Cricopharyngeal achalasia associated with Arnold-Chiari malformation in childhood

Six patients with myelomeningocele and the Arnold-Chiari malformation developed cricopharyngeal achalasia and lower cranial nerve deficits. Diagnosis is established by cine-esophagram. Distortion of the brain stem or cranial nerves secondary to the Arnold-Chiari malformation may produce the autonomic imbalance necessary for cricopharyngeal achalasia. Treatment is supportive and includes verification of cerebral spinal fluid shunt function. Suboccipital craniectomy may reverse progressive lower cranial nerve deficits and reduce cricopharyngeus spasm. Cricopharyngeal myotomy may be considered when the cranial nerve deficits and cricopharyngeal achalasia are fixed, irreversible, and continue to cause disability.