Enlarging Ventriculus Terminalis in a Patient With Polyarteritis Nodosa

Abstract

Ventriculus terminalis (VT) is a cystic embryological remnant within the conus medullaris that normally regresses after birth. In rare cases, it may persist into adulthood and give rise to neurologic symptoms. The pathogenesis remains unclear but is thought to be related to failed embryonic regression with other proposed possible etiologies including vascular disturbances. We present an intriguing case of a slowgrowing VT in a woman with progressive neurologic symptoms who experiences symptomatic relief following thoracic laminectomy and fenestration. Our case is the first to present a unique association with polyarteritis nodosa and only the third to report a case of documented enlargement of the VT over time successfully treated with surgical fenestration.

Cystic dilatation of the ventriculus terminalis: A narrative review

“…The present investigation examined the prevalence of various preoperative MRI abnormalities, the extent of spinal segment involvement, the dimensions of cysts, and associated abnormalities such as tethered cord, dysraphism, and lipoma. [ 3 4 5 , 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 ] The findings underscore the importance of preoperative MRI in understanding the characteristics of VTs and their potential impact on neighboring anatomical structures. There was considerable diversity seen in the distribution of cysts across different spinal segments.…”

“…In total, 290 articles were found after an initial search of the PubMed, Scopus, and Google Scholar databases. In total, 34 studies[ 3 4 5 , 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 ] met the inclusion criteria after being screened for quality via titles, abstracts, and full texts. The present study involved the analysis of patient-level data from a cohort of 90 individuals presenting with ventricularis terminalis.…”

“…This review focused solely on the inclusion of case reports or case series. [ 3 4 5 , 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 ] The research included in the study shown heterogeneity in terms of sample numbers, methodology, and reporting standards. The presence of variability in the data has the potential to generate bias, which in turn might affect the generalizability of the conclusions.…”

Usefulness of cyst-subarachnoid shunt using syringo-subarachnoid shunt tube for symptomatic enlarging ventriculus terminalis: A case report and review of the literature

“…This structure usually forms during embryogenesis and subsequently disappears during the regressive differentiation period. [ 1 - 4 , 8 - 10 ] Expansion in adulthood is a very rare but known phenomenon, although the underlying mechanisms are still unclear. [ 1 , 8 ] Nassar et al .…”

“…Even then, VT is generally asymptomatic and rarely requires treatment because the appearance of symptoms as the cyst enlarges is extremely rare. [ 1 , 3 , 10 ] As a result, treatment strategies for this condition have not been established. Here, we describe three cases of symptomatic, enlarging VT in adulthood and propose the utility of cyst-subarachnoid shunt using a syringo-subarachnoid shunt tube for symptomatic enlarging VT.…”