Cerebrospinal fluid fistula secondary to ecchordosis physaliphora

Abstract

A patient with a large ecchordosis physaliphora extending from the sphenoid sinus into the subarachnoid space of the prepontine cistern and resulting in a cerebrospinal fluid fistula is described. Ecchordoses are most commonly asymptomatic and found only incidently at autopsy. This case report adds to the scant literature on symptomatic ecchordoses. The previously reported cases of symptomatic ecchordoses and intradural chordomas are briefly reviewed. Differentiation of chordoma and symptomatic ecchordosis may be difficult; however, the intradural location and relatively benign behavior of the latter are useful points. A discussion concerning the remnants of the notochord which persist in the adult and their role in the genesis of chordoma and ecchordosis physaliphora is also provided.

Giant ecchordosis physaliphora in an adolescent girl

“…Among the 6 symptomatic cases, 2 patients presented with CSF rhinorrhea due to a skull base CSF fistula, 1 patient presented with facial pain, and a fourth patient presented with diplopia. 2,4,18,26 In these cases, the symptomatology was consistent with the potential effects of the lesion given its proximity to the clivus and pons. In the remaining 2 cases, the reported symptoms were headaches, dizziness, or confusion, which might have been caused by the EP but were very likely coincidental.…”

“…Although these cells usually have an intraosseous location, they occasionally perforate through the dorsal wall of the clivus into the subdural and subarachnoid spaces, which may explain the pathogenesis of EP at the prepontine region. 4,18,28,29,36 With EP, there is often a bony stalk that connects it with the clivus, emphasizing its clival origin. 17,24,32 This potential continuum of cells from the clivus to the intradural space explains the occurrence of lesions anywhere along this path, such as was seen in our case in which the lesion was isolated in the epidural spaces.…”

A case of ecchordosis physaliphora presenting with an abducens nerve palsy: A rare symptomatic case managed with endoscopic endonasal transsphenoidal surgery

“…The major symptoms presented were headache (33.3%), diplopia (25%), and subarachnoid hemorrhage (16.6%) [Table 1]. [1246–810–121415]…”

“…[3–513] However, symptomatic EP cases are extremely rare, as only 11 cases have been reported to date, most of which were managed by resection via craniotomy. [1246–810–121415] Here we report, a rare case of symptomatic EP managed successfully by endoscopic endonasal trans-sphenoidal surgery (ETSS) without resection via craniotomy. We also present a review of the literature and discuss the advantages of ETSS and the differences between EP and intradural chordoma.…”

Intradural Retroclival Chordoma

“…Intradural extraosseous chordoma has clearly different features from those of typical chordoma 17) . These entities show different biological behavior, with gradient evolution of growth and malignancy from the usually asymptomatic ecchordosis, to the slowly evolving intradural chordoma, to the highly malignant and invasive chordoma 10) . Classic chordomas in bone frequently have ill-defined margins, and complete resection is usually not feasible even with extensive surgery because a few tumor cells are often left behind in the bone or dura.…”