meningocele & spina bifida
DESCRIPTION
spinal dysraphysmTRANSCRIPT
MENINGOCELEandSPINA BIFIDASupervisor: dr. Nick, SpBS
Keyne Christa Monintja (2013-061-111) | Angelina Tjokro (2013-061-112)
INTRODUCTION• The 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 ÞNeural plate development -18th day
ÞCranial closure 24th day (upper spine)
ÞCaudal closure 26th day (lower spine)
Etiology & Risk Factor• Unknown• Multifactorial▫Genetic = single gene mutation (eg. Meckel’s
syndrome) & chromosomal abnormalities (trisomy 13, trisomy 18)
▫Nutrition Intake zinc, folic acid reduce spina bifida incidence
▫Environmental factor = lower socioeconomic status▫Racial▫Alcohol consumption▫Maternal problems
Use of anti epileptic drugs, maternal diabetes, hyperthermia, obesity
CLASSIFICATIONSPINAL DYSRAPHYSM
Spina Bifida APERTA
Meningocele
Myelomeningocele
Spina Bifida OCCULTASimple Spina Bifida
OccultaSpinal Dermal Sinus
Lipomyelomeningocele
Diastematomelia
Tethered Spinal Cord
MENINGOCELE• Protrusion 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
MANAGEMENT• Surgical treatment 12 to 24 hours after birth
prevent infection, swelling, and further damage.1. Using general anesthesia2. An incision is made in the sac and some of the
excess fluid is drained off3. The spinal cord is covered with the membranes
(meninges)4. The skin is closed over the protruding meninges,
spinal cord, and nerves.
What is Spina Bifida?
A midline defect of the Þbone, Þskin, Þspinal column, &/orÞspinal cord.
Definition• 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
Spina bifida occulta• The most mild, sometimes called “hidden” spina
bifida• 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.
• Processus spinous always lost, the laminae may be lost
• Occur in the lumbosacral region (L4-S1)
Spina bifida aperta/mielomeningokel• The most often, the most serious type • With this condition, a sac of fluid comes through
an opening in the baby’s back. Part of the spinal cord and nerves are in this sac and are damaged.
• Causes moderate to severe disabilities
Clinical Manifestation• Leg weakness and paralysis• Orthopedic abnormalities (i.e., club foot,
hip dislocation, scoliosis)• Bladder and bowel control problems, including
incontinence, urinary tract infections, and poor renal function
• Pressure sores and skin irritations• Abnormal eye movement• Hydrocphalus
• 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 Detection
ÞAmniocentesisAFP - indication of abnormal leakage
ÞBlood testMaternal blood samples of AFP
ÞUltrasonographyFor locating back lesion vs. cranial signs
Therapy• Surgical 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 antibiotics
• Skin grafts often are required for large defects. • Ventricular shunts, if indicated, are placed
concurrently with myelomeningocele closure or at a later
PROGNOSTIC• children 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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