Spinal neurenteric cysts and their relation to more common aspects of occult spinal dysraphism

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>Neurenteric cysts are infrequently reported congenital abnormalities believed to be derived from an abnormal connection between the primitive endoderm and ectoderm. The authors report a series of 13 patients treated over a 50-year period.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>Of the 13 patients, seven were female and six were male. Their ages at presentation ranged widely from 5 weeks to 52 years of age. Children presented more commonly with cutaneous stigmata of occult spinal dysraphism (OSD) whereas adults presented primarily with pain. Neurological deficit as a presenting symptom was less common in our series, a finding that reflects the slow growth of these lesions. In all but one patient some form of vertebral anomaly was associated with the cystic lesions, including two patients with Klippel–Feil abnormalities. There was a high incidence of associated forms of OSD including split cord malformation, lipoma, dermal sinus tract, and tethered spinal cord. In previous reports the authors have suggested that neurenteric cysts are more common in the cervical region and in a position ventral to the cord. In the present series these cysts most commonly occurred as intradural, extramedullary masses in the thoracolumbar region, situated dorsal to the spinal cord. The median follow-up period was 7.5 years, and postoperative outcome reflected a patient's preoperative neurological status; in no patient was outcome worsened due to surgery.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Complete excision of the neurenteric cyst remains the treatment of choice, as subtotal excision is associated with recurrence.</jats:p></jats:sec>

Rare Cervical Spine Neuroenteric Idem Cyst With Intramedullary Extension

“…Rauzzino, et al reported a series of 13 cases of neurenteric cysts. [27] The cysts were most commonly located dorsal to the cord in the thoracolumbar region. Also, neurenteric cyst was associated with another form of OSD in 69% of the cases.…”

Neurenteric cyst: Case report and a review of the potential dysembryology

“…4). Neurenteric cysts are relatively benign slow-growing lesions with a recurrence rate of 27% in one series (Rauzzino et al, 2001).…”

“…These rare lesions are ectopias in that they are displaced nests of endodermally derived tissue. Historically, neurenteric cysts have been described as enterogenous cysts, enteric cysts, gastrocytomas, dorsal enteric cysts, split notochord cysts, and teratoid cysts (Rauzzino et al, 2001). Neurenteric cysts are often seen in conjunction with other forms of occult spinal dysraphism.…”

A Rare Case of Split Notochord Syndrome

“…[2] Another theory attribute this discrepancy to the varied positions of Hensen's node (accessory neurenteric canal). [3] A recent theory suggests a primary notochord defect resulting in secondary changes to the paraxial mesoderm, which is responsible for the formation of the spinal column, giving rise to a medial interosseous space. Through this space, the endoderm and the underlying primitive intestine herniate, adhere to the dorsal ectoderm, and eventually rupture.…”