Abstract Details

Title A Rare Case of Ecchordosis Physaliphora Presenting with Headache, Abducens Nerve Palsy and Intracranial Hypertension

Topic Neuro-oncology

Presentation(s) P3 - Poster Session 3 (12:00 PM-1:00 PM)

Poster/Presentation
Number 13-007

Objective

Report a rare case of ecchordosis physaliphora presenting with headache, abducens nerve palsy, and intracranial hypertension

Background

Ecchordosis physaliphora is a congenital benign hamartomatous lesion derived from notochord remnants, usually located in the retroclival prepontine region. Unlike chordomas, which are often symptomatic due to brainstem or cranial nerve compression, patients with ecchordosis physaliphora are usually asymptomatic.

Design/Methods NA

Results A 22-year-old female was admitted for severe headache, nausea, and diplopia. The headache, with which she had awoken the previous morning, was located at the occipital region as well as the left frontal region, was associated with nausea, vomiting, and blurred vision, and was aggravated by lying down, cough and bending over. The next day when she awoke in the morning, she had blurred vision and binocular horizontal diplopia. She had been experiencing intermittent occipital headaches for one year. She also reported a 30-lbs unintentional weight-gain over the past year. Neurological exam revealed impaired left eye abduction without papilledema or other neurological deficits. CT head without contrast was unremarkable. In order to rule out subarachnoid hemorrhage, lumbar puncture was performed, which revealed an opening pressure of 37 cm H2O. Brain MRI with and without contrast was then performed, and revealed a non-enhancing retroclival mass with mass effect upon the ventral pons, which had increased signal intensity on T2-weighted sequences and decreased signal intensity on T1-weighted sequences, and was highly suspicious for ecchordosis physaliphora. Although the patient’s headache had improved after the lumbar puncture, the diplopia persisted.
The patient underwent an endoscopic endonasal approach for retroclival mass resection. The tissue analysis of the mass was consistent with ecchordosis pysaliphora.

Conclusions

We report a rare case of symptomatic ecchordosis physaliphora. The diagnosis was revealed by brain MRI and tissue analysis. Ecchordosis physaliphora could have been misdiagnosed as idiopathic intracranial hypertension had the brain MRI not been performed.

Authors/Disclosures
Ruiqing Sun, MD, PhD (UTMB health) PRESENTER	Dr. Sun has nothing to disclose.
Yousaf Ajam, MD (Prevea Health)	Dr. Ajam has nothing to disclose.
Todd Masel, MD	Dr. Masel has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Zeto Inc..

��ɫ��

��ɫ��