Idiopathic intracranial hypertension presenting as bilateral spontaneous lateral intrasphenoidal and transethmoidal meningoceles: a case report and review of the literature

Radonjic et al. Journal of Medical Case Reports https://doi.org/10.1186/s13256-018-1959-6 (2019) 13:62 CASE REPORT Open Access Idiopathic intracranial hypertension presenting as bilateral spontaneous lateral intrasphenoidal and transethmoidal meningoceles: a case report and review of the literature Aleksandar Radonjic3, Abdul Mounem Kassab3, Ioana D. Moldovan1,4, Shaun Kilty2,3,4 and Fahad Alkherayf1,3,4* Abstract Background: Basal meningoceles are rare herniations of the meninges that tend to present unilaterally with cerebrospinal fluid rhinorrhea. Growing evidence suggests that intracranial hypertension contributes considerably to the formation of spontaneous basal meningoceles. Case presentation: A 50-year-old man of Middle East ethnicity presented with a 16-week history of cerebrospinal fluid rhinorrhea, short-term memory loss, and slight decline in cognitive function. We present a case of bilateral spontaneous meningoceles with bone defects in the left lateral sphenoid sinus and right anterior cribriform plate, as well as with a remodeled sella. A neuronavigation-assisted expanded endoscopic endonasal surgery was performed to resect the meningoceles. Postoperative imaging demonstrated complete resolution of the bilateral meningoceles. Conclusions: This case reports the first bilateral basal spontaneous meningoceles in the literature. Furthermore, based on this case’s imaging results and the literature reviewed, elevated intracranial pressure may be a determining factor behind the development of spontaneous meningoceles. Keywords: Bilateral, Spontaneous, Meningocele, Lateral intrasphenoidal, Transethmoidal, Expanded endoscopic, Endonasal, Surgery, Skull base, Case report Background Basal meningocele is a herniation of the meninges through a defect in the bone of the skull base. This disorder almost invariably presents with cerebrospinal fluid (CSF) rhinorrhea, and the clinical history may also include headache, vertigo, seizures, and meningitis [1]. The etiology behind spontaneous forms of this disorder has been debated; however, recent evidence points to increased intracranial pressure (ICP) as a driving cause [2]. Most spontaneous basal meningoceles present unilaterally with CSF rhinorrhea in adults. We present a case * Correspondence: 1 Division of Neurosurgery, Department of Surgery, The Ottawa Hospital, Civic Campus, 1053 Carling Avenue, Room C2218, Ottawa, Ontario K1Y 4E9, Canada 3 Faculty of Medicine, University of Ottawa, Ottawa, Canada Full list of author information is available at the end of the article of bilateral spontaneous left lateral intrasphenoidal and right transethmoidal meningoceles in a 50-year-old man. This is a rare finding in which two types of skull base lesions present concurrently in an adult patient with no previous history of nasal surgery or trauma. Case presentation A 50-year-old man of Middle East ethnicity presented with a 16-week history of CSF rhinorrhea, short-term memory loss, and slight decline in cognitive function. On physical examination, clear watery rhinorrhea, right-beating nystagmus, tongue deviation to the left side, mild facial asymmetry, multiple lipomas, bradycardia (52 beats/minute), and high blood pressure (194/118 mmHg) were detected. Laboratory tests results revealed presence of beta-2 transferrin in rhinorrhea fluid and © The Author(s). 2019 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Radonjic et al. Journal of Medical Case Reports (2019) 13:62 hypokalemia (3 mmol/L). There were no other abnormalities in his hematology (for example, blood count) and chemistry test results (for example, liver function and CSF analysis). His past medical history was significant for: hypertension; Dercum’s disease; right internal carotid dissection with pseudoaneurysm formation which was stable and conservatively treated, and followed with imaging; chronic compensated noncommunicating hydrocephalus secondary to obstruction at aqueduct of Sylvius, and a one-time seizure episode. Computed tomography (CT) showed bony defects in his left lateral sphenoid sinus and right anterior cribriform plate (Fig. 1). CT cisternography revealed adjacent meningocele to the aforementioned defects with pooling of intrathecal contrast, confirming herniation into the left lateral sphenoid and right anterior ethmoid air cells. Magnetic resonance imaging (MRI) demonstrated a 2.9 × 1.8 × 1.8 cm right anterior meningocele traversing the anterior cribriform plate inferiorly into anterior ethmoid air cells and nasopharynx with extension into the right maxillary sinus (Fig. 2a). Another contrast extension from the left middle cranial fossa along its most anterior aspect into the most lateral aspect of the sphenoid sinus was identified suggesting a second meningocele measuring 1 × 1 × 0.9 cm (Fig. 2b). Both lesions were enhanced with gadolinium but no brain parenchyma could be identified within the sacs. Other findings on MRI included a significantly flattened pituitary gland within a remodeled sella and a slightly dilated ventricular system. He underwent neuronavigation-assisted expanded endoscopic endonasal surgery with resection of the anterior skull base meningoceles. The first lesion was right ethmoidal and the second lesion was left sphenoidal. Repair of the dura was carried out with two layers of dural matrix. Insertion of a lumbar drain was done to drain CSF and for injection of fluorescein to help confirm Page 2 of 6 dural seal. Opening pressure upon insertion of the lumbar drain at the time of surgery was 20 mmHg. Septal and anterior ethmoidal flaps were used to support the repair of the sphenoid and anterior ethmoidal lesions, respectively. He recovered uneventfully and postoperative imaging showed complete resolution of the meningoceles bilaterally (Fig. 3). Four weeks after the surgery, he presented to our clinic with CSF leak and headache. MRI revealed evidence of CSF leak noted within the left sphenoid sinus. He underwent an endoscopic repair of the CSF leak and insertion of a ventriculoperitoneal shunt. Postoperation, he recovered well and presented no symptoms. He had 3-year follow up with no recurrence of the meningoceles. Discussion We presented a rare case of a 50-year-old man with bilateral spontaneous lateral intrasphenoidal and transethmoidal meningoceles with nasal herniation and CSF rhinorrhea, associated with a significantly flattened pituitary gland within a remodeled sella. Nasal (...truncated)