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

Abstract

We report a rare case of ecchordosis physaliphora presenting with headache, nausea, and diplopia. On neurological examination, the patient was found to have left abducens nerve palsy. CT of the head without contrast was unremarkable. Brain MRI demonstrated a non-enhancing retroclival mass with a mass effect upon the ventral pons. The mass had increased signal intensity on T2 and decreased signal intensity on T1-weighted sequences. Lumbar puncture revealed an opening pressure of 37 cm H 2 O. The patient underwent an endoscopic endonasal approach for retroclival mass resection three weeks later. The tissue analysis of the mass was consistent with ecchordosis physaliphora. This could have been misdiagnosed as idiopathic intracranial hypertension had the MRI of the brain not been performed.

Extremely Rare Pathologies of the Craniovertebral Junction Region: A Case Series and Review of the Literature

“…However, in rare instances, it can manifest with clinical symptoms, necessitating therapeutic intervention. Up to now, the literature has documented only 40 cases of symptomatic EP that underwent surgical treatment [13][14][15][16][17][18]. The complete removal of the lesion and the SB repair allowed the complete resolution of the symptoms in our patient.…”

Spontaneous rhinorrhea: a possible concealing initial symptom of ecchordosis physaliphora. Illustrative case

“…The intraosseous growth of EP weakens the clivus, making it vulnerable to fractures with even minor trauma or spontaneous fractures. 5,[17][18][19] This "locus of minor resistance" can lead to the formation of a pseudo-meningocele or a CSF leak into the nasal cavity, increasing the patient's risk of developing pneumocephalus and meningitis. 20,21 The patient may experience various symptoms, such as headache, stiffness in the neck, sensitivity to sound and light, and a decline in consciousness due to irritation of the meninges.…”