Evaluation of Congenital Midline Nasal Masses

1
Evaluation of Congenital Midline Nasal Masses

• Camysha H. Wright, MD, Resident
• Matthew Ryan, MD, Faculty
• UTMB Dept of Otolaryngology
• Grand Rounds
• June 7, 2006

2
Outline

• Embryology of the nose
• Dermoid cysts
• Glioma
• Encephalocele/Meningocele
• Evaluation of Nasal Mass
• Imaging Studies
• Surgical Intervention
• Conclusion

3
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

4
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

5
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

6
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

7
Embryology

• Nose develops from frontonasal processes and 2
  nasal placodes
• Medial processes fuse
• Nasomaxillary groove becomes the nasolacrimal duct

8
Embryology

• Scanning electron micrograph

9
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

10
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)

11
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

12
Pediatric Airway
13
Nasal Masses

• Differential Diagnoses
• Inflammatory lesions (abscess)
• Traumatic deformity
• Benign neoplasms (polyps, JNA)
• Malignant neoplasms (rhabdomyosarcoma)
• Congenital masses (teratomas, hemangiomas)

14
Nasal Dermoids

• Can occur as cyst or sinus
• Most common congenital midline nasal mass
• 1-3 of all dermoids
• 10 dermoids of head and neck

15
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

16
Nasal Dermoid

• Superonasal dermoid

17
Nasal Dermoid

• CNS connection variably reported (4-45)
• Associated congenital anomalies (5-41), however
  not found to be associated with syndromes

Aural atresia Mental retardation Spinal column abn hydrocephalus Hypertelorism Hemifacial microsomia
albinism Corpus callosum agenesis Cerebral atrophy Lumbar lipoma Dermal cyst of the frontal lobe Coronary artery anomaly
Cleft lip/palate Tracheo-esophageal fistulas Cardiac anomalies Genital anomalies Cerebral anomalies
18
Nasal Dermoid

• Complications
• Intermittent inflammation
• Abscess
• Osteomyelitis
• Broaden nasal root
• Meningitis
• Cerebral abscess

19
Nasal Dermoid abscess

• Abscess formation

20
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

21
Dermoid Cyst Development
22
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

23
Glioma

• Intranasal glioma

24
Glioma

• Extranasal - 60
• Intranasal - 30
• Both - 10
• Dural connection - 35 intranasal, 9 extranasal
• Overall 15 dural connection
• CSF rhinorrhea, meningitis possible, if dural
  connection exists

25
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

26
Glioma Development
27
Encephaloceles

• Extracranial herniation of meninges and/or brain
• Connection with subarachnoid space
• Rare at 135,000 births
• 30-40 associated anomalies
• microcephaly, hydrocephalus, microopthalmia,
  anopthalmia, agenesis of the corpus callosum,
  porencephaly, cortical atrophy, ventricular
  dilations

28
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

29
Encephaloceles

• Large nasal encephalocele

30
Encephaloceles

• Divided into three categories
• Occipital 75
• Sincipital 15
• Basal 10
• Sincipital-anterior or frontonasal (dorsum of
  nose, orbits, forehead)
• Basal-intranasal mass, nasopharynx, posterior
  orbit because they herniate through the cribiform
  plate or posterior to it (potential for airway
  obstruction in neonate)

31
Encephaloceles

• Basal Encephaloceles
• Transethmoidal-through cribiform plate into
  middle meatus
• Sphenoethmoidal-extends through cranial defect
  between posterior ethmoids and sphenoid to
  nasopharynx
• Transsphenoidal-presents in nasopharynx
• Sphenomaxillary-through superior and inferior
  orbital fissures to sphenomaxillary fossa

32
Encephalocele

• Development
• Dural projection through fonticulus nasofrontalis
  
• Abnormal closure results in herniated
  meninges/brain
• May be closely related to glioma

33
Encephalocele Development
34
Nasal MassesEvaluation

• Most often infants and children
• Dermoids-fistula tract, hair, pus or sebum,
  midline
• Gliomas-firm, noncompressible, does not
  transilluminate, telangiectasias
• Encephaloceles-soft, 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)

35
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)

36
CT Imaging

• Nasal Dermoid axial ct with dermoid anterior to
  nasal and maxillary bones, no bony dehiscence or
  abnormalities noted

37
Nasal Dermoid with Intracranial Extension
38
CT Imaging

• Encephalocele with defect noted in cribiform
  plate and herniation of brain tissue

39
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

40
MRI Imaging

• Nasal dermoid cyst

41
MRI-Glioma

• Saggital T1

42
MRI-Glioma

• Sagittal T2

43
MRI-Glioma

• Coronal T2

44
MRI-Encephalocele

• Coronal T2 weighted and sagittal T1 weighted
  image of sphenoid encephalocele

45
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

46
Treatment

• Surgical Treatment
• Complete excision (open-transcranial vs
  extracranial, vs endoscopic approaches
  described)
• Perform early to avoid nasal distortion, bony
  atrophy, osteomyelitis, meningitis
• dermoids-must excise entire tract to prevent
  recurrence

47
Dermoid

• Can be removed endoscopically or via open
  approach
• Transverse rhinotomy has been described
• Small to moderately sized lesions
• Avoids vertical scar and splaying

48
Nasal Dermoid with intracranial extension. Meher
R, Singh I, et al. J Postgrad Med 20055139-40.

• 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.

49
Glioma

• Can be removed via open or endoscopic approach
• Lateral rhinotomy or alar incisions may be used
  for intranasal gliomas or combined
  intra-extranasal gliomas
• Several authors have reported isolated cases of
  endoscopic excision of small gliomas with and
  without evidence of intracranial extension.

50
Encephaloceles

• In the past required combined approach with
  neurosurgery
• Frontal craniotomy is performed, intracranial
  mass excised, bone-dura defect is repaired
• Extracranial mass is then removed
• More reports describing endoscopic removal

51
Conclusion

• Midline nasal masses are rare but must be
  remembered in the differential
• Furstenbergs test
• Dont biopsy without imaging
• Surgical intervention necessity

52
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