associated with a metamerically consistent
paraplegia, a posterior fossa deformity known as the
Chiari II malformation, hydrocephalus and a constellation
of central nervous system (CNS) dysplasias
 Epidemiology
• It is the most common malformation of CNS
worldwide with an incidence of 0.4-1 per
1000-2000 live births
• It is one of the leading causes of infantile
paralysis in the world today
• A slight female predilection has been noted
• Twice as common in infants of mothers >35
years of age
• Caucasians> Blacks>Orientals
 Pathology
• Myelomeningocele results from failure of fusion of
neural folds dorsally during embryogenesis
• There is a localized defect of the closure of caudal
neuropore with persistence of neural placode (open
spinal cord)
• Location
lumbosacral: ~45%
thoracolumbar: ~30%
lumbar: ~20%
cervical: ~2%
• Associated risk factors are in utero folate deficiency
and in utero teratogen exposure
Associations
(1) Hydrocephalus (70-90%): requiring
ventriculoperitoneal shunt in 90%
25% of patients with hydrocephalus have spina bifida
(2) Chiari II malformation (99%)
(3) Congenital I acquired kyphoscoliosis (90%)
(4) Vertebral anomalies (vertebral body fusion,
hemivertebrae, cleft vertebrae, butterfly vertebrae)
(5) Diastematomyelia (20-46%): spinal cord split above
(31 %), below (25%), at the same level (22%) as the
myelomeningocele
(6) Duplication of central canal (5%) cephalic to+ at level
of placode
(7) Hemimyelomeningocele (10%) =two hemicords in
separate dural tubes separated by fibrous I bony spur: one
hemicord with myelomeningocele on one side of midline,
one hemicord normal I with smaller myelomeningocele at
a lower level impaired neurological function on side of
hemimyelocele
(8) Hydromyelia (29-77%) cranial to placode as a result of
disturbed CSF circulation
(9) Chromosomal anomalies (10-17%): trisomy 18, trisomy
13, triploidy, unbalanced translocation
In 20% no detectable associated anomalies
(10) Tethering of spinal cord (70-90%)
(11) Arachnoid cyst (2%) due to developmental deficiency
during formation of arachnoid I dura mater with a
subdural location
Clinical presentation
• Swelling at the back from birth
• Progressive head enlargement
• Headaches
• apnea, stridor, vomiting, poor tolerance to
changes in food texture with choking, or
respiratory distress.
• Lethargy
• Paraparesis
• Paraplegia, club foot and other deformities
• Delayed mile stones
 Imaging modalities
• Ultrasound scan
• Plain radiographs
• CT scan/ CT myelography
• Myelography (conventional)
• MRI
 Obstetric uss
• Detection rate of 85-90%; sensitivity dependent on GA (fetal
spine may be adequately visualized after 16-20 weeks GA);
• False-negative rate of 24%
• Spinal level estimated by counting up from last sacral
ossification center = S4 in 2nd trimester+ S5 in 3rd trimester
• May have clubfoot I rocker-bottom foot
• Polyhydramnios
• Spine:
loss of dorsal epidermal integrity
soft-tissue mass protruding posteriorly +visualization
of a sac
widening of lumbar spine with fusiform enlargement of
spinal canal:
• Splaying(= divergent position) of ossification
centers of laminae with cup- I wedge-shaped pattern
(in transverse plane =most important section for
diagnosis)
• Absence of posterior line= posterior vertebral
elements (in sagittal plane)
• Gross irregularity in parallelism of lines representing
laminae of vertebrae (in coronal plane)
• Anomalies of segmentation I hemivertebrae (33%)
with
short-radius kyphoscoliosis
• Tethered cord (with lumbar I lumbosacral
myelomeningocele
Head:
• "lemon sign"= concave I linear frontal contour
abnormality located at coronal suture strongly
associated with spina bifida
• "banana sign“ - Prevalence: in 96% of fetuses;
24 weeks; in 91% of fetuses >24 weeks
• "nonvisualization" of cerebellum
• Effaced cisterna magna (100% sensitivity)
• A normal cisterna magna is 3-10 mm deep
and usually visualized in 97% at 15-25 weeks
GA
• BPD <5th percentile during 2nd trimester (65-
79% sensitive)
• HC <5th percentile (35% sensitivity)
• Ventriculomegaly (40-90%) with choroid plexus
incompletely filling the ventricles (54-63%
sensitivity) "dangling" choroid on dependent
side
• Prevalence: in 44% of myelomeningoceles <24
weeks GA; in 94% of myelomeningoceles during
3rd trimester
• Postnatally, transfontanelle scan will show
ventricular dilatation
Prenatal uss showing normal laminae, beginning of posterior
element separation and wider separation of the laminae with
the myelomeningocele
 Prenatal uss (longitudinal and transverse) showing
myelomeningocele and posterior element defects of the fetal
spine
Transfontanelle uss showing dilated ventricles
Transverse Antenatal ultrasonogram shows a
lemon sign and a banana sign
 Plain radiograph
• Bony defect in neural arch
• Deformity+ failure of fusion of lamina
• Absent spinous process
• Widened interpedicular distance
• Widened spinal canal
• Kyphoscoliosis
• soft tissue swelling may also be seen
Plain lumbar spine radiograph showing defects in L4-5 and S1
laminae, absent spinous processes as well as widened
interpedicular distance
Lateral plain radiograph showing a soft tissue
swelling at the lumbar region
 CT scan/ Ct myelography
• May show sac like swelling with varying density at the lumbar
region
• Defects of the body and posterior elements is seen and more
clearly on bone window with the cord and its coverings
herniating through the defect
• Vertebral body fusion, hemivertebrae, cleft vertebrae,
butterfly vertebrae
• CT myelography will show additional findings such as
diastematomyelia
• CT brain may show ventricular dilatation, herniation of the
cerebellar tonsil, medulla, and 4 th ventricle through the
foramen magnum
• Myelography will show diastematomyelia, tethering of the
cord
Axial CT images through the L/S demonstrating absence
of the posterior elements. Sclerosis of the laminae and
widened spinal canal.
Myelograph showing splitting of the cord, a long
tethered cord and widening of the spinal canal
Plain radiograph of the lumbar spine showing bony defects
in the lamina of L2 to S1. myelogram shows a split cord.
 MRI
• MR imaging shows sac like swelling over the
lumbar region
• Bony defects of the posterior elements with
herniation of the cord
• Splitting of the cord may also be noted
• MR imaging of the brain and entire spine may
also reveal features of Chiari II malformation
• Dilated ventricles
• Cervico-medullary kink may also be seen
• Syringomyelia
• Hydromyelia- focal, multiple or diffuse
• When it is multiple or diffuse it gives the
“bamboo appearance” that is classic for
myelomeningocele
• Arachnoid cysts could also be seen
• Vertebral body fusion, hemivertebrae, cleft
vertebrae, butterfly vertebrae
• Tethering of the spinal cord
Axial T1 and T2W images showing
ventricular dilatation
T1W sagittal MR images demonstrating hydrocephalus
with features of Chiari II malformation
Axial T1W MR images showing splitting of the cord
by a septum into 2 hemicords.(Diastematomyelia)
 Sagittal T1W MR image showing dilatation of the central canal of the
cord with hypointense fluid collection which shows multiple transverse
hyperintensities giving it a ‘stacked coin appearance’ of hydromyelia
Sagittal T2W MR image showing tethering
of the cord distally
 T1 W and T2W saggital MR images showing an extradural spinal lipoma of
high signal intensity in contact with the distal part of the cord and is
continous with the subcut fat through a spinal defect(lipomeningomyelocele)
 T2W MR image shows a dorsal midline fusion defect in the L/S region with herniation of
meningeal sac through the defect forming a subcut. Swelling of mixed signal intensity. Low
lying tethered cord with neural elements noted traversing the meningeal sac. Focal
dilatation of the central canal of cord is also seen.
 Conclusion
• An organized approach to imaging findings
with consideration of clinical and
developmental factors allows greater ease in
the diagnosis and management of
myelomeningocele.
THANK YOU
 References
• Dahnert – Radiologic review manual. 7th
Edition. Pg 221-223
• Sharon E. Byrd, Mary Ann Radkowski.
Radiological evaluation of the child with
myelomeningocele
• Grainger and Alison Diagnostic radiology- A text
book of medical imaging. . 5th Edition. Ch 70.
• Medscape.