meningocele & spina bifida
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MENINGOCELEandSPINA BIFIDASupervisor: dr. Nick, SpBSKeyne Christa Monintja (2013-061-111) | Angelina Tjokro (2013-061-112)
Dysraphysm = incomplete closure of raphe; defective fusion; particularly in the neural tube1INTRODUCTIONThe neural structures develop from the ectoderm.The neural tube forms from the neural placode at approximately 21 days of gestation.Failure to form neural tube defects (spinal dysraphism) .The neural tube defects have already formed by the time pregnancy is diagnosed prevention : folic acid prior to 21days of gestation.Neural Tube Development
Normal embryological development Neural plate development -18th dayCranial closure 24th day (upper spine)Caudal closure 26th day (lower spine)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, obesityCLASSIFICATIONSPINAL DYSRAPHYSMSpina Bifida APERTAMeningoceleMyelomeningoceleSpina Bifida OCCULTASimple Spina Bifida OccultaSpinal Dermal SinusLipomyelomeningoceleDiastematomeliaTethered Spinal Cord
MENINGOCELEProtrusion of dura and arachnoid outside the confines of the spinal canal, with neural tissue remaining within the spinal canal confines. No neural elements present no associated neural deficits repair is simpler.Can be at any location in the spine, most common in the lumbar region.MENINGOCELE
MANAGEMENTSurgical treatment 12 to 24 hours after birth prevent infection, swelling, and further damage.Using general anesthesiaAn incision is made in the sac and some of the excess fluid is drained offThe spinal cord is covered with the membranes (meninges)The skin is closed over the protruding meninges, spinal cord, and nerves.
What is Spina Bifida?A midline defect of the bone, skin, spinal column, &/orspinal cord.
DefinitionSpina 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
Spina bifida occultaThe most mild, sometimes called hidden spina bifidaNeural 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.Processus spinous always lost, the laminae may be lost Occur in the lumbosacral region (L4-S1)
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
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.Diagnosis and DetectionAmniocentesisAFP - indication of abnormal leakageBlood testMaternal blood samples of AFPUltrasonographyFor locating back lesion vs. cranial signsTherapySurgical 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),THANK YOU