A patient with spina bifida presents with stridor, swallowing issues, gagging/aspiration, and vocal cord paralysis. These S/S suggest?

• A. Chiari II malformation
• B. Arnold-Chiari I malformation
• C. Syringomyelia
• D. Tethered cord syndrome

Answer: (A)

When a patient with spina bifida develops stridor, swallowing difficulties, gagging/aspiration, and vocal cord paralysis, it points to brainstem involvement from Chiari II malformation. Chiari II occurs with myelomeningocele and involves downward herniation of the hindbrain through the foramen magnum, which can compress the medulla and lower cranial nerves. The nerves responsible for swallowing and vocal cord function (the glossopharyngeal, vagus, and hypoglossal roots) can be affected, leading to impaired swallow, poor gag reflex, aspiration risk, and vocal cord paralysis that may cause stridor and a weak or altered voice. This pattern contrasts with Arnold-Chiari I malformation, which usually presents later with headaches and cerebellar signs and lacks the classic association with myelomeningocele and prominent lower cranial nerve dysfunction. Syringomyelia would show spinal cord symptoms like cape-like loss of pain and temperature or segmental weakness, not the cranial nerve–level signs described. Tethered cord syndrome affects the spinal cord below the conus with progressive leg weakness, scoliosis, or bladder dysfunction rather than brainstem-related swallowing and vocal cord issues.