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Paramedian frontal encephalocele. (a) (T2-weighted sagittal) and (b) (T2-weighted axial) images show herniation of dysplastic brain (star) through the frontal bone on the right side. The herniated content is located in a paramedian location which is an unusual position for the sincipital encephalocele
Source publication
Background
Encephaloceles refer to the herniation of intracranial contents through the defect in the dura and calvarium. This article aims to equip the radiologists with all the necessary information to approach a case of encephalocele in a systematic manner and report in an organized format. Thus reduce the reporting time without overlooking any d...
Contexts in source publication
Context 1
... encephaloceles are often seen in the midline in the inferior part of the metopic suture; however, in rarer events it can be paramedian in location [9,11] (Fig. 2). Most frontoethmoidal encephaloceles occur through the foramen cecum located anterior to crista galli. Frontoethmoidal encephaloceles are further subdivided into nasofrontal, nasoethmoidal, and nasoorbital types based on their relationship with nasal bone and the nasal cartilage [9,12]. Nasofrontal encephalocele occurs through ...
Context 2
... isointense to the cortex on T1W and T2W sequences or may have high signal intensity on T2W sequences as a result of gliosis. An encephalocele might also show a similar imaging picture; however, there will be definite evidence of intracranial extension demonstrated by widened foramen cecum/fonticulus frontalis and eroded/widened crista galli (Fig. 12). The basic embryology of encephalocele and nasal gliomas is the same; hence, nasal gliomas may also be associated with widened foramen cecum/fonticulus frontalis and eroded/ widened crista galli. In such scenarios, heavily T2-weighted images or intrathecal contrast will show communication of the encephalocele with the subarachnoid ...
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Citations
... Anatomically, meningoencephaloceles are classified into four types-parietal, occipital, basal, and sincipital types. Sincipital type is a synonym used for the frontoethmoidal type of meningoencephalocele. Imaging plays an important role in diagnosing and planning the management of meningoencephalocele [3]. ...
... Anatomical classification divides the meningoencephalocele into four major groups: parietal, occipital, sincipital, and basal types [3]. Sincipital meningoencephalocele, a/k/a fronto-ethmoidal meningoencephalocele, is a descriptive term used to describe a bony defect at the level of foramen cecum, where the ethmoid and frontal bones meet. ...
Background
Fronto-ethmoidal meningoencephalocele is an uncommon pathology. Most of the cases present a history of trauma or paranasal surgical intervention. Spontaneous development of such pathology is extremely rare. The aim of this case report was to demonstrate a rare appearance of spontaneous fronto-ethmoidal meningoencephalocele in an adult male with high-quality images in multiple modalities used in radiology.
Case presentation
This case report demonstrates an extremely rare pathology of spontaneous development of fronto-ethmoidal meningoencephalocele in a 50-year-old male. The patient complained of swelling involving the nasal bridge, the left side of the nose, and the left cheek for more than 20 years, which was spontaneous without any trauma or surgical intervention. The patient underwent radiological investigations of ultrasonography, computed tomography of the paranasal sinus, and contrast-enhanced magnetic resonance imaging of the brain. The diagnosis of spontaneous development of left fronto-ethmoidal meningoencephalocele abutting the left orbit was made. No other complications of the brain tissue were noted.
Conclusions
Knowledge about etiopathogenesis and various types of meningoencephalocele helps manage this pathology.
... The most accepted theory in the development of frontoethmoidal is an incomplete joining of the frontal and ethmoid bones. By extension, frontonasal encephaloceles are attributed to a defect in the joining of the nasal bones inferiorly to the frontal bone [3] . This typically allows the brain to herniate through the fonticulus nasofrontalis, creating a mass located in the forehead and nasal bridge. ...
An encephalocele is a cranial defect that allows brain matter to present in other portions of the skull. We present a case of a 57-year-old female who presented to the ICU with fever and unresponsiveness and was diagnosed with meningitis, bacteremia, and pneumonia. After diagnostic imaging was performed, a nasofrontal encephalocele was found and corrected via surgical intervention. The patient's nasofrontal encephalocele caused a predisposition for opportunistic infections due to the exposed and unprotected brain matter, which caused a simple case of sinusitis to turn into a life-threatening case of meningitis and bacteremia.
... Encephalocele is a congenital NTD in which a sac containing the brain, meninges, and CSF develops outside the skull due to a defect [1,2]. Anterior encephalocele are more common in Africa and Russia, with one case occurring in every 3500 to 6000 live births [3]. ...
... An encephalocele should be surgically corrected as soon as possible to avoid deleterious effects [2,6]. The objectives are to repair the bone defect with a water-tight dural closure, remove excess skin, and remove non-functional brain tissue [1]. ...
Introduction and importance:
Encephalocele is an NTD that affects one in every 10,000 live births. A ruptured encephalocele is advised to be operated on as soon as possible, preferably within 48 h. Signs and symptoms of infection should be considered when performing corrective surgery.
Case presentation:
We present the case of an 8-day-old baby who had a ruptured midline frontoparietal encephalocele as a result of delayed corrective surgery. The mass on the patient's head measured approximately 7 × 6 × 5 cm with a leakage of clear and yellowish fluids. The head CT scan revealed a multi-enhancement mass, pedunculated with an ill-defined border protruding from the anterior fontanelle.
Clinical discussion:
The patient underwent an emergency corrective surgery. Excision was performed completely. The defect was covered with a double-layer closure technique in a watertight manner. The patient's postoperative recovery was uneventful.
Conclusion:
An encephalocele can only be resolved through corrective surgery. In our case, the ruptured midline encephalocele was easily resolved with an emergency corrective surgery. Closure of the defect soon after birth is advised, especially if no layer of skin protects the encephalocele.
... Por outro lado, o motivo de estruturas neurais bem definidas estarem no interior do saco herniário mesmo sem o fechamento do tubo neural ainda não é bem explicada, já que o tecido cerebral se forma somente após o período de fechamento do tubo neural. Diante disso, muitos autores apontam que a encefalocele seja ocasionada por um evento pós-neurulação (Pal et al., 2021). Embora diversos casos de encephaloceles Frontoethmoidal tenham sido descritos desde o século XVI, não se tem muito conhecimento acerca dos mecanismos exatos que estão envolvidos no desenvolvimento dessa doença, o que se sabe é que não estão associados a quadros de mortalidade em sua grande maioria. ...
... Além disso, a RM pode ajudar a guiar o planejamento e prognóstico cirúrgico. A tomografia computadorizada também pode ser útil, principalmente em relação ao estudo ósseo, em destaque aos casos incipitais (Pal et al., 2021). ...
A encefalocele é uma doença de característica congênita, quando relacionada a um defeito no tubo neural e formação de sacos herniários que variam de composição e se formam externamente ao crânio por consequência a algum defeito ósseo ou adquirida, em decorrência de traumas, tumores e lesões iatrogênicas. O seu tipo varia de acordo com a localização das alterações. A etiologia dessa patologia ainda não é muito bem esclarecida. Tendo em vista a escassez de estudos atualizados em torno dessa temática, esse trabalho trata-se de uma revisão de literatura que tem como objetivo contribuir, com base em uma revisão de literatura, reunindo os principais achados dessa patologia, integrando aspectos clínicos, radiológicos e terapêuticos. Foi realizado uma revisão narrativa de literatura nos meses de março e abril de 2023, por meio de pesquisa em base de dados: PubMed, SciELO e Google Acadêmico. Os descritores utilizados foram Frontoethmoidal encephaloceles, Frontoethmoidal encephaloceles diagnosis e Frontoethmoidal encephaloceles radiology filtrados nos últimos 10 anos. O seu diagnóstico pode ser feito através de métodos como RM em sequências ponderadas em T1 e T2 em planos sagital, axial, coronal e FLAIR, além da TC. Quadros de encefalocele anterior podem ainda cursar com obstrução nasal, fenda nasal, rinorreia de LCR, telecanto e anomalia orbitária. Portanto, ao analisarmos as manifestações encontradas na encefalocele frontoetmoidal, podemos inferir que é de suma importância reconhecer os sinais clínicos dessa patologia, bem como incentivar novos estudos para esclarecer o assunto em questão.
... An encephalocele is a congenital herniation of intracranial contents throughout a cranial defect. These intracranial contents, which protrude through the defect, may include meninges, cerebrospinal fluid (CSF), and/or brain tissue [1,2]. ...
... The etiology of congenital encephaloceles is not fully understood but many risk factors may be associated such as genetic factor, environmental factors like maternal nutrition deficiencies such as folic acid deficiencies [2]. ...
... Encephaloceles are considered to be a spectrum of neural tube defects caused due to failure of neurulation [2,6]. ...
Background and objective
Encephaloceles are considered to be a spectrum of neural tube defects with a herniation of the brain and the meninges through a bony skull defect to the exterior.
Methods
This retrospective and prospective study was carried out on 30 patients with congenital encephaloceles either vault or basal. The risk factors and the prognostic factors were assessed.
Results
There was a statistically significant association between the content of the sac, outcome and seizures. There was a presence of neural tissue associated with worse outcome and seizures. There was a statistically significant association between the size of the sac and outcome with seizure. There was a statistically insignificant association between site of defect and parent consanguinity, folic acid intake, drug history, seizures, outcome, gender, family history and a statistically significant association between the site of the sac and hydrocephalus. There was a statistically significant association between the outcome and presence of Hydrocephalus, microcephaly and seizures.
Conclusions
There was statistically significant association between the worst outcome and presence of hydrocephalus, microcephaly and seizures, and there is correlation between site of the defect in occipitocervical region and development of hydrocephalus.
... 8 Sementara prognosis dari kelainan ini bergantung pada ada tidaknya jaringan otak dalam kantong cele, ukuran dan letak kantong cele, kelainan bawaan penyerta (hidrosefalus, mikrosefali, infeksi) dan resektabilitas jaringan intrakranial dalam kantong cele tersebut. 8,9 Umumnya, frontoethmoidal meningoencephalocele memiliki prognosis yang lebih baik dibandingkan dengan occipital meningoencephalocele. 2 Berbeda dengan teori, pada kasus ini ditemukan meningoencephalocele pada regio oksipital meskipun pasien berjenis kelamin laki-laki dan berasal dari Asia Tenggara. ...
... Karena seringkali ditemukan pada pasien dengan keluarga menderita keluhan yang sama, latar belakang genetik dianggap berperan, namun hingga saat ini belum diketahui gen yang bertanggung jawab terhadap kelainan ini. 2 Meningoencephalocele dapat didiagnosa sejak periode antenatal menggunakan USG. 9 Beberapa modalitas lain yang dapat digunakan untuk mendeteksi kelainan ini antara lain CT-scan kepala, magnetic resonance imaging (MRI) kepala maupun magnetic resonance angiography (MRA). 2 Dari ketiga modalitas tersebut, MRI merupakan pilihan utama dalam penegakan diagnosis encephalocele. 8 Waktu yang paling tepat untuk mendeteksi kelainan ini adalah pada usia 10 minggu saat osifikasi tulang kranium mulai terbentuk. ...
... Setelah defek ditemukan, disarankan untuk melakukan pemeriksaan serial untuk mengetahui resolusi atau progresifitas defek. 9 Pada kasus ini, tidak ditemukan kelainan bawaan selain selain mikrosefali. Riwayat keluarga dengan keluhan yang sama juga disangkal. ...
Meningoencephalocele is a rare congenital abnormality where duramater and cranium failed to close completely, leaving part of intracranial tissues outside the cranium wrapped in a sac. Neonate’s airway management, pose many challenges due to immaturity of airway anatomy, limited respiratory organ reserve and extreme head-to-body ratios. Occipital meningoencephalocele will also complicate the intubation and airway control during anesthesia and surgery. A nine days old male patient with an occipital lump measuring 17x12x8 cm underwent meningoencephalocele resection. Induction of anesthesia was performed by sevoflurane inhalation, rocuronium to facilitate videolaryngoscopy and intubation in supine position, supported by doughnut-shape pads which fits the size of the cele. Videolaryngoscope was chosen because it provides optimal visualization without hyperextension and over manipulation than conventional laryngoscope, therefore intubation can be done in supine position. Anesthesia and surgery duration was 2 hours and 15 minutes. The patient was treated in the neonatal intensive care unit after surgery, extubated 24 hours later, and discharged home 10 days after surgery. The greatest challenge in anesthesia management for occipital meningoencephalocele resection is securing the airway while ensuring the sac is not ruptured during airway manipulation and positional changes during anesthesia and surgery.
... Encephalocele is defined as the herniation of brain tissue through the defect in the cranial bone. The incidence rate is 1 in every 4000 births [1]. Occipital encephalocele occurs between lambda and foramen magnum, and is divided into supra-and infratorcular types [2]. ...
... Occipital encephalocele occurs between lambda and foramen magnum, and is divided into supra-and infratorcular types [2]. The prognosis of the lesion depends on the size of the calvarial defect and the herniated sac, presence of hydrocephalus (more common in posterior cephalocele due to torsion of aqueduct of Sylvius), the status of the venous sinuses, and other associated congenital brain malformations [1]. Encephaloceles are usually managed electively; however, in case of rupture, immediate surgical repair is done as exposure of meninges and brain carries the risk of central nervous system infection [3]. ...
... The frontoethmoidal variety occurs through the foramen cecum, that is located anterior to the crista galli. The frontoethmoidal encephaloceles are subcategorized into nasofrontal, nasoethmoidal and nasoorbital types [12]. ...
Background
Encephaloceles are herniation of brain parenchyma through the defect in the dura and the skull bones. This case report reveals a rare association of frontonasal encephalocele, subependymal nodular heterotopias and cerebellar dysplasia with review of literature.
Case presentation
Frontonasal encephalocele is a rare entity. We report an 18-months-old male child who presented to the department of pediatrics with chief complaint of congenital swelling in the region of forehead. Swelling was increasing in size as told by the parents of the child. The child was born full term with normal vaginal delivery. There was no history of any obstetrical complications. The swelling was soft, transilluminant and pulsatile in nature. He was referred to the department of radio diagnosis for imaging evaluation. His CT and MRI images revealed defect in the anterior cranial fossa with herniation of meninges and brain parenchyma through it. There was also evidence of subependymal nodular heterotopias in this patient along with hypoplasia of left cerebellar hemisphere and left middle cerebellar peduncle with left cerebellar dysplasia.
Conclusions
This case report describes the coexistence of frontonasal encephaloceles, subependymal nodular heterotopias and cerebellar dysplasia, which is a very rare association.
Encephalocele is a rare and serious deformity that occurs by a protrusion of neural tissue (usually like a sac) through a bony defect in the skull, there are two types congenital (primary) and acquired (secondary) encephaloceles, congenital encephalocele belong to the neural tube defect (NTDs) along with spina bifida and anencephaly. The exact cause of congenital encephalocele is unknown, probably, it is multifactorial and there are roles for genetic and environmental factors, failure of closure of the neural tube during early pregnancy (third to fourth week) is thought the pathogenesis process of congenital encephalocele. According to the contents herniated sac, encephalocele is divided into many subtypes including meningocele, men-ingo-encephalocele, atretic encephalocele, and gliocele. The main classification of encephalocele includes four major types according to the location: occipital, Sincipital, basal, and convexity. The most common type of them depends on geographical region. Usually, encephalocele is present at birth as a visible mass but it may be occult like some cases of basal encephalocele. Ultrasound is the main diagnostic tool for prenatal diagnosis of encephalocele in early pregnancy during the first trimester. MRI plays a major role in diagnosis after birth as MRI demonstrates the contents of the herniated sac and aids in making the surgical decision. in most cases, surgery is the appropriate choice and it is an emergency if there is a risk of meningitis like CSF leakage. Hydrocephalus is the most common complication associated with encephalocele and it should be resolved before the surgery. Morbidity and mortality of encephaloceles have improved in recent years.
Encephalocele is a rare congenital anomaly characterized by the protrusion of intracranial contents through a defect in the skull base or calvarial. In Southeast Asia, frontoethmoidal encephaloceles are more frequently observed compared to their occurrence in Western countries. Typically, frontoethmoidal encephaloceles present as a visible mass adjacent to the nasal region. In this report, we delineate the case of a 9-year-old boy who presented with a palpable mass on the nasal bridge. Subsequent ultrasound and CT scan evaluations identified a nasofrontal defect with a communicating connection to the intracranial compartment.
