Re: Website describing a tethered cord and symptoms

June 6, 2006

i agree with holly, its smart to rule out a tethered cord with MRI

just to be sure, but i would disagree with holly's statement that 99%

of kids with club feet are ideopathic. i have an academic paper

(Bakalis, et.al, 2002) (and a son with a neurlogical disorder and

clubfeet) which shows that only about 50% of kids with clubfeet have

no other issues. its just that the ones that have other issues dont

hang out on this board - the clubfeet are the least of their

problems...

for those that are interested, i can email them the paper.

in this paper, for instance, it also shows, that idiopathic clubfeet

are equally likely to unilateral as bilateral, but that if there is

more going on, it is more likely (70%) that the clubfeet presentation

will be bilateral. the coverse statement is also true, a diagnosis

of bilateral clubfeet results in a " poor outcome " almost 60% of the

time; that of unilateral clubfoot only about 25%. the last

interesting point that i will paraphrase is this: even after all in

utero scans, amnio, etc., if the clubfeet appears idopathic, there

remains a low risk, call it 3% according to the Bakalis paper, of a

complex presentation and poor outcome that is observed postnatally.

bill

>

> 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-

>

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.

>

> __________________________________________________

>

June 6, 2006

That is interesting, Bill. Everything I have ever read has stated that the

numbers are about 90% of kids with club feet having no other complications and

10% of kids with club feet having other complications. The perinatologist gave

us these same stats, as well.

What are the " other complications " which are involved? Do you mind sharing your

experience with your son and more specifically what neurological issues are

involved with him? My daughter has bilateral club feet but has no known other

issues and is developing very well. All the prenatal scans showed normal

development and so far she is meeting milestones and has no sacral dimple, hairy

patch on her back, etc to indicate any issues. My " gut " tells me that she

simply was born with bilateral clubfeet and nothing else, but I do like to look

at research. It behooves us as parents to be aware of the possibilities so we

can have them checked out, as needed. Thanks,

Carol

Re: Website describing a tethered cord and

symptoms