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Unformatted text preview: Evaluation of Congenital Evaluation of Congenital Midline Nasal Masses
Camysha H. Wright, MD, Resident
Matthew Ryan, MD, Faculty
UTMB Dept of Otolaryngology
June 7, 2006 Outline
Outline Embryology of the nose
Evaluation of Nasal Mass
Embryology The critical period in nasal development is in first twelve weeks of fetal development
Abnormalities of development are believed to cause gliomas, dermoids, and encephaloceles Embryology
Embryology Neural tube develops between the third and fourth week of gestation
Closure of the neural tube occurs from the midline and extends cranially and caudally
Neural tube then gives rise to neural crest cells Embryology
Embryology As the neural tube closes neural crest cells migrate anteriorly and laterally around the eyes to the frontonasal process
Nose formed from the medial and lateral prominence and invagination of the nasal pit Embryology
Embryology In most of the body neural crest cells are involved in ectodermal components, in the face the primary role is in the formation of mesenchymal cells
Bone, cartilage, and muscles of the face are all derivatives of neural crest cells Embryology
Embryology Nose develops from frontonasal processes and 2 nasal placodes
Medial processes fuse
Nasomaxillary groove becomes the nasolacrimal duct Embryology
Embryology Scanning electron micrograph Embryology
Embryology During formation of skull base and nose, mesenchymal structures are formed from several centers which will eventually fuse and ossify.
Before their fusion, there are recognized spaces which are important in the development of congenital midline nasal masses
Fonticulus frontalis Prenasal space Foramen cecum Embyrology
Embyrology Fonticulus frontalis – space between the frontal and nasal bones Eventually fuses with foramen cecum to create a separation between intracranial and extracranial structures Prenasal space is between the nasal bones and the nasal capsule (precursor of the septum and nasal cartilages) Pediatric Anatomic Considerations
Pediatric Anatomic Considerations Neonates can suffer from respiratory distress with nasal obstruction
Pediatric airway differs from adults in that neonates are nasal breathers
Epiglottis abuts nasal surface of the soft palate forming anatomic divide between the airway and digestive tracts
Food from oral cavity is shunted laterally into esophagus via the pyriform sinuses
Neonates can functionally eat and breathe concurrently Pediatric Airway
Pediatric Airway Nasal Masses
Nasal Masses Differential Diagnoses Inflammatory lesions (abscess) Traumatic deformity Benign neoplasms (polyps, JNA) Malignant neoplasms (rhabdomyosarcoma) Congenital masses (teratomas, hemangiomas) Nasal Dermoids
Nasal Dermoids Can occur as cyst or sinus
Most common congenital midline nasal mass
13% of all dermoids
10% dermoids of head and neck Nasal Dermoid
Nasal Dermoid Has ectodermal and mesodermal components (ectodermal components only – epidermal cyst, ectoderm, mesoderm and endoderm teratoma) May present as midline nasal pit, fistula, or infected mass anywhere from glabella to columella
Sometimes presents as single cutaneous tract with hair at opening
May secrete pus or sebaceous material Nasal Dermoid
Nasal Dermoid Superonasal dermoid Nasal Dermoid
Nasal Dermoid CNS connection variably reported (445%)
Associated congenital anomalies (541%), however not found to be associated with syndromes
Aural atresia albinism Cleft lip/palate Mental retardatio
Corpus callosum agenesis Tracheo
l fistulas Spinal column abn
Cerebral atrophy hydrocephalu
s Cardiac anomalies Genital anomalies Hypertelorism Hemifacial microsomia Lumbar lipoma Dermal cyst of the frontal lobe
Cerebral anomalies Coronary artery anomaly Nasal Dermoid
Complications: Intermittent inflammation Abscess Osteomyelitis Broaden nasal root Meningitis Cerebral abscess Nasal Dermoid abscess Nasal Dermoid abscess Abscess formation Dermoid Cyst
Dermoid Cyst Development During development a projection of dura projects through the foramen cecum and attaches to skin Dura normally separates from nasal skin and retracts through foramen cecum losing connection If skin maintains attachment to underlying fibrous tissue, nasal capsule, or dura ,epithelial elements may be pulled into the prenasal space with or without dural connection Dermoid Cyst Development
Dermoid Cyst Development Glioma
Glioma Glial cells in a connective tissue matrix
Firm, noncompressible Red or bluish lump
Can be found at glabella, at nasomaxillary suture, intranasally
No connection with subarachnoid space
Do not enlarge with crying
Do not transilluminate
May have telangiectasias Glioma
Glioma Intranasal glioma Glioma
Glioma Extranasal 60%
Dural connection 35% intranasal, 9% extranasal
Overall 15% dural connection
CSF rhinorrhea, meningitis possible, if dural connection exists Glioma – Formation Hypotheses
Glioma – Formation Hypotheses Similar to that of nasal dermoids
Develop from extracranial rests of glial tissue
Abnormal closure of fonticulus nasofrontalis Another theory is that they are possibly encephaloceles which have lost CSF connection Glioma Development
Glioma Development Encephaloceles
Encephaloceles Extracranial herniation of meninges and/or brain
Connection with subarachnoid space
Rare at 1:35,000 births
3040% associated anomalies: microcephaly, hydrocephalus, microopthalmia, anopthalmia, agenesis of the corpus callosum, porencephaly, cortical atrophy, ventricular dilations Encephaloceles
Encephaloceles Bluish, soft, compressible, transilluminate, pulsatile
Enlarge with crying
Positive Furstenberg test (enlarge with bilateral compression of internal jugular veins)
Originate medially in the nose (cannot pass probe medially to this mass, versus with glioma generally can as they originate laterally often) May have associated CSF leak Encephaloceles
Encephaloceles Large nasal encephalocele Encephaloceles
Encephaloceles Divided into three categories: Occipital 75%
Basal 10% Sincipitalanterior or frontonasal (dorsum of nose, orbits, forehead)
Basalintranasal mass, nasopharynx, posterior orbit because they herniate through the cribiform plate or posterior to it (potential for airway obstruction in neonate) Encephaloceles
Encephaloceles Basal Encephaloceles Transethmoidalthrough cribiform plate into middle meatus Sphenoethmoidalextends through cranial defect between posterior ethmoids and sphenoid to nasopharynx Transsphenoidalpresents in nasopharynx Sphenomaxillarythrough superior and inferior orbital fissures to sphenomaxillary fossa Encephalocele
Development Dural projection through fonticulus nasofrontalis Abnormal closure results in herniated meninges/brain May be closely related to glioma Encephalocele Development
Encephalocele Development Nasal Masses
Evaluation Most often infants and children
Dermoidsfistula tract, hair, pus or sebum, midline Gliomasfirm, noncompressible, does not transilluminate, telangiectasias
Encephalocelessoft, compressible, bluish or red, enlarge with crying, positive Furstenburg test
Do not biopsy extra or intranasal mass in a child before imaging (Risk of meningitis or CSF leak if there is an intracranial connection) Imaging
• CT and MRI most used CT findings include: fluid filled cyst, soft tissue mass, intracranial mass, enlargement of foramen cecum, distortion of crista galli CT findings suggestive of intracranial extension are enlarged foramen cecum and bifidity of crista galli Findings valuable if absent, (when present may be false positive) CT Imaging
CT Imaging Nasal Dermoid axial ct with dermoid anterior to nasal and maxillary bones, no bony dehiscence or abnormalities noted Nasal Dermoid with Nasal Dermoid with Intracranial Extension CT Imaging
CT Imaging Encephalocele with defect noted in cribiform plate and herniation of brain tissue MRI
MRI Better delineates soft tissue
Ability to visualize in the sagittal plane
Denoyelle 36 children with dermoids, 2 patients had CT suggestive of intracranial involvement not found at surgery.
Recommended CT followed by MRI to confirm intracranial connection MRI Imaging
MRI Imaging Nasal dermoid cyst MRIGlioma
MRIGlioma Saggital T1 MRIGlioma
MRIGlioma Sagittal T2 MRIGlioma
MRIGlioma Coronal T2 MRIEncephalocele
MRIEncephalocele Coronal T2 weighted and sagittal T1 weighted image of sphenoid encephalocele Workup
Workup History/Physical Examination Nasal obstruction, polypoid intranasal mass, CSF leak, presence of hair or fistulous tract, compressible or firm, presence of pulsations, enlargement with crying or internal jugular compression Radiologic Evaluation (CT and/or MRI) No Intracranial Extension (Dermoid/Glioma) Intracranial Extension Treatment
Treatment Surgical Treatment Complete excision (opentranscranial vs extracranial, vs endoscopic approaches described) Perform early to avoid nasal distortion, bony atrophy, osteomyelitis, meningitis dermoidsmust excise entire tract to prevent recurrence Dermoid
Dermoid Can be removed endoscopically or via open approach Transverse rhinotomy has been described •Small to moderately sized lesions •Avoids vertical scar and splaying Nasal Dermoid with intracranial extension. Nasal Dermoid with intracranial extension. Meher R, Singh I, et al. J Postgrad Med 2005;51:3940. Dermoid cyst with intracranial extension without craniotomy Nasal bones removed along with anterior part of the frontal bone
Followed sac through cribiform plate, incised wall of sac and evacuated contents, and removed all except for its base where it was attached to dura
Destroyed secretory epithelial surface of remnant of sac with bipolar, replaced bone and closed wound in layers
Pt did well postoperative and no recurrence noted during 2 year follow up. Glioma
Glioma Can be removed via open or endoscopic approach Lateral rhinotomy or alar incisions may be used for intranasal gliomas or combined intraextranasal gliomas
Several authors have reported isolated cases of endoscopic excision of small gliomas with and without evidence of intracranial extension. Encephaloceles
Encephaloceles In the past required combined approach with neurosurgery
Frontal craniotomy is performed, intracranial mass excised, bonedura defect is repaired
Extracranial mass is then removed
More reports describing endoscopic removal Conclusion
Conclusion Midline nasal masses are rare but must be remembered in the differential Furstenberg’s test
Don’t biopsy without imaging
Surgical intervention necessity Bibliography
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