cns congenital kel 14
TRANSCRIPT
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INTRODUCTION OF CONGENITAL DISEASE OFCENTRAL NERVOUS SYSTEM ANDHYDROCEPHALUS
Dr. pramDr.Alma
Drg.Saka
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B. TAHAP KEDUA : PEMBENTUKANVENTRIKUALOSISTERNAL
Terjadi pada minggu VII VIII Kehamilan
Minggu VII : Plexus choroideus mulaimenghasilkan LCS.
Minggu VIII : Ujung ekor Ventrikel IV
mengalami perforasi..
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C. TAHAP KETIGA : PROLIFERASI SEL-
SEL.
Sel-sel berproliferasi menjadi Neuroblas.
Gangguan tahap ini mengakibatkan Cerebellar
Hypoplasia dan Dandy Walker Cyst.
Bila proliferasi berlebihan menyebabkan
Neurofibromatosis, Tuber Sclerosis dan Sturge
Weber Pisease
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D.TAHAP IV : MIGRASI NEURONAL
Neuroblast bermigrasi ke lateral membentuk
Mantle Zone yang merupakan bentuk primitif
Basal Ganglia
Minggu VII : Neroblast migrasi kedua
membentuk Cortical Plate ( Bentuk PrimitifSubtansia Grisea ).
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Minggu XX : Cortical Plate menebal
membentuksulci primer. Kegagalanpembentukannya menyebebkan
Lisencephaly (Tak ada sulcus), Microgyria
dan Macrogyria.
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CNS DEVELOPMENT
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CNS DEVELOPMENTAND
CNS CONGENITALANOMALIES(SUMMARY)
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ETIOLOGY OF CONGENITAL BRAINMALFORMATIONS
Unknown (most cases)
Chromosomal (6%)
Single gene (2%)
Environmental (maternal)
Nutrition: folic acid deficiency
Disease: diabetes
Toxins: alcohol, smokingInfections: rubella, toxoplasmosis, CMV, syphilis
Radiation
Famous M, M.D. Dept. of Neurosciences, School of Medicine, Jordan University of Scince and Technology
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Mark S. Dias, MD; Michael Partington, MD. Embryology of Myelomeningocele and Anencephaly, 2004 American Association470598http://www.medscape.com/viewarticle/.of Neurological Surgeons
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Failure of anterior neuropore to close
Brain and calvarium are absent & Replaced by acerebrovasculorum - a tangle of glial andconnective tissue
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HOLOPROSENCEPHALY
Holoprosencephaly, overview Jeanty www.fetus.net"semilobar" form ofholoprosencephaly,
**accompanied by:
midline facial defects
(cleft lip, cleft palate,cyclopia, etc)
Dandy-Walker malformationcerebellumcongenital brain malformation involving the
and the fluid-filled spaces around it
Dandy-Walker Malformation
cause very big head because itth ventricle4obstructs the
Dr. Josefa I. Panlilio,MD. Pediatrics Congenital Anomalies of the CentralNervous System. UERMMMC College of Medicine. Philippines.
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CHIARIMALFORMATIONS,(types I-IV)
congenital hindbrain abnormalitiesaffecting the structural relationshipsbetween the cerebellum, brainstem,
the upper cervical cord,
Type I & Type II
Dr. Josefa I. Panlilio,MD. Pediatrics Congenital Anomalies of the CentralNervous System. UERMMMC College of Medicine. Philippines
PORENCEPHALY Presence of cysts or cavitieswithin the brain that result fromdevelopmental defects or
includingacquired lesions ,Infarction of tissue
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TREATMENT OF CONGENITAL ANOMALIES
Surgery (generally)
Tissue excision and defect closure
Reconstruction
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Moore
Clinically Oriented Anatomy 4th ed
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Moore AJ
Neurosurgery
Principles and Practice
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Additional Examinations
CT scan
MRI
Therapy :
Shunt
Proximal catheter
Distal catheter
Valve
Drugs.