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Congenital Midline Nasal Masses

5/26/2012. rhinoplastyman@yahoo.com. 2. Introduction. Dermoid sinuses and cystsGliomasEncephaloceles. 5/26/2012. rhinoplastyman@yahoo.com. 3. Congenital Midline Nasal Masses. 1:20,000 to 1:40,000 birthsAll three have potential intracranial connectionsMay present as a mass on nasal dorsum or as

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Congenital Midline Nasal Masses

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    1. 5/27/2012 rhinoplastyman@yahoo.com 1 Congenital Midline Nasal Masses Professor Sameer Ali Bafaqeeh Otolaryngology Department KSU

    2. 5/27/2012 rhinoplastyman@yahoo.com 2 Introduction Dermoid sinuses and cysts Gliomas Encephaloceles

    3. 5/27/2012 rhinoplastyman@yahoo.com 3 Congenital Midline Nasal Masses 1:20,000 to 1:40,000 births All three have potential intracranial connections May present as a mass on nasal dorsum or as intranasal mass Biopsy can lead to meningitis and CSF leak Treatment is surgical excision

    4. 5/27/2012 rhinoplastyman@yahoo.com 4 Congenital Midline Nasal Masses Most present in infants and children Any unilateral nasal mass in a child should be evaluated for a congenital midline mass

    5. 5/27/2012 rhinoplastyman@yahoo.com 5 Differential Inflammatory lesions Traumatic deformity Benign neoplasms Malignant neoplasms Congenital masses

    6. 5/27/2012 rhinoplastyman@yahoo.com 6 Topics Embryology Dermoid Sinus Cysts Gliomas Encephaloceles Evaluation Surgical Treatment

    7. 5/27/2012 rhinoplastyman@yahoo.com 7 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

    8. 5/27/2012 rhinoplastyman@yahoo.com 8 Congenital Midline Nasal Masses 3-4 weeks neural fold develops Closure occurs from the midline and extends cranially and caudally

    9. 5/27/2012 rhinoplastyman@yahoo.com 9 Congenital Midline Nasal Masses Neural crest cells play a key role in facial development As the neural groove closes neural crest cells migrate around the eyes to the frontonasal process

    10. 5/27/2012 rhinoplastyman@yahoo.com 10 Congenital Midline Nasal Masses In most of the body neural crest cells are involved in ectodermal components In the face the primary role is mesenchymal cells Bone, cartilage, and muscles of the face are all derivatives

    11. 5/27/2012 rhinoplastyman@yahoo.com 11 Congenital Midline Nasal Masses Nose develops from frontonasal process and two nasal placodes Medial processes fuse Nasal-maxillary groove becomes the nasolacrimal duct

    12. 5/27/2012 rhinoplastyman@yahoo.com 12 Congenital Midline Nasal Masses Mesenchymal structures form in centers which fuse Key spaces are the foramen cecum, fonticulus nasofrontalis, and the prenasal space

    13. 5/27/2012 rhinoplastyman@yahoo.com 13 Dermoids Cyst or sinus Most common congenital midline mass 1-3% of all dermoids 3-12% dermoids of head and neck

    14. 5/27/2012 rhinoplastyman@yahoo.com 14 Congenital Midline Nasal Masses Dermoids ectodermal and mesodermal Midline nasal pit, fistula, or infected mass from glabella to columella Sometimes single cutaneous tract with hair at opening May secrete pus or sebaceous material

    15. 5/27/2012 rhinoplastyman@yahoo.com 15 Complications Intermittent inflammation Abscess Osteomyelitis Broaden nasal root Meningitis Cerebral abscess

    16. 5/27/2012 rhinoplastyman@yahoo.com 16 Dermoids CNS connection variably reported from 4-45% Associated congenital anomalies 5-41%

    17. 5/27/2012 rhinoplastyman@yahoo.com 17 Associated Abnormalities Aural atresia, mental retardation, spinal column abnormalities, hydrocephalus, hypertelorism, hemifacial microsomia, albinism, corpus callosum agenesis, cerebral atrophy, lumbar lipoma, dermal cyst of the frontal lobe, coronary artery anomaly, cleft lip and palate, tracheoesophageal fistula, cardiac, genital and cerebral anomalies

    18. 5/27/2012 rhinoplastyman@yahoo.com 18 Development During development a projection of dura projects through the foramen cecum 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

    19. 5/27/2012 rhinoplastyman@yahoo.com 19 Congenital Midline Nasal Masses

    20. 5/27/2012 rhinoplastyman@yahoo.com 20 Gliomas Glial cells in a connective tissue matrix Red or bluish lump Glabella, nasomaxillary suture, intranasal Firm, noncompressible Do not enlarge with crying Do not transilluminate May have telangiectasias

    21. 5/27/2012 rhinoplastyman@yahoo.com 21 Congenital Midline Nasal Masses Extranasal 60% Intranasal 30% Both 10% Dural connection 35% intranasal, 9% extranasal Overall 15% dural connection CSF rhinorrhea, meningitis

    22. 5/27/2012 rhinoplastyman@yahoo.com 22 Congenital Midline Nasal Masses Develop from extracranial rests of glial tissue Abnormal closure of fonticulus nasofrontalis, possibly encephaloceles which have lost CSF connection

    23. 5/27/2012 rhinoplastyman@yahoo.com 23 Encephaloceles Extracranial herniation of meninges and/or brain Subarachnoid connection Rare at 1:35,000 births 1:6000 live births in Southeast Asia and Russia 30-40% associated anomalies: microcephaly, hydrocephalus, microopthalmia, anopthalmia, agenesis of the corpus callosum, porencephaly, cortical atrophy, ventricular dilations

    24. 5/27/2012 rhinoplastyman@yahoo.com 24 Congenital Midline Nasal Masses Encephaloceles

    25. 5/27/2012 rhinoplastyman@yahoo.com 25 Congenital Midline Nasal Masses Encephaloceles Bluish, soft, compressible, transilluminate, pulsatile Enlarge with crying Positive Furstenberg test (bilateral compression of internal jugular veins) Originate medially in the nose May have associated CSF leak

    26. 5/27/2012 rhinoplastyman@yahoo.com 26 Congenital Midline Nasal Masses Encephaloceles Divided into three categories: Occipital 75% Sincipital 15% Basal 10% Sincipital-dorsum of nose, orbits, forehead Basal- intranasal mass, nasopharynx, posterior orbit

    27. 5/27/2012 rhinoplastyman@yahoo.com 27 Sincipital encephaloceles Nasofrontal Nasoethmoidal Nasoorbital

    28. 5/27/2012 rhinoplastyman@yahoo.com 28 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

    29. 5/27/2012 rhinoplastyman@yahoo.com 29 Development Encephaloceles Dural projection through fonticulus nasofrontalis Abnormal closure results in herniated meninges/brain May be closely related to glioma

    30. 5/27/2012 rhinoplastyman@yahoo.com 30 Evaluation Encephaloceles 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

    31. 5/27/2012 rhinoplastyman@yahoo.com 31 Congenital Midline Nasal Masses Encephaloceles Do not biopsy extra or intranasal mass in a child before imaging Risk of meningitis or CSF leak Get imaging before biopsy

    32. 5/27/2012 rhinoplastyman@yahoo.com 32 Imaging Encephaloceles 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 Pensler et al reported that these findings were only valuable if absent, (when present may be false positive)

    33. 5/27/2012 rhinoplastyman@yahoo.com 33

    34. 5/27/2012 rhinoplastyman@yahoo.com 34 MRI Encephaloceles 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

    35. 5/27/2012 rhinoplastyman@yahoo.com 35 Congenital Midline Nasal Masses Encephaloceles Bloom et al 10 patients with nasal dermoids One false positive, one indeterminate CT Recommends MRI as first-line test due to increase cost of multiple tests, delay in diagnosis, additional risk if anesthesia required

    36. 5/27/2012 rhinoplastyman@yahoo.com 36

    37. 5/27/2012 rhinoplastyman@yahoo.com 37 Surgical Treatment Encephaloceles Complete excision Perform early to avoid nasal distortion, bony atrophy, osteomyelitis, meningitis Denoyelle et al reported a recurrence rate of 5.5% of dermoids-must excise entire tract

    38. 5/27/2012 rhinoplastyman@yahoo.com 38 Dermoids Pollock reviewed surgical treatment: Access to all midline cysts and ability to perform medial and lateral osteotomies Exposure for repair of cribiform defects, permit control of CSF rhinorrhea Allow reconstruction of nasal dorsum Offer probability of a favorable scar

    39. 5/27/2012 rhinoplastyman@yahoo.com 39 Congenital Midline Nasal Masses Dermoids Transverse rhinotomy Small to moderately sized lesions Avoids vertical scar and splaying

    40. 5/27/2012 rhinoplastyman@yahoo.com 40 Transverse Rhinotomy Fistulous opening excised with transverse fusiform incision Tract cannulated with lacrimal probe Second incision made over dermoid

    41. 5/27/2012 rhinoplastyman@yahoo.com 41 Tripod-eversion rhinotomy Larger lesions, especially lower 2/3 of nose Transverse columellar incision, transfixion incision carried laterally and between upper and lower lateral cartilages

    42. 5/27/2012 rhinoplastyman@yahoo.com 42 Congenital Midline Nasal Masses Paraalar incisions will permit upward rotation of the nose Tract opening-fusiform excision Cannulate fistulous tract Operating microscope may be used to improve visualization

    43. 5/27/2012 rhinoplastyman@yahoo.com 43 Zig-Zag rhinotomy Large lesions Underlying bone or cartilage damage Known intracranial extension Provides wide exposure Scar improved over straight vertical rhinotomy

    44. 5/27/2012 rhinoplastyman@yahoo.com 44 Congenital Midline Nasal Masses Incisions designed with limbs which extend vertically >40 degrees <90 degrees Fusiform excision of fistulous tract opening Scar improved over straight vertical rhinotomy, limbs less than 90 degrees to RSTL running horizontally across the nose

    45. 5/27/2012 rhinoplastyman@yahoo.com 45 Congenital Midline Nasal Masses Rohrich recommends open rhinoplasty for the following reasons: Improved aesthetic results Ease of exposure Wide exposure of entire nasal dorsum More control over osteotomy Better visualization of the cribiform plate

    46. 5/27/2012 rhinoplastyman@yahoo.com 46 Congenital Midline Nasal Masses Weiss et al described two cases of endoscopic dermoid excision in lesions with little or no skin involvement

    47. 5/27/2012 rhinoplastyman@yahoo.com 47 Congenital Midline Nasal Masses Inferior portion removed via bilateral intercartilaginous and membranous septum incision Aufricht retractor in place 0 and 30 telescope sinus tract followed to defect

    48. 5/27/2012 rhinoplastyman@yahoo.com 48 Congenital Midline Nasal Masses Lateral rhinotomy may be used for intranasal gliomas or combine intra-extranasal Several authors have reported isolated cases of endoscopic excision of small gliomas without evidence of intracranial extension.

    49. 5/27/2012 rhinoplastyman@yahoo.com 49 Encephaloceles Will require combined approach with neurosurgery Frontal craniotomy is performed, intracranial mass excised, bone-dura defect is repaired Extracranial mass is then removed Turgut et al reported mortality of 46% when there is brain tissue in the encephalocele

    50. 5/27/2012 rhinoplastyman@yahoo.com 50 Key Points Midline nasal masses are rare but must be remembered in the differential Dont biopsy without imaging Furstenbergs test

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