7/27/2019 CNS Congenital Kel 14

1/17

INTRODUCTION OF CONGENITAL DISEASE OFCENTRAL NERVOUS SYSTEM ANDHYDROCEPHALUS

Dr. pramDr.Alma

Drg.Saka

7/27/2019 CNS Congenital Kel 14

2/17

7/27/2019 CNS Congenital Kel 14

3/17

B. TAHAP KEDUA : PEMBENTUKANVENTRIKUALOSISTERNAL

Terjadi pada minggu VII VIII Kehamilan

Minggu VII : Plexus choroideus mulaimenghasilkan LCS.

Minggu VIII : Ujung ekor Ventrikel IV

mengalami perforasi..

7/27/2019 CNS Congenital Kel 14

4/17

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

7/27/2019 CNS Congenital Kel 14

5/17

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 ).

7/27/2019 CNS Congenital Kel 14

6/17

Minggu XX : Cortical Plate menebal

membentuksulci primer. Kegagalanpembentukannya menyebebkan

Lisencephaly (Tak ada sulcus), Microgyria

dan Macrogyria.

7/27/2019 CNS Congenital Kel 14

7/17

CNS DEVELOPMENT

7/27/2019 CNS Congenital Kel 14

8/17

CNS DEVELOPMENTAND

CNS CONGENITALANOMALIES(SUMMARY)

7/27/2019 CNS Congenital Kel 14

9/17

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

7/27/2019 CNS Congenital Kel 14

10/17

Mark S. Dias, MD; Michael Partington, MD. Embryology of Myelomeningocele and Anencephaly, 2004 American Association470598http://www.medscape.com/viewarticle/.of Neurological Surgeons

7/27/2019 CNS Congenital Kel 14

11/17

Failure of anterior neuropore to close

Brain and calvarium are absent & Replaced by acerebrovasculorum - a tangle of glial andconnective tissue

7/27/2019 CNS Congenital Kel 14

12/17

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.

7/27/2019 CNS Congenital Kel 14

13/17

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

7/27/2019 CNS Congenital Kel 14

14/17

TREATMENT OF CONGENITAL ANOMALIES

Surgery (generally)

Tissue excision and defect closure

Reconstruction

7/27/2019 CNS Congenital Kel 14

15/17

Moore

Clinically Oriented Anatomy 4th ed

7/27/2019 CNS Congenital Kel 14

16/17

Moore AJ

Neurosurgery

Principles and Practice

7/27/2019 CNS Congenital Kel 14

17/17

Additional Examinations

CT scan

MRI

Therapy :

Shunt

Proximal catheter

Distal catheter

Valve

Drugs.