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

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

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

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