Chiari I with syrinx and skull base anomalies

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Short neck with restricted neck motions.

Patient Data

Age: 16 years
Gender: Female
This study is a stack
Sagittal
T1
This study is a stack
Sagittal
T2
This study is a stack
Sagittal
STIR
This study is a stack
Coronal
T2
This study is a stack
Axial
T2
This study is a stack
Axial
T2
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Info

The MRI sequences demonstrate:

  • caudal descent of the cerebellar tonsils through the foramen magnum 18 mm below the McRae line and 12 mm below the McGregor line.
  • dilatation of the supratentorial ventricular system and 4th ventricle.
  • skeletal anomalies with basilar invagination (the tip of the odontoid process projects 2 mm above the McRae line and 7 mm above the McGregor line) with compression of the bulbomedullary junction.
  • intramedullary cystic cavity of the upper dorsal spinal cord in keeping with syrinx.
This study is a stack
Axial
non-contrast
This study is a stack
Sagittal
non-contrast
This study is a stack
Axial bone
window
This study is a stack
Sagittal
bone window
This study is a stack
Coronal
bone window
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Info

The CT scan demonstrates:

  • dextroscoliosis.
  • hypoplasia of the right occipital condyle.
  • midline defect of the anterior arch of the atlas, with agenesis of the posterior arch.
  • hypoplasia of the lateral masses of C1 (more prominent on the left)
  • hypoplasia of the odontoid process and the body of C2.
  • basilar invagination compressing the bulbomedullary junction.
  • vertebral block from C2-C3 to C4-C5 level (Klippel-Feil malformation type 2)

Case Discussion

CT and MRI features of a Chiari I malformation with associated hydrocephalus, syrinx and skeletal anomalies.

 

Additional contributor: ZE. Boudiaf, MD CHU, Constantine, Algeria

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