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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 Grand Rounds June 7, 2006 Outline Outline Embryology of the nose Dermoid cysts Glioma Encephalocele/Meningocele Evaluation of Nasal Mass Imaging Studies Surgical Intervention Conclusion Embryology 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 n Corpus callosum agenesis Tracheo­ esophagea 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 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% Intranasal ­ 30% Both ­ 10% 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% Sincipital 15% 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 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 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 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 Bibliography Agirdir B, Derin A, Ozbilim G, et al. Endoscopic management of intranasal glioma. J pediatric Surg 2004;39:1571­3. Bilkay U, Gundogan H, Ozek C, Tokat C, Gurler T, Songur E, et al. Nasal dermoid sinus cysts and the role of open rhinoplasty. Ann Plast Surg 2001;47:8­14 Bloom DC, Carvalho DS, Dory C, Brewster DF, Wickersham JK, Kearns DB. Imaging and surgical approach of nasal dermoids. Int J Pediatr Otorhinolaryngol 2002;62:111­22 Brown K, Brown OE. Congenital Malformations of the nose. In: Cummings CW, ed. Pediatric Otolaryngology Head & Neck Surgery 3rd edition, St. Louis, Mosby; 1998:92­98 Burckhardt W, Tobon D. Endoscopic approach to nasal glioma. Otolaryngology­Head and Neck Surgery 1999;120:747­748. Clark WD, Bailey BJ, Stiernberg CM. Nasal dermoid with intracranial involvement. Otolaryngol Head Neck Surg 1985;93:102­4 Denoyelle F, Ducroz V, Roger G, Garabedian EN. Nasal dermoid sinus cysts in children. The Laryngoscope 1997;107:795­800. Dimov P, Rouev P, Tenev K, Krosneva R, Valkanov P. Endoscopic surgery for removal of a nasal glioma: case report. Otolaryngol Head Neck Surg 1991;124:690. Edward J. Wladis, MD ∙ Rudolph S. Wagner, MD. Dermoid cysts in children. Pediatric Ophthamology. Tutorial. www.ophthalmic.hyperguides.com Haafiz AB, Sharma R, Faillace WJ. Congenital Midline Nasofrontal Mass. Clinical Pediatrics September 1995:482­486 Hayashi T, Utsanomiya H, Hashimoto T. Transethmoidal encephalocele. Surg Neurol 1985; 24: 651­655 Huisman TA, Schneider JF, Kellenberger CJ et al. developmental nasal midline masses in children:neuroradiological evaluation. Eur Radiol 2004; 14(2):243­49. Bibliography Bibliography Ingraham FD, Matson DD. An unusual nasopharyngeal encephalocele. New Eng J Med 1943;228:815­820 Mahapatra AK, Tandon PN, Dhawan IK, Khazanchi RK. Anterior encephaloceles. A report of 30 cases. Childs Nervs Syst 1994; 10: 501­504 Mahapatra AK. Anterior encephaloceles. Indian J Pediatr 1997; 64: 699­704. Marxhall AH, Jones NS, Robertson, IJ. Endoscopic repair of basal encephaloceles. J Laryngol Otol 2001;115:545­47. Meher R, Singh I, Aggarwal S. Nasal dermoid with intracranial extension. J Postgrad Med 2005;51:39­40. Pensler J, Bauer B, Naidich T. Craniofacial dermoids. Plast Reconstr Surg 1988;82:953­958. Pollock RA. Surgical approaches to the nasal dermoid cyst. Annals of Plastic Surgery. 1983;10:498­501. 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Nasal dermoid sinus cysts: Association with intracranial extension and multiple malformations. Cleft Palate Craniofac J 1991;28:87­95 Bibliography Bibliography Weiss DD, Robson CD, Mulliken JB. Transnasal endoscopic excision of midline nasal dermoid from the anterior cranial base. Plastic and Reconstructive Surgery 1998;101(6):2119­2123. Woodsworth BA, Schlosser RJ, Faust RA, et al. Evolutions in the management of congenital intranasal skull base defects. Arch Otolaryngol Head Neck Surg 2004;130:1283­88. Yokoyama M, Inouye N, Mizuno F. Endoscopic management of nasal glioma in infancy. Int J Pediatr Otorhinolaryngol 1999;51:51­54. Zerris VA, Annino D, Heilman CB. Nasofrontal dermoid sinus cyst: Report of two cases. Neurosurgery 2002;51:811­4 ...
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