Tumores nasales congénitos de la linea media / Congenital nasal tumors of the midline in ENT

Las masas nasales congénitas de la línea media se presentan con una frecuencia muy baja ­1/20.000 a 1/40.000 nacidos vivos­. Se trata de hallazgos asintomáticos en el recién nacido y son resultado de anomalías congénitas del desarrollo embrionario, que suelen aparecer como masas en la línea media nasal en un punto cualquiera entre glabela y columela. Estas tumoraciones presentan un riesgo elevado de extensión al sistema nervioso central, lo que es especialmente importante tener en cuenta para prevenir consecuencias tales como la fístula de líquido cefalorraquídeo y/o la aparición de meningitis recidivante. Existen gran cantidad de tumores nasales de la línea media que aparecen en el recién nacido o en el lactante y que constituyen diagnósticos diferenciales de las lesiones congénitas antes descriptas. Describiremos brevemente los más frecuentes según nuestra experiencia. AU

Congenital nasal masses of the midline are very rare ­ 1/20,000 to 1/40,000 live births ­. Nasal tumors are asymptomatic findings in the neonate and are caused by congenital abnormalities during fetal development, usually appearing at the nasal midline between the glabella and columella. These tumors are associated with a high risk of extension to the central nervous system; therefore, it is especially important to prevent the development of a cerebrospinal fluid fistula and/or recurrent meningitis. There is a large number of nasal tumors of the midline in neonates or infants in the differential diagnosis of the above-described congenital lesion. Here we briefly describe the most common nasal tumors seen at our department. AU

Nasal glioma presenting with strabismus.

Congenital midline nasal masses are rare anomalies that occur in about 1 in 20,000 to 40,000 live births. Nasal gliomas are thought to be collections of heterotopic tissue of neurogenic origin, which have lost their intracranial connection. It rarely cause ocular distortion and deformity in the medial orbital wall. We describe here a case of a 12-month-old baby girl diagnosed as extranasal glioma at the lateral nasal wall and medial orbital wall presenting with strabismus and subsequently treated in our service and perform a literature review.

[Congenital nonpulsatile midline nasal masses].

These tumours are rare, benign abnormalities including dermoids, gliomas and encephaloceles that result from aberrant embryologic development. They can cause severe deformity of the midface and nasal structures and may have an intracranial extension that requires neurosurgical consultation. Thus preoperative manipulations, i.e. biopsies, are contraindicated as it can lead to cerebrospinal fluid leak and meningitis. The treatment is surgical excision and should be performed early. Neuroimaging is essential in the evaluation of specific type, presence of intracranial extension and presurgical planning.

Does nasal neuroglial heterotopia represent a risk for the newborn during delivery?

Neuroglial heterotopia is a rare developmental abnormality. Most frequently the diagnosis is established at birth or in early childhood by a typical clinical presentation. Neuroglial heterotopia can be intracranial or extracranial. A typical example of extracranial heterotopia is nasal glioma, which can be isolated or can communicate directly with the intracranium. The most sensitive investigation for the confirmation of its site is magnetic resonance imaging. Histological investigation is crucial in establishing the diagnosis. The authors present the case of postnatally assessed nasal glioma. They emphasize the importance of detailed prenatal investigation as most important in preventing birth trauma and consequent complications.

The external rhinoplasty approach for congenital nasal lesions in children.

OBJECTIVE: Congenital lesions of the nose can be challenging to excise. While some lesions carry infection risks, in most cases surgery is primarily performed for cosmesis. Some lesions may extend up to the skull base and this can occasionally be missed on MRI scans. Surgical access has to allow complete excision in all circumstances, but access must be balanced against cosmetic results. We present our experience of the external rhinoplasty approach which allows wide access with little visible scarring. METHODS: Retrospective chart review of all cases performed between November 2003 and October 2009. RESULTS: 15 children underwent excisional surgery using the external rhinoplasty approach. They were aged 1-5 years at the time of surgery, and 12 were male. Pathology comprised congenital midline nasal dermoid cysts in 13 (of which 4 extended intracranially), extranasal glioma in 1 and non-resolving haemangioma in 1. The surgical approach provided adequate visualisation in all cases. The children with intracranial dermoids had resection and repair of the dura as part of their procedure. No post-operative CSF leaks occurred. One child with nasal dermoid had a small cyst recurrence in the skin of the nasal tip requiring further surgery but no deep recurrences occurred. Follow up ranges from 3 months to 6 years. Children with widened nasal bones before surgery have all shown rapid bony remodelling after surgery. CONCLUSIONS: The external rhinoplasty approach offers excellent access in young children, even for intracranial lesions.

Congenital midline nasal mass: cases series and review of the literature.

Encephalocele, glioma and dermoid cyst are the most common midline nasal masses. Given their potential for intracranial extension, prompt treatment is necessary to prevent complications. Herein, we present two cases of midline nasal masses. A comparison was made to delineate the differences between their clinical courses, treatments and outcomes. Case 1 was a baby girl with respiratory distress beginning at birth. Nasal glioma without definite intracranial extension was present. The mass was completely excised with the aid of a video-assisted endoscope without complications. At follow-up two years after surgery, no recurrence was noted. Case 2 was a two-year-old boy with a midline nasal dermoid cyst. Extirpation of the lesion through a vertical-dorsal approach was performed. He was discharged three days after surgery with a satisfactory aesthetic result.

In vivo detection of inducible nitric oxide synthase in rodent gliomas.

Increased iNOS expression is often found in brain tumors, such as gliomas. The goal of this study was to develop and assess a novel molecular MRI (mMRI) probe for in vivo detection of iNOS in rodent models for gliomas (intracerebral implantation of rat C6 or RG2 cells or ethyl nitrosourea-induced glioma). The probe we used incorporated a Gd-DTPA (gadolinium(III) complex of diethylenetriamine-N,N,N',N'',N''-pentaacetate) backbone with albumin and biotin moieties and covalent binding of an anti-iNOS antibody (Ab) to albumin (anti-iNOS probe). We used mMRI with the anti-iNOS probe to detect in vivo iNOS levels in gliomas. Nonimmune normal rat IgG coupled to albumin-Gd-DTPA-biotin was used as a control nonspecific contrast agent. By targeting the biotin component of the anti-iNOS probe with streptavidin Cy3, fluorescence imaging confirmed the specificity of the probe for iNOS in glioma tissue. iNOS levels in glioma tumors were also confirmed via Western blots and immunohistochemistry. The presence of plasma membrane-associated iNOS in glioma cells was established by transmission electron microscopy and gold-labeled anti-iNOS Ab. The more aggressive RG2 glioma was not found to have higher levels of iNOS compared to C6. Differences in glioma vascularization and blood-brain barrier permeability between the C6 and the RG2 gliomas are discussed. In vivo assessment of iNOS levels associated with tumor development is quite feasible in heterogeneous tissues with mMRI.

An unusual midline nasal mass in a newborn.

We report a rare case of glial heterotopia that presented as an unusual mass over the bridge of an infant's nose. Nasal gliomas arise due to defective closure of the anterior neuropore during embryological development and represent encephaloceles that have lost their intracranial connection. They are treated by excision, which also allows definitive histological diagnosis.

Neuroglial heterotopia causing neonatal airway obstruction: presentation, management, and literature review.

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 clinical course and treatment are discussed, along with their radiology and histology. The relevant scientific literature is reviewed.

Respiratory distress secondary to nasopharyngeal glial heterotopia.

Nasal glial heterotopia (nasal glioma) is a rare congenital malformation of neural origin. We present a newborn baby with life-threatening respiratory distress secondary to nasopharyngeal glial heterotopia that obstructed the nasopharyngeal or nasal airway. A high degree of suspicion, early diagnosis and surgical management are essential to cure this rare and potentially life-threatening disorder.

Nasal glioma.

Nasal gliomas are uncommon congenital lesions arising from abnormal embryonic development. Clinically, these masses are firm and incompressible. Histologically, they are made up of astrocytes and neuroglial cells, embedded in fibrous and vascular connective tissue. Proper management of a nasal glioma requires a multidisciplinary approach including an otorhinolaryngologist, radiologist, and neurosurgeon. Radiological investigations such as computed tomography or magnetic resonance imaging should be performed to exclude intracranial extension. The mainstay of treatment is conservative surgical excision because nasal gliomas are slow-growing, rarely recurrent, and have no malignant potential. We report one case of nasal glioma in a Chinese infant. He had an uncomplicated surgical intervention with a good cosmetic result. A review of the clinical features of and diagnostic approach to nasal gliomas is also presented.

[Nasal glial heterotopia: embryological and clinical approaches]. / Hétérotopie gliale nasale: approches clinique et embryologique.

INTRODUCTION: Nasal gliomas or heterotopia are nonhereditary congenital malformations composed of heterotopic neuroglial tissue. They usually present in infancy. Evaluation should include preoperative imaging with CT scan and/or MRI to rule out intracranial extension. There have been several cases reported in which nasal gliomas were misdiagnosed as capillary hemangiomas. The differential diagnosis includes prenasal space developmental impairment, which are nasoethmoidal meningoencephaloceles, nasal dermoid and epidermoid cysts. CASE REPORT: We describe the case of a newborn male infant presenting at birth with a paramedial nasal glioma. An embryological and clinical analysis of nasal gliomas is proposed. DISCUSSION: Nasal glioma is an uncommon congenital lesion presenting as a large panel of midline craniofacial anomalies. The embryological and anatomical origins of nasal gliomas are reviewed. The most known embryological theory was described by Grünwald in 1910 and is called the "prenasal space" theory. This theory is very attractive because of the embryopathogenic continuum proposed among dermoids, gliomas, and encephaloceles. In this article, we discuss major embryological theories on nasal gliomas pathogenesis and propose that while the prenasal space theory can explain the occurrence and the continuum between basal anterior or prenasal encephaloceles and gliomas, it cannot explain the occurrence of craniofacial demoids of the same topography. Better knowledge of embryological mechanisms implicated in the pathogenesis of nasal gliomas can help clinical management of this kind of malformations.

Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging.

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.