- Department of Neurosurgery, Sina Hospital, Tehran University of Medical Science, Tehran, Iran
- Department of Oto-Rhino-Laryngology, Amir Alam Hospital, Tehran University of Medical Science, Tehran, Iran
Department of Neurosurgery, Sina Hospital, Tehran University of Medical Science, Tehran, Iran
DOI:10.4103/2152-7806.106260Copyright: © 2013 Sanjari R 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.
How to cite this article: Sanjari R, Mortazavi SA, Amiri RS, Samimi Ardestani SH, Amirjamshidi A. Intrasphenoidal Meningo-encephalocele: Report of two rare cases and review of literature. Surg Neurol Int 18-Jan-2013;4:5
How to cite this URL: Sanjari R, Mortazavi SA, Amiri RS, Samimi Ardestani SH, Amirjamshidi A. Intrasphenoidal Meningo-encephalocele: Report of two rare cases and review of literature. Surg Neurol Int 18-Jan-2013;4:5. Available from: http://sni.wpengine.com/surgicalint_articles/intrasphenoidal-meningo-encephalocele-report-of-two-rare-cases-and-review-of-literature/
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.
Keywords: Basal encephalocele, cerebrospinal fluid rhinorrhea, intra-sphenoidal encephalocele, lateral cranio-pharyngeal canal, trans-sellar meningo-encephalocele
Encephalocele may be of congenital, spontaneous, and traumatic origin.[
Trans-sphenoidal encephalocele can occur through a defect in the sella, planum sphenoidal, or posterior ethmoid sinus.[
We intend to report cases of a 15-year-old boy with large intra-sphenoidal meningocele and a 45-year-old female with laterally located sphenoidal meningomyelocel both presenting with CSF leakage and treated by endoscopic endonasal approach. Discussing the management of these cases might be informative for young neurosurgeons interested in the field of pediatric skull base surgery.
A 17-year-old boy was referred complaining from headache and intermittent CSF rhinorrhea of 2 years duration. In physical examination, hypertelorism, cleft lip, high arch palate, and see saw nystagmus were detected. Endocrinological examinations showed deficient growth hormone (GH), adrenocorticotropic hormone (ACTH), and luteinizing hormone (LH). Brain magnetic resonance imaging (MRI) revealed intrasphenoidal meningocystocele with infandibular recess descending into the meningocele and no other brain anomaly [
A 45-year-old female was admitted complaining of refractory headache and recent CSF rhinorrhea. She had no history of head trauma, meningitis or previous surgical operation. In physical exam, cranial nerves were intact and laboratory tests including hormonal studies were all normal. In MRI, the sella was enlarged and filled with CSF compatible with the pattern of empty sella [Figure
(a) T1W image showing CSF existing within the sella and extending into the sphenoid sinus through a possible defect located in the left lateral side of the sinus cavity, (b) and (c) contrast enhanced T1W images in horizontal and coronal views showing herniation of the glial tissue along with the arachnoidal pouch through the Sternberg's canal (white arrow), (d) and (e) T2W images in horizontal, coronal and sagittal views showing empty sella containing CSF and CSF containing cyst within the sella, (f) white arrow denoting extension of the glial tissue into the sella via the Sternberg's canal, (g) and (h) metrizamide CT cisternography in sagittal and coronal views showing CSF containing cavity both within the sella and the sphenoid cell
Incidence and review of literature
The incidence of congenital encephalocele is approximately 1 in 3000-5000 live births.[
There are multiple theories attempting to explain the formation of these basal encephalocele, among them, the incomplete closure of the neural tube leading to herniation of meninges and neural tissue and the persistence of the craniopharngeal canal.[
Incomplete fusion of the precursor of the greater wing of sphenoid, presphenoid and basi sphenoid can result a persistent channel termed the lateral craniopharyngeal canal of Sternberg.[
The clinical presentation of patients with trans-sphenoidal encephalocele is variable depending upon the age (i.e., the type described previously). Excluding the characteristic face of some of these patients with hypertelorism, the diagnosis can be delayed into adolescence or adulthood when an unexplained rhinorrhea, meningitis, endocrine dysfunction or progressive visual field deficit prompt evaluations leading in diagnosis. Both our cases presented with CSF leakage without history of trauma.
MRI is essential in evaluation of trans-sphenoidal encephalocele to confirm the extent of lesion, the possible associated abnormalities and to plan for the safest approach. MR angiography is helpful to evaluate intracranial vasculature.[
The indications for treatment and choice of surgical approach for trans-sphenoid encephaloceles remain controversial. The main indication for intervention are: Obstruction of respiratory pathway, repeated meningitis, rhinorrhea, and progressive visual defect attributable to the lesion.[
The management of the trans-sphenoidal encephalocele require a multidisciplinary approach.[
Repair of persistent CSF leakage is the major indication for surgery in intrasphenoidal encephalocele. Trans-sphenoidal or trans-ethmoidal approaches must be decided individually depending upon the exact location of the defect in skull base diagnosed by the precise preoperative imaging and the experience of the surgeon.[
Regarding the delicate points worthy to be considered in the technique of surgery, in median perisellar lesions such as our case 1, a trans-nasal, trans-meatal, or trans-oral trans-palatal route is safe. In encephalocele within the lateral recess of the sphenoid sinus, that is, coming through the Sternberg's canal, the endoscopic trans-pterygoid approach can be a better passage than the traditional tans-nasal and trans-ethmoid avenue.
In asymptomatic patients with true trans-sphenoidal encephalocele, or in patients with endocrine disorders or stable visual deficits, indications for operative repair are less clear. In symptomatic patients, the surgical approach must be tailored according to the individual patient's age, anatomical characteristics and associated anomalies.[
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