Spontaneous rhinoliquorrhea caused by ecchordosis physaliphora

Introduction Ecchordosis physaliphora (EP) is a congenital retroclival remnant of notochordal tissue. It is usually asymptomatic and is considered an incidental radiological finding or occures in post-mortem autopsy.

Case We report a case of a 45-year-old patient with atraumatic rhinoliquorrhea and cephalgia as part of an EP. CT and MRI showed a fistula in the prepontine cistern with lateral bone pedicle as a typical correlate of EP. Endonasal, endoscopic therapy was decided in the event of severe liquorrhea. Preoperatively, the patient received a Tuohy drainage (TD) with intrathecal administration of fluorescein (1ml 5%) 8 hours before the operation. Intraoperatively, the flow of cerebrospinal fluid could be optimally localized through the fluorescein on the roof of the sphenoid sinus. The fistula was covered with belly fat, Tachosil and an autologous free concha nasalis flap. Postoperatively, 50 ml liquorfluid was drained daily for 6 days. A malignant process could be excluded histologically. The postoperative course was uncomplicated.

Discussion and conclusion This rare case of spontaneous rhinoliquorrhea shows the EP as an important differential diagnosis. Histologically a chordoma must be ruled out as a malignant differential diagnosis. Using a TD with intrathecal fluorescence for detecting a liquor fistulas is an off-label use that is successfully used in fistulas that are difficult to visualize. While 3-layer coverage is the standard procedure in the case of an EP-related skull base defect, the indication for TD remains very variable. The present case shows usefull combination of a TD for the pre-operative administration of fluorescein and simultaneous post-operative control of the liquorfluid pressure to support the healing of the defect.

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Publication History

Article published online:
13 May 2021