Meningocele & Spina BifidaPresented by:Maya Okta Lestari 2012061057Haris Cakrasana 2012061058

AnatomyThe spinal cordextendsfrom the foramen magnum where it is continuous with the medulla to the level of the first or second lumbar vertebrae. The spinal cord is 40 to 50 cm long and 1 cm to 1.5 cm in diameter.Two consecutive rows of nerve roots emerge on each of its sides. These nerve roots join distally to form 31 pairs of spinal nerves. The spinal cord = cylindrical structure of nervous tissue composed of white andgray matter, is uniformly organized and is divided into four regions: cervical (C), thoracic (T), lumbar (L) and sacral (S)The spinal nerve contains motor and sensory nerve fibers to and from all parts of the body. Each spinal cord segment innervates a dermatome (see below and Figure 3.5).

Development of Neural TubeBy about 16 days after fertilisation, the intraembryonic mesoderm is formed and the embryo becomes a trilaminar disc is called gastrulation.

One or two days after gastrulation, the notochord can be seen along the midline of the embryo. The notochord induces a wide strip of ectoderm overlying it to get thickened to form the neural plate.

Some cells at the junction between the neural plate and the rest of ectoderm become specialised to form the primordia of the neural crest.

By the 21st day (end of 3rd week), the neural plate gets depressed along the midline and a groove is formed (neural groove) on the neural plate.

The neural groove gradually deepens; the two edges of the neural plate come closer and eventually fuse to become the neural tube.

The neural tube initially closes in the middle. The fusion starts around 23rd day and proceeds both cranially and caudally. The anterior neuropore closes by the 25th day and the posterior neuropore by the 27th day. The closure of neural tube is completed by the end of 4th week.

DefinitionDisgraphism Spinal term group of congenital anomalies of spinal fusion caused by the failure of neural tube both bones and skin. Spina bifida is a congenital abnormality, a defect in the posterior arch of the spine as a result of the failure of neural tube closure in the early embryo

EpidemiologyApproximately 400.000 infants with spina bifida are born worldwide each year.The incidence in Europe and North America ranges between 1-2.5 per 1,000 live births The incidence in Asian countries, including Indonesia ranges from 0.1-0.3 per 1,000 live births

Etiology & Risk FactorUnknownMultifactorialGenetic = single gene mutation (eg. Meckels syndrome) & chromosomal abnormalities (trisomy 13, trisomy 18)NutritionIntake zinc, folic acid reduce spina bifida incidenceEnvironmental factor = lower socioeconomic statusRacialAlcohol consumptionMaternal problemsUse of anti epileptic drugs, maternal diabetes, hyperthermia, obesity

TypesSpina bifida aperta/mielomeningokelSpina bifida okultaSpina bifida cystica

Spina bifida occultaThe most mild, sometimes called hidden spina bifidaNot discovered until late childhood or adulthood.Neural defect covered by skin. There is a small gap in the spine, but no opening or sac on the back. The spinal cord and the nerves usually are normal.Prosessus spinous always lost, the laminae may be lost Epidemiology = 25% population Occur in the lumbosacral region (L4-S1) This type of spina bifida usually does not cause any disabilities.

Spina bifida aperta/mielomeningokelThe most often, the most serious type With this condition, a sac of fluid comes through an opening in the babys back. Part of the spinal cord and nerves are in this sac and are damaged.Causes moderate to severe disabilities

MeningoceleWith meningocele a sac of fluid comes through an opening in the babys back may or may not be covered by skin.But, the spinal cord is not in this sac. There is usually little or no nerve damage.This type of spina bifida can cause minor disabilities.

PathophysiologyNeural tube defects are the result of a teratogenic process that causes failed closure and abnormal differentiation of the embryonic neural tube.Myelomeningocele results when the closure of caudal neuropore is disrupted during days 26-30

open lesion or sac that contains dysplastic spinal cord, nerve roots, meninges, vertebra, skin

Clinical ManifestationLeg weakness and paralysisOrthopedic abnormalities (i.e.,club foot,hip dislocation,scoliosis)Bladder and bowel control problems, including incontinence,urinary tract infections, and poor renal functionPressure soresand skin irritationsAbnormal eye movementHydrocphalus

A sac sticking out of the mid to lower back, dimpling of the sacral area.Motor, sensory and autonomic deficit related to the level of the defect.Symptoms include : loss of bowel and bladder control, paralysis or weakness of the legs, loss of sensation.

DiagnosisClinical manifestationUSG

TherapySurgical closure of the myelomeningocele is undertaken within 24 to 48 hours of birth to avoid CNS infection. The defect covered by moist sterile dressings,and given prophylactic antibioticsSkin grafts often are required for large defects. Ventricular shunts, if indicated, are placed concurrently with myelomeningocele closure or at a later

PROGNOSTICchildren with myelomeningocele, 60% to 70% will ultimately require a shunt insertion, whereas only 15% to 30%(24% over a 25-year period), cognitive development (75% have an IQ higher than 80 if adequately treated for hydrocephalus),

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