Posterior Fossa Neurenteric Cysts Can Expand Rapidly: Case Report

Abstract

Neurenteric cysts are considered congenital lesions that may slowly expand over time. Although more commonly found in the spinal canal, they may be found intracranially, particularly in the posterior fossa. Here, we present an unusual case of a large, rapidly expanding histologically confirmed posterior fossa neurenteric cyst in a 53-year-old woman, who presented with quadriparesis. Computed tomography imaging done ~1.5 years before admission failed to demonstrate any obvious abnormality; however, the lesion had grown to 4 cm in maximal dimension at presentation with significant mass effect. The lesion was resected microsurgically using a retrosigmoid approach. The patient improved postoperatively and was neurologically intact at last follow-up. We could find no other documented case of marked, rapid expansion of a neurenteric cyst in the literature. We conclude that, although neurenteric cysts are thought to be congenital, they can undergo rapid expansion even in adults. The mechanism of expansion is unknown but may involve increased secretion, hemorrhage, or inflammation. We discuss the surgical management and review the literature in view of this surprising finding.

A dorsally located giant posterior fossa neurenteric cyst in a Chinese woman

“…The fluid of the cyst, however, is often clear or yellowish. A brown oily substance, similar to that seen in our patient, has also been reported [3,6]. Xanthomatous changes also can occur [7].…”

“…On gross examination, yellow, white, gray, and red cyst walls have all been reported [6]. The fluid of the cyst, however, is often clear or yellowish.…”

On the Enigma of the Human Neurenteric Canal

“…Furthermore, the neurenteric canal may allow the migration of multipotent endodermal cells producing neuroendodermal cysts (or neurenteric cysts in the old nomenclature of de Oliveira et al [2005]) along the entire neuroaxis including the supratentorial space (the cavity cranial to the cerebellum) in infants and middle-aged adults [Mittal et al, 2010]. These cysts can grow enormously in a short time [Priamo et al, 2011], causing neurological symptoms such as paraplegia [Rizk et al, 2001] and the need for neurosurgical intervention with close diagnostic follow-up by magnetic resonance imaging [Yang et al, 2015]. The neurenteric canal has also been suggested to be involved in the development of rare but severe abnormalities summarized as the split notochord syndrome which includes dorsal enteric fistula, a split vertebral column, and intestinal alterations [Bentley and Smith, 1960;Holschneider and Fendel, 1971;Singh and Singh, 1982;Gupta and Deodhar, 1987;Dhawan et al, 2017].…”

Ventral foramen magnum neurenteric cyst presenting as acute rapidly progressive quadriparesis and respiratory compromise: A case report and review of literature

“…Neurologic examination revealed quadriparesis with motor power of 3/5 in all four limbs; all deep tendon reflexes were brisk, jaw jerk was absent, and bilateral Babinski sign was present. [7] Bone abnormalities, which are commonly associated with spinal neurenteric cysts, are rarely noted with intracranial neurenteric cysts. There were no bony anomalies in the radiograph of the cervical spine.…”

“…There were no bony anomalies in the radiograph of the cervical spine. [7,9] Aspiration followed by complete microsurgical resection of the cyst wall is the treatment of choice, as aspiration alone generally results in recurrence. Next morning, she developed breathing difficulty and further neurological deterioration.…”