Craniocervical Intradural Neurenteric Cysts

Abstract

Neurenteric cysts are rare lesions of the central nervous system that are lined by epithelium of intestinal nature. They result from abnormal separation of germ cell layers in the 3rd week of embryonic life, leading to persistence of entodermal elements in the spinal canal. The common location is cervical and ventral to the spinal cord. The lesions have been recognized as being entodermal in origin by such markers as vertebral anomalies, gut cysts, bowel reduplication and the presence of keratin markers. Three unusual cases of childhood craniocervical intradural neurenteric cysts in patients aged 4-8 years are described. These cysts were located in the ventral aspect of the spinal canal ranging from the craniocervical junction to the C6 level and were associated with bony anomalies such as a bifid clivus, hemivertebrae and blocked vertebrae. Two patients presented with signs of cervical cord compression and 1 with recurrent meningitis. One child with an anterior cervicomedullary region mass had undergone unsuccessful transoral transpalatal exploration and subsequently required excision with a dorsolateral approach. The second patient, after two successful attempts at drainage via laminectomies, required myelotomy of the spinal cord to allow excision. Thus the patient with the lesion from C4 through C6 underwent vertebrectomy and anterior excision with a C4 through C7 strut graft fusion. There were bony and vascular anomalies which would have complicated a dorsal approach. Pathological diagnosis of neurenteric cysts was based on the findings of nonciliated mucin producing small cuboidal or columnar epithelium lining a cystic cavity. Neurenteric cysts are uncommon congenital anomalies which may become symptomatic in the pediatric population. Their intradural and not infrequent intramedullary ventral location makes surgical approach and management difficult. An anterior approach allows visualization of the bony vertebral and vascular anomalies and permits excision which cannot be accomplished from a dorsal route.

Intraparenchymal Supratentorial Neurenteric Cyst

“…Furthermore, cyst leakage has been known to cause meningeal irritation and infection. 11 The case presented in this report and the cases presented by Bavetta et al 12 and Cheng et al 13 have all involved intracranial cysts that were diagnosed only when they reached a very large size (4-5 cm). This is probably due to their frontal location.…”

“…3,11,13,[15][16][17][18] Intracranial neurenteric cysts are more frequent in the posterior fossa in comparison to the supratentorial area. Cyst locations in the posterior fossa include the fourth ventricle, the prepontine space, adjacent to the jugular foramen, the cerebellopontine angle, the cerebellar vermis and within the medulla.…”

“…1 5 Spinal neurenteric cysts can occur over a similar wide range of ages as intracranial cysts but are more common in the pediatric age group and have a distinct 2-3:1 male predominance. 4,11 The signs and symptoms produced by neurenteric cysts are usually secondary to compression or irritation of surrounding neural structures (Table). There is usually a long history of symptoms that progress over months to years with an acute worsening once the cyst reaches a critical size.…”

“…3,16 Alternatively, some intracranial and spinal neurenteric cysts may present with a fluctuating course of episodic symptoms that have occasionally led to a misdiagnosis of multiple sclerosis. 11,17,21,24 Intermittent leakage of cyst contents may explain the fluctuating symptoms. Furthermore, cyst leakage has been known to cause meningeal irritation and infection.…”

“…8,9,11,22 The majority of previously reported supratentorial neurenteric cysts were completely excised on initial treatment (9/10 cases) with improvement or no worsening of the preoperative condition of the patient (Table). None of these cysts have recurred.…”