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]

• Soft Tissue Mass

  Figure 1: Endoscopic picture of right ear, showing lobulated pulsatile mass occupied whole external canal Click here to view Figure 2: High-resolution computed tomography (axial) revealed a postoperative mastoid bowl with soft-tissue mass on the right [indianjotol.org]

  To better visualize the soft tissue mass, and its contents and boundaries, high-resolution computed tomography (CT), or cone beam CT, in conjunction with magnetic resonance (MR) is recommended in almost all the cases, considering the complementarity of [entokey.com]

  Because the contents of the soft-tissue mass could not be defined at CT, MR cisternography was recommended. [pubs.rsna.org]

• Crying

  Convexity Frontal Parietal Temporal Occipital Neurofibromatosis type 1 (NF1): sphenoid greater wing Fracture or surgery: orbital roof and/or medial wall Orbital mass effect causes proptosis and/or downward/medial displacement of globe: Straining and crying [aao.org]

  The baby had a spontaneous cry at birth with Apgar score of 9 and 9 at 1 and 5 min, respectively. His birth weight was 1650 g (10th–25th percentile). [casereports.bmj.com]

  The neonate was not irritable, and his cry was normal. Preanaesthetic evaluation was thoroughly carried out. [asja.springeropen.com]

• Chronic Infection

  Mealey J Jr Chronic cerebrospinal fluid otorrhea: report of a case associated with chronic infection of the ear. Neurology 1961;11996- 998PubMedGoogle ScholarCrossref 14. [jamanetwork.com]

• Malocclusion

  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]

• Strabismus

  Fracture or surgery: orbital roof and/or medial wall Orbital mass effect causes proptosis and/or downward/medial displacement of globe: Straining and crying might result in increased size of mass Pulsation of globe or mass Vision loss might result from strabismus [aao.org]

• Fracture

  where low-velocity blunt trauma only fractures the inner table, as opposed to a growing skull fracture in which both the inner and outer tables are disrupted.[ 11 12 ] The fracture of the inner table results in a dural tear; as the skull recoils, a [surgicalneurologyint.com]

  Agenesis of corpus callosum, Dandy-Walker malformation, hydrocephalus, holoprosencephaly (Bersani, OPRS 2006) Traumatic skull base or orbital roof fracture Relatively small (2–4 mm) roof fractures can cause delayed encephaloceles in children (Cayli, Pediatr [aao.org]

  Other considerations included a fracture of the cribriform plate with subsequent brain herniation or a CSF fistula. This case illustrates a rare etiology for CNS infections that should be considered in cases of recurrent meningitis. Dragos A. [n.neurology.org]

  Acquired causes include iatrogenic injury from skull base surgeries, erosion from skull base tumors, traumatic fracture, and radiation.[3] Nonspecificity of the clinical signs and symptoms of this situation often lead to delay in diagnosis. [indianjotol.org]

  Traumatic CSF fistulas are the most common type (accounting for 80%–90% of all cases) of acquired CSF fistula, with most cases secondary to a skull base fracture (1,6,10–14). [pubs.rsna.org]

• Ear Discharge

  She was well and discharged at day 2 after surgery. [indianjotol.org]

• Ear Fullness

  Case Report A 24-year-old female was referred to our otolaryngology clinic from another private hospital, presented with progressive right ear fullness for 6 months. It was associated with an intermittent gush of clear fluid. [indianjotol.org]

• Vertigo

  She denied vertigo, purulent ear discharge. On clinical examination, she was comfortable with intact cranial nerves. Nystagmus and fistula test were all absent. There were no signs of meningitis. [indianjotol.org]

  Right temporal meningoencephalocele in a 59-year-old man with long-standing idiopathic facial palsy and intermittent vertigo. [pubs.rsna.org]

  She denied headaches, fevers, vertigo, otalgia, and tinnitus and she had no history of meningitis, trauma, or prior cranial surgery. [jamanetwork.com]

• Aphasia

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

  Keywords: Intradiploic, meningoencephalocele, trauma INTRODUCTION In 1976, Kosnik et al. published the first report of an intradiploic encephalocele.[ 6 ] The patient was a 57-year-old man who presented with generalized seizures and expressive aphasia [surgicalneurologyint.com]

  Other less common presenting symptoms include vertigo, pulsatile tinnitus, facial nerve paresis, temporal lobe epilepsy, and aphasia (a result of traction or herniation of the involved cortex) (3,14,17,25–27,30,41,44,45). [pubs.rsna.org]

• Stroke

  The mother had a history of chronic hypertension, type 1 diabetes mellitus, stroke and asthma. Her medications included enoxaparin, insulin, nifedipine, methyldopa, inhaled albuterol and inhaled corticosteroids. She received regular prenatal care. [casereports.bmj.com]

  […] associated with craniofacial abnormalities, optic nerve atrophy, coloboma, and megalopapilla. 28 Moyamoya disease is a progressive, steno-occlusive arteriopathy involving the internal carotid artery terminus and its branches that can cause recurrent strokes [entokey.com]

• Irritability

  Merriam-Webster unabridged Odd Habits and Quirks Which of the following best describes an easily irritated person? Test your knowledge - and maybe learn something along the way. TAKE THE QUIZ [merriam-webster.com]

  The neonate was not irritable, and his cry was normal. Preanaesthetic evaluation was thoroughly carried out. [asja.springeropen.com]

• Nystagmus

  Nystagmus and fistula test were all absent. There were no signs of meningitis. [indianjotol.org]

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]

• Polyps

  The main entities to consider are the encephalocele, dermoid cyst, glioma, and polyp. Defining characteristics are listed in Table I, but there remains overlap. Table I. [dermatologyadvisor.com]

  The fiberoptic endoscopic examination of the nose showed a normal appearance of the nasal mucosa, mild and non-obstructive septal deviation, no evidence of intranasal mass or polyps. [casereports.bmj.com]

  It may be difficult to recognize in an operated ear by junior doctors as it could mimic polyp or granulation tissues arise deep within from an infected mastoid bowl. [indianjotol.org]

  Nerve and nasal septum cartilage may also be present. 8 Differential diagnosis should include mainly nasal polyps, nasal gliomas, or dermoid cysts. 39 A special mention has to be made for these latter two entities, since they are supposed to share the [entokey.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]