Website describing a tethered cord and symptoms

[Guest guest]

Information on tethered cord...page down to # 2 as it describes orthopedic

problems.

Remember that 99% of kids with club feet are just that....only have a club

foot. I just want you to be aware in case you see other things along with

it...smart to rule out a tethered cord with an MRI just to be sure.

Holly

Website below

http://www.lfsn.org/lipomyel.htm

Lipomyelomenigocele and other OSD's

What is LMC

Symptoms of OSD's

What is LMC?

(lipomyelomeningocele)

Midline lumbosacral masses are usually some form of lipmyelomeningocoele. In

this first image , the mass can be seen extending from the subcutaneous tissues

into the spinal column and then into the spinal cord. Some of these lesions are

referred to plastic or general surgeons who might consider excising the

subcutaneous portions of the lesion, but fail to remove the intradural portion.

This is not in the best interests of the child. We now believe that excision of

the extradural portion of the lesion without intradural evaluation leads to

dense subarachnoid scarring and neurologic dysfunction. Furthermore, secondary

excision of the intradural portion of the lesion is associated with greater

morbidity than primary excision. lipomyelomeningocele that enter the cord

dorsally are more amenable to surgical excision. Primary excision of these

lesions has been facilitated by use of the ultrasonic aspirator. In these next

images , the approach to surgical excision is illustrated.

In the majority of patients, the lipomyelomeningocele enters dorsally and the

dissection can be performed by coring out the lipoma within the cord.

The most difficult lipomyelomeningocele are those that enter the cord caudally .

These lesions are more difficult technically , but can be considered for

operation realizing; first, we now know that these lesions will progress and

produce neurologic impairment, and that we have the ability to dissect out these

lesions with the ultrasonic aspirator or the CO2 laser and tease the fat away

from the functioning nerve roots.

Taken from: Lumps, Bumps, and Holes: A Primer on Occult Spinal Dysraphism -W.

Jerry Oakes, M.D. Professor and Chief Section of Pediatric Neurosurgery ,The

University of Alabama at Birmingham ,The Children's Hospital of Alabama.

****Reprinted with permission

Lipomyelomeningeoceles (spinal lipomas) represent a spectrum of disorders all

involving a component of fat that is contiguous with the spinal cord. They may

be seen in association with myelomeningocele or more commonly, as an isolated

occult dysraphic malformation having an intact skin covering. Almost all are

confined to the caudal (lumbosacral) spinal cord and/or filum terminale. Two

general configurations have been described. In the first (and more frequent)

type, the fat forms a subcutaneous mass of variable size that is contiguous with

the subarachnoid space through a dorsal vertevral and dural defect. In the

second type, the dura and posterior vertabral elements are intact, and the fat

is present only within the subarachnoid space. In both cases, the spinal cord is

open

dorsally at the level of the lipomyelomeningocele. and the fat enters the cord

through the defect and is contiguous with the central lumen of the cord. The

dorsal nerve roots, which normally arise from the neural folds just lateral to

the site of dorsal midline tusion, are located immediately lateral to the

junction between the fat and the dorsal cord. McLone has suggested that

lipomyelomeningeoceles arise through a disorder of neural tube closure in which

the cutaneous ectoderm separates prematurely from the approximating neural fold

before neural tube closure is complete; the surrounding mesenchma enters the

central lumen of the neural tube and is induced to form fat.

***We have permission to reprint for educational uses

Taken from:

Chapter 15 Spinal Dysraphism- Mark S. Dias and G. McLone The Pediatric

Spine:Principles and Practice- S.L. Weinstein, Editor- Raven Press., Ltd., New

York © 1994

http://www.jamestgoodrich.com/lipomyelomeningocele_image.html#lippo1 -

pictures of lipo.

Symptoms of OSD's

7/11/99 - This brochure is a new edit of the old one- it has not yet been

approved by a doctor

**This brochure was put together by a member of LFSN- Sharon

Dreyfus. She holds the sole copyright for this brochure- if you would like a

copy, please contact her. We are so grateful to have people so involved in

awareness in LFSN- Sharon really has a passion for making sure no one goes

undiagnosed.

HIDDEN NEURAL TUBE DEFECTS:

OCCULT SPINAL DYSRAPHISMS AND TETHERED CORD SYNDROME

Much publicity has been recently given to the congenital spinal cord defect

Spina bifida (Myelomeningocele), a condition affecting one in 1000 births, in

which the neural tube does not close during early embryonic development and the

baby is born with the spinal cord exposed in a sac at the its back. But not all

spinal defects are so obvious.

WHAT IS OCCULT SPINAL DYSRAPHISM?

Occult spinal dysraphism (OSD) refers to any hidden spinal cord defect which is

associated with neurological involvement. In OSD, the spinal cord is not exposed

and the defect may be much more likely to go undetected. OSD malformations

include distortion of the spinal cord or its nerve roots by fibrous or fatty

bands and adhesions (Tight/Fatty Filum Terminale) or other spinal cord fixations

(eg. Meningocele Manque); fatty tumors in the spine, under the skin, or in

surrounding fibrous tissue (Lipomyelomeningocele); cysts in the skin or just

under it (Neurenteric Cyst); a syrinx in the spinal cord (Syringomyelia);

divisions in the spinal cord itself (Diastematomyelia); abnormalities in the

bones of the vertebrae or sacrum (eg. Spina Bifida Occulta); or tracts which

extend from the skin to the spinal cord (Dermal Sinus Tracts). Perhaps one in

1500 people are born with OSD. Symptoms of an OSD may be absent, minimal, or

severe depending on the degree of neural involvement.

Symptoms may be static or slowly progressive. Symptoms may

exhibit from birth on or may begin to show in adulthood or during adolescent

growth spurts. People with OSD typically have less severe neurological symptoms

than those with classic Spina bifida.

WHAT IS SPINA BIFIDA OCCULTA?

Spina Bifida Occulta (SBO) is an incomplete closure of the vertebral column of

the lower spine, but without protrusion of the cord because the neural tube has

closed. In other words, this defect doesn’t show to the naked eye. There is no

hole at the baby’s back; no obvious bulge. SBO is thought to occur in 5% (one

recent study cites an astounding 17%) of the U.S. population. The vast majority

of these people have no neurological involvement. However, a small percentage

either exhibit neurological symptoms from birth or develop them during life.

WHAT IS LIPOMYELOMENINGOCELE?

A child born with a lump of subcutaneous fat at the lumbar or sacral region, may

simply have an extra pad of fat (lipoma), which may be cosmetically removed at

some later date. However, there is a possibility that the fat is an indicator of

an OSD, in which the fat enters a defect in the spinal column during foetal

development to merge with the neural tissue. This is a form of Spinabifida which

is often overlooked in diagnoses and which is often subject to neurological

symptoms. Surgery is indicated with this condition. The fat

must be carefully excised from the neural tissue to avoid further nerve damage.

There is also some danger that cosmetically removing the fat outside of the

neural tube without also freeing the nerves from the fat inside of the neural

tube can increase neurological dysfunction.

WHAT IS FIBROUS/THICK OR FATTY FILUM TERMINALE?

The filum terminale is a stretchy band of filament which attaches the bottom of

the spinal cord to the pelvis and provides “give” to the spinal cord so that the

less flexible cord will not become overstretched during foetal development and

other growth periods. In rare instances, the filum may become too fibrous or

form fat around it during the differentiation phase of foetal development. This

prevents the filum from stetching. The result is that the spinal cord becomes

overstretched and pulled down, resulting in nerve damage. Often, no signs will

be visible on the back. Surgery to resect the filum is indicated to prevent

further neurological damage as the child grows.

WHAT IS TERMINAL SYRINGOMYELIA?

Syringomyelia (or Hydromyelia) is a cerebrospinal fluid-filled cyst within the

spinal cord. Though often a result of injury to the back, it may also be

associated with - and an indicator of - a tethered spinal cord, particularly

when located within the lower thoracic and lumbar level of the spine. The syrinx

may need to be drained, but often will decrease once the tethered cord is

released.

ARE THERE ANY SIGNS ON THE BACK?

There may be one or more characteristic signs of OSD on the lower back, (usually

along the midline around the lumbar-sacral junction of the spine), such as:

*a skin depression, dimple, or sinus tract

*a tuft of dark hair

*areas of increased pigmentation

*a fatty lump under the skin

*skin defects

*abnormal skin appendages, tags, tails

Or, there may be no tell-tale signs on the back at all.

WHAT IS TETHERED CORD SYNDROME?

Most symptoms of an OSD are caused by Tethered Cord syndrome (TCS). Tethered

Cord refers to any adhesion of the spinal cord to an immovable structure, (be it

bone, fat, skin, tumor, or other tissue, such as scar tissue), which causes

interference of the free movement of the cord. The spinal cord is then fixed

between two points--at the tethering structure and at the base of the brain.

With movement of the vertebral structures, be it as a result of growth, daily

activity or pathological skeletal changes such as curvature of

the spine--the spinal cord will be forced to stretch abnormally. The result is

that this segment of tethered spinal cord is stretched beyond its tolerance,

circulation to the spinal cord can become compromised, leading to damage of

nerve tracts and nerve cells of the spinal cord, and subsquent loss of function.

This is known as Tethered Cord Syndrome (TCS). (source: Neil Feldstein,MD,

Director of Pediatric Neurosurgery Babies and Childrens' Hospital of New York

Columbia-Presbyterian Medical Center)

Symptoms of TCS may include any of the following dysfunctions or changes in

function:

1) bowel/bladder dysfunction:

loss or lack of bowel & /or bladder control (incontinence); constant leaking;

bladder spasms; lack or loss of sensation in bladder or bowel; lack of urge;

inability to void completely; lack of strong stream urination; lack of motor

control of anal or bladder sphincters; in newborns, lack of anal wink; chronic

constipation, diarrhea or both; unusual straining at the toilet; fecal smearing

on underwear; recurrent urinary tract infections; adult onset may involve

anorectal pain, followed by weakness and incontinence.

2) orthopedic problems:

foot deformities, particularly club foot; shortened heel tendons; ankle

rigidity; foot size or leg length differences; weakness & /or sensory lack or

loss in the legs and feet: including lack of reflexes, reduced or spotty

sensation, numbness, tingling, ankle flaccidity, or foot paralysis; tendency to

get blisters or cuts in feet and not realize it; stiffness, pain, tremors, or

spasms (contractures) in hamstrings, calves,feet or toes; deformity of legs or

hips; hip dislocation; change in alignment of knees, ankles and feet; in

preschoolers, change in foot positioning or tone & /or changes in general

posture; in older children, loss of strength, hamstring tightness, and increased

valgus deformity of the foot.

3) gait problems:

decreased strength in legs; muscle weakness resulting in fatigue when walking,

muscle atrophy, brittle bones; legs “suddenly giving out”; abnormal gait;

clumsiness or balance problems; stumbling or falling a lot; progressive

deterioration of gait; delays in large motor skills: rolling over, crawling,

walking.

4) back and postural problems:

early development of rapidly-increasing curvature of the spine (scoliosis);

tendency to tilt the head, curve the back, or tilt the hips; increasing lumbar

lordosis; increasing back or leg pain; lower back pain; sciatica in young age

groups; desire to arch or otherwise curve the back to relieve discomfort.

5) Other symptoms which have been noted relative to OSD, and which may be as the

result of accompanying Syringomyelia or Arnold Chiari Malformation, might

include:

difficulty swallowing, weak or poor cry (weakness of vocal cord), inspiratory

rasp or wheeze (stridor/tracheomalacia), sustained arching of the head, and

possibly facial weakness. In children and adolescents, ACM may appear as

numbness, tingling, tremors, stiffness or spasming of the arms or hands and may

be accompanied by loss of pain and/or temperature sensation. Other reported

symptoms include drooling, excessive snuffling after crying, frequent hiccups,

occipital headaches, slurred speech, hypersensitive gag reflex, dizziness,

double vision, eye movement disorder, hearing problems, seizures, nausea,balance

problems and problems in the ability to coordinate movement.

WHAT SHOULD A CONSCIENTIOUS PARENT OR HEALTH PRACTITIONER KNOW?

It is important to become aware of the signs and symptoms of OSD and TCS because

early detection is the key to reducing or avoiding neurological deterioration.

So little information has been publicly available, that signs and symptoms may

go undetected, even by health care providers whom we trust to

know--pediatricians, neurologists, orthopedists, urologists, radiologists, etc.

All too often, babies with a mass of fat on the lower back will be sent home for

later cosmetic surgery, with the assumption that since there are no symptoms

yet, there is no neurological involvement. A baby born with a club foot may

never be checked for neurogenic symptoms of bowel and bladder, but simply put in

a foot cast with expectations that this is a simple orthopedic problem rather

than a neurological one. An incontinent child may never be given a urodynamics

exam. An adolescent may develop scoliosis and receive

only an x-ray without examining further. An adult may suddenly develop chronic

sciatica pain or numbness in a leg or foot and seek Chiropractic treatment

alone, believing that a vertebra simply needs adjustment.

In each of these cases, further examination may be indicated to rule out TCS.

Because if the cord is tethered and the situation goes untreated, neurological

deterioration may continue with progressive loss of function. And once damaged,

the spinal nerves will not regenerate.

WHAT SHOULD BE DONE TO DIAGNOSE AN OSD?

If your child has any of the above signs or symptoms, he or she should be

checked by a Medical Professional who is very familiar with Occult Spinal

Dysraphism (OSD) to determine whether SBO or any other anomally causing TCS

exists. A spinal x-ray will show any bony abnormality. However, the definitive

test is Magnetic Resonance Imaging (MRI), which will show the anatomy of the

cord. This will show important secondary signs such as areas of spinal cord

atrophy, fluid-filled cysts (called syrinxes) in the lower

third of the spinal cord which are often associated with OSD, ventral

compression of the spinal cord, or small dermoid tumors (which can occur from

elements of the skin being closed deep into the spinal canal) as well as any

other structural anomalies of the spinal cord or column, such as fatty or

thickened filum. (The MRI should be of the whole spinal cord in order to rule

out any cervical or hindbrain neurological involvement.) An MRI is painless and

takes little time, but it is expensive to insurance companies

and physicians are sometimes reluctant to order them. Therefore, a concerned

parent may have to be prepared to insist.

If the spinal cord is found to be tethered, neurosurgery may be indicated,

especially if the child is still growing or if there are any symptoms present.

Infants should be especially well-checked because statistically they achieve or

maintain better neurological function following surgical intervention than

adults do.

Evaluation by a pediatric urologist is also indicated, including ultrasound of

the kidneys and ureters to rule out reflux or kidney damage. A lifelong program

of bladder and bowel maintenance may need to be instituted. In addition,

somatosensory evoked potentials and EMG tests will evaluate nerve conduction and

the transmission of electrical impulses through the spinal

cord, indicating the level of sensation and motor loss. Orthopedic aids may be

recommended to give strengthening and stabilizing assistance in walking.

In any case, where SBO or any other Spinal Cord Dysraphism is found, the

condition should be regularly monitored throughout life for signs of cord

tethering. In most cases, the child should be seen regularly in a spinal defects

clinic, by a multidisciplinary team of practitioners, including a Physiatrist,

Physical Therapist, Orthopedist, Urologist, Neurosurgeon, and GI Nurse.

REMEMBER: THE NATURAL COURSE OF THE CONGENITAL SPINAL LESIONS THAT PRODUCE

TETHERED CORD IS ASSOCIATED WITH PROGRESSIVE NEUROLOGIC DETERIORATION AND

DISABILITY. EARLY RECOGNITION, EVALUATION AND DIAGNOSIS ARE POSSIBLE AND

EXTREMELY IMPORTANT. EARLY OPERATIVE TREATMENT PREVENTS DETERIORATION AND

MAY RESULT IN NEUROLOGIC RECOVERY. OPERATION BEFORE SYMPTOMS AND ABNORMAL

FINDINGS APPEAR MAY PRESERVE NORMAL NEUROLOGIC, ORTHOPEDIC AND UROLOGIC

FUNCTION.

COPYRIGHT 1999 SHARON ALEXANDER

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