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Introduction Nasal gliomas - or nasal glial heterotopias - are rare congenital malformations, which correspond to 5% of the congenital nasal masses. It is a mass composed of mature glial tissue that can be located outside, inside or near the nasal region, and may or may not be connected to the brain by a fibrous pedicle. This report addresses a case of nasal glioma that suffered recurrence after endoscopic treatment.

Nasal glial heterotopia (NGH), also known as nasal glioma, is a rare congenital malformation in which a mature glial cell mass is found in a location other than the central nervous system. These benign masses are divided into 3groups based on localization: the intranasal, extranasal, and mixed types. Here we present a case study of a young boy with an extranasal-located NGH who underwent surgical resection. Radiological examination is indispensable to differentiate between other midline masses (hemangiomas, dermoid cysts, and encephaloceles). Magnetic resonance imaging should exclude communication with the central nervous system. Computed tomography is used to verify the integrity of the anterior skull base. The proper management of NGH is a complete resection, often by a multidisciplinary approach involving otorhinolaryngology, neurosurgery, and reconstructive surgery. [ABSTRACT FROM AUTHOR]

Developmental midline nasal masses including nasal dermoids (NDs), encephaloceles (EPHCs), and nasal glial heterotopias (NGHs) are a consequence of disrupted embryonal developmental processes in the frontonasal region. Surgery is the only method of treatment in order to prevent local and intracranial inflammatory complications as well as distant deformities of the facial skeleton. Due to their rarity, similar location, and clinical and radiological symptoms, meticulous preoperative differential diagnostics is mandatory. The aim of this thorough literature review was to present and discuss all clinical, histopathological, and radiological aspects of NDs, NGHs, and EPHCs that are crucial for their differential diagnosis. [ABSTRACT FROM AUTHOR]

Developmental midline nasal masses including nasal dermoids (NDs), encephaloceles (EPHCs), and nasal glial heterotopias (NGHs) are a consequence of disrupted embryonal developmental processes in the frontonasal region. Surgery is the only method of treatment in order to prevent local and intracranial inflammatory complications as well as distant deformities of the facial skeleton. Due to their rarity, similar location, and clinical and radiological symptoms, meticulous preoperative differential diagnostics is mandatory. The aim of this thorough literature review was to present and discuss all clinical, histopathological, and radiological aspects of NDs, NGHs, and EPHCs that are crucial for their differential diagnosis.

A 3-month-old boy presented with an intranasal polypoidal mass protruding out of the nostril which was present since birth and growing slowly. The mass was non-pulsatile and soft to firm in consistency. It did not increase in size on coughing, crying, or compression of the jugular vein. Magnetic resonance imaging and contrast-enhanced computed tomography (CT) revealed a lobulated well-circumscribed soft tissue mass in the left nasal cavity with no intracranial communication. Complete surgical excision of the mass was carried out via an intranasal endoscopic approach. Histopathological examination confirmed the diagnosis of intranasal glioma.

Nasal Glioma (NG) represents a rare congenital abnormality of the neonate, which can be associated with skull defects or even a direct communication to the central nervous system. MRI serves valuable information for differentiation from encephalocele, dermoid cyst and congenital hemangioma. Complete resection remains the treatment of choice. We present two cases of NG, which were both suspected during prenatal ultrasound and MRI. In the first case, postnatal MRI showed a transcranial continuity. Mass excision was performed and the defect was covered by a glabellar flap allowing a good cosmetic result. Postnatal MRI excluded a trans-glabellar communication in the second case. After surgical excision, the resulting skin defect was covered with a full thickness skin graft harvested from the right groin. In cases of NGs complete resection and cosmetic appealing results can be achieved and might necessitate a multidisciplinary approach.

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 detail. Main text: An extensive literature review was performed searching through the standard textbooks and electronic databases to obtain thorough information on the various encephaloceles particularly from a radiological point of view. The author's observations were also incorporated in the review. Among the available imaging modalities, magnetic resonance imaging is the most versatile and considered the imaging modality of choice because of superior anatomic resolution in the characterization of central nervous system malformation. This study provides the reporting radiologist with a methodical guide to approach a case of encephalocele discussing the imaging protocol, relevant anatomy, classification, associated abnormalities, and imaging prognostic factors. Conclusion: Encephalocele has always been a subject of predicament for radiologists. Understanding the relevant terminologies, anatomy, imaging protocols, classification, associated malformations/anomalies, and imaging prognostic factors will help to methodically approach each case and provide a systematic and comprehensive report. [ABSTRACT FROM AUTHOR]

Glial heterotopias are the displacement of neuroglial tissue in extracranial sites. Nasal glial heterotopias can be of three typesextranasal, intranasal and mixed. Root of the nose is the most common location. These are rare anomalies with an incidence of 1 case in 20,000-40,000 live births. Here we report the case of a 6-month-old infant with a congenital mass located at the root of the nose. Non-contrast computed tomography studies showed no evidence of intracranial communication of the lesion. The mass was excised, and on histopathological examination, it showed glial tissue with astrocytes in a fibrillary background and fibroconnective tissue. Masson's trichrome stain showed the red staining of the glial tissue, whereas the background fibrosis was stained blue. On immunohistochemistry, glial fibrillary acidic protein was positive. Hence, the diagnosis of nasal glial heterotropia was made. The patient had an uneventful postoperative period. [ABSTRACT FROM AUTHOR]

Congenital midline nasal masses are rare anomalies of which nasal glial heterotopia represents an even rarer subset. We report a case of a 25-day-old male child with nasal glial heterotopia along with cleft palate suggesting embryonic fusion anomaly which was treated with excision and primary closure for nasal mass followed by palatal repair at later date.

Neuroglial heterotopias are rare congenital masses. Nasal heterotopies are termed as nasal glial heterotopia or nasal glioma. The differential diagnosis includes hemangioma, dermoid/epidermoid cysts, encephalocel and tumors. Surgical excision is the treatment of choice. We report an extremely low birth weight infant with nasal glioma as firts time in the literature. [ABSTRACT FROM AUTHOR]

Introduction: Lesions affecting the anterior skull base represent a rare group of craniofacial pathologies. A tumor of the facial midline, meningitis, or rhinoliquorrhea may be indicative of malformations like dermoid cysts, gliomas, encephaloceles, or nasal fistulas. Methods: We present a case series of 13 children (4 months to 12 years; 8 males, 5 females) with lesions involving the anterior skull base, which were treated surgically in an interdisciplinary setting. This case series includes cases of nasal fistulae (n = 5), nasal cysts (n = 5), aneurysmal bone cyst, nasal glioma, and meningoencephalocele (n = 1). Results: All lesions were resected with a transnasal, transcutaneous, and/or transcranial approach with reconstruction of the anterior skull base if intracranial/intradural extension was detected. In 5 cases, a dura leakage was visible, which was sealed via Onlay-technique in 3 cases, whereas in 2 cases involving a greater dural defect, the GAP-CAS technique was performed. No complications occurred, and no recurrence was visible in a long-term follow-up. An algorithm for a systematic approach to these various pathologies is provided. Conclusion: Congenital pathologies of the anterior skull base are rare, challenging to diagnose, and present as clinical emergencies. An interdisciplinary surgical approach is needed for best functional and aesthetic results. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: To describe our experience and current management approach for congenital nasal neuroglial heterotopia (NGH) and encephaloceles.Study Design: Retrospective chart review at a tertiary pediatric hospital from 1970 to 2013.Methods: Thirty patients met inclusion criteria: 21 NGH and nine encephaloceles. Data including demographics, pathology, imaging modality, surgical approach, resection extent, outcomes, and complications were analyzed.Results: Fourteen NGH patients (67%) presented with an internal nasal mass and nasal obstruction. Three patients (14%) presented with an external nasal mass and four (19%) had a mixed lesion. Median age at surgery was 0.51 years (interquartile range 1.32 years). Thirteen (62%) had an intranasal endoscopic approach. Median operative time was 1.6 hours (interquartile range 1.2 hours), and there were no major complications. Nine patients with encephalocele were identified: six (67%) presented with transethmoidal encephaloceles, two (22%) presented with nasoethmoidal encephaloceles, and one (11%) presented with a nasofrontal lesion. The median age at surgery was 1.25 years (interquartile range 1.4 years). All patients required a craniotomy for intracranial extension. Median operative time was 5 hours (interquartile range 1.9 hours), and eight patients (88%) had a total resection. Length of stay ranged from 3 to 14 days.Conclusion: Nasal neuroglial heterotopia and encephaloceles are very rare lesions that require multidisciplinary evaluation and management. At our institution, there has been a shift to magnetic resonance imaging alone for the evaluation of NGH to avoid radiation exposure. Endoscopic extracranial resection is feasible for most intranasal and mixed NGH without an increase in operative time, residual disease, or complications.Level Of Evidence: 4. Laryngoscope, 126:2161-2167, 2016. [ABSTRACT FROM AUTHOR]

Congenital midline nasal masses are rare anomalies of which nasal glial heterotopia represents an even rarer subset. We report a case of a 25-day-old male child with nasal glial heterotopia along with cleft palate suggesting embryonic fusion anomaly which was treated with excision and primary closure for nasal mass followed by palatal repair at later date. [ABSTRACT FROM AUTHOR]

Objectives. Diagnosis and treatment challenges in similar clinical appearance nasal malformations. The authors present 2 nasal external malformations. Material. Patients admitted, evaluated and operated into the pediatric ENT department. Results. The evolution of operated patients was fine, with no postoperative complications. Discussions. 2 clinically similar malformations were compared mostly from the surgical perspective. Imagistics failed to fully delineate the extent of the disease in one case. Surgical approach was differently decided, considering patients ages. The main surgical issues and dilemmas were analyzed. Conclusion. The investigation and surgery for nasal malformations in children can be challenging from the decision and the monitoring point of view. [ABSTRACT FROM AUTHOR]

Introduction: Nasal glioma is a rare, benign congenital midline facial lesion. Materials and Methods: Prenatal ultrasound diagnosis performed at 2nd trimester of pregnancy revealed a right-sided mass at the level of the fetal face extending from the right internal canthus to the nasal bridge. Conclusion: Differential diagnosis of facial mass in the fetus represents a critical issue because is essential in guiding the prenatal counselling of the couple and in guiding the prenatal and/or postnatal management. Alternative diagnoses such as dacryocystocele, dermoid cyst, retinoblastoma or teratoma, hemangioma, and encephalocele that can not completely be excluded prenatally are discussed. Embryology, pathology, prenatal ultrasound diagnostic clusters of the lesion as well as MR imaging findings are discussed together with review of the literature. [ABSTRACT FROM AUTHOR]

Objectives: We report a rare case of a nasal glioma found incidentally in an adult, presenting with visual loss, optic nerve oedema and proptosis. Case report: A 41-year-old woman presented with bilateral proptosis, impairment in visual acuity (6/60 bilaterally) and loss of colour vision. Computed tomography and magnetic resonance imaging showed proptosis, bilateral optic nerve swelling and a heterogeneous mass occupying the left nasal cavity and extending through a skull base defect into the anterior cranial fossa. Biopsy confirmed a nasal glioma. Treatment with intravenous dexamethasone resolved the proptosis, and the patient's visual acuity recovered to 6/9 bilaterally. At the multidisciplinary team meeting, it was felt that the nasal glioma probably represented an incidental finding and was not directly responsible for the patient's proptosis and transient visual loss. Conclusion: To our knowledge, this is the first report in the English language literature of adult nasal glioma presenting with visual loss. The management of nasal gliomas in adults is contentious and the relevant literature is reviewed. This case was managed conservatively with regular follow up. [ABSTRACT FROM AUTHOR]

Nasal glioma is a rare congenital midline malformation composed of heterotopic masses of neuroglial tissue. We report a case of fetal nasal glioma diagnosed by sonography at 22 weeks' gestation as a vascular hypoechoic mass located on the left nasal bone. Fetal MRI excluded an underlying bone defect. At birth, the lesion appeared as a reddish mass. Post natal imaging confirmed the vascularisation within the lesion with an arterial low-flow velocity and a high-resistance spectrum, consistent with a glioma. The child underwent surgery at 5 months and final diagnosis was made on pathological examination. Therefore, a vascular lesion and a clinical aspect mimicking a haemangioma should not be considered sufficient to reach the final diagnosis. [ABSTRACT FROM AUTHOR]

Summary: In this report, we describe an infant with a nasal tip glioma treated by excision via external rhinoplasty. A 3-month-old full-term, otherwise healthy infant presented with a slowly enlarging midline mass of the nasal tip. Clinically the mass appeared cystic without trans-illumination, enlargement with crying, or signs of infection. On successive CT scans at 3 and 5 months old the mass appeared as an enlarging focal prominence to the left of midline with central hypodensity without evidence for intracranial extension. Preoperatively the differential diagnosis included nasal dermoid, glioma, and hemangioma. The mass was removed at 6 months of age via an open rhinoplasty approach with conchal cartilage grafting to reconstruct the left upper lateral cartilage. The final pathological diagnosis was nasal glioma (glial heterotopia) with fibrosis. Pre-operative, intra-operative, and post-operative photos were taken. The patient has been followed up for 6 months without evidence of recurrence or other complications. A review of the literature on nasal glioma and external nasal surgery in infancy is presented. [Copyright &y& Elsevier]

Abstract: Infantile hemangiomas are frequent benign vascular tumors that are often easily recognized. However, the diagnosis between infantile hemangiomas and other vascular tumors, whether benign or malignant, may be difficult. This chapter describes the different clinical presentations of hemangiomas and details the investigations that are needed to confirm the nature of the lesion and to diagnose the potentially associated anomalies. Knowledge on differential diagnosis enables clinicians to detect hemangiomas that can lead to complications and that necessitate a multidisciplinary approach. [Copyright &y& Elsevier]

The newborn, infant, or young child who presents with a midline frontonasal mass often poses a diagnostic challenge to the clinician. The most pressing issue is whether the mass extends intracranially. The development of the frontonasal region or anterior neuropore is complex. Aberrant embryogenesis leads to three main types of anomalies: nasal dermal sinus, anterior cephalocele, and nasal glioma. Understanding the developmental anatomy of the anterior neuropore and postnatal maturation will serve the radiologist well when it comes to imaging frontonasal masses. Pitfalls particularly common to CT imaging interpretation include the evolving ossification of the frontal, nasal and ethmoid bones in the first year of life, morphology and size of the foramen cecum, and the natural intumescence of the anterior nasal septum. Determination of the presence of a connection between the frontonasal mass and the anterior cranial fossae is crucial in the imaging assessment and clinical management. In the case of the nasal dermal sinus, failure to appreciate the intracranial components of the malformation can lead to fatal meningitis. MR imaging is the modality of choice for assessing the pediatric frontonasal region. Its advantages include multiplanar imaging, distinguishing the interface among cartilage, bone, brain and fluid, diffusion imaging to detect epidermoid tumors, and the capacity to evaluate the brain for associated cerebral anomalies. [ABSTRACT FROM AUTHOR]

Summary: PHACE syndrome consists of the constellation of manifestations including Posterior fossa anomalies of the brain (most commonly Dandy-Walker malformations), Hemangiomas of the face and scalp, Arterial abnormalities, Cardiac defects, and Eye anomalies. We present the case of a patient who presented with respiratory distress at birth secondary to a large nasal glioma. She was subsequently found to have a ventricular septal defect (VSD), a facial hemangioma, and a malformation of the eye and optic nerve head. The nasal glioma, which extended to the cribriform plate, has not been described in this syndrome. The tumor was resected through a coronal incision, midline nasal bone osteotomy, and a retrograde dissection from the nasal bones to the anterior skull base. Glioma of the skull base is a novel and serious manifestation of this uncommon condition. [Copyright &y& Elsevier]

Objective and Patient: Both nasal cerebral heterotopia and encephalocele are rare congenital benign masses of neurogenic origin caused by an embryonic developmental abnormality. It is generally accepted that nasal heterotopia is a sequelae to encephalocele. This report presents an unusual case of nasal cerebral heterotopia and encephalocele arising in the same patient. The patient had a firm, solid mass measuring 1.5 ⊗ 1.0 cm on the bridge of the nose covered with normal skin and another mass in the nasal cavity obstructing the right nasal cavity. Computed tomography (CT) demonstrated that the nasal bone separated these masses. CT also showed a bony defect at the skull base. Surgery consisted of dividing the encephalocele and closure of the skull base fistulae, along with nasal subcutaneous mass enucleation. Results: Intraoperative examination indicated the existence of a pit on the nasal bone where the pedicle of the nasal subcutaneous mass connected. Microscopic examination of the nasal cavity mass demonstrated meningoencephalocele, and examination of the nasal subcutaneous mass demonstrated nasal cerebral heterotopia, which was confirmed by immunohistochemical staining. After 10 months, complete removal of the subcutaneous nasal mass was recognized and there was no evidence of recurrence. Conclusion: Findings in this case suggest that the nasal cerebral heterotopias will result from encephalocele. [ABSTRACT FROM AUTHOR]

Developmental nasal midline masses in children are rare lesions. Neuroimaging is essential to characterise these lesions, to determine the exact location of the lesion and most importantly to exclude a possible intracranial extension or connection. Our objective was to evaluate CT and MRI in the diagnosis of developmental nasal midline masses. Eleven patients (mean age 4.5 years) with nasal midline masses were examined by CT and MRI. Neuroimaging was evaluated for (a) lesion location/size, (b) indirect (bifid or deformed crista galli, widened foramen caecum, defect of the cribriform plate) and direct (identification of intracranially located lesion components or signal alterations) imaging signs of intracranial extension, (c) secondary complications and (d) associated malformations. Surgical and histological findings served as gold standard. Nasal dermoid sinus cysts were diagnosed in 9 patients. One patient was diagnosed with an meningocele and another patient with a nasal glioma. Indirect CT and MRI signs correlated with the surgical results in 10 of 11 patients. Direct CT findings correlated with surgery in all patients, whereas the direct MRI signs correlated in 9 of 11 patients. In 2 patients MRI showed an intracranial signal alteration not seen on CT. Neuroimaging corrected the clinical diagnosis in 1 patient. One child presented with a meningitis. In none of the patients was an associated malformation diagnosed. Intracranial extension is equally well detected by CT and MRI using indirect imaging signs. Evaluating the direct imaging signs, MRI suspected intracranial components in 2 patients without a correlate on CT. This could represent an isolated intracranial component that got undetected on CT and surgery. In 9 patients CT and MRI matched the surgical findings. The MRI did not show any false-negative results. These results in combination with the multiplanar MRI capabilities, the different image contrasts that can be generated by MRI and the lack of radiation favour the use of MRI as primary imaging tool in these young patients in which the region of imaging is usually centred on the radiosensitive eye lenses. [ABSTRACT FROM AUTHOR]

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