Craniocervical Neurenteric Cyst without Associated Abnormalities

Abstract

Spinal neurenteric (NE) cyst is an uncommon congenital cyst and frequently found in the cervical region. The clinical symptoms associated with this entity depend on the site of the lesion and are not typical for all such cysts. A definitive diagnosis can only be made by biopsy and histological examination. MRI can confirm these cystic masses and is the method of choice for their imaging investigation. They are often connected by a fibrous tract, fistula or cleft to structures derived from the primitive gut in the thoracic or abdominal cavities and are commonly associated with anterior spina bifida or other vertebral anomalies. We report a case of craniocervical NE cyst without associated abnormalities and discuss the implications for clinical diagnosis and management by a thorough review of the literature.

Far-lateral transcondylar approach: surgical technique and its application in neurenteric cysts of the cervicomedullary junction

“…6,8,13,14 Spinal neurenteric cysts are often connected by a fibrous tract, fistula, or cleft to structures derived from the primitive gut in the thoracic or abdominal cavities and are commonly associated with vertebral anomalies such as vertebral body dysgenesis, split spinal cord malformations, hemivertebra, segmentation abnormalities, and spina bifida. 7,12,22,24 This is not the case with intracranial neurenteric cysts. 6 Although the exact pathogenesis remains unknown, intracranial neurenteric cysts are thought to arise from a failure of dissolution of the transient neurenteric canal between the foregut or the respiratory buds and the notochord during notochordal development.…”

“…Neurenteric cysts that are located in the anterior cervicomedullary junction are even rarer, and often require a skull base approach for adequate resection. 1,12,14,16,20,21 We describe two cases of neurenteric cysts arising from the cervicomedullary junction that were resected via a far-lateral transcondylar approach. We discuss the surgical approach and operative nuances involved in removing these lesions, and we review the clinical presentation of neurenteric cysts in this region as well as the neuroimaging characteristics, histopathological findings, and surgical management.…”

Staged bilateral far-lateral approach for bilateral cervicomedullary junction neurenteric cysts in a 10-year-old girl

“…13 To our knowledge, only 26 cases of intracranial neurenteric cysts, including the present case, have been identified in patients 18 years of age or younger (Table 1). 5,[8][9][10]12,14,16,17,20,21,[23][24][25][27][28][29][30][32][33][34] All of the pediatric cases previously reported in the literature involved children who presented with unilateral neurenteric cysts. Of the 25 patients, 10 were male and 15 were female; the patients' mean age was 7.27 years (range 1 day-18 years).…”

Neurenteric Cyst of the Craniocervical Junction in an Infant -Case Report-

“…2,5,9) Neurenteric cysts are lined with gastrointestinal mucosa and have direct contact with the central nervous system. 4) Bony anomalies are associated with half of the neurenteric cysts in the spinal cord, but rarely with intracranial neurenteric cysts.…”

“…6,11) Neurenteric cyst usually occurs in the first two decades, with most patients aged under 40 years and a 3:1 male to female predominance. 5) Neurenteric cyst is predominantly located ventral to the spinal cord in the lower cervical and upper thoracic spinal subarachnoid space, or at the lumbar and sacral levels, and infrequently involve the posterior fossa. 1,3,4) Neurenteric cysts of the craniocervical junction occurring in infancy are extremely rare, and in only two patients aged under 3 years.…”