Basal encephalocele in an adult patient presenting with minor anomalies: a case report

Abstract

IntroductionBasal encephalocele is rare in adults. Congenital and acquired cases have been reported with regard to the developmental mechanism, and the pathology has not been elucidated in detail.Case presentationWe encountered an adult with basal encephalocele strongly suggesting congenital development because of the presence of minor anomalies: strabismus and ocular hypertelorism. The disease manifested as persistent spontaneous cerebrospinal fluid rhinorrhea and repeated meningitis in a 66-year-old Japanese man. On computed tomography, brain tissue protruded through a part of the ethmoid bone of his right anterior skull base, and it was diagnosed as transethmoidal-type basal encephalocele. Regarding his facial form, the distance between his bilateral eyeballs was large compared to his facial width, and his canthal index (defined as inner to outer inter canthal ratio × 100) was calculated as 38.5, based on which it was judged as ocular hypertelorism. In addition, his right eyeball showed strabismus. A right frontotemporal craniotomy was performed for spontaneous cerebrospinal fluid rhinorrhea, and the defective dura mater region was patched with temporal fascia.ConclusionsMild minor anomalies that require no treatment are overlooked in adults, but the presence of several anomalies increases the possibility of congenital disease. Therefore, it may be necessary to examine minor anomalies in cases of adult basal encephalocele when considering the possibility that the disease may be congenital.

Brain and Optic Chiasm Herniation into Sella after Pituitary Tumor Apoplexy

“…The vast majority is diagnosed at birth or first year of life. However, there have been some cases diagnosed during adulthood, as facial abnormalities can be subtle or absent ( 33 ). In these cases, clinical presentation includes CSF rhinorrhea, visual impairment including visual field defects and amaurosis, endocrine abnormalities (hypopituitarism, hyperprolactinemia), and an epipharyngeal soft tissue mass ( 31 , 34 , 35 ).…”

Large Primary Transethmoidal Encephalocele Presenting in an Adult

“…If untreated, the latter can lead to recurrent meningitis before a diagnosis is made [1,3,4]. Notably, in this case, the patient did not experience meningitis as a sequela of CSF rhinorrhea in contrast to the majority of previous reports of transethmoidal encephalocele presenting in adulthood [4,6]. However, the patient did experience a lifetime of symptoms, incorrect diagnoses, and unsuccessful treatments, representing an enormous burden on quality of life and medical costs.…”

Cerebrospinal Fluid Rhinorrhea due to Transethmoidal Encephalocele Following Occipital Meningioma Resection in an Adult: A Case Report

“…12 Congenital transethmoidal encephalocele in adults can also be associated with minor anomalies (e.g., ocular hypertelorism or strabismus). 13 In our case, the specific etiology was difficult to determine. Acquired transethmoidal encephalocele in adults is generally more frequent than congenital.…”