Cns cong anomalies
Transcript of Cns cong anomalies
CONGENITAL CONGENITAL ANOMALIES OF CNSANOMALIES OF CNS
ETIOLOGYETIOLOGY Hyperthermia Drugs Malnutretion Nutrition: folic acid deficiency Disease: diabetes Toxins: alcohol, smoking Infections: rubella, toxoplasmosis, CMV,
syphilis Radiation Unknown (most cases)
Primary neurolationPrimary neurolation Occur in 3-4 week gestation Neural tube (NT) internally (gives rise to the
CNS above S2 segment), epidermis externally & neural crest (NC) at the junction of NT &
Epidermis mesoderm cells which migrate away from the
neural tube and give rise to the PNS,skull, facial and jaw bones, pigment cells, adrenal medulla
Neural tube closureNeural tube closure Start at the cervical area (cervico-
medularly junction) Extend cranially and caudally Cephalic neuropore close around day
25 Caudal neuropore closes around day
29 and correspond to S2 segment
Disorders of primary Disorders of primary neurolationneurolation
Split cord syndrome Craniorachischisis Neuroenteric cyst Anencephaly Myeloschisis Arnold chiari malformation Meningocele, myelomeningocele Dermal sinus Encephalocele
Dermal sinus Dermal sinus tracttract
result from incomplete disjunction of neuroectoderm from cutaneous ectoderm
The sinus tracts extend deep into the subcutaneous tissues, reaching the spinal canal in one-half to two-thirds of cases.
it may be attached to the dura, causing tenting of the thecal sac.
AnencephalyAnencephaly:: Failure of anterior neuropore to close
Brain and calvarium are absent & Replaced by a cerebrovasculorum - a tangle of glial and connective tissue
Spina Bifida OccultaSpina Bifida OccultaCommon anomaly
Midline defect of vertebral bodies without protrusion of spinal cord or meningesAsymptomaticSpine X RayMRI
Meningocele and Meningocele and myelomeningocelemyelomeningocele
Primary failure of neural tube closure Myelomeningocele Meningocele
Most frequently located in the thoracolumbar area.
MeningoceleMeningoceleMeninges herniate through a defect in the
posterior vertebral arches
Spinal cord is normal
Fluctuant midline mass
Covered with skin
MeningoceleMeningoceleINVESTIGATIONSINVESTIGATIONS::
Plain X RayUltrasonography
MRICT Scan
MyelomeningoceleMyelomeningocele Severe form of neural tube defectSevere form of neural tube defect1/40001/4000
- -Located anywhere esp. lumbosacralLocated anywhere esp. lumbosacral - -Low sacral region: no motor impairment, anesthesia in Low sacral region: no motor impairment, anesthesia in
perineal areaperineal area - -Mid lumbar region: saclike cystic structure covered with thin Mid lumbar region: saclike cystic structure covered with thin
layer of partially epithelialized tissuelayer of partially epithelialized tissue . .
Remnant of neural tissue are visibleRemnant of neural tissue are visibleFlacid paralysisFlacid paralysis
Clinical MinifestaionClinical Minifestaion
EncephaloceleEncephalocele Bony defects in midline Brain tissue can protrude through
hole Most common in occipital region Prognosis depends on quantity of
cerebral tissue that herniates into the defect
Surgical repair is required
CHIARI MALFORMATIONCHIARI MALFORMATIONHerniation of post. Fossa contents through Herniation of post. Fossa contents through foramen magnumforamen magnum..
Normal: upto 5 mm of tonsillar descent Normal: upto 5 mm of tonsillar descent through foramen magnumthrough foramen magnum..
Classified into Chiari I and II based on Classified into Chiari I and II based on amount of descentamount of descent..
CLINICAL FEATURESCLINICAL FEATURES
Presents in infants or young adultsPresents in infants or young adults..
Headache aggravated by coughing or Headache aggravated by coughing or strainingstraining..
Signs of brainstem compressionSigns of brainstem compression
TREATMENTTREATMENT
DECOMPRESSIONDECOMPRESSION
CRANIOSYNOSTOSISCRANIOSYNOSTOSIS
Premature closure of cranial suturesPremature closure of cranial sutures..May affect single or multiple suturesMay affect single or multiple suturesPresent with abnormal head shapePresent with abnormal head shape..Associated with rare genetic Associated with rare genetic abnormalitiesabnormalities..Surgery is required to correct itSurgery is required to correct it..
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