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

Abstract

We report a 13-year-old female who presented to an outside emergency room following blunt trauma to the head. As part of her evaluation, an unenhanced CT of the face and radiographs of the cervical spine were performed. She was referred to our clinic with "abnormal" imaging. Radiographs revealed dysmorphic cervical spine and ventral clefting of the vertebral bodies from C7-T2. A subsequent MRI was obtained that demonstrated a large spinal cord mass. MRI demonstrated an unenhanced intramedullary mass of the cervicothoracic spinal cord measuring approximately 1.5 cm in greatest axial diameter. Surgical exploration of her intraspinal mass revealed a neurenteric cyst. Spinal neurenteric cysts are categorized in the spectra of occult spinal dysraphism and most likely arise from incomplete separation of the developing notochord and foregut in the embryo. We discuss potential etiologies for the formation of such cysts. The clinician should consider neurenteric cysts in their differential diagnosis of patients with spinal dysraphism and/or intraspinal masses.

Intramedullary Cervical Neurenteric Cyst Mimicking an Abscess

“…Intramedullary neurenteric cysts are rare and account for less than 5% of all reported cases. Amongst the 26 cases of pediatric intramedullary neurenteric cysts reported in the literature, 14 are located in the cervical region, suggesting that less than 50% of the spinal neurenteric cysts occur in the cervical region; intramedullary location is still rare [3,4,5,6,7,8,9,10,11,12]. Out of the 14 reported cervical neurenteric intramedullary cysts, 8 cases were in isolated form (table 1).…”

Clinical presentation and surgical outcomes of intramedullary neurenteric cysts

“…In our 13 cases, bone anomalies were identified in 30.8% of the patients, which was a slightly higher rate than that in the literature on intramedullary NECs. 3,18,19,21,24,37,40,41 In contrast, bone anomalies were more common in extramedullary NECs. 12 Thus, the diagnosis of an isolated intramedullary NEC without concurrent malformations becomes even more challenging.…”

“…8,14,19,26,40 Although commonly associated with other abnormalities, intraspinal NECs may be found without any abnormalities, and bone abnormalities were found in only 8 patients (21.1%) with an intramedullary NEC. 3,18,19,21,24,37,[40][41][42] Surgical methods ranged from cyst puncture and aspiration 25,39 to resection with cyst marsupialization or cyst-subarachnoid shunting. 21,32,37 In the 38 reported cases, 33 patients underwent surgical resection, and GTR was achieved in 17 cases.…”

“…However, it cannot explain the formation of NECs in their entirety, such as formation of intracranial lesions. 40,41 Thus, various pathogenic mechanisms may cause these lesions. …”

“…22 To our knowledge, only 38 cases of NEC (with or without concurrent malformations) have been described since the publication of the earliest report in 1954. [2][3][4]8,10,[12][13][14]16,[18][19][20][21][22][24][25][26][27][29][30][31][32][33][34][35][37][38][39][40][41][42]44,45 Most previous publications regarding intramedullary NECs have been case reports. Therefore, the optimal management for this lesion has not been fully determined.…”