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

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 1:35,000 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 2005;51:39-40. 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 Furstenberg’s test Don’t biopsy without imaging Surgical intervention necessity

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