Case 10 Chiari malformation type 1 (CM-I)

Figure 10.1: MRI of the patient Pre and post operative.

Clinical presentation

• A 39-year-old female patient presented with neck pain and weakness and atrophy in both hands (more pronounced on the right) for the past 6 months.
• History of suboccipital headaches dating back 2 years.
• Noted weakness and atrophy in hands starting 4 months ago.
• Lower Limbs: Full power
• Upper Limbs: Right—shoulder, elbow G4; hand G3. Left—shoulder, elbow full power; hand G4.
• Hyperreflexia in the lower limbs.

• Q1: Can you describe the pathology seen in the MRI?

Sagittal T1 and T2-weighted image findings:

• The cervical curvature is straightened with good vertebral alignment. There is no vertebral structural collapse or marrow signal alteration.
• The tonsils are pointed and peg-tailed, with tonsillar descent to the level of the C1 arch. There is an oval cyst at the beginning of the cord, accompanied by hyperintensity extending throughout the cervical cord and downwards, representing syringomyelia.

• Q2: What is the aim of surgery in the case of Chiari 1 malformation?
• The aim of surgery in the case of Chiari 1 malformation is to enlarge the posterior fossa and reestablish the flow of cerebrospinal fluid (CSF) between the cranial and spinal compartments. This can be achieved through multiple techniques ranging from simple bony decompression, which ensures CSF flow as verified by ultrasound, to more extensive procedures involving arachnoid lysis, tonsillar coagulation, and duroplasty.

Figure 10.2: diagrammatic represent explanation of syringomyelic symptoms.

• Pain : Dysthetic pain without dermatomal distribution.
• Motor : syringomyelic syndrome : segmental weakness, decrease tendon jerk, dissociated sensory loss.
• Sensory : Cape distribution , Dissociated ( loss of pain and temp with preserved light touch ) ,Gloves and stocking , may predispose to trophic ulcers
• Miscellaneous : Horner’s in cervical ,Scoliosis and torticollis.