Trans-Sellar Trans-Sphenoidal Herniation of Third Ventricle with Cleft Palate and Microophthalmia: Report of a Case and Review of Literature

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Abstract

Trans -sellar trans-sphenoidal encephalocele is an extremely rare entity. We present the case of an 18-month old boy who presented with a trans-sellar, trans-sphenoidal encephalocele associated with cleft lip, cleft palate and microphthalmia. This patient was treated successfully by a trans-cranial extra-dural route. In this paper, we discuss the clinico-radiological findings as well as various surgical options in managing these rare lesions and briefly review the literature.

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Sternberg's canal as a cause of encephalocele within the lateral recess of the sphenoid sinus: A report of two cases

Damián Bendersky, Federico Landriel, Pablo Ajler … (2011)

Background: Intrasphenoidal encephaloceles are extremely rare findings. Sternberg's canal is a lateral craniopharyngeal canal resulting from incomplete fusion of the greater wings of the sphenoid bone with the basisphenoid. It acts as a weak spot of the skull base, which may lead to develop a temporal lobe encephalocele protruding into the lateral recess of the sphenoid sinus (SS). Case Description: We present two cases of intrasphenoidal encephalocele due to persistence of the lateral craniopharyngeal canal. The first case presented with cerebrospinal fluid (CSF) rhinorrhea and the second one was referred to the neurosurgical department with CSF rhinorrhea and meningitis. Radiological investigations consisted of computed tomography (CT) scan, CT cisternography and magnetic resonance images in both cases. These imaging studies identified a herniated temporal lobe through a bony defect which communicates the middle cranial fossa with the lateral recess of the SS. Both patients underwent a transcranial repair of the encephalocele because of the previous failure of the endoscopic surgery. There was no complication related to the surgical procedure and no recurrence of CSF leakage occurred 2 and 3 years after surgery, respectively. Conclusion: Encephalocele within the lateral recess of the SS is a rare entity which must be suspected in patients who present with spontaneous CSF rhinorrhea. Congenital intrasphenoidal encephaloceles, which are located medial to the foramen rotundum, seem to be due to persistence of the Sternberg's canal. Transcranial approach is a good option when a transnasal approach had failed previously.

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Intrasphenoidal encephaloceles--a clinical entity.

M Buchfelder, P Thierauf, R Fahlbusch … (1986)

In the current classification basal encephaloceles are grouped together with real transsphenoidal encephaloceles. But those encephaloceles extending only into but not through the sphenoid sinus seem to represent a specific clinical entity and therefore should be regarded as a rare subgroup of sphenoidal encephaloceles. One personal case and six cases from the literature are reviewed, the own case being associated with an empty sella turcica. The initial sign is rhinorrhea, almost invariably. The association with other intracranial anomalies is uncommon. The extradural transsphenoidal or transethmoidal midline approach accompanied by a shunting procedure today is the most suitable method of surgical treatment.

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Transsphenoidal meningoencephalocele.

Maria Iannelli, F Formica, G. PALUDETTI … (2002)

Encephaloceles are rare cranio-facial developmental anomalies. In this paper, we discuss the case of an 8-month-old girl affected by a transsphenoidal meningoencephalocele and try to give landmarks of the state of art in the surgical treatment of this rare pathology.

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Author and article information

Journal

Journal ID (nlm-ta): Asian J Neurosurg

Journal ID (iso-abbrev): Asian J Neurosurg

Journal ID (publisher-id): AJNS

Title: Asian Journal of Neurosurgery

Publisher: Medknow Publications & Media Pvt Ltd (India )

ISSN (Print): 1793-5482

ISSN (Electronic): 2248-9614

Publication date (Print): Jul-Sep 2018

Volume: 13

Issue: 3

Pages: 782-785

Affiliations

[1] Department of Neurosurgery, SGPGIMS, Lucknow, Uttar Pradesh, India

Author notes

Address for correspondence: Dr. Sanjay Behari, Department of Neurosurgery, SGPGIMS, Raibareilly Road, Lucknow - 226 014, Uttar Pradesh, India. E-mail: sbehari27@ 123456yahoo.com

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Publisher ID: AJNS-13-782

DOI: 10.4103/1793-5482.238003

PMC ID: 6159010

PubMed ID: 30283546

SO-VID: d7522cc0-4251-44ba-981b-9e0947ccba1c

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Trans-Sellar Trans-Sphenoidal Herniation of Third Ventricle with Cleft Palate and Microophthalmia: Report of a Case and Review of Literature

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Most cited references 21

Sternberg's canal as a cause of encephalocele within the lateral recess of the sphenoid sinus: A report of two cases

Intrasphenoidal encephaloceles--a clinical entity.

Transsphenoidal meningoencephalocele.

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