An Incidental Finding of Ecchordosis Physaliphora in a Case of Abducens Nerve Palsy: Case Report

Abstract

Ecchordosis physaliphora (EP) is a rare non-malignant mass that originates from remains of the notochord and is typically asymptomatic. A 42-year-old man presented with sudden onset of painless horizontal diplopia and his neurological exam showed sixth cranial nerve palsy. Magnetic resonance imaging (MRI) identified a non-enhanced retroclival mass (EP) with increased signal intensity on T2 and decreased signal intensity on T1-weighted sequences. He was treated with methylprednisolone, completely recovered in four weeks and has remained symptom free. Conservative management should be attempted before surgery in all cases since symptoms can resolve spontaneously and EP could be an incidental finding.

A Rare Case of Benign Long-Standing Ecchordosis Physaliphora

“…According to Ilorah et al, atypical features of EP include an absent bony stalk, a T2-hypointense protrusion from the clivus, T2 hypointensities bordering the lesion, a T2-hypointense center within the lesion, and a T2 hyperintensity on the pharyngeal surface or dorsum sellae [ 10 ].…”

An Ecchordosis Physaliphora, a Rare Entity, Involving the Central Nervous System: A Systematic Review of the Literature

“…There are no definite diagnostic criteria for EP, and diagnosis is usually made based on imaging characteristics. Typical EP lesions are cystic T2 hyperintensities, a T1 hypointensity with no contrast enhancement in the midline craniospinal axis along the dorsal aspect of the posterior clivus, are often at the level of Dorello’s canal [ 22 ], areless than six centimeters and have a stalk [ 3 ]. Atypical features of EP include an absent bony stalk, a T2 hypointense protrusion from the clivus, T2 hypointensities bordering the lesion, a T2 hypointense center within the lesion and a T2 hyperintensity on the pharyngeal surface or dorsum sellae [ 22 ].…”

“…Early studies mentioned a surgical approach throughsuboccipital craniotomy [ 36 , 44 , 47 ], retrolabyrinthrinepresigmoid [ 28 , 37 ] and transmaxillary [ 12 ], which were associated with higher morbidities, complications and increased hospital stays. Few studies stuck to conservative management with steroids for acute states where patients presented with diplopia and VI CN palsy due to compression from EP and have shown satisfactory results [ 5 , 22 ]. Alkan et al conservatively treated with osmotic diuretic for brain stem edema after intratumoral hemorrhage, causing a mass effect on the pons.…”

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

“…The “inverted bottle effect” refers to the situation in which prolonged and profuse nasal liquorrhea in the cranial cavity creates negative pressure, resulting in air entering through an existing defect in an extracranial manner. 22 Compression of the cranial nerve VI as it travels through Dorello’s canal, 23 which may be narrowed by extrinsic compression of the tumor or a subarachnoid herniation, is a potential cause of diplopia. 24 The prepontine cistern is the most common location for an EP, with a frequency of 67%.…”