Abstract
ABSTRACT Objective Neurenteric cysts account for 0.7–1.3% of all spinal cord tumors. These rare lesions are composed of heterotopic endodermal tissue. Methods A 26-year-old woman with a 13-month history of severe cervicalgia and brachial paresthesia. Clinically she had mildbilateral brachial paresis (4/5), generalized hyperreflexia and a left Babinski Sign. Past medical history was significant for a cervical fistula closure when she was 1yearold. The superior somatosensory evoked potentials revealed medullary axonal damage with a left predominance. A cervical magnetic resonance imaging of the neck was performed showing a dorsal homogeneous cystic intradural extramedullary lesion with high signal intensity on T2. Computed tomography revealed a Klippel-Feil syndrome. Results A posterior laminectomy and surgical excision were performed without complications. Post-operative follow-up showed a complete recovery of arm strength. The histopathological report confirmed the preoperative diagnosis of neurenteric cyst. Most neurenteric cysts are located in the spine, mainly in a ventral position. A total of 95% of neurenteric cysts are found in the intradural/intramedullary compartment. Symptomatic neurenteric cysts typically appear in the second and third decades of life and are 1.5 to 3 times more common in men. In 50% of the cases, other vertebral malformations have also been reported. In this case, a congenital dorsal enteric cyst and a Klippel-Feil syndrome were observed. Conclusions The intraspinal neurenteric cysts are rare lesions that must be included in the differential diagnosis of a dorsal, intradural cystic structure. The diagnosis may be overlooked, especially in cases of chronic neck pain without neurological deficit. Level of evidence V; Expert Opinion.
Highlights
Neurenteric cysts account for between 0.7 and 1.3% of all axial spinal cord tumors.[1]
The intraspinal neurenteric cysts are rare lesions that must be included in the differential diagnosis of a dorsal, intradural cystic structure
We present a case of a neurenteric intradural extramedullary cyst of atypical localization due to its location dorsal to the spinal cord, associated with the Klippel-Feil syndrome and a posterior cutaneous cervical fistula treated via posterior approach
Summary
Neurenteric cysts account for between 0.7 and 1.3% of all axial spinal cord tumors.[1]. During the third week of embryogenesis the incomplete separation of the notochordal plate and the endoderm produces heterotopic remnants of the gastrointestinal and respiratory epithelium and the possible formation of compressive cystic lesions of the medulla.[2,3,4,5,6] Classically, neurenteric cysts are reported in locations anterior or anterolateral to the spinal cord.[6]. We present a case of a neurenteric intradural extramedullary cyst of atypical localization due to its location dorsal to the spinal cord, associated with the Klippel-Feil syndrome and a posterior cutaneous cervical fistula treated via posterior approach. The only significant history was the closure of a cervical fistula performed when she was 1 year old. Upon admission, she presented bilateral brachial paresis (4/5 on the Daniels scale) with global hyperreflexia and the Babinski sign on the left side. The pathology report confirmed the preoperative diagnosis of a neurenteric cyst
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