• SSx: nasal pit present in 45% to 84%, may contain tuft of hair; masses are firm, nontender, noncompressible, nonpulsatile, and do not transilluminate

• Dx: CT and/or MRI to evaluate for intrancranial extension, negative Furstenburg test

• Complications: repeat infection with cellulitis and abscess formation, nasal skin necrosis, cartilaginous and bony destruction, recurrent meningitis, CSF leak

• Rx: early complete excision; requires removal of entire cyst and tract, along with involved bone, cartilage, and septum; open rhinoplasty approach advocated; allows for wide exposure of the entire nasal dorsum, controlled external osteotomies, and improved cosmesis

Nasal Glioma

• Present shortly after birth but may present later in life

• Composed of ectopic glial tissue

• Intracranial connection for 20% of gliomas retained through fibrous stalk

• Histology: demonstrates mass covered by normal nasal mucosa or skin and contains astrocytic neuroglial cells with fibrous and vascular connective tissue

• Pathophysiology: sequestered glial tissue, results in unencapsulated collection of heterotopic glial cells

• Location: 60% external, 30% internal, 10% combined, not typically midline

• SSx: firm, nontender, noncompressible, does not transilluminate; external gliomas are red and may have overlying telangiectasias; intranasal masses glistening gray or purple arising from lateral aspect of the nose

• Dx: CT and/or MRI, negative Furstenberg test

• Complications: cosmetic deformity, nasal obstruction, meningitis

• Rx: complete surgical excision; external nasal approach or lateral rhinotomy for extracranial gliomas; gliomas with significant intracranial extension may require craniotomy with exploration of the anterior cranial fossa

Encephalocele

• Congenital herniation of CNS tissue through a skull base defect

• Incidence: 1 per 4000 live births

1. Meningocele: meninges only

2. Meningoencephalocele: meninges and brain

3. Meningoencephalocystocele: meninges, brain, and part of the ventricular system

• Types by Location

1. Posterior/occipital: most common, 75% to 80% of encephaloceles

2. Anterior: subdivided into sincipital and basal types

– Sincipital (frontoethmoidal): defect between frontal and ethmoid bones at foramen cecum; three types

a. Nasofrontal: glabellar lesion

b. Nasoethmoid: lateral nose lesion

c. Nasoorbital: medial orbital wall lesion

– Basal: Four types

a. Transethmoidal

b. Sphenoethmoidal

c. Transsphenoidal

d. Sphenomaxillary

• Histologically consist of meningeal sac containing glial tissue

• Pathophysiology: failed closure of neuropore or failed migration of neural crest cells results in ependymal-lined meninges herniating through the base of the skull; communicates with the subarachnoid space (CSF filled)

• SSx: external encephaloceles present at or near the glabella as soft compressible masses; intranasal lesions bluish, pedunculated masses medially and high in the nasal cavity; masses compressible and pulsatile, may enlarge with crying or internal jugular vein compression (positive Furstenberg sign)

• Dx: CT and/or MRI reveals bony defect, positive Furstenberg sign

• Complications: meningitis, nasal obstruction, cosmetic deformity, hydrocephalus

• Rx: surgical resection and repair of skull base defect to prevent CSF rhinorrhea or further herniation

1. Endoscopic repair: transnasal approach, encephalocele bipolared to skull base defect; multi-layer closure performed with many options; classically, fat underlay, fascia lata underlay, +/− bone graft, with free mucosal graft or pedicled nasoseptal flap overlay

2. Intrathecal fluorescein aids in identification of encephalocele or CSF leak, 0.1 ml of 10% fluorescein in 10 ml of patient’s CSF infused at 1 cc/min

• Primary congenital tumors that arise from all three embryonic germ layers

• Incidence: 1 in 4000 live births

• Most common locations: sacrococcygeal, retroperitoneal, mediastinal, and head and neck

• Head and neck teratomas comprise 2% to 9% of all teratomas; cervical and nasopharyngeal most common

• Occur in children <1 year of age

• SSx: propensity for rapid growth, can cause dyspnea at birth, intermittent airway obstruction, and dysphagia with pedicled lesions

• Imaging: calcifications pathognomonic, seen in 15% of cases; CT helps delineate mass and potential intracranial extension; MRI provides additional detail

• Rx: secure airway initially, surgical excision recommended given rapid growth and small risk of malignant transformation

Cysts

Nasoalveolar Cyst (Klestadt’s Cyst)

• Pathogenesis: arises from nasal epithelium trapped in a cleft between the maxillary medial and lateral nasal processes; may also arise from displaced nasolacrimal duct tissue

• Female preponderance 3:1

• Right and left sides equally affected, 10% bilateral

• Histology: cyst composed of mature, condensed connective tissue lined by pseudostratified columnar epithelium with or without goblet cells

• SSx: facial asymmetry, unilateral nasal obstruction or pain; effacement of nasolabial fold, nose displaced anteriorly, gingivobuccal sulcus obliterated on involved side; firm, non-tender, fluid-filled mass

• CT shows homogenous, non-enhancing cystic mass anterior to the pyriform aperture; bony remodeling in long-standing lesions

• Rx: surgical excision; traditional excision via sublabial incision; transnasal endoscopic marsupialization of the cyst into the nasal cavity shows good results

Nasopalatine Duct Cyst

• Incidence: 1% of population; most common nonodontogenic cyst

• Presents between fourth and sixth decades; more common in males

• Histology: cyst lined with respiratory epithelium superiorly by the nasal cavity, followed by cuboidal and then squamous epithelium closer to oral cavity

• SSx: usually asymptomatic; hard palate swelling, pain, drainage, nasal obstruction

• Imaging: CT shows well-circumscribed round, ovoid, or heart-shaped lesions of the maxilla

• Rx: surgical enucleation with 0% to 11% recurrence rate

Nasolacrimal Duct Cyst (Dacryocystocele)

• Pathophysiology: failure of opening of the distal nasolacrimal duct with crust formation from accumulation of secretions

• Location: anteriorly in the inferior meatus along nasolacrimal duct

• SSx: commonly asymptomatic, epiphora, nasal obstruction; respiratory distress and difficulty feeding in infants with large obstructive cysts (obligate nasal breathing)

• Dx: nasal exam, nasal endoscopy, CT, or MRI

• Rx: endoscopic marsupialization for symptomatic cysts (presence of infection, respiratory obstruction, feeding difficulties)

Thornwaldt’s Cyst

• Pathogenesis: during sixth week of fetal development, notochord begins to regress from its most cephalic extent; transient communication may develop between the notochord and the primitive foregut (pharynx); communication provides a pathway for ingrowth of respiratory epithelium and formation of a potential space in the midline; obstruction of the nasopharyngeal opening of this pharyngeal bursa creates Thornwaldt’s cyst

• Location: midline nasopharynx

• SSx: usually asymptomatic, post nasal drainage, ear fullness or pain, serous otitis media, headache, cervical myalgia; exam midline smooth submucosal mass in the midline nasopharynx

• Imaging: CT imaging midline mass high in the nasopharynx between longus capitis muscles, containing low density fluid that does not enhance; MRI high T1 and T2 signal

• Rx: observation, endoscopic marsupialization for symptomatic cysts

Rathke’s Pouch Cyst

• Lined by a single layer of epithelium, contain goblet cells

• Commonly found in fifth to sixth decades of life; more common in females; often incidental

• SSx: commonly asymptomatic, enlarging cysts compress adjacent structure including the pituitary gland, optic chiasm, or hypothalamus causing headaches, visual impairment, and endocrinopathy

• Imaging: MRI well circumscribed, cystic masses with thin wall in the sella turcica; may have suprasellar component and generally displace the pituitary gland and stalk posteriorly, T1 hypointense, T2 hyperintense

• Rx: endoscopic transsphenoidal approach with cyst marsupialization and drainage

• Dx: Generally treated without laboratory testing; serologic, tissue culture, PCR assays available

• Rx: supportive, rest, hydration, topical/systemic decongestants, mucolytics, nasal irrigation; no role for steroids (topical and systemic)