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.

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.

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.

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.

Osteoporosis-pseudoglioma syndrome.

Two patients with osteoporosis pseudoglioma syndrome are described. Both are single children, born to nonconsanguineous, healthy parents. The first patient, a 17-year-old girl, had serious visual impairment since birth. She is severely dwarfed and has major skeletal deformities resulting in inability to walk since age 2 years. The second patient is an 18-year-old girl with unilateral neonatal blindness, short stature and deformities, mainly of pelvis and lower limbs. She has been able to walk with support up to now. The clinical and radiological findings in these 2 patients reflect the clinical variability of the condition. Results of collagen studies in both patients are normal and differentiate this condition clearly from severe osteogenesis imperfecta, which it resembles.

Management of the congenital midline nasal mass: a review.

The nasal encephalocele, the glioma, and the dermoid are the most common of the congenital midline nasal masses. Due to similar embryologic development, each of these lesions may be associated with bony cranial defects and intracranial abnormalities, as well as CSF leakage and the potential for fatal meningitis if not handled properly. Properative manipulation should be avoided. Radiologic studies are instructive only if they are positive. If intracranial attachments are identified radiologically or suspected clinically, neurosurgical consultation should be obtained, and intracranial exploration and resection should be carried out as the initial procedure. Extra-cranial resection of the remaining mass may be performed immediately after intracranial resection, may be postponed, or may become unnecessary.

Congenital nasal masses: CT and MR imaging features in 16 cases.

The imaging studies of 16 children with pathologically proved nasal encephaloceles (eight), nasal dermal sinuses/nasal dermoids (seven), and nasal cerebral heterotopias, more commonly known as nasal gliomas (one), were retrospectively reviewed and compared with normal control subjects to define the normal anatomy and analyze deformities caused by these lesions. Nasal encephaloceles were always identified as complex masses of mixed soft tissue and CSF intensity that were contiguous with intracranial structures. The nasal glioma appeared as a mixed-intensity mass that, on the basis of the CT scan, appeared to be continuous with intracranial structures. Nasal dermal sinuses could only be identified as they coursed through the skin and subcutaneous soft tissue. They could not be identified when intraosseous. Moreover, on CT and, particularly, on MR, a number of potential diagnostic pitfalls were encountered. The most important of these was the normal fat deposition that occurs within bone during normal maturation and during aeration of the frontal sinuses and nasal bones. These fatty changes can easily be mistaken for fatty tumors if they are not recognized as normal anatomic changes. Interestingly, the classic plain film findings for congenital nasal masses were present only in the encephaloceles and nasal glioma; dermoids and dermal sinuses showed none of the classic plain film findings. In the six patients who had both CT and MR, the masses were easily identified and characterized by each imaging method. Congenital nasal masses are well characterized by both CT and MR. It is important to understand the normal changes in the anatomy of the nasofrontal region in the pediatric age group to avoid false-positive diagnoses in this region.

A neonatal huge subependymal giant cell astrocytoma: case report.

We report a neonate with a huge subependymal giant cell astrocytoma associated with tuberous sclerosis, with atypical magnetic resonance imaging findings. The neonatal subependymal giant cell astrocytoma is rare, and we discuss the difficulty in its diagnosis and treatment.

Nasal glioma.

Six cases of nasal gliomas, which are rare ectopic rests of neural tissue found at the root of the nose, are presented. It is important to distinguish nasal tumors from basofrontal encephaloceles to avoid inadvertent exposure of the brain during the surgical removal of mass lesions. Because of their related embryologic origins, the distinction between nasal gliomas and basofrontal encephaloceles may not be clear clinically. Nasal gliomas may be treated by several surgical specialties, and only a proper awareness of their relatonship to encephaloceles can assure the selection of a flexible and adequate surgical approach. This paper emphasizes the salient clinical characteristics of nasal gliomas, their clinical distinction form and embryologic relationship to encephaloceles, and the options for treatment.

Nasal glioma or nasal glial heterotopia? Morphological, immunohistochemical and ultrastructural study of two cases.

The term nasal glioma has been used to describe a congenital benign tumor of the nasal region containing neural tissue. The nature of these lesions remains open to controversy, because of the different locations of the heterotopic neural tissue involved, the deficient development of the bony structures and the persistence or not of the structural relations with the central nervous system. More recent terms define these lesions as ectopic nervous tissue. A clinical, morphological, ultrastructural and immunohistochemical study is made of two cases of nasal glioma, one associated with agenesis of the corpus callosum. In this case, the mother had been treated with clomiphene. In such cases, morphological and immunohistochemical findings support that "nasal glioma" remain valid as a descriptive term defining a congenital benign tumor composed of heterotopic neural tissue within the nasal region and covered by skin, that may recur following incomplete surgical resection.

Congenital glioblastoma of the cerebellum with cytofluorometric deoxyribonucleic acid analysis.

A case of congenital glioblastoma arising from the cerebellum is presented with special reference to microspectrophotometric deoxyribonucleic acid (DNA) analysis of the tumor cells. Seven cases of this condition are already in the literature. The prognosis is poor and most of the reported cases survive no more than 2 months. Cytofluorophotometric DNA studies were helpful in elucidating the extreme heterogeneous distribution of DNA contents of the tumor cells, suggesting a biological aggressiveness probably related to the rate of proliferation.

Midline nasal mass in infancy: a nasal glioma case report.

Congenital midline nasal masses are rare anomalies that occur in about one in 20,000-40,000 live births. The most common are dermoid/epidermoid tumors, nasal cerebral heterotopias (nasal gliomas), and nasal encephaloceles; some have an actual or potential central nervous system connection. Nasal gliomas are CNS masses of neurogenic origin which have lost their intracranial connections and present as an obvious external or intranasal mass at birth without associated surgical symptoms. Careful evaluation is required to confirm the diagnosis and appropriate management. The interpretation of CT and MR images can be difficult but is useful in differentiating nasal gliomas from other congenital nasal masses. The presence of a fibrous stalk may be associated with cranial defects and CSF leak. Excisional biopsy allows histopathologic diagnosis and is the definitive treatment. They are benign lesions, and recurrences are rare, so conservative cosmetic surgical techniques should be chosen for gliomas where there is no proven intracranial extension. The authors report an illustrative nasal glioma case in a one-year-old male infant with extranasal and intranasal components, and discuss the therapeutic options.

Pediatric congenital nasal masses.

Nasal dermal sinus cysts, encephaloceles, and gliomas are the most common congenital nasal masses with actual or potential central nervous system connections. They can present intranasally, extranasally, or as external nasal masses with or without nasal obstruction. This paper presents several illustrative cases and reviews recent concepts in embryology and management, which hold that all congenital midline nasal masses potentially have central nervous system connections and should be managed as such.

[Fronto-ethmoidal meningo-encephaloceles and nasal gliomas (author's transl)]. / Méningo-encéphalocèles fronto-ethmoïdales et gliomes nasaux.

Meningoencephalocoeles and nasal gliomas are congenital malformations rare in Europe but much better known in Morocco and in Thailand. These lesions have embryonic relations which are such that certain authors consider them to be only clinical varieties of the same malformation. They cause extra nasal, intra nasal or mixed tumours discovered most often at birth. Treatment is surgical either via a high intracranial approach or via a low facial approach (both approaches sometimes being used in association) and the prognosis is dominated by the problem of the dura mater if there are connections between the tumour mass and the subarachnoid spaces. When the problem is resolved by dural plasty, the later prognosis is excellent and, in particular, the psychomotor development of the children appears to be normal. Cosmetic problems, and in particular hypertelorism, may nevertheless require later surgery.

Congenital midline nasal masses: dermoids, gliomas, and encephaloceles.

Congenital midline nasal masses are rare entities and therefore easily misdiagnosed or missed altogether. This article reviews the three most common congenital midline nasal masses: dermoids, gliomas, and encephaloceles. Discussion entails embryology, diagnosis, radiologic workup, surgical therapy, and complications.

Nasal cerebral heterotopia: a case report.

Nasal cerebral heterotopia (nasal glioma) are rare congenital benign masses of neurogenic origin with intranasal location, or both. An extranasal case is reported in a 2-year-old-boy without any bony defect or connection with brain. Histology and immunohistochemistry confirmed the diagnosis.

[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.