English

Abstract

Trans-sellar trans-sphenoidalencephaloceles are rare and the trans-sellar variety is the least common. We present a 9-year-old male patient with trans-sellar trans-sphenoidal nasopharyngeal encephalocele which herniated into the nasopharynx through the congenital defect in the floor of sella. Patient presented with complains of nasal obstruction, nystagmus& visual disturbance in left eye. A trans-sellar trans-sphenoidal nasopharyngeal encephalocele was found on computed tomography (CT) and magnetic resonance imaging (MRI).We discuss our clinical findings and the results of preoperative computed tomography and magnetic resonance imaging along with review of this rare condition. INTRODUCTION: Encephaloceles occur in approximately1 in 3000 to 5000 live births1, 2, 3. Basal meningoencephaloceles are rare anomalies, reportedly constituting 1% 10% of all encephaloceles and originate from a congenital opening in the midline region of the skull base, which permits meninges, neural tissue or both to herniated from the intracranial space 4, 5, 6, 7. Basal encephaloceles occur with an estimated incidence of one in every 35, 000 live births1. Pollock classifies them as follows:(1) Sphenopharyngeal or trans-sphenoidal, when they protrude into the epipharynx and/or sphenoidalsinus; (2) spheno-orbital, when the protrusion is through the superior orbital fissure into the superior orbit producing unilateral exophthalmus; (3) sphenoethmoidal, when the cerebral mass herniates through the sphenoid and ethmoidal bones into the posterior nasal cavity; (4) transethmoidal, whenencephalocele extends into the anterior nasal cavity; and (5) sphenomaxillary, when the meningo-encephalocele passes through the superior orbital fissure into the orbit and through the inferior orbital fissure into the pterygopalatine fossa8, 9. Transsphenoidalencephaloceles are rare and the transsellar variety is the least common variety. Wediscuss our clinical findings and the results of preoperativecomputed tomography and magnetic resonance imaging of trans-sellartrans-sphenoidal nasopharyngeal encephalocele along with review of this rare condition. CASE REPORT: A 9 year old patient with normal built for age presented with complaints of nasal obstruction with anterior nasal discharge, nystagmus, headacheand visual disturbances more in left eye since 1 year. Clinical examination showed large nasopharyngeal soft tissue mass lesion causing near total occlusion of nasopharynx. Thesite of origin of the mass was not able to make out.Visual examination showed horizontal nystagmus in both eyes with decreased vision on left side.He had no history of CSF rhinorrhea, meningitis/seizures. His physical exam was unremarkable and the patient was referred for further work-up. Radiograph of skull showed defect in the floor of sella [Fig.1]. Axial non enhanced computer tomography (NECT) showed large cerebrospinal fluid (CSF) density cystic lesion extending from sella, sphenoid sinus into nasopharynx[Fig 2] Bony defect noted in thesellar floor with widening of sella [Fig.3].Rest of the brain was normal. Diagnosis of trans-sphenoidalencephalocele was made.