Neurenteric Cyst

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Status

Done

Parent item

Spinal anomalies

General

• Aka:
• Enterogenous cyst
• Teratomatous cyst
• Intestinoma
• Archenteric cyst
• Enterogene cyst
• Endodermal cyst.

Definition

• CNS cyst lined by epithelium primarily resembling that of the GI tract, or less often, the respiratory tract.

Numbers

• 1% of all spinal cord tumours

• 16% of CNS Cyst

Location (Extramedullary> intramedullary)

• Spine (most) -Can occur anywhere along the path of neuro-enteric canal
• Upper thoracic spine
• Lower cervical spine

• Intracranial (Rare): because the neurenteric canal forms caudal to the brain
• CP angle
• Ponto-medullary region
• Parasellar area

• Never occur supratentorially because the canal never goes cranial enough

Embryology

• Persistence of neurenteric canal of Kovalevsky
• Normally neurenteric canal closes by 3rd wk

• Congenital. Not true neoplasms.

Pathology

• Spinal neurenteric cysts (NEC) may have a fistulous or fibrous connection to the GI tract (through a spinal dysraphism) and some call these endodermal sinus cysts
• Endodermal elements

• Lined by respiratory or GI epithelium

• Can have smooth mucosa underlying fibrovascular tissue

• Bony abnormality

• Attached to anterior cord

Clinical presentation

• Pain

• Myelopathy

• Cardio-respiratory compromise

• Meningitis (from fistulous tract)

• Manifest as early as prenatally and late as adulthood

• Associated developmental vertebral anomalies
• Diastematomyelia
• Klippel-feil syndrome

Evaluation

• MRI
• Best
• CSF intensity on T1/2
• Non enhancing

Treatment

• Spinal NEC
• Surgical removal usually reverses the symptoms.
• Recurrence is uncommon with complete removal of cyst wall.

• Intracranial NEC
• Capsule adherent to brainstem may prevent complete resection, which predisposes to delayed recurrence.
• Apparently successful treatment by evacuation of contents and marsupialization has been reported (5 cases, mean follow-up: 5 yrs).
• Incomplete removal requires long-term follow-up.
• Hydrocephalus is shunted if indicated.

DDx

• Ependymal cyst

• Epidermoid cyst

• Dermoid cyst

• Mets (Intracranial)
• Primary adenocarcinoma

• Dorsal enteric fistula
• Rare and severest of the complex dysraphic states.
• A cleft connecting bowel with dorsal skin is present.
• The tract traverses the prevertebral soft tissues, vertebral bodies, spinal canal and cord, and neural arches.
• Due to a persistent connection between the endoderm and ectoderm resulting in a fistulous tract.
• Whereas neurenteric cysts are cystic lesions believed to arise from an abnormal connection that is form from the neurenteric canal during embryonic development.