Imaging in developmental malformations of the brain
•Download as PPTX, PDF•
23 likes•2,873 views
brief description of imaging in common congenital anomalies of brain
Recommended
Recommended
More Related Content
What's hot
What's hot (20)
Viewers also liked
Viewers also liked (20)
Similar to Imaging in developmental malformations of the brain
Similar to Imaging in developmental malformations of the brain (20)
Recently uploaded
Recently uploaded (20)
Imaging in developmental malformations of the brain
- 1. Imaging in Developmental Malformations of the Brain Presenter :Dr.Chakradhar
- 2. Over view 1.Introduction 2.Normal development of Brain 3.Classification of malformations 4.Imaging features of anomalies 5.conclusion Imaging in developmental Malformations of the brain
- 3. Introduction Congenital anomalies of the brain Extremely complex Best studied by correlating with embryological development. Imaging in developmental Malformations of the brain
- 4. NORMAL BRAIN DEVELOPMENT MYELINATION DORSAL INDUCTION Formation of neural tube VENTRAL INDUCTION Formation of vesicles and Segmentation MYGRATION, and PROLIFERATION a.Primary Neurulation b.Secondary neurulation Imaging in Developmental malformations of the brain
- 5. Stages of brain development Imaging in Developmental malformations of the brain Three phases: Neurulation, Canalization, Retrogressive differentiation Stage 1: Dorsal Induction: Formation and closure of the neural tube Occurs at 3-5 weeks
- 6. Dorsal induction defects: . Imaging in Developmental malformations of the brain Stages of brain development Neural tube defects -Anencephaly, -Cephalocele, - Chiari malformations and -Spinal dysraphic disorders
- 7. Stage 2: Ventral Induction: Formation of the brain segments and face Occurs at 5-10 weeks of gestation Stages of brain development Imaging in Developmental malformations of the brain Three vesicles Prosencephalon - cerebral hemispheres/thalamus, Mesencephalon- midbrain and Rombencephalon- cerebellum/brain stem
- 8. Ventral induction disorders: Imaging in Developmental malformations of the brain Stages of brain development Holoprosencephalies, Dandy Walker malformation, Cerebellar hypoplasia/dysplasia Joubert syndrome, Rhombencephalosynapsis, Septo -optic dysplasia, and Facial anomalies.
- 9. Stage 3: Migration and Differentiation Occurs at 2-5 months of gestation . Imaging in Developmental malformations of the brain Stages of brain development Neuronal migration from germinal matrix to the surface resulting in Cortical organization
- 10. Migration Disorders: Imaging in Developmental malformations of the brain Stages of brain development Heterotopias, Agyria-pachygyria, Polymicrogyria, Lissencephaly, Schzencephaly, Corpus callosal agenesis, Lhermitte-Duclos disease
- 11. Differentiation and proliferation disorders: Imaging in Developmental malformations of the brain Stages of brain development Aqueductal stenosis, Arachnoid cyst, Megalencephaly, Micoencephaly, Neurocutaneous syndrome (phakomatoses) Congenital vascular malformation, and Congenital neoplasms
- 12. Stage 4: Myelination Begins at 6 months of gestation, Matures by 3 years. Imaging in Developmental malformations of the brain Stages of brain development Progresses from Caudal to Cephalic, Dorsal to Ventral, and Central to Peripheral Disorders of Myelination Dysmyelinating diseases, Leukodystrophies.
- 13. DISORDERS OF NEURAL TUBE FORMATION DISORDERS OF SEGMENTATION DISORDERS OF MYGRATION and CORTICAL ORGANISATION DISORDERS OF MYELINATION a.Anencephaly b.Cephaloceles c.Chiari Malformations a.Holoprosencephaly 1.Alobar 2.Semilobar 3.Lobar b.Corpus callosal agenesis c.Dandy Walker malformations a.Hetrotropias b.Lissencephaly, Schizencephaly Classification of congenital malformation of brain a.Leukodystrophies b.Dysmyelinating Disorders Imaging in Developmental malformations of the brain
- 14. Neural Tube Defects Imaging in Developmental malformations of the brain
- 15. Anencephaly Neural Tube Defects Imaging in Developmental malformations of the brain complete or partial absence of the cerebral structures, cranial vault and skull base. It results from failure of closure of the cephalic portion of the neural tube
- 16. Cephaloceles Herniation of intra cranial structures through congenital defects in Dura and skull Cephaloceles MeningoencephalocystoceleMeningoencephaloceleMeningocele MeningesMeninges Meninges Neural tissue Neural tissue Ventricles Neural Tube Defects Imaging in Developmental malformations of the brain Neural Tube Defects
- 17. Encephalocele Fronto Nasal Encephalocele Occipital Encephalocele Imaging in Developmental malformations of the brain Neural Tube Defects Herniation of brain and Meninges through a defect in the skull. It is typically covered by skin (closed defect) or a Thin layer of epithelium (open defect).
- 18. Fronoto Nasal, Naso Orbital Encephalocele Nasal Glioma (D/d for encephaloele) Imaging in Developmental malformations of the brain Neural Tube Defects
- 19. Type of Chiari malformation Imaging features(core) Associated findings Type 1 (Tonsillar Ectopia) Elongated cerebellar tonsils with herniation into cervical canal Syringohydromyelia(30-60%) Type 2 (Arnold Chiari) Herniation of vermis ,tonsils, medulla 1.Small and shallow posterior fossa 2.Myelomeningocele (nearly 100%) 3.Upward herniation of cerebellar hemispheres 4.Corpus callosal dysgenesis, heterotopias, polymicrogyria Type 3 Type 2 + occiputal encephalocele Type 4 Cerebellar Aplasia/Sever Hypoplasia The chiari malformations Imaging in Developmental malformations of the brain Neural Tube Defects
- 20. Chiari I Malformation Imaging in Developmental malformations of the brain Neural Tube Defects Level below the Foramen Magnum First Decade: 6mm Second /Third decade: 5mm Fourth-Eight Decade:4mm and Nienth Decade:3mm
- 21. Chiari II Malformation Imaging in Developmental malformations of the brain Neural Tube Defects
- 22. Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation
- 23. Holoprosencephaly Disorders of Diverticulation and Segmentation Cerebellum and brain stem are relatively normal Imaging in Developmental malformations of the brain Developing Forebrain (prosencephalon) fails to divide into hemispheres and lobes.
- 24. Normal brain Alobar Holoprosencephaly semilobar Holoprosencephaly Lobar Holoprosencephaly Types of Holoprosencephaly Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation
- 25. Alobar Holoposencephaly Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation • Single cresent shaped ventricle • No Separation of the cerebral hemispheres • Fused thalami and basal ganglia • Absence of septum pellucidum, corpus callosum, falx cerebri, and interhemispheric fissure
- 26. Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation Semilobar holoprosencephaly • Interhemispheric fissure is formed posteriorly •Rudimentary occipital and temporal horns •Thalami and basal ganglia are partially separated •Septum pellucidum is absent • callosal splenium may be formed
- 27. Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation Lobar holoprosencephaly • Ventral portions of the frontal lobes remains fused • Rudimentary frontal horns are formed • Septum Pellucidum is absent • Thalami and basal ganglia well separated
- 28. Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation Septo optic Dysplasia (de Morsier syndrome) • Milder form of lobar holoprosencephaly • Hypoplastic optic nerves, optic chiasma, • Absent septum pellucidum •Squared frontal horns Associated hypoplasia of Hypothalamic-Pituitary axis seen in 2/3rd cases
- 29. Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation Corpus Callosum Agenesis Corpus callosal development starts at 12 weeks , and completes by 20 weeks Formation is from anterior to posterior direction, Starts with Genu- Body-Splenium. The rostrum is last to develop Corpus Callosum Agenesis Complete agenesis Partial agenesis
- 31. Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation Complete callosal agenesis •Entire corpus callosum, cingulate gyrus and sulcus are absent •Widely separated, parallel and non-converging lateral ventricles. • Colpocephaly (dilated occipital horns) •Frontal horns are small and pointed
- 32. Complete corpus callosal agenesis colpocephaly Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation
- 33. Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segemtation Partial callosal agenesis •Splenium and rostrum absent • Genu and body present
- 34. Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation Associated anomalies •Migration disorders (heterotopias, lissencephaly, schizencephaly) • Chiari II malformation • Dandy-Walker malformation • Holoprosencephaly • Corpus callosal lipoma
- 35. Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation of Posterior fossa
- 36. Dandy walker spectrum Dandy Walker syndrome Dandy Walker variant Mega Cysterna Magna Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation of Posterior fossa
- 37. Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation of Posterior fossa Joubert’s Syndrome (Congenital Vermian Hypoplasia) • Vermian dysgenesis •Enlarged superior cerebellar peduncles and • High riding fourth ventricle.
- 38. Imaging in Developmental malformations of the brain Associated anomalies • Occipital encephalocele (30%), • Callosal dysgenesis, • Cortical dysplasia, • Hypothalamic hamartoma, and • Ocular, hepatic & renal diseases. Disorders of Diverticulation and Segmentation of Posterior fossa
- 39. Classified into two groups: Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation of Posterior fossa Pontocerebellar hypoplasia (PCH). • Combined hypoplasia of both the cerebellum and the pons •These are primarily genetic disorders
- 40. • Hypoplastic Brain stem and cerebellum that is close to the tentorium • Cerebellar hemisphares are wing like, appear to float in posterior fossa . Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation of Posterior fossa -
- 41. Imaging in Developmental malformations of the brain Disorders of Diverticulation and Segmentation of Posterior fossa Differentials of pontocerebellar hypoplasia CDG syndromes-(disialotransferrin) will be elevated in CDG Dandy-Walker syndrome - Normal Pons Dandy-Walker variant -Normal Pons
- 42. Disorders of Migration Imaging in Developmental malformations of the brain
- 43. Ectopic Migration Cobble stone-Lissencephaly (type 2) Over Migration Classic Lissencephaly (type1) Under Migration Migration Disorders Band Heterotropia Subcortical Subependymal Heterotrpia Imaging in Developmental malformations of the brain Disorders of Migration
- 44. Imaging in Developmental malformations of the brain Disorders of Migration Lissencephaly (Agyria-Pachygyria): Refers to “smooth brain” with absent or poor sulcation. Types: Complete (agyria) Incomplete (pachygyria).
- 45. . Imaging in Developmental malformations of the brain Disorders of Migration • Thickened cortex with flat broad gyri •Smooth gray-white matter Interface • Colpocephaly Oblique and shallow • Sylvian fissures- figure eight Type I (classical) Lissencephaly Associated with Miller-Dieker syndrome
- 46. Type II(Cobblestone) Lissencephaly Imaging in Developmental malformations of the brain Disorders of Migration • Thickened cortex • Polymicrogyric appearance. • Hypomyelination of underlying white matter Associated with Fukuyama congenital muscular dystrophy, Walker-Warburg syndrome and muscle-eye-brain syndrome
- 47. Two types: Nodular type(common), Band/laminar type (uncommon) Imaging in Developmental malformations of the brain Disorders of Migration Heterotopias Presence of normal neurons at abnormal sites Best appreciated on medium tau inversion recovery sequences Nodular type:
- 48. Imaging in Developmental malformations of the brain Disorders of Migration • A layer of neurons interposed between the ventricle and cortex •Overlying cortex has pachygyria or polymicrogyria Band or laminar type
- 49. Disorders of Cortical organisation Imaging in Developmental malformations of the brain
- 50. Non lissencephalic Cortical Dysplasia Disorders of Cortical organisationImaging in Developmental malformations of the brain Disorders of Cortical organisation Polymicrogyria • Thickened cortex •Irregular, bumpy gyral pattern • Irregular gray-white matter junction • Underlying white matter signal changes
- 51. Pachygyria Imaging in Developmental malformations of the brain Disorders of Cortical organisation •Thickened and flattened cortex • Blurred gray-white matter Junction • Underlying white matter signal changes
- 52. Schizencephaly Imaging in Developmental malformations of the brain Disorders of Cortical organisation Cleft lined by Heterotopic gray matter extends from the ventricular (ependyma) to the periphery (pial surface) of the brain, traversing through the white matter. Two types: Closed lip (type I) Open lip (type II)
- 53. Closed lip (type I) schizencephaly Openlip (type2) schizencephaly Imaging in Developmental malformations of the brain Disorders of Cortical organisation
- 54. Porencephalic cyst resembles schizencephaly but CSF space is lined by gliotic white matter, in contrast to gray matter as in Schizencephaly Porencephalic cyst Imaging in Developmental malformations of the brain Disorders of Cortical organisation
- 55. Anomalies Associated with Schizencephaly : Heterotopias, Septo-optic dysplasia, Absence of septum pellucidum Callosal dysgenesis Imaging in Developmental malformations of the brain Disorders of Cortical organisation
- 56. Hemimegalencephaly Hamartomatous overgrowth of a part or all of one cerebral hemisphere Imaging in Developmental malformations of the brain Disorders of Cortical organisation Associated with : • Epidermal nevus syndrome, • Klippel-Trenaunay–Weber syndrome, • Neurofibromatosis type 1
- 57. • Linear Nevus Sebaceous Syndrome(LNSS) Imaging in Developmental malformations of the brain Disorders of Cortical organisation
- 58. Conclusion • Variety of congenital anomalies of brain of brain coexist • Clinical Presentation of various anomalies is more or less same • Imaging plays an important role in diagnosing them
- 59. Thank you
Editor's Notes
- DIFFERRATION ANDKEE
- S
- Porencephalic cyst