Intrasphenoidal Meningo-encephalocele: Report of two rare cases and review of literature

Abstract

Background:Transsphenoidal encephaloceles represent a rare clinical entity with wide range of symptoms in adult. Such lesions require accurate diagnosis and surgical treatment. The incidence of congenital encephalocele is very low occurring in approximately 1 in 3000-5000 live births. Even though 63 similar cases have been reported in the literature not all of them have been discussed completely. Due to the rare occurrence of these lesions, we will focus on the main clues in the diagnosis and management of such lesions, which are challenging.Case Description:We intend to present our experience with two cases of trans-sphenoidal meningoencephalocels, one located medially and the other herniating through the Sternberg's canal. The younger was 17 and the elder was 47 years old and both of them presented with cerebrospinal fluid (CSF) leakage. Both patients were treated successfully using pure endoscopic endonasal approach.Conclusion:Ideal surgical approach for such patients is still not clear due to lack of adequate experience in the literature, it is suggested that full preoperative imaging studies might lead the surgeons to undertake minimally invasive skull base approaches in similar patients.

Morning glory syndrome associated with transsphenoidal encephalocele and panhypopituitarism

“…Surgical repair of encephalocele, as advocated by many authors, was not performed because it is often not beneficial due to the risk of damaging functioning tissue. 9 Our patient had bilateral MGS associated with transsphenoidal encephalocele with endocrine changes, including GH and gonadotropin deficiency with a late diagnosis of hypothalamic-pituitary involvement, despite the fact that the history of the syndrome was known. We therefore emphasize the need for early endocrinological work-up and long-term follow-up, because hormone deficiencies may appear years after the initial diagnosis.…”

Caudal Regression and Encephalocele: Rare Manifestations of Expanded Goldenhar Complex

“…Encephalocele is a protrusion of the brain and/or meninges through a defect in the skull and can be numbered among neural tube defects (NTDs) [3]. It can be due to failure of neural tube closure, resulting in a bony defect through which herniation of neural tissue may occur.…”

“…It can be due to failure of neural tube closure, resulting in a bony defect through which herniation of neural tissue may occur. Based on the location, a commonly accepted system classifies encephaloceles as occipital encephalocele and encephalocele of the cranial vault, which represent about 80% of all encephaloceles, and frontoethmoidal encephalocele and basal encephaloceles, collectively known as nasal encephaloceles [3]. The incidence of encephaloceles in western countries is 1 in 35,000 to 40,000 births [4].…”

The expanding role of the endonasal endoscopic approach in pituitary and skull base surgery: A 2014 perspective

“…To date, the fully endonasal endoscopic approach (EEA) has been described to access the anterior middle fossa through the cribriform plate,[ 47 54 ] the suprasellar cistern through the planum sphenoidale and tuberculum sellae,[ 35 54 87 ] the prepontine and premedullary cisterns through the clivus,[ 48 55 78 ] the ventral cervicomedullary junction,[ 53 74 ] Meckel's cave,[ 58 59 ] the middle cranial fossa,[ 57 ] the petrous apex,[ 90 ] the jugular foramen,[ 32 52 ] and the pterygopalatine[ 9 ] and infratemporal fossae. [ 6 59 ] Although benign tumors represent the majority of pathology addressed with the fully EEAs, malignant tumors,[ 6 58 ] congenital lesions,[ 74 85 ] inflammatory processes,[ 74 ] and vascular lesions[ 23 29 ] have been reported. The following review provides an update on the indications and outcomes of endoscopic endonasal skull base surgery with coverage of the most relevant and impactful articles from 2011 to 2014.…”