Abstract:Neuroglial heterotopias are rare congenital masses that are thought to represent encephaloceles that become sequestered on the extracranial side of the skull base. Although most often adjacent to bony skull base defects, they lack communication to the subarachnoid space. They contain mature neuroglial tissue and specialized central nervous system elements, such as a functioning choroid plexus. A case is presented of neonatal airway obstruction due to neuroglial heterotopia in the nasopharynx. The patient's cli… Show more
“…Dermoids, teratomas, and gliomas are uncommon lesions of the nasopharynx. 31,32 Nasopharyngeal teratoma can be associated with duplication of the adenohypophysis, as in our case. 33 The epignathus type of teratoma is a large neoplasm that fills the oral cavity and has been reported in association with a CPC.…”
Section: 1213supporting
confidence: 52%
“…Although there are reported cases of nasopharyngeal gliomas associated with midline skull base defects, none are explicitly associated with CPCs or extend intracranially as our cases did. 32 As in type 3 CPCs with cephalocele, identifying intracranial extension of a dermoid, teratoma, or glioma into a type 3 CPC is important for surgical planning to avoid inadvertent CSF leak and potential meningitis. Limitations of this series were its relatively small size and retrospective nature.…”
BACKGROUND AND PURPOSE:The craniopharyngeal canal is a rare, well-corticated defect through the midline of the sphenoid bone from the sellar floor to the anterosuperior nasopharyngeal roof. We reviewed a series of craniopharyngeal canals to determine a system of classification that might better our understanding of this entity, highlight the range of associated pathologic conditions, and optimize patient treatment.
“…Dermoids, teratomas, and gliomas are uncommon lesions of the nasopharynx. 31,32 Nasopharyngeal teratoma can be associated with duplication of the adenohypophysis, as in our case. 33 The epignathus type of teratoma is a large neoplasm that fills the oral cavity and has been reported in association with a CPC.…”
Section: 1213supporting
confidence: 52%
“…Although there are reported cases of nasopharyngeal gliomas associated with midline skull base defects, none are explicitly associated with CPCs or extend intracranially as our cases did. 32 As in type 3 CPCs with cephalocele, identifying intracranial extension of a dermoid, teratoma, or glioma into a type 3 CPC is important for surgical planning to avoid inadvertent CSF leak and potential meningitis. Limitations of this series were its relatively small size and retrospective nature.…”
BACKGROUND AND PURPOSE:The craniopharyngeal canal is a rare, well-corticated defect through the midline of the sphenoid bone from the sellar floor to the anterosuperior nasopharyngeal roof. We reviewed a series of craniopharyngeal canals to determine a system of classification that might better our understanding of this entity, highlight the range of associated pathologic conditions, and optimize patient treatment.
“…Neuroglial heterotopia is a rare lesion composed of differentiated neuroectodermal tissue without communication with the brain, spinal cord or meninges [1]. The commonest site of involvement is the nasal cavity while the orbit, middle ear, pharynx, palate, lips, tongue and neck have been reported [4].…”
“…In 1999, Gyure et al [1] defined the term ''intracranial extracerebral neuroglial heterotopia'' to describe a case with involvement of the middle and anterior cranial fossae. It should be noted that because of its benign pathological and clinical nature, this type of lesion is considered to be a developmental malformation rather than a true neoplasm [1,6]. IENHs are exceedingly rare lesions and, unlike meningoencephalocoeles, they do not have any connection with the subarachnoid space [1].…”
ABSTRACT. We report an unusual case of intracranial extracerebral neuroglial heterotopia (IENH) presenting with parapharyngeal extension. Neuroglial heterotopias are rarely reported pre-natally and, to our knowledge, there are no reported cases presenting pre-natal imaging findings of IENH with parapharyngeal extension. Because pre-and post-natal imaging studies are essential to diagnose IENH and plan surgical treatment, radiologists should be familiar with this rare condition. Neuroglial heterotopias (NHs) are rare congenital lesions characterised by the presence of differentiated neuroectodermal tissue in locations where neuronal and glial cells do not typically occur [1]. These lesions usually involve the head and neck regions and are more commonly described in extracranial locations such as the nose and nasopharynx, oral cavity, oropharynx, palate, tongue, lips, scalp and orbit. Although intracranial extracerebral NH has been described more frequently with the increased availability of MRI, it remains a very rare condition [2].Intracranial extracerebral neuroglial heterotopias (IENHs) with parapharyngeal or oropharyngeal extension have been presented in a limited number of reports [2,3]. To our knowledge, pre-natal imaging findings of this uncommon combination have not been reported before. We report a case of IENH with parapharyngeal extension and describe its antenatal and post-natal imaging features.
Case reportA 28-year-old pregnant female (gravida 3, para 2) was referred to our institution after an obstetric ultrasound examination at 25 weeks' gestation identified a foetal intracranial mass with extension to the left side of the neck. A repeat obstetric ultrasound performed in our department showed an osseous malformation in the left frontotemporal area of the cephalic pole resulting from a solid, round, predominantly hypoechoic intracranial mass (4.864.2 cm) with an anechoic area inside. This mass appeared to be contiguous with a predominantly cystic mass (6.765.9 cm) on the left side of the neck (Figure 1). A foetal MRI was performed at 27 weeks gestation; this showed a solid cystic mass lesion in the left anterolateral portion of the craniofacial region (Figure 2). Subsequent ultrasound evaluations showed that the mass had remained stable during pregnancy.The female infant was delivered pre-term by the ex utero intrapartum treatment procedure (EXIT) at 36 weeks gestation because of the risk of airway compression. Apgar scores at 1 min and 5 min were 9 and 10, respectively. The patient, who weighed 3045 g at birth, was admitted into the neonatal intensive care unit on mechanical ventilation. On clinical examination, the neonate had a large, soft, compressible cervical mass involving the frontotemporal region of the head.An MRI scan performed at the age of 1 day showed a large heterogeneous lesion with solid and cystic components. The solid component was an intracranial extracerebral lesion located in the left middle cranial fossa that presented a signal intensity similar to that of cerebral parenchyma...
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