International Journal of Neuroimaging

International Journal of Neuroimaging

International Journal of Neuroimaging

Current Issue Volume No: 1 Issue No: 1

Case Report Open Access Available online freely Peer Reviewed Citation

A Treatable Cause of Progressive Visual Loss

1Consulant Neurologist, Tiruvalla Medical Mission Hospital

2Head of Department of Neurology, Tiruvalla Medical Mission Hospital


Sphenoethmoidal encephalocoele is a rare clinical entity with varying clinical presentation. It can be seen in association with corpus callosal agnesis and midline facial defects. We are reporting a case of 44 year old female presented with progressive vision loss of right eye, and surgical correction of meningoencephalocoele and the defect prevent further visual detiortion.

Author Contributions
Received 23 Feb 2023; Accepted 30 Oct 2023; Published 18 Dec 2023;

Academic Editor: Jamal Uddin, Assistant Professor, Computer Science Department, Qurtuba University Of Science & Information Technology, Peshawar, KP, Pakistan.

Checked for plagiarism: Yes

Review by: Single-blind

Copyright © 2023 Miny Susan Abraham, et al.

Creative Commons License     This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Competing interests

The authors have declared that no competing interests exist.


Miny Susan Abraham, Nikhil Gladson (2023) A Treatable Cause of Progressive Visual Loss. International Journal of Neuroimaging - 1(1):1-3.

Download as RIS, BibTeX, Text (Include abstract )

DOI Coming Soon

Case Discussion

Reporting a case of 44 year old female presented with history of gradually progressive painless visual loss of right eye for past 6 months which was insidious in onset .She had nasal block on the right side, no nasal discharge. No history of diplopia, headache, vomiting or fever. Past history was remarked with pthisis bulbi of left eye following a trauama at 13 years and history of surgical correction of cleft lip and palate. Her right eye vision was fairly normal till 6 months back; the only functioning eye. She is a diabetic and hypertensive on treatment. On examination her higher mental function was normal, visual acuity was only counting finger at 1 feet on right eye, no vision left eye, fundus showed pale disc on right side with features of diabetic retinopathy. VEP showed reduced P100 amplitude on right side. MRI had been done which showed sphenoethmoidal encephalocoele with corpus callosal agenesis. She was referred to higher centre for trannasal endoscopic repair, following which her visual acuity on the right side became CF at 3 feet on 7 th day post-surgery and no further progression of symptoms and improved to 6/60 at one month. Postoperative CT scan showed no evidence of hemorrhage infarct or midline shift. 1

Images and description

MRI showed parallel running lateral ventricles and colpocephaly (Racing Car appearance), large central sphenoethmoidal meningoencephalocoele with large bony defect in anterior sellar floor, planum sphenoidale and ethmoid bone. Herniated sac (4.1x3.5x2.6) contains bilateral optic nerves olfactory nerves, infundibulum of pituitary gland and csf. Pthisis bulbi of left eye noted.Figure 1

Figure 1.Fundus Images and OCT – right optic atrophy ; pale optic disc
 Fundus Images and OCT – right optic atrophy ; pale optic disc

Encephalocoeles can be of spontaneous /congenital or traumatic in origin. Transphenoidal encephalocole is a rare clinical entity and may have varying clinicalpresentation. Prompt diagnosis and surgical treatment is required in such patients. Reports of association of sphenoethmoidal meningoencephalocoele with corpus callosal agenesis have been there in the literature. sphenoethmoidal encephalocoele causing progressive visual loss have not been reported yet.2

Running Title

Sphenoethmoidal meningoencephalocoele should be considered as one of the differential diagnosis of progressive vision loss in patients with midline craniofacial defects. Prompt diagnosis and surgical repair may prevent further visual deterioration.3


  1. 1.D S Acherman, D K Bosman, Horst C van der. (2003) Cleft Palate Craniofac J. Sphenoethmoidal encephalocele: a case report. 40(3), 329-33.
  1. 2.Pradhan Prajina, Phuyal Subash, Ritesh Lamsal DM, Pooja A, Raju P. (2021) Transsellar trans-sphenoidal encephalocele with cleft lip, cleft palate and agenesis of corpus callosum: Radiology Case Reports.
  1. 3.Sanjari Reza, Seyed A Mortazavi, R Shams Amiri, Ardestani S H Samimi, Amirjamshidi Abbas. (2013) Intrasphenoidal Meningo-encephalocele: Report of two rare cases and review of literature ;: Surg Neurol Int. 4, 5.