- Department of Neurosurgery, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India.
Bhanu Pratap Singh Chauhan, Department of Neurosurgery, GB Pant Institute of Postgraduate Medical Education and Research, New Delhi, India.
DOI:10.25259/SNI_341_2023Copyright: © 2023 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Bhanu Pratap Singh Chauhan, Binita Dholakia, Daljit Singh. Adult transsphenoidal meningoencephalocele: Clinical image. 23-Jun-2023;14:215
How to cite this URL: Bhanu Pratap Singh Chauhan, Binita Dholakia, Daljit Singh. Adult transsphenoidal meningoencephalocele: Clinical image. 23-Jun-2023;14:215. Available from: https://surgicalneurologyint.com/surgicalint-articles/12377/
Background: Encephalocele is herniation of cranial contents through a skull defect, classified according to their contents and location, and is usually seen in pediatric age group. The transsphenoidal type represents
Case Description: A 19-year-old female complaining of breathing difficulties during sleep and exertional dyspnea was diagnosed with a transsphenoidal meningoencephalocele, likely representing patent craniopharyngeal canal. On exploration through bifrontal craniotomy, the defect was identified in the sellar floor after completely reducing the contents into the cranial cavity and was repaired. She had immediate symptomatic relief and an uneventful postoperative course.
Conclusion: There can be significant symptomatic relief with minimal postoperative morbidity after transcranial repair of such large transsphenoidal meningoencephaloceles, through traditional skull base approaches.
Keywords: Adult presentation, Open repair, Transsphenoidal meningoencephalocele, Surgical repair
Encephalocele is herniation of cranial contents through a skull defect, classified according to their contents and location, and is usually seen in pediatric age group. The transsphenoidal type represents <5% of all basal meningoencephaloceles, with an incidence of one in 700,000 live births.[
A 19-year-old female student presented with complaints of difficulty in breathing during sleep since childhood, right vision loss (congenital cataract), and exertional dyspnea for 2 years. She was born prematurely at 7 months by cesarean section with history of neonatal intensive care unit stay. There were associated menstrual irregularities since 1 year. On general examination, she had frontal bossing, a short neck, telecanthus, and slanting palpebral fissures with normal higher mental functions. Visual examination showed that the right pupil was dilated, not reactive, with no perception of light, with the left eye best-corrected vision measured as 6/24. Chromosomal analysis, 2D echo study, and hormonal profile were normal.
She consulted an otolaryngologist, and on finding no significant clinical cause, a contrast-computed tomography (CT) scan was performed, which was suggestive of a well-defined corticated midline defect replacing the body of sphenoid measuring 2.4 × 1.6 cm. communicating with the nasopharynx and 3rd ventricle [
(a) Coronal section contrast computed tomography (CT) scan showing a nonenhancing isointense soft-tissue mass herniating into the sphenoid sinus reaching up to the floor. (b): Sagittal section bone window CT scan showing defect size of 2.4 cm in the sellar floor with herniating soft-tissue contents.
(a) Intraoperative microscopic view showing approximately 3 × 2 cm defect in the sellar floor with herniating meninges and pituitary stalk into the sphenoid sinus. (b) Intraoperative microscopic view showing defect (in oval) after complete reduction of contents into the cranial cavity. (c) The defect was repaired with pericranium, Surgicel, and glue (in oval).
Occurrence of cephaloceles is approximately one in every 3000–5000 live births without any gender predilection, with 30% incidence of associated anomalies.[
Based on the integrity of the sphenoid sinus floor, Jabre et al. noted two types of transsphenoidal meningoencephaloceles: the intrasphenoidal (extending into the sphenoid sinus but confined by its) and the true transsphenoidal (traversing the floor of the sphenoid sinus and protruding into the nasal cavity or nasopharynx).[
Treatment options vary from minimally invasive endoscopic transsphenoidal repair to open surgical exploration and repair. Endoscopic approaches are preferred for herniations into lateral sphenoid sinus, which include transnasal, transpterygoid, and transethmoid approaches.[
There can be significant symptomatic relief with minimal post operative morbidity after transcranial repair of such large transsphenoidal meningoencephaloceles, via traditional skull base approaches.
Patient’s consent not required as patient’s identity is not disclosed or compromised.
Publication of this article was made possible by the James I. and Carolyn R. Ausman Educational Foundation.
There are no conflicts of interest.
The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.
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