Neurenteric cyst

Neurenteric cyst (also known as enterogenous cyst, endodermal cyst, or neuroenteric cyst) is a rare congenital malformation classified under other specified congenital malformations of the spinal cord (ICD-10: Q06.8). These cysts are benign, endoderm-lined developmental lesions that arise from abnormal persistence of the neurenteric canal, which is a transient embryonic connection between the yolk sac (future gut) and the amniotic cavity through the developing notochord. They are most commonly found in the intraspinal compartment, particularly in the lower cervical and upper thoracic spine (ventral to the spinal cord), but can also occur intracranially, most often in the posterior fossa. Rarely, they may be found in the mediastinum or other locations. Clinical presentation depends on the location and size of the cyst. Spinal neurenteric cysts typically present with progressive myelopathy, back or neck pain, radiculopathy, sensory deficits, motor weakness, and in some cases bowel or bladder dysfunction due to spinal cord compression. Intracranial neurenteric cysts may cause headaches, cranial nerve palsies, cerebellar signs, or symptoms of raised intracranial pressure. Associated vertebral anomalies such as spina bifida, hemivertebrae, or diastematomyelia are frequently observed. Symptoms may present at any age but are most commonly identified in childhood or young adulthood. The primary treatment for neurenteric cysts is surgical excision. Complete resection is the goal, as it offers the best chance of cure and minimizes recurrence. However, complete removal may not always be possible when the cyst is densely adherent to the spinal cord or brainstem, and subtotal resection carries a risk of recurrence. There is no established pharmacological therapy. Long-term follow-up with serial imaging (MRI) is recommended to monitor for recurrence, particularly after incomplete resection. Prognosis is generally favorable when complete excision is achieved, though neurological deficits present before surgery may not fully resolve.

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