Publication:

Symptomatic cerebrospinal fluid leak secondary to sphenoidal Ecchordosis physaliphora: case report and literature review

Ecchordosis physaliphora (EP) is a rare, benign, and hamartomatous tumor that is driven from ectopic notochordal remnants. It has a usual asymptomatic presentation and is often incidentally detected in about 2% of autopsies. A 50-year-old female presented to rhinology clinic with clear nasal discharge for 6 weeks associated with frontal headache. Unremarkable neurological and ENT examination. Brain computed tomography (CT) scan demonstrated a focal bony defect at the posterior wall of sphenoid sinus with partial opacification of the sphenoid sinus showing air fluid level suspicious for cerebrospinal fluid (CSF) leakage with a small cyst. Further assessment with MRI of the brain showed signs of rhinorrhoea secondary to ecchordosis physaliphora. Patient was managed successfully with endoscopic trans-nasal skull base reconstruction. Upon follow-up at two years post-operatively, no evidence of recurrence was detected. EP is indistinguishable from chordoma histopathologically hence imaging plays a crucial role in diagnosis. EP appears as hyperintense in T2-weighted images and hypointense in T1-weighted images. Symptomatic EP cases are extremely rare and most of these cases are managed by resection via craniotomy. In addition, endoscopic endonasal trans-sphenoidal surgery (ETSS) provides a good approach. Imaging is crucial for the diagnosis of these lesions. Surgical treatment is considered the gold standard for symptomatic ecchordosis physaliphora. Endoscopic endonasal transnasal skull base reconstruction is effective for management, it is a minimally invasive approach for complete resection of the lesions with reduced hospitalization time and less postoperative complications.