Spina Bifida Facts Video -- What Is Spina Bifida? - CHOP (2 of 12)


Uploaded by ChildrensHospPhila on 10.06.2011

Transcript:
>> SPINA BIFIDA OR A MORE SEVERE FORM, MYELOMENINGOCELE,
IS THE MOST COMMON CENTRAL NERVOUS SYSTEM OR NEUROLOGIC
BIRTH DEFECT IN THE WORLD.
>> MYELOMENINGOCELE IS A DEFECT IN THE SPINE.
IT'S FAILURE OF THE SPINAL CORD TO CLOSE.
>> THE SPINAL CORD, OR THE NEURAL TUBE AS THE EMBRYOLOGISTS SAY,
FORMS VERY EARLY IN GESTATION, CERTAINLY BEFORE 8 WEEKS GESTATION.
AND SPINA BIFIDA IS THOUGHT TO BE A FAILURE OF THE FORMATION
AND CLOSURE OF THAT NEURAL TUBE.
SO THAT THE SPINAL CORD ELEMENTS ARE EXPOSED
TO THE INTRAUTERINE ENVIRONMENT.
>> IT'S A PROGRESSIVE PROCESS.
IT'S A PROGRESSIVE INJURY.
SO THAT BY THE END OF PREGNANCY, THERE'S BEEN DIRECT TRAUMATIC
INJURY TO THE SPINAL CORD, AND THERE'S BEEN INJURY
FROM THE AMNIOTIC FLUID ITSELF.
>> BECAUSE OF CHANGES IN PRESSURE AND FLUID FLOW,
BABIES ARE ALSO AT RISK FOR DEVELOPING HYDROCEPHALUS.
>> --WHICH IS A FLUID BUILDUP ON THE BRAIN AND WHICH DAMAGES
THE DEVELOPING BRAIN.
>> VIRTUALLY EVERY CHILD WITH MYELOMENINGOCELE OR SPINA BIFIDA
HAS AN ARNOLD CHIARI MALFORMATION ON X-RAY.
AND WHAT IT IS, IS THAT THE BACK PART OF THE BRAIN CALLED
THE "CEREBELLUM" THAT HAS A LOT TO DO WITH CONTROL OF MOTOR
MOVEMENTS IS WEDGED DOWN INTO YOUR NECK.
>> THE CEREBROSPINAL FLUID THAT CUSHIONS THE BRAIN AND CUSHIONS
THE SPINAL CORD LEAKS OUT THROUGH THE BACK THROUGH
THE OPEN SPINA BIFIDA DEFECT.
THAT LEADS TO THE BACK PART OF THE BRAIN GETTING SUMPED DOWN
INTO THE UPPER PART OF THE SPINAL CANAL.
THAT, IN TURN, CAN BLOCK THE CIRCULATION OF CEREBROSPINAL
FLUID IN THE BRAIN BECAUSE THE FLUID IS PRODUCED IN THE BRAIN
AND THEN THERE'S NO WAY FOR IT TO CIRCULATE BEYOND
THAT BLOCKAGE POINT.
>> SO IF THERE'S A BLOCKAGE IN THE FLOW OF THAT FLUID,
THEN THE WATER BUILDS UP INSIDE THE VENTRICLE.
SO YOU'VE PROBABLY HEARD THE TERM "WATER ON THE BRAIN."
THE WATER ISN'T ON THE BRAIN.
IT'S INSIDE THE BRAIN.
IF YOU DON'T TREAT THAT THEN YOU END UP WITH THE MASSIVELY
ENLARGED HEAD.
YOU DIE.
OR YOU END UP WITH SEVERE MENTAL RETARDATION.
>> SO IN ORDER TO MINIMIZE THAT ONGOING DAMAGE OF PRESSURE
FROM THE FLUID, THE PEDIATRIC NEUROSURGEON PLACES A SHUNT TUBE.
>> IT'S A SIMPLE DEVICE.
THIS IS A SHUNT.
IT'S MADE OUT OF SILICONE.
THIS END GOES THROUGH A HOLE IN THE BACK OF THE HEAD,
INTO THAT WATER CAVITY, AND TUNNELS DOWN UNDER THE SKIN,
AND GOES INTO THE ABDOMEN WHICH IS ABLE TO ABSORB THE FLUID.
IT'S NOT A CURE.
IT TREATS THE SYMPTOM.
IT DOESN'T TREAT THE DISEASE.
>> ONCE YOU HAVE A SHUNT THERE ARE MULTIPLE COMPLICATIONS
THAT CAN ARISE FROM IT.
>> THE SHUNT CAN BECOME INFECTED, WHICH IS A SERIOUS COMPLICATION
BECAUSE THEN THE BRAIN AND THE LAYERS OF THE BRAIN CAN BECOME
INFECTED WITH MENINGITIS ENCEPHALITIS.
AND WITH EACH BOUT OF INFECTION, THAT CAN EFFECT THE BRAIN FUNCTION.
>> AND THEN, OF COURSE, THERE ARE MECHANICAL PROBLEMS.
THEY CLOG.
THEY BREAK.
THEY GET TO BE TOO SHORT.
THEY MIGRATE.
>> SO IT'S NOT UNUSUAL FOR A CHILD WITH SPINA BIFIDA
WHO HAS A SHUNT TO REQUIRE THAT SHUNT, AND NOT ONLY FOR LIFE,
BUT TO HAVE THAT SHUNT CHANGED AT LEAST FIVE OR SIX TIMES
DURING THE COURSE OF THEIR LIFE.
>> IT'S IMPORTANT FOR PARENTS TO UNDERSTAND THE POTENTIAL
COMPLICATIONS OR RAMIFICATIONS OF SPINA BIFIDA
AFTER THE DIAGNOSIS IS MADE.
>> A CHILD MAY EXPERIENCE DIFFICULTY WITH APNEA,
FORGETTING TO BREATHE.
THEY MAY ALSO HAVE BREATHING PROBLEMS, CHOKING, BLUE SPELLS.
>> IN THE WORST CASE, YOU END UP WITH A CHILD
THAT'S ON A VENTILATOR, WITH A TRACHEOSTOMY,
AND A TUBE IN THE STOMACH TO EAT.
AND THAT IS A HUGE IMPACT ON YOUR QUALITY OF LIFE.
>> SPINA BIFIDA CAN CAUSE PARALYSIS IN THE LOWER EXTREMITIES.
THAT CAN INTERFERE WITH THE BABY'S ABILITY TO WALK,
OR THEY MAY EVEN BE WHEELCHAIR DEPENDENT.
>> THEY ALSO HAVE DIFFICULTY WITH THEIR BOWEL
AND BLADDER FUNCTION.
>> MOST OF THESE KIDS, WHEN THEY BECOME OLDER,
HAVE TO CATHETERIZE OR ELSE THEY HAVE TO WEAR DIAPERS.
>> THEY CAN DEVELOP SIGNIFICANT CURVATURES OF THE SPINE
BECAUSE OF INSTABILITY-- BONY INSTABILITIES
OF THE SPINE IF THEY HAVE A HIGH LESION.
>> BECAUSE OF THE LACK OF INNERVATION TO THE FEET
AND THE ANKLES, THERE CAN BE THE FORMATION OF CLUB FEET,
OR THE MEDICAL TERM IS "TALIPES" AS A CONSEQUENCE.
SO THAT REQUIRES ORTHOPEDIC INTERVENTION AFTER BIRTH.
>> AND THEN THERE ARE PROBLEMS THAT CAN COME ON LATER ON IN LIFE.
IT'S NOT EVEN NECESSARILY DONE WHEN YOU'RE A BABY.
YOU CAN GET TETHERED CORD, WHICH MEANS THAT THE NERVE TISSUE GETS
CAUGHT UP IN SCAR TISSUE, AND YOU CAN LOSE FUNCTION.
SO IT'S A LIFELONG ISSUE OF DISABILITY.