Extensive parapharyngeal and skull base neuroglial ectopia; a challenge for differential diagnosis and treatment: case report

Abstract

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...

An unusual presentation of neuroglial heterotopia: case report

“…Clinical presentation of nasopharyngeal heterotopias is usually with respiratory distress, neck mass and feeding difficulties. 6 In our case, although the lesion is situated in the nasopharynx, the delayed presentation is presumably due to its small size.…”

“… 5 Over 181 cases of neuroglial heterotopia have been documented in the literature with 98% presenting in early infancy. 6 A careful review of literature in PUBMED during 1990–2018 revealed about 14 cases of glial heterotopias reported in the nasopharyngeal region, and all presented during neonatal or infancy period with symptoms of respiratory distress or airway obstruction. Extra nasal sites are rare and include the orbit, palate, submandibular region, and overlying the spine.…”

A Rare Case of Nasopharyngeal Glial Heterotopia in a Neonate

“…Nasopharyngeal glial heterotopia is an extremely rare congenital malformation presenting frequently at birth or early childhood according to a case series of glial heterotopia cases between 1961 and 2009. 5 Its embryological origin is not clear but it may be derived from an encephalocele that has lost its connection with the subarachnoid space or may originate as an extracranial separation of embryonic neuroglial tissue beyond 10th week of gestation. 6 Prenatal diagnosis is possible with antenatal ultrasonography and fetal MRI.…”