Cervical persistent neurenteric canal results from a failure of proper notochord formation during embryogenesis. The neuroenteric canal is a transient structure formed by a fusion of the notochordal plate and the definitive endoderm, facilitating communication between the yolk and amniotic sacs during gastrulation. It normally closes when the notochordal plate ends fuse to form the notochord proper prior to neurulation. Improper closure results in an inappropriate and persistent connection between the gut and upper spinal column. The degree of neurological damage depends on the degree of spinal disruption.

The subject grossly exhibits mild edema around the neck and upper back. The cervical spine demonstrates scoliosis and spina bifida with meningocele.

Internally, the upper cervical vertebrae exhibit ventral failure of closure. The vertebral bodies themselves are highly asymmetric and dysplastic.

The stomach is stretched superiorly and passes dorsally behind the larynx. The esophagus is hypoplastic. The lumen of the esophagus is in continuity (on microscopy) with the central canal of the spinal cord.
The cervical spinal cord is duplicated and asymmetrically and ventrally herniated, and the central canal is dilated.

Note the thoracic and lumbar vertebral column is spared from deformity, as is the brain.

See related content for external views of the front and back, radiograph, and photographs and micrographs of internal structures.