Spinal Neurenteric Cysts and Their Relation to More Common Aspects of Occult Spinal Dysraphism

, , , , and , Peninsula Neurosurgical Associates, Hampton, Virginia; Brigham and Women's Hospital, Boston, Massachusetts; and Section of Pediatric Neurosurgery, Division of Neurological Surgery, Department of Surgery, University of Alabama at Birmingham, Alabama

Neurosurg Focus. 2001;10(1) 

In This Article

Abstract and Introduction

Object. 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.
Methods. 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.
Conclusions. Complete excision of the neurenteric cyst remains the treatment of choice, as subtotal excision is associated with recurrence.

Spinal neurenteric cysts are infrequently reported congenital abnormalities believed to be derived from an abnormal connection between the primitive endoderm and ectoderm during the 3rd week of life. Neurenteric cysts are not confined to the spinal column but may be found within the brain, mediastinum, abdomen, pelvis, or even in a subcutaneous location.[1,3,16,17,28,48,50] These lesions are rare. Intraspinal neurenteric cysts represent 0.3 to 0.5% of all spinal "tumors."[35] They are, in fact, not tumors, which differentiates them from teratomas; instead, they are more similar to harmatomas -- displaced nests of endodermally derived tissue. The terminology for these lesions is problematic, as they have been reported as a neurenteric cyst, enterogenous cyst, enteric cyst, gastrocytoma, dorsal enteric fistula, split notochord syndrome, and teratoid cyst.[6,12,20,26,27,37,49] Part of the confusion in naming these cysts is that they are not uniform; likely they exist as a spectrum of lesions. The presentation may vary from a simple isolated intraspinal cyst with no other abnormalities to that of the so-called split notochord syndrome, as proposed by Bentley and Smith [6] in which there may be multiple visceral and vertebral anomalies.

Neurenteric cysts are considered to be a form of OSD, as are the following entities: lipoma, lipomyelomeningocele, SCMs, meningocele manqué, dermal sinus tract inclusion cysts (dermoids and epidermoids), terminal syringohydromyelia, and myelocystocele. A consistent association between neurenteric cysts and these other forms of OSD has not been reported. In analyzing our 13 cases, we were struck by both their fascinating case histories as well as the incredible diversity of associated findings (typically either other forms of OSD or the various cutaneous and orthopedic stigmata of OSD). We present these patients in detail, compare them with other large reported series, and discuss possible causes of intraspinal neurenteric cysts.

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