Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that

limit the movement of the spinal cord within the spinal column. Attachments may occur
congenitally at the base of the spinal cord (conus medullaris) or they may develop near the
site of an injury to the spinal cord. These attachments cause an abnormal stretching of the
spinal cord. The course of the disorder is progressive. In children, symptoms may include
lesions, hairy patches, dimples, or fatty tumors on the lower back; foot and spinal
deformities; weakness in the legs; low back pain; scoliosis; and incontinence. This type of
tethered spinal cord syndrome appears to be the result of improper growth of the neural tube
during fetal development, and is closely linked to spina bifida. Tethered spinal cord
syndrome may go undiagnosed until adulthood, when pain, sensory and motor problems, and
loss of bowel and bladder control emerge. This delayed presentation of symptoms is related
to the degree of strain placed on the spinal cord over time and may be exacerbated during
sports or pregnancy, or may be due to narrowing of the spinal column (stenosis) with age.
Tethering may also develop after spinal cord injury and scar tissue can block the flow of
fluids around the spinal cord. Fluid pressure may cause cysts to form in the spinal cord, a
condition called syringomyelia. This can lead to additional loss of movement, feeling or the
onset of pain or autonomic symptoms.
Is there any treatment?
MRI imaging is often used to evaluate individuals with these symptoms, and can be used to
diagnose the location of the tethering, lower than normal position of the conus medullaris, or
presence of a tumor or fatty mass (lipoma). In children, early surgery is recommended to
prevent further neurological deterioration. Regular follow-up is important: retethering may
occur in some individuals during periods of rapid growth and may be seen between five to
nine years of age. If surgery is not advisable, spinal cord nerve roots may be cut to relieve
pain. In adults, surgery to free (detether) the spinal cord can reduce the size and further
development of cysts in the cord and may restore some function or alleviate other symptoms.
Other treatment is symptomatic and supportive.
What is the prognosis?
With treatment, individuals with tethered spinal cord syndrome have a normal life
expectancy. However, some neurological and motor impairments may not be fully
correctable. Surgery soon after symptoms emerge appears to improve chances for recovery
and can prevent further functional decline.
What research is being done?
The NINDS conducts and supports research on disorders of the spinal cord. The goals of this
research are to find ways to prevent, treat, and cure these disorders.




Sindrom sumsum tulang belakang ditambatkan adalah gangguan neurologis yang disebabkan oleh
lampiran jaringan yang membatasi pergerakan dari sumsum tulang belakang dalam kolom tulang
belakang . Lampiran dapat terjadi kongenital di dasar sumsum tulang belakang ( konus medularis )
atau mereka dapat mengembangkan dekat lokasi cedera sumsum tulang belakang . Lampiran ini
menyebabkan abnormal peregangan dari sumsum tulang belakang . Jalannya gangguan yang
progresif . Pada anak-anak , gejala mungkin termasuk lesi , patch berbulu , lesung , atau tumor lemak
pada punggung bawah , kaki dan deformitas tulang belakang , kelemahan di kaki , nyeri pinggang ,
scoliosis , dan inkontinensia . Jenis sindrom sumsum tulang belakang ditambatkan tampaknya
merupakan hasil dari pertumbuhan yang tidak benar dari tabung saraf selama perkembangan janin ,
dan berhubungan erat dengan spina bifida . Sindrom sumsum tulang belakang ditambatkan mungkin
tidak terdiagnosis sampai dewasa , ketika rasa sakit , masalah sensorik dan motorik , dan hilangnya
kontrol usus dan kandung kemih muncul . Ini presentasi tertunda gejala berkaitan dengan tingkat
ketegangan ditempatkan pada sumsum tulang belakang dari waktu ke waktu dan dapat diperburuk
selama olahraga atau kehamilan , atau mungkin karena penyempitan tulang belakang ( stenosis )
dengan usia . Tethering juga dapat berkembang setelah cedera tulang belakang dan jaringan parut
bisa menghalangi aliran cairan di sekitar sumsum tulang belakang . Tekanan fluida dapat
menyebabkan kista terbentuk di sumsum tulang belakang , suatu kondisi yang disebut syringomyelia
. Hal ini dapat mengakibatkan kerugian tambahan gerakan , perasaan atau timbulnya rasa sakit atau
gejala otonom.
Apakah ada pengobatan ?

Pencitraan MRI sering digunakan untuk mengevaluasi individu dengan gejala ini , dan dapat
digunakan untuk mendiagnosa lokasi tethering , lebih rendah dari posisi normal dari konus medularis
, atau adanya tumor atau massa lemak ( lipoma ) . Pada anak-anak , bedah awal dianjurkan untuk
mencegah kerusakan neurologis lanjut . Regular tindak lanjut adalah penting : retethering dapat
terjadi pada beberapa individu selama periode pertumbuhan yang cepat dan dapat dilihat antara 5-9
tahun . Jika operasi ini tidak dianjurkan , akar saraf sumsum tulang belakang dapat dipotong untuk
menghilangkan rasa sakit . Pada orang dewasa , operasi untuk gratis ( detether ) sumsum tulang
belakang dapat mengurangi ukuran dan pengembangan lebih lanjut dari kista di kabel dan dapat
mengembalikan beberapa fungsi atau meringankan gejala lainnya . Pengobatan lainnya adalah
simtomatik dan suportif .

Apa prognosis ?

Dengan pengobatan , individu dengan sindrom sumsum tulang belakang ditambatkan memiliki
harapan hidup normal. Namun, beberapa gangguan neurologis dan motorik mungkin tidak
sepenuhnya diperbaiki . Pembedahan segera setelah gejala muncul tampaknya meningkatkan
peluang untuk pemulihan dan dapat mencegah penurunan lebih lanjut fungsional .

Penelitian apa yang sedang dilakukan ?

The NINDS melakukan dan mendukung penelitian tentang gangguan sumsum tulang belakang .
Tujuan dari penelitian ini adalah untuk menemukan cara untuk mencegah , mengobati , dan
menyembuhkan gangguan ini .


Introduction
When there is disturbed or incomplete closure of the neural tube during embryonic life it
leads to a developmental congenital defect called spina bifida (SB). Some of the vertebrae of
the spinal cord are not completely formed and remain open to produce a defect in the
vertebral column that may or may not be covered by the skin. The most common site of this
defect is the lumber or the sacral segment of the spinal cord. The incidence of SB is about
one in every 1000 births
1,2
.
Disorders associated with spina bifida
There are various disorders which are similar to SB or which may be associated with SB.
These disorders include:
Anencephaly
Encephalocele
Tethered Spinal Cord
Chiari Malformation
Meckel syndrome
Dandy-Walker-Malformation
1. Anencephaly

This disorder refers to the absence of a large portion of the brain or the skull. It is also termed
as aprosencephaly or with an open cranium. It is the neural defect that occurs when the
superior portion of neural the tube fails to close. The possible risk factors for this abnormality
include low folic acid level in pregnant mothers and exposure to certain toxins or drugs
during pregnancy. Its incidence is one in 10,000 births
3
.
Clinical features of anencephaly include:
Absence of the skull or a part with exposure of brain tissue
Absence of the brain or a portion
Facial abnormalities
Valvular defects of the heart
3

Diagnosis: The following tests can be carried out to diagnose this defect in a pregnant
mother:
Abdomenial ultrasonography
Amniocentesis
Alpha-fetoprotein level
Test of pre-pregnancy serum folate level
Urine estriol level
3

Treatment: There is no current therapy except genetic counseling.
Prognosis: The anomaly usually results in death within a few days of birth.
Prevention: Adequate folic acid intake may prevent this malformation from developing
3
.
2. Encephalocele

This is another of the neural tube defects which result from the incomplete closure of superior
part of the neural tube. Its other name is cranium bifidum. It sticks out of the skull through
openings in the form of protrusions of brain tissue with its covering membranes. It has many
similarities with spina bifida. It is a rare anomaly that occurs at a rate of one in 5000 live
births
4,5
.
Types: If the protruded part of encephalocele contains only CSF along with membranes, it
may be termed as meningocele and if the brain tissue also protrudes with it, it is called
encephalomeningocele. Encephaloceles include the following types
4
:
Naso-frontal
Naso-ethmoidal
Naso-orbital
Risk factors: Recent studies show an association of these congenital anomalies with the
following risk factors:
Exposure to cancer causing agents
Exposure to tryptan blue- a stain used to color dead tissues
Decreased level of folic acids
5

Clinical manifestations: Patients with this malformation can have following features:
Hydrocephalus
Weakness of lower limbs
An abnormally small sized head
Disturbed muscle movement
Developmental delay
Vision problems
Mental and growth retardation
Seizures
5

Meningitis, both pre-operative and post operative
Hydrocephalus
Blindness
6

Complications: These include:
Management: The following investigations are conducted with pregnant women to diagnose
this anomaly:
Ultrasonography of the abdomen
MRI
AFP-level
5,6

Reparative surgery is the treatment of choice at an early stage. Other treatment options are
supportive therapy, counseling, physical therapy, psychotherapy etc
5, 6
.
3. Tethered Cord Syndrome

This syndrome is characterized by pulling of the spinal cord at the base of the spinal canal
along with consequent clinical manifestations. It has many different forms like:
Dermiods
Dermal sinus tracts
Cystoceles
Tight filum terminale
Lipomenengiomyelocele
Split cord malformation
7

.
Aetiology: Causes of TCSinclude:
Disturbed growth of the neural tube during fetal development
Spina bifida
Later spinal cord injury
Syringomyelia
8

Clinical manifestations: Clinical manifestations of this anomaly include:
Skin lesions like hairy patches, dimples, or fatty tumors on the lower back
Paralysis of both lower limbs
Spine and feet deformities
Kyphoysis and scoliosis
Backache
Urinary incontinence
Urinary retention
Weakness of muscle and loss of sensations of touch, vibration, temperature, pain and
proprioception
Urinary frequency, urgency, feeling of incomplete urination
Rectal prolapse
Constipation
Bowel control dysfunction
Motor neuron findings like amyotrophy, hypereflexia and up-going planter
Chronic recurrent kidney infections and stones
9, 10

Treatment: Treatment options include:
Untethering by surgery
NSAIDS and opiates to relieve the pain
Antidepressants for severe pain
Muscle relaxing exercises
Treatment of urinary tract infection
Physiotherapy
9,10

4. Chiari Malformation

This is another medical condition associated with neural tube defects. In this instance there is
an extension of the cerebellum into the spinal canal and thus blocking the flow of CSF. There
may be associated symptoms of upper body weakness, difficulty in swallowing and breathing
and hydrocephalus
5
.
5. Meckel syndrome

This problem is associated with neural tube defects and it has following features:
Occipital encephalocele along with micrecephaly
Cleft palate
Polycystic kidney disease
Polydactyly
Ambiguous genitals
5

6. Dandy-Walker-Malformation

It is characterized by enlargement of the 4
th
ventricle along with a cyst that extends into the
posterior cranial fossa. This malformation is also associated with neural tube defects
5
.
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