Abstract:Heterotopic neuroglial tissue must be considered in the differential diagnosis of airway obstruction in the newborn. Management is surgical resection, with attention to vital structures and function-analogous to surgery for lymphangioma. Multiple surgical procedures might be necessary in the treatment of these patients.
“…The high soft tissue resolution of MRI enables the specific characterisation of size and extent of the mass and any displacement of adjacent structures. In all pulse sequences, NH has a signal intensity that is similar to normal brain tissue [4]. The vascularity of the lesion may affect the degree of contrast enhancement noted.…”
Section: Discussionmentioning
confidence: 99%
“…Since Reid first reported a case of NH in 1852 [4], several terms have been used to describe this type of lesion, including glioneural or neuroglial choristoma, glioneural hamartoma, brain heterotopia, heterotopic brain tissue and ectopic brain, among others [5]. The term choristoma is used synonymously with heterotopia to define histologically normal tissue occurring in an abnormal location [5].…”
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...
“…The high soft tissue resolution of MRI enables the specific characterisation of size and extent of the mass and any displacement of adjacent structures. In all pulse sequences, NH has a signal intensity that is similar to normal brain tissue [4]. The vascularity of the lesion may affect the degree of contrast enhancement noted.…”
Section: Discussionmentioning
confidence: 99%
“…Since Reid first reported a case of NH in 1852 [4], several terms have been used to describe this type of lesion, including glioneural or neuroglial choristoma, glioneural hamartoma, brain heterotopia, heterotopic brain tissue and ectopic brain, among others [5]. The term choristoma is used synonymously with heterotopia to define histologically normal tissue occurring in an abnormal location [5].…”
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...
“…It remains rare, with reports on neuroglial heterotopic tissue in the scalp, neck, palate, lips and middle ear, while there are fewer descriptions on neuroglial heterotopia in the pharynx and parapharyngeal space. 1,2 The etiology is unclear. Several mechanisms have been proposed but none have been able to explain this abnormality satisfactorily.…”
Section: Introductionmentioning
confidence: 99%
“…The lesion is usually found in newborn infants for whom no gestational abnormalities were observed, and it is very rare in adults. 2 The classical symptoms are respiratory distress, neck mass and feeding difficulties. Computed tomography (CT) and magnetic reso-nance imaging (MRI) are useful for ruling out diagnoses of dura mater communication and cranial defects.…”
Section: Introductionmentioning
confidence: 99%
“…Almost all reports agree on early surgical excision, but some authors advocate delayed resection and even conservative treatment. 2,4 We present a rare case of extensive parapharyngeal and skull base neuroglial heterotopia in a six-month-old girl who presented respiratory and feeding obstruction at birth in another service, from which she was referred to our institution for treatment. The literature is reviewed and the diagnostic tools, histopathology and surgical management are discussed.…”
CONTEXT: Neuroglial ectopia has been defined as a mass composed of differentiated neuroectodermal tissue isolated from the spinal canal or cranial cavity and remains rare. This lesion has to be considered in the differential diagnosis among newborn infants with classical symptoms of respiratory distress, neck mass and feeding difficulties. We present a rare case of extensive parapharyngeal and skull base neuroglial ectopia in 6-month-old girl who presented respiratory and feeding obstruction at birth.
CASE REPORT:A six-month-old girl who presented upper respiratory and feeding obstruction at birth and was using tracheostomy and gastrostomy tubes was referred to our institution. Complete surgical excision of the mass consisted of a transcervical-transparotid approach with extension to the infratemporal fossa by means of a lateral transzygomatic incision, allowing preservation of all vital neurovascular structures. The anatomopathological examination showed a solid mass with nests of neural tissue, with some neurons embedded in poorly encapsulated fibrovascular stroma, without mitotic areas, and with presence of functioning choroid plexus in the immunohistochemistry assay. Neurovascular function was preserved, thus allowing postoperative decannulation and oral feeding. Despite the large size of the mass, the child has completed one year and six months of follow-up without complications or recurrence. Neuroglial ectopia needs to be considered in diagnosing airway obstruction among newborns. Surgical treatment is the best choice and should be performed on clinically stable patients. An algorithm to guide the differential diagnosis and improve the treatment was proposed.
RESUMOCONTEXTO: Tecido neuroglial ectópico é definido como uma massa composta de tecido neuroectodermal diferenciado, isolado do canal espinhal ou da cavidade craniana e permanece raro. Esta lesão tem de fazer parte do diagnóstico diferencial em neonatos com os clássicos sintomas de obstrução respiratória, massa cervical e dificuldade para alimentação. Descreve-se um raro caso de tecido neuroglial ectópico extensor em parafaringe e base de crânio em uma menina de seis meses de idade que apresentou os sintomas de obstrução respiratória e alimentar ao nascimento.
RELATO DE CASO:Menina de seis meses de idade que apresentou obstrução respiratória alta e alimentar ao nascimento, usando traqueostomia e gastrostomia, foi encaminha à instituição. A ressecção cirúrgica completa da massa utilizou um acesso transcervical-transparotídeo com extensão para fossa infratemporal pela incisão lateral transzigomática, permitindo identificação e preservação das estruturas neurovasculares vitais. O exame anatomopatológico evidenciou uma massa sólida com ninhos de tecido neuronal com alguns neurônios imersos em estroma fibrovascular, de pouca cápsula, sem áreas de mitoses e presença de plexo coroide funcionante ao exame imunoistoquímico. As funções neurovasculares foram preservadas na cirurgia, permitindo a decanulação e alimentação via oral no pós-operatório. Ape...
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