Midline nasal region lesions
A variety of congenital midface anomalies occur in children. Although rare, these disorders are clinically important because of their potential for connection to the central nervous system. Lesions presenting as a midline nasal mass include:
- nasal glioma
- nasal encephalocele
- nasal dermoid cyst
- nasal epidermoid cyst
- haemangioma or lymphangioma
- dacryocystocele
- dacryocystitis
Treatment and prognosis
When a dermoid, glioma, or encephalocele is a suspected diagnosis a biopsy should not be performed before an intracranial connection is ruled out because of the risk of causing meningitis or CSF leak.
Both CT and MR imaging are often required to adequately evaluate the bone, brain, and soft-tissue components of midface anomalies, especially congenital masses and craniofacial syndromes.
The treatment of nasal dermoids, gliomas, and encephaloceles is by complete surgical excision.
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Malformations of the central nervous system
-
malformations of cortical development
- abnormal cell proliferation or apoptosis
- abnormal brain size
-
microcephaly
- with normal to simplified cortical pattern
- microcephaly with lissencephaly
- microcephaly with extensive polymicrogyria
- macrocephalies (megalencephaly/macrocephaly)
-
microcephaly
- abnormal cell proliferation
- non-neoplastic
- cortical hamartomas of tuberous sclerosis
- hemimegalencephaly
-
focal cortical dysplasia (Type I and Type IIb)
- Palmini classification (2004)
- Barkovich classification (2005)
- Blumcke classification (2011)
- neoplastic
- non-neoplastic
- abnormal neuronal migration
- lissencephaly
- lissencephaly type I: subcortical band heterotopia spectrum (band heterotopia): undermigration
- lissencephaly type II (cobblestone complex)
- heterotopia: ectopic migration
- subependymal heterotopia
- subcortical heterotopia (not including band heterotopia)
- marginal glioneuronal heterotopia
- lissencephaly
- abnormal cortical organisation
- mild malformations of cortical development (previously microdysgenesis)
-
polymicrogyria and schizencephaly
- bilateral polymicrogyria syndromes
- schizencephaly
- focal cortical dysplasia (Type IIa)
- abnormal brain size
- not otherwise classified
- malformations secondary to inborn errors of metabolism
- mitochondrial and pyruvate metabolic disorders
- peroxisomal disorders
- other unclassified malformations
- malformations secondary to inborn errors of metabolism
- abnormal cell proliferation or apoptosis
-
midline abnormalities of the brain
- absent septum pellucidum
- cephaloceles
- midline nasal region lesions
- cerebral hemispheres
-
holoprosencephaly/septo-optic dysplasia spectrum
- septo-optic dysplasia
- lobar holoprosencephaly
- semilobar holoprosencephaly
- alobar holoprosencephaly
- middle interhemispheric variant/syntelencephaly
-
holoprosencephaly/septo-optic dysplasia spectrum
- corpus callosum
- intracranial lipoma
-
malformations of the cerebellum
-
cerebellar hypoplasia
- focal hypoplasia
- generalized hypoplasia
- with enlarged fourth ventricle
- normal fourth ventricle
- with normal pons
- with small pons
- normal foliation
- pontocerebellar hypoplasias of Barth, types I and II
- cerebellar hypoplasias, not otherwise specified
- normal foliation
- cerebellar dysplasia
- focal dysplasia
- isolated vermian dysplasia
- molar tooth malformations including Joubert syndrome
- rhombencephalosynapsis
- isolated hemispheric dysplasia
- focal cerebellar cortical dysplasias/heterotopia
- Lhermitte-Duclos-Cowden syndrome
- isolated vermian dysplasia
- generalized dysplasia
- congenital muscular dystrophies
- cytomegalovirus
- lissencephaly with RELN mutation
- lissencephaly with agenesis of corpus callosum and cerebellar dysplasia
- associated with diffuse cerebral polymicrogyria
- diffusely abnormal foliation
- focal dysplasia
-
cerebellar hypoplasia
- malformations of the brainstem
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