J Pediatr Neurosci. 2011 Oct; 6(Suppl1): S31–S40.

PMCID: PMC3208922
doi: 10.4103/1817-1745.85707: 10.4103/1817-1745.85707 PMID: 22069428

Spinal dysraphism
N. K. Venkataramana

Department of Neurosurgery, Advanced Neuroscience Institute, BGS Global Hospital, Bangalore, India
Address for Correspondence: Dr. N. K. Venkataramana, Department of Neurosurgery, Advanced Neuroscience
Institute, BGS Global Hospital, BGS Health and Education City, No. 67, Uttarahalli Road, Kengeri, Bangalore - 560
060, India. E-mail: drnkvr@gmail.com

Copyright : © Journal of Pediatric Neurosciences

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-
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Abstract
To review the clinical features and current understanding of spina bifida with an
emphasis on the Indian Scenario. Selected articles and current English language texts
were reviewed. The authors experience was also reviewed and analysed. Spina bifida is a
common congenital anomaly encompassing a wide spectrum of neural tube defects.It is
broadly classified as spina bifida aperta and occulta. With the prenatal screening, the
incidence of aperta is gradually declining, whereas the detection of occulta has increased
with the advent of magnetic resonance imaging. Over the years, the understanding of
pathophysiology has made a significant changein the management of these anomalies.
Early detection and complete correction can significantly reduce the neurological
disability. This article is an overview of spina bifida with a special emphasis on Indian
scenario.

Keywords: Aperta, Indian scenario, occulta, spina bifida, tethered cord syndrome

Introduction
Spina bifida literally means “spine in two parts” or “open spine”.[1] Spinal dysraphism
involves a spectrum of congenital anomalies resulting in a defective neural arch through
which meninges or neural elements are herniated, leading to a variety of clinical
manifestations.[1,2] They are divided into aperta (visible lesion) and occulta (with no
external lesion).[1,2] Meningocele, myelomeningocele, lipomeningomyelocele,
myeloschisis and rachischisis are the usual names associated depending on the
pathological findings. Meningocele by definition involves only the meninges with no
neural involvement; others have variable extent of neural involvement. The spina bifida
aperta is usually associated with skin defect with an impending risk of CSF leak
constituting “open defects,” whereas the occult forms have normal skin cover. Both forms
demand different approaches in their management. The clinical importance of occult
lesion has grown tremendously in the recent years.
Indian scenario
The number of children suffering from spina bifida and hydrocephalus was large though
the detection was sporadic by the pediatricians in the past. With the awareness and
growth of neurosurgery in the country, few referral centers established special clinics
with an ability to address these problems. Gradually, with the availability of magnetic
resonance imaging (MRI), several such anomalies were identified, especially of the occult
variety. This also helped to visualize a variety of underlying anomalies and their clinical
consequences. After pediatric neurosurgery became a subspeciality, several
neurosurgeons took interest in this field and contributed in early detection and treatment
and also with several good publications. The number of centers has increased across the
country. Several unusual anomalies and rare anomalies have been detected and
published. Meanwhile, there has been a steady decline in these anomalies over the last
decade. Prenatal diagnosis and the medical termination of pregnancy as well as folic acid
fortification have contributed. As a result, the anomalies which are not compatible with
life and severe anomalies with major defects have reduced, leading to social comfort.
Prenatal diagnosis and genetic analysis has become a routine across the country. Few
specialized centers have involved in clinical genetics and metabolic workup of these
children, contributing to the etiology of many developmental and metabolic diseases.
Hence, there has been a shift from open lesion to greater detection and treatment of
occult lesions. Considering the size and numbers in the country, a lot needs to be done to
provide uniform standards of care in all centers. But it is a happy moment to all of us that
several neurosurgeons are practicing pediatrics as their special interest and many are
becoming dedicated pediatric neurosurgeons, thanks to ISPN CME Programs for educating
neurosurgeons across the country. This phenomenal change and specialization has
become a reality by such educational programs.

Prevalence
The estimated incidence of spinal dysraphism is about 1–3/1000 live births.[2] The
prevalence of spinal dysraphism has been in decline the world over in the last few
decades due to the better nutrition for women, folic acid supplementation, improved
antenatal care and high-resolution ultrasound for prenatal screening and biochemical
markers.

Embryology
Embryogenesis in the first 2 months of gestation can be divided into 23 stages. Neural
plate is formed in stage 8 around the 18th day, followed by neural folds and their fusion.
The expansion of the neural tube and subsequent closure is completed by day 28. Open
defects occur when the caudal neuropore fails to close. The secondary neurulation sets
the spinal cord formation. Defects at this stage result in occult dysraphism connecting the
epidermis and the mesenchymal tissues, leading to variety of anomalies and tethered
cord.[2,3]

Failure of primary neurulation leads to open dysraphism posing the risks of CSF leakage
and exposure of neural placode. Extent and severity of neurological deficit depends on
the degree of malformation of the neural placode and also the level of the defect. The
higher the level, usually worse is the prognosis. A spectrum of neurological abnormalities
like hydrocephalus, Chiari malformation, syrinx, gyral malformations, skeletal
malformations and uro-vesical defects can be associated. In occult dysraphism, the
overlying skin is intact but the spinal cord is anchored to various tissues starting from
skin, subcutaneous tissue, adipose tissue or cartilage.

Etiology
Spina bifida has a multifactorial causation, involving both genetic and environmental.
Recent information stressed on the importance of maternal nutrition and folic acid
supplementation which has contributed to its reduction.[3,4] In India, certain regional
factors and consanguinity seem to play a role [hospital-based epidemiological study from
NIMHANS (unpublished)].

Symptomatology
Open dysraphism presents with a swelling over the back which is noticed at birth [
Figure 1]. Symptoms are primarily referable to CSF leak or the exposed spinal cord. Since
the skin over the swelling is poorly developed, it usually gives way during labor, resulting
in CSF leak, contamination and meningitis. Defects predominantly involve
thoracolumbar, lumbosacral, lumbar, thoracic, sacral and cervical in the order of
frequency of occurrence. Incidence of high cervical lesions is about 3.9%.[5] Neurological
deficits include motor, sensory and sphincter dysfunction, depending upon the severity
and level. In severe cases, hypotonic areflexic limbs, sphincter atonia with rectal prolapse
may be associated. Chiari malformation presents with lower brainstem and lower cranial
nerve dysfunction. The presence of large head usually indicates hydrocephalus. The
associated skeletal abnormalities are kyphosis, scoliosis, and deformities of the long
bones and feet, hemivertebrae, defective ribs, etc.

Assessment
Initial assessment of the newborn is extremely important, preferably both by a
pediatrician and a neurosurgeon. Examination of head and neck involves the assessment
of head size, shape, skull bones, and openness of fontanelles, lacunar skull defects and the
size of the posterior fossa. Examination of the back needs assessment of the neural
placode, level of the lesion, condition of the skin, extent of skin defect and associated
deformities. Examination of the lower limbs for detection of the deformities of the foot
and abnormalities of long bones is important. A detailed neurological examination is
necessary to assess the level of motor weakness, sensory level and sphincter dysfunction.

Prenatal diagnosis

Prenatal screening for neurological abnormalities is based on ultrasound performed

routinely or oriented by maternal Alpha Feto Protein (AFP) screening. It should be
performed around 12, 22 and 32 weeks. Maternal serum screening can detect up to 80%
cases of spina bifida and 90% cases of anencephaly.[6,7] Sonography may identify up to
90% cases of myelomeningoceles. Over the last few decades, the diffusion of routine
ultrasound has changed the spectrum of the neonatal neurological malformations. Gross
lethal abnormalities nearly always result in termination of pregnancy, depending on the
regional law system. A growing number of more subtle abnormalities including midline
or posterior fossa abnormalities are being discovered. But their postnatal outcome cannot
always be predicted accurately, despite the use of fetal MRI. Maternal serum screening for
chromosomal abnormalities is also increasingly used. Only in select situations,
amniocentesis is contributory.[6,8–13]
Radiological assessment
Radiological assessment [Figures 2–4] should be done at the earliest depending on the
clinical condition. Plain X-rays reveal the skull defects, spine deformities and bony
anomalies. MRI is the investigation of choice to study the neural tissue abnormalities and
also to assess the severity of hydrocephalus and Chiari malformation. However,
ultrasonography can be used to obtain quick information regarding hydrocephalus.
[1,14,15]

Management
Management of these children needs multidisciplinary approaches. Complete clinical
evaluation and appropriate investigations are necessary. Parents need to be counseled
and informed regarding the immediate as well as long-term management strategy.[16]

Surgical treatment
The aim of surgery is to free the placode from the surrounding abnormal skin and
reposition into the spinal canal with reconstruction of the dura and coverings to prevent
CSF leak and infection. The surgical technique depends on the size and the level of the
lesion. The help of pediatric, orthopedic and plastic surgeons may be necessary. Several
attempts for maternal fetal surgeries to improve their outcomes have been made. The
role of fetal surgery for myelomeningocele is yet to be proven.[17]

Timing of intervention primarily depends on the clinical condition of the child and the
impending risks. Surgery need not be done as a compelling emergency but should be
undertaken as soon as it is practical.[18] In case of suspected meningitis or CSF infection
or colonization of the wound, prophylactic antibiotics and anticonvulsants form the
initial treatment. Child is nursed in an incubator; routine blood counts and serum
electrolytes are monitored. Blood grouping and cross matching is done for possible
transfusion. Careful assessment of body weight is essential for intraoperative
management. The newborn child with myelomeningocele should have saline dressings. It
is essential not to use corrosive agents, spirit or antiseptics indiscriminately over the open
defects to avoid damage to the underlying exposed neural tissue.

Surgical technique
To obtain successful repair, it is essential to study the surface anatomy and its
relationships to the surrounding structures. At the apex of the myelomeningocele usually,
the flat neural placode is located and from its edge the remnants of the arachnoid
membrane get attached at the nerve root entry zone. From this junction, the nerve roots
emerge and exit through neural foramina located ventrally. They are seen through the
transparent arachnoid membrane which is fused with the skin at the lateral edges of the
lesion. The dura matter which is defective posteriorly is loosely adherent to the
underlying soft tissue of the back and densely adherent to the bony structures
underneath. Rostrally, the dura forms tube and the neural placode continues into it,
which leads to functional spinal cord.

The child is operated under general anesthesia with endotracheal intubation in prone
position, with the head and neck placed comfortably. The first step is to isolate the neural
placode, while salvaging the nerve roots as much as possible irrespective of their
functional status. It is essential that no skin element becomes buried in the repair to
prevent the possibility of an implantation dermoid. Vertical midline incision is preferable.
In a circumferential fashion, arachnoid is lifted and the nerve roots are identified. This
technique is continued all around till the neural placode is completely free. The neural
placode can then be inverted and sutured. The dura is dissected from the underlying soft
tissue. The dural closure needs to be made water tight with a graft, if necessary. The
overlying skin is dissected from underlying fascia and musculature, mobilized and
approximated. If necessary, relaxing incisions or flaps may be used to close larger defects.
Kyphotic deformity or gibbus can pose special problems. Reigel had advocated resection
of the kyphus with primary spinal fusion at the time of surgical repair.[19] When the
dysplastic skin is large causing larger skin defects, balloon tissue expanders can be used
to enlarge the normal skin area which can then cover the defect.[20]

Postoperative care is equally important. To nurse in prone position, protect the wound
from fecal and urinary contamination. Symptomatic hydrocephalus or arnold chiari
malformation (ACM) needs to be treated simultaneously. Supportive nutrition and
antibiotics are required. Adequate urological treatment can prevent future complications
of the upper urinary tract.[21–23] Early management, maintaining a low intravesical
pressure and continuing intermittent catheterization with or without pharmacotherapy,
is beneficial. But urological management should ideally be based on and modified by the
urodynamic studies.[21–28]

Tethered cord syndrome

Tethered cord syndrome has been defined as progressive neurological deficits from the
restraint of the spinal cord movement and traction due to either anatomical or
physiological reasons. Advent of MRI and the present understanding of embryogenesis
have made this syndrome popular in pediatric neurosurgical practice. The neurological
deficits associated are insidious, progressive, and may be motor, sensory, urorectal, pain
and/or scoliosis.[29–31]

Tethered cord can result from a variety of conditions. This can be broadly classified into
the following:

1. Anatomical in which the conus is placed at a lower level than the normal.

2. Physiological due to tight or thick filum. In this variety, the conus may be at normal
level, but the movement of spinal cord is restrained resulting in neurological deficits
and vesicle dysfunction called “neurogenic nonneurogenic bladder.”

3. Developmental in which it could be secondary to a variety of conditions like

congenital anomalies, terminal lipoma, intraspinal lipoma, lipomyelomeningocele,
split cord malformations, sacral agenesis and other occult dysraphic states.

4. Postoperative tethering that occurs after surgery for myelomeningocele or dermoid

or recurrent adhesions from previous surgery often termed retethering.[32,33]

Symptoms

The predominant symptoms can be motor in the form of insidiously progressive distal
weakness and spasticity, and sensory ones like sensory loss, paresthesias and trophic
ulcers. Neurovesical dysfunction is one of the commonest presentations, ranging from a
minor bladder disturbance to frank urinary incontinence, occasionally associated with
bowel movement dysfunction. At times, pain can be the major symptom. Pain may be
radicular or funicular, low back or calf muscle pain. The scoliosis due to tethering is a
much debated issue, but with enough evidence to suggest its role.
Neurological examination
A detailed neurological assessment is mandatory. Neurocutaneous markers, like tuft of
hair, nevous, dysplastic skin, angiomatous patches, lipoma, dermal sinus, presence of
human tail and absence of gluteal folds, can be the strong indicators [Figures 5–9].[34]
Neurovesical dysfunction, leg pain, scoliosis, and motor or sensory deficits should prompt
the clinician to look for possible tethering even in the absence of cutaneous markers.

With tethering being a congenital phenomenon, these children often present to their
pediatricians with vague leg pains, to orthopedicians with gait disturbance and
deformities, to urologists with bladder symptoms like frequent persistent bed wetting,
and finally to neurosurgeons when the neurological deficits become overt. Hence,
awareness about the recent developments among the medical fraternity is necessary.

Pathophysiology
The name tethered cord implies that the spinal cord is attached tightly to a congenitally
abnormal structure in the lumbosacral area, as well as at the craniovertebral junction.
With growth, the spinal cord is stretched between these two points, resulting in insidious
and progressive neurological deterioration. The popular belief that the deterioration
occurs mostly during the phases of rapid growth is not confounded. During embryonic
life, the spinal cord lies way down at sacral segments, and gradually the conus ascends
and reaches the adult level at the time of birth, as confirmed by ultrasonographic studies.
In this pathological situation, the spinal cord is tethered to anatomical structures lower
down, preventing the ascent of the conus in parallel to the vertebral growth. On the other
hand, there is disproportionate growth between the spine and the spinal cord, resulting in
progressive traction. Normally, when a child bends forward, there is an ascent of the
spinal cord by one to two segments. The filum can be short and thick, with anatomically
variant fibroelastic fibrous tissue, making it tight and preventing such movement.

Several theories have been proposed to explain the neurological deterioration in tethered
cord syndrome. With ongoing stretching, central fibers are subjected to traction injury,
resulting in structural damage. Yamada et al., have shown that the mechanical stretching
of the cord during physical activity leads to changes in intracellular respiration, reduction
in cytochrome oxidase and shift in redox curves.[31] The third possible mechanism is by
ischemic injury. Traction and elongation of the spinal cord can compress the radial
perforating vessels, resulting in ischemic damage. Once the neurological deficit occurs, it
is rarely reversible. Therefore, it is important to treat them prophylactically.[35–38]

The diagnosis should be based on the high index of clinical suspicion, a detailed
neurological examination and investigation for confirmation. MRI is the choice of
investigation to identify the anatomical location of the conus and the associated
abnormalities. The classical dorsal displacement of the conus with a large, ventral CSF
space is often suggestive [Figures 10 and 11]. Early appearance of lumbar potentials in
somato sensory evoked potentials (SSEP) has been found valuable, though often difficult
to record in children.[39,40]

Indication for treatment

Progressive neurological deterioration in terms of motor, sensory or urinary dysfunction

in a child is a clear indication. Pain, spasticity, gait abnormality, and persistent or
progressive scoliosis are the other indications. Knowing the mechanisms of neurological
deterioration, prophylactic surgery is logical. If the neurological deficit has already
occurred, surgery should be done as soon as the diagnosis is established.

The principles of surgery

1. Complete untethering of the spinal cord

2. Proper dural reconstruction with adequate CSF space around the spinal cord to
prevent retethering.

Retethering
A theoretical risk of retethering exists after the treatment of any tethered cord or some of
these complex anomalies. Every known precaution should be adopted to prevent such a
possibility. This includes complete untethering of all the contributory structures, proper
concealing of rough and possible adhesive surfaces by pial sutures, laminectomy to
accommodate thickened cord and to facilitate upward movement, duraplasty to create a
reservoir of CSF around the repaired structures and epidural fat grafts to prevent fibrosis.
Some studies reported advantage of nursing the child in prone position postoperatively.
Avoid intradural lumbar drains as far as possible. During follow-up, subtle neurological
worsening or progression of deformities should be looked for. MRI should be repeated at
the earliest suspicion. At present, there is no foolproof investigation to demonstrate
retethering. MRI in prone position to demonstrate CSF dorsal to the conus has been
suggested. Serial electrophysiological and urodynamic studies seem to be indicative. Till
such time, clinical diagnosis remains the gold standard. All symptomatic children due to
suspected retethering should be reoperated to prevent further deterioration. But surgery
on pure radiological suspicion for retethering is controversial [Figure 12].

Lipomyelomeningocele
Lumbosacral lipomyelomeningocele is a subcutaneous fibrofatty mass that traverses the
lumbodorsal fascia, causing a spinal laminar defect, penetrating dura and tethering the
spinal cord [Figure 13]. Lipomas form one of the prominent causes of spinal cord
tethering, almost all of them with low-lying conus. The term lipomyelomeningocele is
used despite the overlying skin being normal. They arise from the disorder of
embryogenesis, usually consist of single cell type but rather demonstrate fibrous tissue,
muscle cells, neural tissue and a variety of other cell types that arise from all embryonic
layers. Incidence is approximately 1 in 4000 births in USA, with a slightly female
preponderance. True incidence in terminal lipomas is not known. MR studies represent
13- 26% of lesions accounting for tethering.[41,42] Symptoms and signs are mainly related
to the traction on the lower spinal cord, leading to motor deficits, sensory disturbance,
spasticity, urodynamic, urorectal dysfunctions and skeletal deformities.[43] Pathogenesis
and natural history of these complex anomalies are not clearly understood. But it is
certain that almost all deteriorate in their neurological state over course of time. Proper
treatment at the appropriate time prevents their neurological deterioration. But surgical
treatment is complex as it poses several challenges and risk of neurological deterioration.
Various factors that can influence the outcomes are as follows:

Anatomical features like size of the lipoma, location (midline or paramedian), wide bony
defect, defective muscles and fascia, poor cleavage at neurolipomatus junction.

Physiological factors like degree of traction, ability of cord to withstand the effects of
traction.
Pathological factors like vascularity of fibrolipomatous structures and associated
anomalies.[44–46]

Lumbosacral lipomyelomeningoceles have been classified by Chapman as dorsal,

transitional and terminal types, and by Aris into five types as dorsal, caudal, combined,
filar and lipomeningomyelocele.[47,48]

Dorsal variety lipomas enter into the segment of spinal cord dorsally through fibrofatty
pedicle, below which normal cord and dura exists. Transitional variety is essentially a
dorsal type which covers the entire conus and extends up to the filum. Distally, no normal
cord exists, whereas in terminal variety the normal looking conus ends into lipoma
through a dural defect and all the sacral roots are cranial to the lipoma.

Diagnosis in 90% of children is made by the presence of lipomatous mass or a cutaneous

marker. Associated hydrocephalus or cranial anomalies are extremely low though other
spinal anomalies can be associated. MRI (craniospinal screening) is the choice of
investigation for a comprehensive assessment [Figures 14–16]. The surgical treatment is
aimed at complete untethering and prevention of retethering of the cord. Based on the
progressive irreversible worsening, prophylactic surgery has been recommended by
many authors.[49] There is no controversy regarding lipomas of filum terminale. But a
report from Paris group showing 18.7% delayed deterioration on 10-year follow-up of
asymptomatic conus lipomas and a report from Osaka group stating that 88% of their
children operated prophylactically deteriorated with time had created further debate on
this issue.[41,49–53]

Surgical technique
Broad principles include vertical skin incision, dissection of subcutaneous lipoma up to
the fascia, excision of extraspinal component of lipoma, identification of upper and lower
laminae and performing laminectomy, identification of normal dura above and below
and separation of dural tube from the thoracolumbar fascia all around. These are
followed by dural opening (circumferential) around the fibrofatty stalk, removal of
intradural lipoma, gentle dissection of fibrofatty pedicle up to the neural structures over
the dorsal surface of cord and removal of fibrous tissue up to the neuro fibrolipomatous
junction, removal of other fibrous bands and finally detachment of filum.

All known precautions to prevent retethering, like pial closure to cover the raw dorsal
surface of the cord and duraplasty to enlarge the subarachnoid spaces and to facilitate
free CSF circulation around the repaired surface of the cord, are to be adopted.
Replacement of free fat over the dura fills the dead space, protects the soft tissue,
prevents CSF leak and prevents the skin edge necrosis. The spinal cord should be
eventually made free from five structures, i.e. skin, subcutaneous tissue, thoracolumbar
fascia, dura and filum terminale, to achieve complete untethering. Surgery for terminal
lipomas is relatively easy. One needs to separate dura from the lipoma and disconnect the
neural tissue at neurolipomatous junction which is often evident clearly.[18,53–59]

Myelocystocele is an occult form of spinal dysraphism with a localized, cystic dilatation of

the central canal of the spinal cord herniated through a posterior spina bifida. Terminal
myelocystocele truly is an anomaly of the caudal cell mass associated with anomalies of
anorectal system, lower genitourinary system and vertebrae such as anal atrecia, cloacal
extrophy, lordosis, scoliosis and variable degree of sacral agenesis. It constitutes 4-8% of
occult dysraphism and rarely occurs in lower thoracic region. The lesion consists of skin-
covered lumbosacral spina bifida, arachnoid lined meningocele directly continuous with
subarachnoid space and a low-lying hydromyelic spinal cord that traverses the
meningocele and forms a distal sac which does not communicate with the subarachnoid
space. MR appearance is distinctive and characterized by trumpet-like flaring of the distal
cord central canal into an ependyma-lined terminal cyst. Surgical correction can not only
release untethering, but also prevent the complications.[2,60–62]

Dermal sinus
Congenital dermal sinuses are a unique form of occult dysraphism presenting with
meningitis, tethering or neurological compression, with an incidence of 1 in 1500 births.
The dermal sinus tracts are lined by squamous epithelium and may penetrate anywhere
in the midline from the lumbosacral region to the occiput or nasion. Despite innocuous
external look, the tract may extend over several spinal levels before entering the dura or
getting attached to the filum of the spinal cord. Dermoid and epidermoid nodules are
frequently associated with these tracts. The small sinus osteum is quite often overlooked
unless discharge is noted by the parents. The neurological examination is nearly always
intact. The MRI is diagnostic. Embryologically, the formation of the dermal sinuses may
reflect incomplete dysjunction. Focal, incomplete separation of the cutaneous ectoderm
from the neural ectoderm during the 4th week of fetal development retains adherence of
these layers. Altered dysjunction can lead to dermal sinus termination from the
subcutaneous tissues to the intramedullary location. The most common location is
lumbosacral area. Thoracic and cervical dermal sinuses are present in 10%. There are
usually in midline, providing a portal for infection leading to meiningitis or intraspinal
abscesses. The most common organisms are Staphylococcus aureus and Escherichia coli
followed by Proteus species and anaerobes. Multiple organisms may be cultured, and
recurrent meningitis is one of the classical presentations. Neurological examination is
normal except in situations where the dermoid or epidermoids lead to cord compression.
The treatment includes antibiotics and complete surgical excision of the sinus, tract and
the associated lesions. Incomplete removal is the usual cause for recurrences.[63–69]

Syndromes of the tethered cord

Tethered cord syndrome may occur with conus in normal position with or without
associated neurocutanoeus markers. The affected children usually present with
hyperreflexic neurogenic bladder which is earlier referred as nonneurogenic bladder.
Urodynamic studies often reveal detrusor hyperreflexia. There is growing evidence that
sectioning of filum improves the bladder function in 96% of patients.[70–72]

Tethered cord syndrome in adults

Though unusual, it is a well-established entity. The late onset of presentation may be
related to the cumulative effects of repeated microtrauma. Distinct precipitating events
proceeding to the symptoms like heavy weight lifting, trauma and lithotomy position have
been described in 60% In many respects, it is similar to the pediatric population except
that the incidence of the low back pain and leg pain is significantly high in adults. MRI is
diagnostic [Figure 17]. Though indications for surgery are still controversial and not clear,
majority improve with proper surgical sectioning of the filum.[36,73–76]

Neurogenic bladder with conus at normal position

Children with this condition usually present to the urologists with urinary incontinence
and/or the associated complications. Tethered cord syndrome has been postulated as a
cause of neurogenic hyperreflexic bladder even in the presence of a conus medullaris at a
normal position. These conditions were earlier referred by different names like
nonneurogenic-neurogenic bladder. They usually present in children less than 10 years.
They do not have associated regional, neurological or any vertebral associated anomalies.
Urodynamics are highly suggestive. Release of filum terminale has shown significant
improvement in the bladder control and also the urodynamic abnormalities. Selcuki et al.,
described 96% improvement in bladder function.[77–80]

Footnotes
Source of Support: Nil.

Conflict of Interest: None declared.

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Figures and Tables

Figure 1

Myelomeningocele
Figure 2

Bifid spine
Figure 3

Bifida spine
Figure 4

Meningocele
Figure 5

Dysplastic skin
Figure 6

Lipoma with tuft of Hair

Figure 7

Tail
Figure 8

Tuft of hair
Figure 9

Dysplastic skin
Figure 10

Imaging of tethering
Figure 11

MRI of tethered cord

Figure 12

Retethering
Figure 13

Lipoma
Figure 14

Terminal lipoma
Figure 15

Lipomyelomeningoceles
Figure 16

Lipomyelomeningocele
Figure 17

Dermal sinus

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