The most common presenting symptom was persistent hearing loss in 14 patients, followed by aural fullness and headache, which were present in 5 patients each. [jamanetwork.com]

Case presentation A 56-year-old Caucasian woman presented with an intermittent unilateral rhinorrhea on the right side for several months associated with chronic ipsilateral fronto-temporal headaches. [casereports.bmj.com]

She first presented with generalized tonic-clonic seizure. Her physical exam was notable for a palpable hard protuberance on her right frontal calvarium. [surgicalneurologyint.com]

Nasal, nasopharyngeal, or pharyngeal obstruction symptoms were not present, Valsalva and Furstenberg tests were negative, and externally visible mass was not present. No changes were found during nasal endoscopy. [hindawi.com]

Occipital encephaloceles present as masses overlying the occiput or posterior neck. [dermatologyadvisor.com]

• Asymptomatic

  Small temporal encephaloceles are increasingly recognized as causes of refractory epilepsy in some individuals 10 and are also encountered as asymptomatic sequelae of idiopathic intracranial hypertension. [radiopaedia.org]

  The transsphenoidal encephalocele generally is asymptomatic or an occasional finding, being the presentation most common for a mass located in the cranial line media (5). [arquivosdeorl.org.br]

  […] to reuse the content in many different ways. ear, nose and throat headache (including migraines) head and neck surgery Background The incidence of congenital skull base meningoencephaloceles is estimated to be 1 in 35 000 births and they are usually asymptomatic [casereports.bmj.com]

  However, they may sometimes be asymptomatic. In detail, most of frontoethmoidal ECs present as skin-covered masses or visible protrusions on the face, over the nose, glabella, or forehead. [entokey.com]

  Moreover, they may remain asymptomatic or be incidentally found at preoperative endoscopy or CT of the sinonasal region or temporal bone (1,4–6,10–12,27,30,41). [pubs.rsna.org]

• Epilepsy

  She has been following up with the neurology department as a case of epilepsy for 7 years. For three years, the patient's epilepsy was controlled with phenytoin 100 mg and Keppra 500 mg twice daily. [sciencerepository.org]

  Approach. 61 Sreenath SB...Sindwani R 33761785 2021 3 Nasoseptal Flap for Skull Base Reconstruction in a Three-Year-Old Child With Nasofrontal Meningoencephalocele. 61 Eden JK...Betz CS 33645258 2021 4 Potential Efficacy of Multimodal Mixed Reality in Epilepsy [malacards.org]

  Small temporal encephaloceles are increasingly recognized as causes of refractory epilepsy in some individuals 10 and are also encountered as asymptomatic sequelae of idiopathic intracranial hypertension. [radiopaedia.org]

  Epilepsy surgery for refractory epilepsy due to encephalocele: a case report and review of the literature. Epileptic Disord 2010; 12(2):160–166. Medline, Google Scholar 4 Gacek RR, Gacek MR, Tart R. [pubs.rsna.org]

  Neurological manifestations such as epilepsy, facial neuropathy, and aphasia are uncommon. [indianjotol.org]

• Surgical Procedure

  The manifestation of common pathologies of superior airways in this age group, united to the finding of a nasal endoscopic examination, without radiological complementation, can result in inadequate a surgical procedure in this case. [arquivosdeorl.org.br]

  Reconstruction defects in the forehead, nose, or brow often require multiple procedures as the child ages (Figure 4). Surgical resection of the extracranial portion alone is not advised. [dermatologyadvisor.com]

• Turkish

  Vazirnezami et al., “Transcranial approach for spontaneous csf rhinorrhea due to Sternberg's canal intrasphenoidal meningoencephalocele: case report and review of the literature,” Turkish Neurosurgery, vol. 22, no. 2, pp. 242–245, 2012. [hindawi.com]

• Rhinorrhea

  CSF Rhinorrhea and recurrent meningitis caused by transethmoidal meningoencephaloceles. Indian Pediatr. 2005;42(10):1033-1036. Habu M, Niiro M, Toyoshima M, Kawano Y, Matsune S, Arita K. [mediasphera.ru]

  […] a patient presenting with recurrent unilateral rhinorrhea and chronic headaches. [casereports.bmj.com]

  Cerebrospinal fluid (CSF) rhinorrhea is observed if there is any tear in the meninges, usually accompanied by increased intracranial pressure [1]. [cureus.com]

  She was instructed to observe for clear rhinorrhea, CSF leak precautions, and emergency visit instructions were given. [sciencerepository.org]

  The patient recalled intermittent watery rhinorrhea for 1 year preceding the first meningitis episode, but without head trauma. The anterior cranial fossa defect was repaired, without recurrence of rhinorrhea or meningitis in 2-year follow-up. [n.neurology.org]

• Nausea

  Bilateral osteodural defects in the median fossa floor in a 60-year-old man with a 7-year history of seizures characterized by an inexplicable feeling of nausea, sweating, and impaired awareness and followed by a brief period of confusion and difficulty [pubs.rsna.org]

• Suggestibility

  Lauren et al15 suggested that middle turbinate (MT) preservation is not appropriate for skull base defects located lateral to the MT attachment and in these cases, MT resection is necessary for a better exposure of the defect. [casereports.bmj.com]

  […] would allow to identify a specific aneuploidy but it was possible to verify that the numerical variation affected both the sex chromosomes and those of the first large acrocentric complement pair, as well as other remaining chromosomal elements, which suggests [go.gale.com]

  Two predominant hypotheses are currently suggested to explain ECs formation. Neurulation disorder: Congenital ECs share the common finding of a median skull defect, which corresponds to the site where the neural tube closes along the midline. [entokey.com]

  Both environmental and genetic factors have been seen to contribute to the cause of encephaloceles.[3] Some studies have revealed a higher occurrence in female embryos, suggesting a genetic cause.[4] Research has indicated that teratogens (substances [en.wikipedia.org]

• Hypertelorism

  Facial findings: Hypertelorism Elongation of face Nasal airway obstruction Dental malocclusion Associated intracranial abnormalities: Hydrocephalus Microcephaly Cerebral dysplasia Agenesis of corpus callosum Sometimes associated with other ocular anomalies [aao.org]

  The facial deformities are almost always present, and they found characteristics include hypertelorism, median nasal fissure, widened nasal base, cleft lip or palatal, syndrome of the cleft facial medial or bifid cranium occult frontal (8,9). [arquivosdeorl.org.br]

  The herniation was associated with cleft lip and palate, microphthalmia, hypertelorism, bilateral optic nerve hypoplasia, and severe bilateral deafness. [entokey.com]

  Meningoencephalocele is associated with facial anomalies including broad nasal root, hypertelorism, cleft lip, cleft palate, polydactyly, polycystic kidneys, ambiguous genitalia (features of Meckel-Gruber syndrome) [6], microcephaly, microagnathia and [cnjournal.biomedcentral.com]

  Since encephaloceles are caused by neural tube insufficiency in embryos, numerous ocular and craniofacial abnormalities can occur as associated anomalies, such as hypertelorism and cleft lip and palate [11]. [sciencerepository.org]

• Meningism

  All rights reserved. me·nin·go·en·ceph·a·lo·cele (mĕ-ning'gō-en-sef'ă-lō-sēl'), A protrusion of the meninges and brain through a congenital defect in the cranium, usually in the frontal or occipital region. [medical-dictionary.thefreedictionary.com]

  June 11, 2013; 80 (24) Resident and Fellow Section First published June 10, 2013, DOI: https://doi.org/10.1212/WNL.0b013e318296e8e0 A 34-year-old woman presented initially with meningococcal meningitis and with Streptococcus mitis meningitis 6 months [n.neurology.org]

  Acquired: history of craniofacial trauma, orbital or skull base surgery Clinical features Herniation of intracranial tissue into orbit: Meningocele: meninges Encephalocele: brain tissue (often disorganized or infarcted) Meningoencephalocele: meninges [aao.org]

  In the case that the meninges only protrude, the term meningocele is used [1]. Cerebrospinal fluid (CSF) rhinorrhea is observed if there is any tear in the meninges, usually accompanied by increased intracranial pressure [1]. [cureus.com]

  Meningioceles contain meninges alone, without actual brain tissue herniating through the defect. If both brain and meninges are contained in the mass, the term meningoencephalocele is used. [dermatologyadvisor.com]

Multidetector CT The use of CT to depict skull base defects has become a mainstay in the workup of patients with CSF fistulas because of its ability to depict osseous structures (4,6,10,14,24,27,33,38). [pubs.rsna.org]

• Occipital Encephalocele

  OUTCOME • Occipital encephalocele: The prognosis is better in occipital meningocele than in encephalocele. • Prognosis is worse if a cerebral tissue is present in the sac, if the ventricles extend into the sac, or if there is hydrocephalus. • Less than [fr.slideshare.net]

  encephalocele [en-sef´ah-lo-sēl″] hernial protrusion of brain substance and meninges through a congenital or traumatic opening of the skull. occipital encephalocele an encephalocele in the occipital region, the most common kind seen in the Western Hemisphere [medical-dictionary.thefreedictionary.com]

  Prognostic factors in patients with occipital Encephalocele. Pediatr Neurosurg. 2010;46:6–11. Nejat F, El-Khashab M, Andarabi Y. Progressive skin necrosis of a huge occipital encephalocele. Indian J Plast Surg. 2008;41:82–4. Agthong S, Wiwanitkit V. [cnjournal.biomedcentral.com]

  A fairly common classification still widely used is that first described by Suwanwela and Suwanwela in 1972 which divides the encephaloceles according to location 4: occipital encephalocele: ~75% of cases sincipital frontoethmoidal encephalocele nasoethmoidal [radiopaedia.org]

  Chapman PH, Swearingen B, Caviness VS: Subtorcular occipital encephaloceles: anatomic considerations relevant to operative management. J Neurosurg 1989;71:375–381. [karger.com]

Endoscopic treatment is highly successful. [cureus.com]

The treatment efficacy was 110 (78%) patients for primary surgery and 26 (84%) patients for repeated surgery. Conclusion. [mediasphera.ru]

Treatment of anterior basal MEC is still under development. [hindawi.com]

Treatment Options Medical Options (topical, systemic) Other than careful observation, there are no medical treatments to treat meningoceles. [dermatologyadvisor.com]

Preoperative MRI provides useful information to assess prognosis and plan the surgical management of occipital meningoencephalocele. [minervamedica.it]

OUTCOME • Occipital encephalocele: The prognosis is better in occipital meningocele than in encephalocele. • Prognosis is worse if a cerebral tissue is present in the sac, if the ventricles extend into the sac, or if there is hydrocephalus. • Less than [fr.slideshare.net]

The prognosis was related to the localization and the content of the cell sac, and to additional congenital anomalies. Nine children died and five are severely disabled. For 38 children the result is good, with no major disabilities. [pubmed.ncbi.nlm.nih.gov]

Several factors influence the prognosis of patients with Meningoencephalocele. The sac size and the amount of herniated brain tissue determines the prognosis. [cnjournal.biomedcentral.com]

They are most likely a variant form of growing skull fractures and may be the underlying etiology of many spontaneous intradiploic encephaloceles. [surgicalneurologyint.com]

It has multiple etiologies and is classified into congenital and acquired.[4] Improper development and ossification of petrosquamous junction were believed to be the reason behind congenital LTE, and it is less common than the acquired type. [indianjotol.org]

Learning points Spontaneous transethmoidal meningoencephaloceles are rare entities with no underlying clear etiologies. [casereports.bmj.com]

This case illustrates a rare etiology for CNS infections that should be considered in cases of recurrent meningitis. Dragos A. [n.neurology.org]

ETIOLOGY Two main theories*: •Arrested closure of normal confining tissue allows herniation through persistent defect •Early outgrowth of neural tissue prevents normal closure of cranial coverings *Greenberg M., 2016. [fr.slideshare.net]

Epidemiology of frontoethmoidal encephalomeningocoele in Burma. Journal of epidemiology and community health. 1984;38(2):89-98. Rifi L, Barkat A, Khamlichi AE, Boulaadas M, Ouahabi AE. [journals.aiac.org.au]

[…] conditions of bone, meninges, or sinuses (e.g., eosinophilic granuloma, natural killer T-cell lymphoma, cocaine abuse) Gorham-Stout syndrome ("disappearing bone disease") (Krohel, AJO 2002) Rare idiopathic osteolytic condition, often affects skull and face Epidemiology [aao.org]

Clinical and epidemiologic notes Approximately 20% of infants with encephalocele will have at least one additional unrelated major birth defect: Polydactyly and/or renal anomalies. May co-occur with Dandy-Walker malformation or Chiari malformation. [cdc.gov]

Mitchell LE (2005) Epidemiology of neural tube defects. Am J Med Genet C Semin Med Genet 135C: 88-94. [Crossref] 6. [sciencerepository.org]

[citation needed] Epidemiology[edit] Encephaloceles occur rarely, at a rate of one per 5,000 live births worldwide. [en.wikipedia.org]

Tirumandas M, Sharma A, Gbenimacho I, Shoja MM, Tubbs RS et al. (2013) Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst 29: 739-744. [Crossref] 7. [sciencerepository.org]

Gbenimacho et al., “Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications,” Child's Nervous System, vol. 29, no. 5, pp. 739–744, 2013. View at: Publisher Site | Google Scholar M. [hindawi.com]

Etiology Pathophysiology The key underlying event in the development of meningoceles, encephaloceles and gliomas is faulty closure of the anterior neuropore. [dermatologyadvisor.com]

Other theories on the pathophysiologic characteristics of spontaneous CSF fistulas have been proposed. [pubs.rsna.org]

ETIOLOGY Two main theories*: •Arrested closure of normal confining tissue allows herniation through persistent defect •Early outgrowth of neural tissue prevents normal closure of cranial coverings *Greenberg M., 2016. [fr.slideshare.net]

[citation needed] Proper levels of folic acid have been shown to help prevent such defects when taken before pregnancy, and early in pregnancy. [en.wikipedia.org]

British journal of preventive & social medicine. 1972;26(3):193-8. Bourgeois C, Olson L, Comer D, Evans H, Keschamras N, Cotton R, et al. Encephalopathy and fatty degeneration of the viscera: A clinicopathologic analysis of 40 cases. [journals.aiac.org.au]

Anaesthetic management of these children requires careful attention to positioning, airway management with careful securing of endotracheal tube to avoid accidental extubation, monitoring of the body temperature to prevent hypothermia, estimation of blood [asja.springeropen.com]

Suwanwela, “Geographical distribution of fronto-ethmoidal encephalomeningocele,” British Journal of Preventive & Social Medicine, vol. 26, no. 3, pp. 193–198, 1972. View at: Google Scholar A. Kubo, K. Sakata, J. Maegawa, and I. [hindawi.com]