ARTICLE

Early Identification of
Tethered Cord Syndrome:
A Clinical Challenge
Tiffany Sanchez, MS, CPNP, & Rita Marie John, EdD, DNP, CPNP, CMHS

ABSTRACT
Tethered cord syndrome (TCS) is a progressive clinical condition that arises from excessive spinal cord tension. The
clinical signs and symptoms of TCS may be cutaneous, neurologic, musculoskeletal, genitourinary, and/or gastrointestinal. Patients also may be asymptomatic, which does not
exclude the diagnosis of TCS. Although the exact etiology
is unknown, early identification and lifelong surveillance or
surgical treatment is an essential component of patient management. In this article we review the pathophysiology, various etiologies, clinical presentation, and long-term sequelae
of TCS. This information will help pediatric nurse practitioners identify TCS early and anticipate the patients needs
and management requirements. J Pediatr Health Care.
(2014) 28, e23-e33.

KEY WORDS
Tethered cord syndrome, pediatrics, spinal dysraphism,
scoliosis, diagnosis, treatment, outcome

Although it is rare, tethered cord syndrome (TCS) is

one of the most common pediatric spinal disorders
(Lad, Patil, Ho, Edwards, & Boakye, 2007). A tethered
cord is defined as a progressive functional disorder
Tiffany Sanchez, Pediatric Nurse Practitioner, School of Nursing,
Columbia University, New York, NY.
Rita Marie John, Assistant Professor, School of Nursing, Columbia
University, New York, NY.

caused by an abnormal pathologic fixation of the spinal

cord in the vertebral column (Hsieh, Perry, Gupta,
Pearson, & Nguyen, 2006). The incidence of TCS is estimated at 0.25 per 1000 births and is more common
in females (Bademci et al., 2006; McGirt et al., 2009).
In the past, the diagnostic criteria for TCS was
determined by a conus medullaris below the L1-L2
space and/or a filum terminale >2 mm in thickness.
The diagnostic criteria has evolved, and it is now defined as a fixation of the spinal cord to the spinal column
as a result of primary (congenital) or secondary (acquired) conditions, such as spinal dysraphism (i.e., congenital anomalies), physiologic conditions (e.g., thick
filum terminale), anatomic conditions (e.g., low conus
medullaris), or retethering as a result of a previous
spinal surgery. Although diagnosis is confirmed
through spinal imaging, recent studies have revealed
that history and physical examination are often the
hallmarks of diagnosing TCS (Filippidis, Kalani,
Theodore, & Rekate, 2010; Lew & Kothbauer, 2007;
Venkataramana, 2011). Because of the complexity of
this condition and the lack of clear guidelines,
diagnosis and management are often challenging.
In this article we discuss the embryology,
pathophysiology, etiology, and symptomatology of
TCS and the role of the pediatric nurse practitioner
(PNP) in diagnosing and managing patients with TCS
in a primary care setting.

Conflicts of interest: None to report.

Correspondence: Tiffany Sanchez, MS, CPNP, 630 W 168th St,
New York, NY 10032; e-mail: tssanche@gmail.com.
0891-5245/$36.00
Copyright Q 2014 by the National Association of Pediatric
Nurse Practitioners. Published by Elsevier Inc. All rights
reserved.
Published online August 9, 2013.
http://dx.doi.org/10.1016/j.pedhc.2013.06.007

www.jpedhc.org

EMBRYOLOGY
To appreciate the subsequent pathophysiology associated with TCS, it is essential to understand the three
stages of embryological development contributing
to normal spinal development. Embryologic errors
may occur during any of the three stages: gastrulation,
primary neurulation, and secondary neurulation.
Figure 1 provides an illustration of the following
discussion.
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FIGURE 1. Spinal cord development. (Image

by Chris Montague. Printed with permission.)

tion or a sacral meningeal cyst (Rossi et al., 2006). Definitions of select terms are provided in Table 1.
Primary Neurulation
Primary neurulation is a process in which the neuroectoderm is formed from the interaction of the ectoderm
and notochord during weeks 3 to 4 of gestation. Initially, the neuroectoderm lies flat; however, folding
along the midline in a bidirectional fashion forms the
neural tube. By day 27, full closure of the neural tube
in the lumbar region (L1-L2) is attained and is referred
to as the caudal neuropore. Failure of neural tube closure results in open spinal dysraphism such as meningocele, lipomyelomeningocele, dermal sinus tract,
myelomeningocele, myelocele, split cord malformations, or intraspinal lipoma. The severity and extent of
neurologic impairment is correlated with the location
and degree of the malformation. A worse prognosis is
likely when the lesion is higher on the spinal cord
(Bui, Tubbs, & Oakes, 2007; Hertzler et al., 2010;
Rossi et al., 2006; Venkataramana, 2011).

Gastrulation
Gastrulation is a period of cellular proliferation and migration of the ectoderm (one of the three germ layers)
during weeks 2 and 3 of gestation. During this embryonic stage, the neural tube forms from folding of the
neural plate. The neural tube begins to close by day
22 to 23 in a cephalo-caudal fashion with full closure
of the posterior neuropore by day 25 to 27 (Hertzler,
DePowell, Stevenson, & Mangano, 2010). At the conclusion of this stage, the forming notochord serves as
the building block of the axial skeleton. Abnormal fusion of the germ layers during this stage may result in
congenital malformations such as split cord malforma-

Secondary Neurulation
Secondary neurulation follows the conclusion of primary neurulation and continues until day 48 of gestation. This stage is marked by the formation of the
caudal tube from undifferentiated cells below the posterior neuropore. During this stage, the distal neural
tube is formed via fused vacuoles that are derived
from the caudal cell mass. Before the end of this
stage, the ventriculus terminalis is formed at the
coccygeal level and serves as a marker for the conus
medullaris (Lew & Kothbauer, 2007; Rossi et al.,
2006). After secondary neurulation, regression occurs
and continues into the early postnatal period. The
terminal spinal cord undergoes changes, forming the
cauda equina and filum terminale. As the vertebral
column lengthens during periods of growth, the filum
terminale elongates and the conus medullaris begins
to ascend. By 2 months of age, the conus medullaris
is in its normal adult position at L1-L2, with some normal variation (Bui et al., 2007; Lew & Kothbauer,
2007). Disruption during this stage is attributed to

TABLE 1. Definition of commonly used terminology

Terminology

Definition

Anterior sacral meningocele

A presacral herniation of a cerebral spinal fluid filled sac that protrudes through the vertebral
column and often found in patients with caudal agenesis (Rossi et al., 2006)
Agenesis of the caudal portion of the spine that may be total or partial (Rossi et al., 2006)
A bundle of nerve roots (Hertzler et al., 2010)
The caudal end of the spinal cord (Lew & Kothbauer, 2007)
Incomplete closure of the neural tube with malformation of the spine
A mass composed of subcutaneous fat contained within a dural sac that may laterally
displace the spinal cord (Rossi et al., 2006)
A cystic structure that will be the future site of the conus medullaris (Lew & Kothbauer, 2007)

Caudal agenesis
Cauda equina
Conus medullaris
Dysraphism
Lipoma
Ventriculus terminalis

Data from Hertzler et al., 2010; Lew & Kothbauer, 2007; and Rossi et al., 2006.

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Journal of Pediatric Health Care

abnormalities of the mesenchymal tissue and epidermis

resulting in occult (closed) spinal dysraphisms, such as
lipoma, caudal agenesis, low conus medullaris, anterior
sacral meningocele, or tethered cord (Rossi et al., 2006;
Venkataramana, 2011).
PATHOPHYSIOLOGY
The filum terminale is an elastic band that stabilizes the
conus medullaris and enables it to hang freely and move
during normal and abnormal flexion/extension of the
spine and during skeletal growth (Figure 2). When the
normal elasticity of this structure is compromised, increased tension and stress is placed on the conus medullaris. This phenomenon is thought to prevent the conus
medullaris from ascending to its normal adult position at
L1-L2 by 6 months of age, resulting in secondary (ana-

FIGURE 2. Posterior view of spinal cord,

nerves, conus medullaris, and filum terminale.
(Image by Chris Montague. Printed with
permission.)

tomic) cord tethering in which the spinal cord fixates

to the spinal column. Over time, stretching of the fixed
spinal cord causes biochemical and electrophysiologic
changes that damage the blood vessels, nerve fibers,
and nerve cells in the spinal cord (Bademci et al.,
2006; Bui et al., 2007; Kang, 2008; Selden, 2007).
The underlying pathophysiologic effects of the TCS
are best explained by the ischemic hypothesis
(Yamada et al., 2007). The results of the work of Yamada
and colleagues suggested that the degree of caudal traction decreases blood flow and oxidative metabolism,
resulting in cellular hypoxia and eventually ischemic injury. This disrupts oxidative metabolism causes a shift in
the redox ratio by reducing cytochrome a,a3 and adenosine triphosphate, which ultimately impairs nerve
function (Kang, 2008; Stetler, Park, & Sullivan 2010;
Yamada & Won, 2007). The functional changes
supported by the ischemic phenomenon affects the
lower motor neurons, manifesting as dysfunction and
symptomatology in the lower extremities (Husain &
Shah, 2009; Macejko et al., 2007).
Follow-up studies reveal that surgical untethering
may result in varying degrees of neurologic recovery
by reversing the decreased metabolic status and improving oxygenation. The degree of reversibility is
largely based on the degree and duration of spinal
cord traction. Therefore patients with mild to moderate
traction often experience complete recovery, whereas
severe traction often result in minimal or incomplete recovery (Filippidis et al., 2010; Yamada et al., 2007).
ETIOLOGY
The exact etiology of cord tethering is extensive and not
well understood but likely is influenced by genetic and
environmental factors. The following section will discuss primary and secondary conditions associated
with TCS.
Risk Factors
Myelomeningocele
Spina bifida refers to any birth defect involving incomplete closure of the spine. Myelomeningocele is the
most common type of spina bifida and often requires repair soon after birth. Hertzler et al. (2010) stated that up
to 32% of these persons experience cord retethering as
a result of scar tissue anchoring the spinal cord and preventing it from ascending. This phenomenon is most
commonly seen between the ages of 5 and 9 years
when the vertebral column rapidly lengthens, resulting
in spinal cord tension, but it can occur at any age from
sudden stretching or repetitive movements (Hertzler
et al., 2010).
Scoliosis
The incidence of tethered spinal cord and other spinal
cord anomalies is estimated to be 20% in persons with

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TABLE 2. Congenital syndromes associated with tethered cord syndrome

Syndrome

Clinical manifestations

Rubinstein-Taybi syndrome

Gait abnormalities
Short stature
Short limbs
Characteristic facies
Developmental delay
Tethered spinal cord
Vertebral malformations (including tethered
cord syndrome)
Anal atresia
Cardiac anomalies
Transesophageal fistula
Renal anomalies
Limb abnormalities

VACTERL

Genetic association
Chromosome 16

Deletions of GLI2 and GLI3

transcription genes

Data from ONeill et al., 2010, and Tanaka et al., 2006.

congenital or juvenile scoliosis. Some patients may

have a minor degree of cord traction and remain asymptomatic throughout most of early childhood. This subset of patients often present in adolescence or later in
life with neurologic deficits likely induced by repeated
factors aggravating the spine, such as spinal stenosis,
trauma over time, an increase in physical activity, or
pregnancy (Hertzler et al., 2010).
Genetic Association
Recent genetic and twin studies suggest that TCS
may be genetically inherited. Mitsuka, Horikoshi,
Watanabe, & Kinouchi (2009) described two 11-yearold identical twin boys with complaints of unremitting
leg pain within 11 months of each other. Magnetic resonance imaging (MRI) revealed a normal-lying conus
medullaris and fatty filum terminale in both cases, suggesting a genetic association. Bassuk et al. (2005) found
an association between the TBX1 gene located in the
22q11.2 locus and TCS. Findings suggest that a missense
mutation in TBX1 or a deletion of 22q11.2 present with
abnormalities in the caudal spine (Bassuk et al., 2005;
Filippidis et al., 2010). This deletion is among the
most common and is estimated to exist in 1:2000 persons. Although a deletion 22q11.2 is most notable for
being associated with congenital heart diseases, this
gene is highly variable and has been associated with
180 various anomalies (Robin & Shprintzen, 2005). It
is important for PNPs to consider TCS in patients with
22q11.2 deletion syndromes or TBX1 mutations or
whose siblings are affected.
Congenital Syndromes
Several congenital syndromes are associated with TCS
(see Table 2). Rubinstein-Taybi syndrome is a rare condition associated with mutations that affect chromosome 16. This gene is responsible for regulating
cellular division and cellular growth and is a central
component for normal fetal development (Tanaka,
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Ling, Rubinstein, & Crone, 2006). VACTERL association

(Vertebral anomalies, Anal atresia, Cardiac defect, Tracheo Esophageal fistula, Renal abnormalities, and
Limb abnormalities) is associated with deletions of
GLI2 and GLI3 transcription genes. In a retrospective
study of infants with VACTERL, 39% were diagnosed
with TCS via ultrasound screening during the neonatal
period and required cord untethering. Several studies
suggest an increased incidence of TCS if a transesophageal fistula, anal atresia, imperforate anus, or urogenital
anomaly is present (Kuo et al., 2007; ONeill, Yu, &
Tyler-Kabara, 2010). Persons with a genetic syndrome
often present with severe developmental delays or
symptoms overlapping with other conditions that
are often treated independently of TCS. PNPs need to
consider TCS when caring for patients with these
syndromes.
HISTORY
A clinical history suggestive of TCS varies based on age,
onset of symptoms, and the underlying cause (Table 3).
In neonates and toddlers, cutaneous stigmata may be
the only indication of a tethered spinal cord; however,
other features consistent with TCS may exist (Lew &
Kothbauer, 2007). A history remarkable for decreased
lower extremity movement or failure to meet developmental milestones suggests the presence of neurologic
symptoms. A history of urinary dribbling, decreased dry
periods between diaper changes, or frequent accidents
(i.e., enuresis or encopresis) in a previously potty
trained toddler suggests genitourinary and/or gastrointestinal disturbances. According to Hertzler and
colleagues (2010), it is difficult to detect in infants and
toddlers who have not gained full continence (i.e., are
not potty trained) or lack the ability to communicate
such complaints (Hertzler et al., 2010; Lew &
Kothbauer, 2007).
In young children and adolescents, neurologic deterioration is a common manifestation. The young childs
Journal of Pediatric Health Care

www.jpedhc.org

TABLE 3. Relevant history questions and vital components of the physical examination
System
General

Cutaneous

Neurologic

Musculoskeletal

Genitourinary and
gastrointestinal

Pertinent history questions

1. Has a past or recent trauma occurred? If so, explore the cause and timing
2. Does the patient have a history of spinal surgery? If so, why was it performed,
and when?
3. Obtain a history of any sports or physical activities the child has participated in
and the length of participation
1. Does the patient have any history of tufts of hair, nevi, dimples, or
hemangiomas in the newborn or infant period? If so, was any surgical
treatment performed?

May/June 2014

1. Does the patient have any changes in muscle tone, especially in the calf and
buttock region?
2. Does the patient have any changes in the ability to extend (kick) the lower
extremities?
3. Has the patient had any delay in motor milestones, such as walking? Does he
or she have an unsteady gait, drag one or both feet when walking, have a wide
stance when ambulating, or exhibit clumsiness?
4. Are there any changes in sensation in the lower extremities?
5. Does the patient have pain? If so, ask him or her to take one finger and point to
where he or she feels pain
6. If pain is present, is it made worse by back extension or flexion? Explore pain
using the PQRST (provokes, quality, radiates, severity, time) method
7. Does the patient have a history of ulcerations on the lower extremities, and if
so, do the ulcerations heal?
Infant/toddler:
1. Explore developmental milestones with respect to standing and walking: age
of standing, first step, difficulty walking, and decline in previously attained skills
School-age child/adolescent:
1. Does the patient have a history of scoliosis, and if so, what was the age at
diagnosis, and has any treatment been provided?
2. Has the patient had any difficulties with walking or running?
Infants:
1. Ask parent(s)/caregiver if there are any dry periods between diaper changes
Toddlers:
1. Has the child started to toilet train?
2. Does the child experience accidents during the day or at night?
School-age child/adolescent:
1. Does the child experience frequent urina?
2. Does the child experience difficulty with bladder or bowel control during the
day, night, or both? Explore symptoms of frequency, urgency, poor voluntary
control, and incomplete voiding
3. Does the child have a history of constipation?

Physical examination

Examine the dorsal spine; assess for midline stigmata such as sacral dimples,
tufts of hair, hypertrichosis, hemangiomas, dimple, lipoma, dermal sinus tract,
or other cutaneous stigmata; assess for atretic meningocele and lumbosacral
skin appendages
Assess motor abilities: decreased deep tendon reflexes, spasticity, clonus,
Babinski reflex, gait disturbances; assess sensory disturbances in older
children: proprioception, light touch, painful stimulation (pinprick), skip lesions;
assess for decreased sensation in the perineal region, activity level not
consistent with age, or developmental milestones not consistent with age;
look for painless skin ulcerations; observe for gait disturbances

Assess the dorsal spine for any evidence of scoliosis, kyphosis, or lordosis;
assess the lower extremities for orthopedic deformities (e.g., clubfoot,
hammer ties, talipes, and arches), leg length discrepancy, hip subluxation and
ankle imbalance; assess muscles (calf and buttocks) for symmetry or signs of
atrophy; look for asymmetric muscle weakness

Check for anorectal malformations and sphincter disturbances: lack of anal wink
(difficult to detect in children <1 year); ask about dribbling

Data from Michelson & Ashwal, 2004 (regarding history questions) and Lew & Kothbauer, 2007 (regarding the physical examination).

e27

history may be remarkable for gait disturbances (e.g.,

wobbly gait, wide gait, or delayed ambulation), delayed milestones, or regression of previously
The young childs
attained milestones.
history may be
Conversely, older chilremarkable for gait
dren and adolescents
may report progresdisturbances (e.g.,
sion of motor disturwobbly gait, wide
bances as evidenced
gait, or delayed
by a history of asymmetrical lower extremambulation),
ity weakness, gait
delayed
disturbances
(e.g.,
milestones, or
a clumsy leg or dragging of the affected
regression of
foot), or difficulty
previously attained
running
(Lew
&
milestones.
Kothbauer, 2007). A
history of functional
changes to the genitourinary and gastrointestinal system includes fecal incontinence, constipation, urinary
incontinence, urinary frequency, urinary urgency, urinary retention, or recurrent urinary tract infection
(Hertzler et al., 2010; Pinter & Bognar, 2009).
A history of pain is common in the adolescent and
adult population but is rare in young children. It has
been hypothesized that children younger than 6 years
lack the ability to localize pain and communicate this
manifestation. If a young child reports pain from TCS,
it is often insidious, poorly localized, confined to the sacral region, and rarely radiates to the lower extremities.
Conversely, more than 90% of school-age children, adolescents, and adults aged between 7 and 25 years experience unrelenting nondermatomal pain, described
as a shock-like feeling with localization to the lower extremities, lumbosacral spinal region, or perineal region
(Kang, 2008; Kang et al., 2009; Lew & Kothbauer, 2007).
Although pain is almost always a universal symptom in
adolescents, young children may still describe pain
using unfamiliar vocabulary or unusual behavior.
Table 3 provides a list of relevant history questions
based on system and age.

2010). Cutaneous stigmata are often seen midline in

the lumbosacral region and may include subcutaneous
lipoma, midline hypertrichosis, hemangioma, dermal
sinus tracts, dermal pit, masses, asymmetrical gluteal
crease, atretic meningocele, or lumbosacral skin appendages as illustrated in Figure 3 (Hertzler et al.,
2010; Lew & Kothbauer, 2007).
Neurologic Manifestations
Neurologic deterioration is attributed to impaired nerve
function and metabolic function of gray matter in the
lumbosacral region of the spinal cord. Patients with
TCS often experience one or more signs of neurologic
deterioration (Al-Holou, Muraszko, Garton, Buchman,
& Maher, 2009; Michelson & Ashwal, 2004). Neurologic dysfunction may consist of motor or sensory
deficits, with the former being more common. On
physical examination, lower motor neuron signs of
decreased reflexes may be present, which are often
unilateral and reflect involvement of the cortico-spinal
tract. Other signs may include abnormal gait, decreased
tone, or abnormal reflexes (i.e., upward going Babinski
reflex). Although they are less common, sensory
deficits to the lower extremities present in a nonsegmental patchy pattern (i.e., a decreased sensation to
FIGURE 3. Lumbosacral cutaneous stigmata in
a 2-month-old girl with split cord malformation
and tethered cord syndrome. (Image courtesy
of Dr. Rita Marie John. Reprinted with
permission.)

PHYSICAL ASSESSMENT
Physical assessment is vital for diagnosis, and findings
vary based on the childs age (Table 3). The physical assessment allows the provider to detect deterioration because patients may present with one or more clinical
manifestations from the following systems.
Cutaneous Stigmata
Cutaneous manifestations are among the most common
physical findings consistent with TCS and are present in
approximately 59% of patients, with 3% being neonates
(Bui et al., 2007). This coexistence is caused by their
common origin from the ectoderm (Drolet et al.,
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BOX 1. Differential diagnosis of tethered cord

syndrome

BOX 2. Indications for imaging with regard to

lumbosacral lesionsa

Neoplastic diseases
 Ependymoma
 Astrocytoma
 Medulloblastoma/primitive neuroectodermal tumors
 Intracranial germ cell tumors
Infectious diseases
Inflammation resulting from trauma
Inherited degenerative diseases
 Spinocerebellar degeneration
 Sensory neuropathy
 Friedreich ataxia
Neurologic condition
 Demyelinating condition
 Global developmental delay

1. Sacral dimples that are >0.5 mm, extend through the

dural/subarachnoid space, and are located above
the gluteal crease
2. Midline mass near L5
3. Hemangioma
4. Midline hypertrichosis
5. Asymmetrical gluteal crease
Data from Hertzler et al., 2010; Lew & Kothbauer, 2007;
and Splete, 2007.
a
Rule of thumb: All midline lesions are suggestive of
a spinal anomaly until proven otherwise.

Data from Michelson & Ashwal, 2004.

light touch, temperature, and pinprick), and painless

ulcerations on the perineum or feet may be present
(Bui et al., 2007; Kang, 2008; Lew & Kothbauer, 2007;
Michelson & Ashwal, 2004).
Musculoskeletal Manifestations
Musculoskeletal changes are present in 90% of patients
with TCS, with foot deformities being the most common
(Bui et al., 2007). These changes are attributed to muscle tone imbalance in the lower extremities. The lower
extremities should be evaluated for foot asymmetry,
foot deformities (e.g., high arches or club foot), gluteal
asymmetry, leg length discrepancy, or hip subluxation.
Muscular atrophy may be apparent but is less visible in
infants because of excess subcutaneous fat (Michelson
& Ashwal, 2004). Evaluation of the spine for vertebral
abnormalities may reveal abnormal curvature, which
is estimated to be present in up to 25% of persons
with intraspinal pathology. This clinical presentation
is diagnosed as idiopathic left thoracic scoliosis that rapidly progresses in a child younger than 11 years, with
pain as the primary manifestation (Hertzler et al.,
2010; McGirt et al., 2009; Michelson & Ashwal, 2004).
Although many of these signs may be attributed to
other etiologies, surgical untethering has proven to
successfully improve symptoms. An underlying spinal
cord anomaly or worsening TCS should be considered in persons with musculoskeletal deformities
or progressive clinical deterioration.
Genitourinary and Gastrointestinal
Manifestations
Bladder and bowel dysfunction are two manifestations
resulting from disrupted sphincter innervation (ONeill
et al., 2010). Abnormalities may vary from subclinical
presentation that is only detectable though urodynamic
studies to severe dysfunction based on patient history
www.jpedhc.org

(Bui et al., 2007). Although an anorectal malformation

may be present on physical examination, detection
may be based solely on abnormal urodynamic studies (ONeill et al., 2010; Pinter & Bognar, 2009). The
ability to distinguish between neurogenic and nonneurogenic bowel and bladder dysfunction is critical.
DIFFERENTIAL DIAGNOSIS
Very minor differences in a patients history and physical examination may distinguish the diagnosis of TCS
from another disorder (Box 1). These conditions often
affect the lumbosacral spinal cord and present with
changes in deep tendon reflexes and/or progressive
lower extremity weakness during childhood. Based
on the overlap in clinical presentation, a diagnosis of
TCS often remains inconclusive until imaging studies
are performed to exclude other possible causes
(Michelson & Ashwal, 2004).
DIAGNOSIS
In the vast majority of pediatric patients, the diagnosis
of TCS is based on a high index of suspicion obtained
from a detailed history and physical examination.
Clinical evidence of cutaneous stigmata, neurologic
manifestations, musculoskeletal manifestations, or
genitourinary/gastrointestinal manifestations consistent
with TCS should be confirmed with diagnostic studies.
Midline spinal lesions are seen in 70% of persons with
closed spinal dysraphism, which may be an underlying
cause of TCS (Pinter & Bognar, 2009). Although common, not all lesions are suggestive of a spinal anomaly,
and therefore it is important that the PNP be familiar
with indications for imaging with regard to lumbosacral
lesions. All midline lesions are suggestive of a spinal
anomaly until proven otherwise (Box 2). Indications include sacral dimples that are >0.5 mm deep, extend
through the dura/subarachnoid space, and are located
above the gluteal crease; a midline soft mass near L5,
which may suggest a lipomyelomeningocele; a hemangioma, which may suggest an intradural anomaly; midline lumbar hypertrichosis; and an asymmetric gluteal
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crease. However, non-midline lesions and sacral dimples in the natal cleft region are often benign and do
not require diagnostic imaging (Hertzler et al., 2010;
Lew & Kothbauer, 2007; Splete, 2007).
Diagnostic Imaging Studies
Multiple diagnostic neuroimaging studies may be performed to provide the clinician with findings associated
with TCS. Plain radiographs are readily available and
provide the clinician with information regarding the existence of vertebral anomalies; however, they are not
routinely performed because of their low sensitivity
and specificity. Infants younger than 3 months may
have incomplete ossification, and ultrasonography is
used as the first-line screening method to evaluate the
spinal cord.
Ultrasonography is an inexpensive and effective
screening tool for determining the level of the conus
medullaris or to investigate underlying spinal dysraphism in infants younger than 3 months. Although
this technique does not expose the infant to radiation,
interpretation of results is entirely dependent on the operator. Beyond 3 months of age, the conus medullaris
ascends in the spinal column to the L1-L2 region and
is no longer visible because of vertebral ossification
(Bademci et al., 2006; Bui et al, 2007; Lew &
Kothbauer, 2007; Miyasaka et al., 2009). If ultrasound
findings are abnormal, an MRI should be performed
(Ben-Sira, Pogner, Miller, Beni-Adani, and Constanti,
2009; Schenk et al, 2006).
MRI is the gold standard imaging for the evaluation
and diagnosis of intraspinal pathology in persons
3 months of age and older. MRI of the lumbosacral spinal region is used to determine the anatomic cause of
spinal tethering, motion of the spinal cord, the level
of the conus medullaris, and to identify the nature of
the filum terminale (Bademci et al., 2006; Miyasaka
et al., 2009; Pinter & Bognar, 2009). Recent studies
show that there is a high correlation of TCS with
imperforate anus or lumbosacral hemangioma during
infancy, and it is recommended that these persons be
screened with MRI (Drolet et al., 2010; Miyasaka et al.,
2009; Tarcan et al., 2012). Computed tomography
scans may be used in conjunction with MRI to
evaluate the septum if spinal tethering is attributed to
split cord malformation or to evaluate the anatomy of
the bone (Lew & Kothbauer, 2007). Although MRI and
computed tomography scans are useful in determining
the anatomic and pathologic conditions of the distal
spine, children routinely require sedation to obtain
quality results.
Urodynamics
As previously stated, urologic dysfunction is a prevalent
symptom in the pediatric population (Pinter & Bognar,
2009; Stavrinou et al., 2010). These findings are difficult
to diagnose from clinical presentation or history alone.
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Urodynamic studies have been deemed a useful

assessment for determining sphincter dysfunction in
children with urologic symptoms or subclinical
symptoms with high suspicion of TCS (Hsieh et al.,
2006). Urodynamic studies are routinely undertaken
in the vast majority of patients diagnosed with TCS, including infants, because they provide an objective assessment both before and after surgery. They also
detect further deterioration when supportive treatment
is suggested (Hertzler et al., 2010; Selden, 2007).
CURRENT MANAGEMENT OF TCS
Despite the morbidity associated with TCS and improved diagnostic studies to better understand the etiology, a search of National Guidelines Clearing House
did not produce any specific guidelines for the treatment of pediatric patients. The type of management is
largely based on the patients age, clinical presentation,
and institutional protocol. Although a fine line exists
between treatment approaches, the ultimate goal is to
minimize symptoms and risks associated with the tethered spinal cord.
Surgical Management
Surgical management is often recommended in the
pediatric population to prevent further neurologic deterioration and possibly reverse, stabilize, or improve
symptoms. Several authors suggest that patients with
a spinal dysraphism,
Surgical
low-lying conus medullaris, fatty filum
management is
terminale, and/or prooften
gressive clinical derecommended in
terioration are ideal
candidates for spinal
the pediatric
cord
untethering
population to
(Bowman, Mohan, Ito,
prevent further
Seibly, & Mclone, 2009;
Drake, 2007; Ostling,
neurologic
Bierbrauer, & Kuntz,
deterioration and
2012). The goal of
possibly reverse,
surgery is to promote
spinal cord movement
stabilize, or
by relieving tension,
improve
decreasing cellular hysymptoms.
poxia, and improving
cellular
conduction
(Gupta, Heary, & Michaels, 2010; Husain & Shah,
2009; Metcalfe et al., 2006). Although evidence is
conflicting on an optimal time to perform spinal
cord untethering, Lew & Kothbauer (2007) suggested that early intervention provided the best
outcome in terms of neurologic functioning, urologic
functioning, postsurgical complications, risk for
infection, and risk for neural damage. Early
diagnosis by the primary care PNP and referral to
neurosurgery and the neurosurgical PNP allows
Journal of Pediatric Health Care

FIGURE 4. Diagnosis and management flowchart for the pediatric nurse practitioner (PNP). CT,
Computed tomography; MRI, magnetic resonance imaging.

communication between the specialty PNP and the

primary care PNP.
LONG-TERM SEQUELAE
The prognosis of TCS is variable based on the etiology
of each individual. Recent clinical studies suggested
that further deterioration is imminent in the pediatric
population and that the diagnosis of TCS alone should
be an indication for surgery (Bui et al., 2007; Cornips
et al., 2011). One study suggested that tethered cord
release provides immediate clinical improvement in
urologic symptoms (72%) and fecal symptoms (91%)
in pediatric patients (Metcalfe et al., 2006). Other studies have suggested that tethered cord release halts curve
progression or provides curve stabilization in patients
with scoliosis once skeletal maturity has been achieved
(McGirt et al., 2009; Mehta et al., 2011; Samdani, Asghar,
Pahys, Andrea, & Betz, 2007). Conversely, 26% who
underwent surgery for a spinal dysraphism or cord
untethering experienced symptomatic retethering
(Samuels et al., 2009). The prevalence is highest during
ages 6 and 13 years when the spinal cord remains anchored at the previous surgical repair site as the child
grows (Al-Holou et al., 2009).
ROLE OF THE PEDIATRIC NURSE
PRACTITIONER
Approximately 75% of pediatric patients with TCS experience a constellation of deficits that increase progressively with age. Because multiple systems are
www.jpedhc.org

involved, the clinical symptoms may initially be treated

independently of TCS (Gupta et al., 2010). Because of
the risks associated with untreated TCS, it is imperative
that PNPs correctly identify patients with TCS.
If the PNP has a high index of suspicion of TCS based
on a thorough history and physical examination
(Table 3), an MRI (which is the gold standard) should
be performed to view the spinal anatomy, cause of tethering, and extent of damage to surrounding tissue. If the
findings are consistent with TCS, a referral to a neurosurgeon is indicated to prevent further deterioration,
especially in young children who have not yet attained
full skeletal maturity. The acute care PNP specializing in
neurosurgery plays an active role in the care of these patients. Communication between the primary and acute
care PNP can help families understand the management
of the patient. Joint referral to other subspecialists
should be arranged when indicated, and appropriate
diagnostics should be performed. Specialists may
include orthopedic surgeons, neurologists, urologists,
rehabilitation medicine practitioners, and/or gastroenterologists. Additionally, education should be provided
on the condition and activity limitations because repetitive flexion/extension result in further cord tethering.
Finally, collaboration between the referring primary
care PNP and neurosurgical PNP should be maintained
to coordinate care and remain updated on clinical status
to ensure that optimal care and treatment are provided.
These patients may be seen in multidisciplinary clinics
depending on their availability.
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e31

Although operative procedures release the tethered

spinal cord, in some cases, clinical manifestations may
still persist because of irreversible nerve damage that
is sustained. Therefore
some children may still
PNPs should
experience urinary inmonitor patients for
continence, difficulty
the development of
with ambulation, or
other clinical maniearly symptoms
festations. PNPs may
that suggest cord
prescribe
adaptive
retethering or
equipment or refer
such patients to a physprogressive
iatrist, physical therapy,
worsening of
occupational therapy,
symptoms.
early intervention (if
the patient is younger
than 3 years), or social work for remediation. The goal
is to aid with physical and developmental impairments
to achieve the best possible level of functioning for
each individual.
Long-term surveillance is imperative. During routine
well-child visits, it is important that PNPs closely
monitor high-risk patients, particularly those who underwent lumbosacral spinal surgery for spinal dysraphism or tethered cord, especially during growth
spurts. PNPs should monitor patients for the development of early symptoms that suggest cord retethering
or progressive worsening of symptoms. Encouraging
regular follow-up with subspecialists and ensuring adequate communication between the subspecialist and
the primary PNP is critical.
The psychosocial effects experienced by patients
with TCS should be evaluated at every visit. The PNP
should assess each patients strengths, weaknesses, developmental level, social situation, environmental constraints, and family strengths to ensure optimal support
and treatment. Interventions include educating the
family and patient about the condition, providing answers to questions, scheduling regular follow-up visits,
referring patients to therapists or community support
groups, and/or collaborating with the patients school
to ensure that a supportive environment is provided
(Perrin, Gnanasekaran, & Delahaye, 2012).
The PNP provides primary care by meeting the needs
that are specific to each patient. Optimal care requires
open communication with subspecialists, adequate
family support, and patient advocacy. The PNP needs
to stay up to date with current research on TCS and incorporate the most recent evidence into the clinical setting to ensure that quality care is provided to each
patient (Figure 4). The ultimate goal of the primary
care PNP and the neurosurgical PNP is to ensure optimal growth and development and that functional and
medical needs are met through a family-centered and
multidisciplinary team.

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Volume 28  Number 3

CONCLUSION
Diagnosing and managing TCS remains a challenge for
many providers. Despite the evolving definition of TCS,
the exact etiology is variable and not well understood.
To date no definitive guidelines exist with respect to
diagnosis and treatment. Despite the inconsistency in
presentation and diagnostic findings, it is vital that
PNPs be familiar with the distinct findings and recognize TCS in its early stages to provide necessary management and help prevent irreversible damage,
reverse deficits, and ultimately improve the quality of
life of each affected pediatric patient.
We thank Chris Montague for assisting us with his
technical artistic ability.
REFERENCES
Al-Holou, W. N., Muraszko, K. M., Garton, H. J., Buchman, S. R., &
Maher, C. O. (2009). The outcome of tethered cord release in
secondary and multiple repeat tethered cord syndrome. Journal
of Neurosurgery: Pediatrics, 4(28), 28-36.
Bademci, G., Saygun, M., Batay, F., Cakmak, A., Basar, H., Anbarci,
H., & Unal, B. (2006). Prevalence of primary tethered cord syndrome associated with occult spinal dysraphism in primary
school children in turkey, Prevalence of primary tethered cord
syndrome associated with occult spinal dysraphism in primary
school children in turkey. Pediatric Neurosurgery, 42(1), 4-13.
Bassuk, A. G., Craig, D., Jalali, A., Mukhopadhyay, A., Kim, F., Charrow, J., . Kessler, J. A. (2005). The genetics of tethered cord
syndrome. American Journal of Genetics, 132A(4), 450-453.
Ben-Sira, L., Pogner, P., Miller, E., Beni-Adani, L., & Constanti, S.
(2009). Low-risk lumbar skin stigmata in infants: The role of
ultrasound screening. Journal of Pediatrics, 155(6), 864-869.
Bowman, R. M., Mohan, A., Ito, J., Seibly, J. M., & Mclone, D. G.
(2009). Tethered cord release: A long term study in 114 patients. Journal of Neurosurgery Pediatrics, 3(3), 181-187.
Bui, C. J., Tubbs, R. S., & Oakes, W. J. (2007). Tethered cord syndrome in children: A review. Neurosurgical Focus, 23(2), 1-9.
Cornips, E. M. J., Vereijken, I. M. O., Beuls, E. M. A., Weber, J. W.,
Soudant, D. L. H. M., Van Rhijn, L. W., . Vles, J. S. H.
(2011). Clinical characteristics and surgical outcome in 25
cases of childhood tight filum syndrome. European Journal of
Paediatric Neurology, 16(2), 103-117.
Drake, J. M. (2007). Surgical management of the tethered spinal
cordwalking the fine line. Neurosurgical Focus, 23(2), 1-4.
Drolet, D. A., Chamlin, S. L., Garzon, M. C., Adams, D., Baselga, E.,
Haggstrom, A. N., . Frieden, I. J. (2010). Prospective study of
spinal anomalies in children with infantile hemangiomas of the
lumbosacral skin. Journal of Pediatrics, 157(5), 789-794.
Filippidis, A. S., Kalani, M. Y., Theodore, N., & Rekate, H. L. (2010).
Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord
syndrome. Neurosurgical Focus, 29(1), 1-5.
Gupta, G., Heary, R. F., & Michaels, J. (2010). Reversal of longstanding neurological deficits after a late release of tethered spinal
cord. Neurosurgical Focus, 29(1), 1-4.
Hertzler, D. A., II, DePowell, J. J., Stevenson, C. B., & Mangano, F. T.
(2010). Tethered cord syndrome: A review of the literature from
embryology to adult presentation. Neurosurgical Focus, 29(1),
1-9.
Hsieh, M. H., Perry, V., Gupta, N., Pearson, C., & Nguyen, H. T.
(2006). The effects of detethering on the urodynamics profile
in children with a tethered cord. Journal of Neurosurgery,
105(5), 391-395.

Journal of Pediatric Health Care

Husain, A. M., & Shah, D. (2009). Prognostic value of neurophysiologic intraoperative monitoring in tethered cord syndrome surgery. Journal of Clinical Neurophysiology, 26(4), 244-247.
Kang, J. K. (2008). Pathophysiology and clinical features of tethered
cord syndrome. In M. M. Ozek, G. Cinalli & W. J. Maixner (Eds.),
Spina bifida: Management and outcome (1st ed., pp. 275-280).
Milan, Italy: Springer.
Kang, J. K., Yoon, K. J., Ha, S. S., Lee, I. W., Jeun, S. S., & Kang,
S. G. (2009). Surgical management and outcome of tethered
cord syndrome in school-aged children, adolescents, and
young adults. Journal of Korean Neurosurgery Society, 46(5),
468-471.
Kuo, M. F., Tsai, Y., Hsu, W. M., Chen, R. S., Tu, Y. K., & Wang, H. S.
(2007). Tethered spinal cord and VACTERL association. Journal
of Neurosurgery, 106(3), 201-204.
Lad, S. P., Patil, C. G., Ho, C., Edwards, M. S. B., & Boakye, M.
(2007). Tethered cord syndrome: Nationwide inpatient complications and outcomes. Neurosurgical Focus, 23(2), 1-5.
Lew, S. M., & Kothbauer, K. F. (2007). Tethered cord syndrome: An
updated review. Pediatric Neurosurgery, 43(3), 236-248.
Macejko, A. M., Cheng, E. Y., Yerkes, E. B., Meyer, T., Bowman,
R. M., & Kaplan, W. E. (2007). Clinical urological outcomes following primary tethered cord release in children younger than
3 years. Journal of Urology, 178(4 Part 2), 1738-1743.
McGirt, M. J., Mehta, V., Garces-Ambrossi, G., Gottfried, O., Solakoglu, C., Gokaslan, Z. L., . Jallo, G. I. (2009). Pediatric tethered
cord syndrome: Response of scoliosis to untethering. Journal of
Neurosurgery: Pediatrics, 4(3), 270-274.
Mehta, V. A., Gottfried, O. N., McGirt, M. J., Gokaslan, Z. L., Ahn,
E. S., & Jallo, G. I. (2011). Safety and efficacy of concurrent pediatric spinal cord untethering and deformity correction. Journal
of Spinal Cord Techniques, 24(6), 401-405.
Metcalfe, P. D., Luerssen, T. G., King, S. J., Kaefer, M., Meldrum,
K. K., Cain, M. P., . Casale, A. J. (2006). Treatment of the occult tethered spinal cord for neuropathic bladder: Results of
sectioning the filum terminale. Journal of Urology, 176(4 Part
2), 1826-1830.
Michelson, D. J., & Ashwal, S. (2004). Tethered cord syndrome in
childhood: Diagnostic features and relationship to congenital
anomalies. Neurological Research, 26(7), 745-753.
Mitsuka, K., Horikoshi, T., Watanabe, A., & Kinouchi, H. (2009). Tethered cord syndrome in identical twins. Acta Nuerochirurgica,
151(1), 85-88.
Miyasaka, M., Nosaka, S., Kitano, Y., Ueoka, K., Tsutsumi, Y., Kuroda, T., & Honna, T. (2009). Utility of spinal MRI in children with
anaorectal malformation. Pediatric Radiology, 39(8), 810-816.
ONeill, B. R., Yu, A. K., & Tyler-Kabara, E. C. (2010). Prevalence of
tethered spinal cord in infants with VACTERL. Journal of Neurosurgery: Pediatrics, 6(2), 177-182.
Ostling, L. R., Bierbrauer, K. S., & Kuntz, C. (2012). Outcome, reoperation, and complications in 99 consecutive children operated
for tight or fatty filum. World Neurosurgery, 77(1), 187-191.

www.jpedhc.org

Perrin, J. M., Gnanasekaran, S., & Delahaye, J. (2012). Psychological

aspects of chronic health conditions. Pediatrics in Review,
33(3), 99-109.
Pinter, A. B., & Bognar, L. (2009). Dermal sinus and tethered cord. In
M. Hallawarth & P. Puri (Eds.), Pediatric surgery: Diagnosis and
management (1st ed., pp. 789-795). Berlin: Springer.
Robin, N. H., & Shprintzen, R. J. (2005). Defining the clinical spectrum of deletion 22q11.2. Journal of Pediatrics, 147(1), 90-96.
Rossi, A., Gandolfo, C., Morana, G., Piatelli, G., Ravegnani, M., Consales, A., . Tortori-Donati, P. (2006). Current classification and
imaging of congenital spinal abnormalities. Seminars in Roentgenology, 41(4), 250-273.
Samdani, A. F., Asghar, J., Pahys, J., Andrea, L. D., & Betz, R. R.
(2007). Concurrent spinal cord untethering and scoliosis correction. Spine, 32(26), E832-E836.
Samuels, R., McGirt, M., Attenello, F., Ambrossi, G., Singh, N., Solakoglu, C., . Jallo, G. (2009). Incidence of symptomatic retethering after surgical management of pediatric tethered cord
syndrome with or without duraplasty. Childs Nervous System,
25(9), 1085-1089.
Schenk, J. P., Herweh, C., Gunther, P., Rohrschneider, W., Zieger,
B., & Troger, J. (2006). Imaging of congenital anomalies and
variations of the caudal spine and back in neonates and small
infants. European Journal of Radiology, 58(1), 3-14.
Selden, N. R. (2007). Minimal tethered cord syndrome: Whats necessary to justify a new surgical indication. Neurosurgical Focus,
23(2), 1-4.
Splete, H. (2007). A sacral dimple can be benign or a sign of a spinal
anomaly. Pediatric News, 41(5), 43.
Stavrinou, P., Kunz, M., Lehner, M., Heger, A., Muller-Felber, W.,
Tonn, J. C., & Peraud, A. (2010). Children with tethered cord
syndrome of different etiology benefit from microsurgery: A
single institution experience. Childs Nervous System, 27(5),
803-810.
Stetler, W. R., Park, P., & Sullivan, S. (2010). Pathophysiology of adult
tethered cord syndrome: Review of the literature. Neurosurgical
Focus, 29(1), 1-5.
Tanaka, T., Ling, B. C., Rubinstein, J. H., & Crone, K. R. (2006).
Rubinstein-Taybi syndrome in children with tethered spinal
cord. Journal of Neurosurgery: Pediatrics, 105(4), 261-264.
Tarcan, T., Tinay, I., Temiz, Y., Alpay, H., Ozek, M., & Simsek, F.
(2012). The value of sacral skin lesions in predicting occult spinal
dysraphism in children with voiding dysfunction and normal neurological examination. Journal of Pediatric Urology, 8(1), 55-58.
Venkataramana, N. K. (2011). Spinal dysraphism. Journal of Pediatric
Neurosciences, 6(3), 31-40.
Yamada, S., & Won, D. J. (2007). What is the true tethered cord syndrome? Childs Nervous System, 23(4), 371-375.
Yamada, S., Won, D. J., Pezeshkpour, G., Yamada, B. S., Yamada,
S. M., Siddiqi, J., & Colohan, A. R. T. (2007). Pathophysiology of
tethered cord syndrome and similar complex disorders. Neurosurgical Focus, 23(2), 1-9.

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