by nour eldin mohammed l.
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Neuroradiology Congenital Malformation of CNS. By: Nour-Eldin Mohammed . Callosal Dysgenesis. Definitions: One or all segments of CC absent (if partial, body remains). CC segments front to back: Lamina rostralis (unmyelinated) • Rostrum (myelinated) Genu • Body • Splenium.

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by nour eldin mohammed


Congenital Malformation of CNS

By:Nour-Eldin Mohammed

callosal dysgenesis
Callosal Dysgenesis


One or all segments of CC absent

(if partial, body remains)

  • CC segments front to back:
  • Lamina rostralis (unmyelinated)
  • • Rostrum (myelinated)
  • Genu
  • • Body
  • • Splenium
callosal dysgenesis3
Callosal Dysgenesis
  • General Features:
  • Axial: Parallel lateral ventricles
  • Coronal: "Trident" anterior horns resemble "viking helmet" or "moose head“
  • Location: Midline anomaly
  • Size: CC remnants vary in size, shape , Remnant may be paper thin or bulbous
callosal dysgenesis4
Callosal Dysgenesis

Radially arrayed gyri "point to" 3rd ventricle

Absent Cingulate Gyrus

callosal dysgenesis5
Callosal Dysgenesis

Lateral ventricles are key to diagnosis:

• Parallel (non-converging)

• Widely separated

• Occipital horns often dilated (colpocephaly)

• Pointed frontal horns

callosal dysgenesis6
Callosal Dysgenesis
  • Coronal T2WI MR
  • shows agenesis of the corpus
  • Callosum
  • trident shaped lateral ventricles
  • vertical hippocampi (open arrow),
  • enlarged, "keyhole" shaped
  • temporal horns and Probst
  • bundles (curved arrow).
callosal dysgenesis7
Callosal Dysgenesis

Axial NECT with widened "windows" shows colpocephaly, calcified (arrow) midline lipoma that extends through choroid fissures into lateral ventricles (open arrows).

Axial TlWI MR shows parallel ventricles, colpocephaly, and a midline lipoma. Note lipoma (arrow) protruding into the lateral ventricles.

callosal dysgenesis8
Callosal Dysgenesis
  • Coronal T2WI MR in fetus shows trident shaped lateral
  • ventricles, agenesis of Cc
  • "Probst bundle" (curved arrow)
  • vertical hippocampus (arrow).
differential diagnosis of callosal dysgenesis
Differential Diagnosis of Callosal Dysgenesis

Partial Absence of Callosal Dysgenesis


Differential Diagnosis of Callosal Dysgenesis

Destruction of CC

• Surgery (callosotomy),trauma (Acquired interhemispheric disconnection syndrome)

• Hypoxic ischemic encephalopathy (HIE), infarcts

• Metabolic

differential diagnosis of callosal dysgenesis11
Differential Diagnosis of Callosal Dysgenesis

Stretched Corpus Callosum (e.g.: Hydrocephalus)

• Thinned CC but all parts present

• Severe hydrocephalus often present

  • Definitions
  • • Mass of mature non-neoplastic adipose tissue
  • CNS lipomas are congenital malformations, not true
  • neoplasm

General Features

• Best diagnostic clue: Well-delineated lobulated extra-axial mass with fat attenuation/intensity

• Location : Midline location common

80% supratentorial :

40-50% interhemispheric fissure (over corpus callosum; may extend into lateral ventricles, choroid plexus)

15-20% suprasellar(attached to infundibulum, hypothalamus)

10-15% pineal region (usually attached to tectum)

20% infratentorial

• Cerebellopontine angle (may extend into lAC, vestibule)

• Uncommon: Jugular foramen, foramen magnum


Sagittal Tl WI MR

Sagittal Tl WI MR with fat-saturation

(a small interhemispheric lipoma (arrows) above the corpus callosum, found incidentally at MR imaging in this patient with headache.


Sagittal TlWI MR shows a well-circumscribed high signal lesion in the pineal region (arrow).The patient was asymptomatic.

Axial gross pathology shows a quadrigeminal lipoma (arrow)