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Midline Nasal Masses


Introduction

Midline nasal masses and nasolacrimal duct cysts (NLDC) are pathologies that afflict infants and children. The pathophysiology of the midline nasal masses and NLDC is distinct, and the treatment of each of them is just as distinct. When a child presents with a mass near or within the nose, congenital nasal masses and NLDC are in the differential diagnosis.

NLDC are intranasal cysts occurring in the inferior meatus. NLDC occur when a dacryocystocele expands into the nasal cavity under the inferior turbinate. A dacryocystocele forms when there is distal obstruction of the valve of Hasner and proximal dysfunction of the valve of Rosenmeuller. NLDC should be ruled out in any infant presenting with a dacryocystocele, dacryocystitis, or nasal obstruction.

Congenital midline nasal masses consist of multiple pathologies, but for the purposes of this chapter, I will discuss nasal dermoid cyst, glioma, and encephalocele, due to their similarities in anomalous embryogenesis of the anterior neuropore. These masses can present as an external or intranasal mass, and there can be intracranial extension. Other lesions included in the differential diagnoses are tumor, hemangioma, and vascular malformation.

Nasal dermoid cyst is a congenital cyst that can occur externally anywhere from the tip of the nose to the glabella and internally, along the nasal septum, prenasal space, at the level of the skull base near the foramen cecum and crista galli, and intracranially. This is the most common midline nasal mass, and theoretical embryogenesis is related to failure of dural and dermal precursors to detach from one another, resulting in dermal elements becoming trapped. Intracranial extension of this mass occurs about 20% of the time.

Glioma is also known as glial heterotopia. Gliomas are composed of neural tissue without direct intracranial connection, although they may connect to the intracranial space through a fibrous stalk. These masses commonly occur near the glabella and can be midline or off midline externally. Externally, they can be smooth, noncompressible masses, but intranasally they can also occur as a pale mass.

Unlike nasal dermoid cysts or glial heterotopia, encephaloceles have a communication with the intracranial space. An encephalocele will contain cerebrospinal fluid, meninges, and neural tissue herniating through a defect in the skull base. This can result in an external or intranasal mass. Encephaloceles can occur through many different defects in the skull base. Frontonasal encephaloceles are those occurring via a defect in fonticulus frontalis, while nasoethmoidal encephaloceles protrude via the foramen cecum. Other encephaloceles occur in various regions of the skull base.

Key Operative Learning Points

  • Always assess the contralateral nasal cavity for an occult NLDC.

  • Preoperative imaging is mandatory in the assessment of congenital nasal masses and allows for a more accurate surgical plan.

  • A variety of approaches could be used in each mass encountered. Consider using the approach that best allows visualization, exposure, and minimization of morbidity. Sometimes a combination of approaches provides the best solution.

  • Do not biopsy a midline nasal mass prior to imaging studies.

Preoperative Period

History

  • When was the mass recognized? How long has it been present? Most midline nasal masses will be noticed at birth but not necessarily. External nasal masses can be more easily seen, but even external nasal dermoid cysts can be present but escape notice by the parents. NLDC are located within the nasal cavity and will usually not present unless they obstruct the nasal cavity.

  • What color is the mass?

  • Is there any nasal obstruction or breathing problems?

  • Is there difficulty feeding?

  • Does the patient have epiphora, eyelid swelling, redness?

  • Has there been any recent infection?

  • Has there been any trauma to that area of the face?

  • Is there an opening or pit in the skin? Has drainage from it been noticed?

  • Is there any change to the mass induced by crying (i.e., increased intracranial pressure)?

Physical Examination

  • NLDC

    • Facial examination: The facial examination will often be abnormal, especially if the infant is presenting with dacryocystitis. If there is no dacryocystitis, there can still be a mild abnormal swelling near the medial canthus of the eye.

    • Nasal examination: NLDC will be visualized in the inferior meatus. When it is associated with acute infection, the cyst can be more forceful and displace the inferior turbinate superiorly ( Fig. 190.1 ).

      Fig. 190.1, Intranasal endoscopy demonstrating a large nasolacrimal duct cyst (arrow) in the inferior meatus causing superior-medial displacement of the inferior turbinate (asterisk) .

    • Ocular examination: This is most important when there is an infected dacryocystocele. This can cause erythema and edema of the inferior eyelid, and a proper ophthalmologic examination is important to rule out other ocular pathology.

  • Congenital nasal mass

    • Examination of the face: A mass in the face can often by missed by parents. It is important to look for a mass along the dorsum of the nose from the nasal tip to the glabella. Most often the mass will be in the midline, but paramedian locations can also occur. Hypertelorism can also occur. Discoloration of the lesion is more consistent with a glioma. A positive Furstenberg sign occurs if crying or occlusion of the jugular vein results in enlargement of the mass. This suggests that there is an intracranial communication related to the mass. The presence of a pit or sinus in the skin, with or without a hair, suggests a nasal dermoid cyst is suspected ( Fig. 190.2A and B ).

      Fig. 190.2, A, Preoperative photograph of child with active drainage from the midline sinus tract of a nasal dermoid cyst. B, Small punctum noted near nasal tip (arrow) .

    • Examination of the nose: It is always important to make sure that there is no evidence of an intracranial portion of the mass. Some masses can be solely intranasal, some combined intranasal and extranasal, and some entirely extranasal.

    • Examination of the oral cavity: In very large midline nasal masses, there can be extension of the mass into the oral cavity. Examine for a cleft in the hard or soft palate, as it could be an associated defect of the midline.

    • Examination of the eyes: This is usually normal, but assessment for hypertelorism is important.

Imaging

  • NLDC

    • Imaging studies are not warranted for most presentations of NLDC, as there will be a cyst visualized in the middle meatus in a patient presenting with nasal obstruction or dacryocystitis. If the presentation seems atypical, however, imaging with facial computed tomography (CT) or magnetic resonance imaging (MRI) is recommended.

  • Midline nasal mass

    • Plain films: not indicated

    • Ultrasound: not indicated

    • Computed tomography scan of the face and brain: Computed tomography (CT) highlights the ossified bony anatomy and can also depict the mass. The anterior midline skull base is not ossified at birth, and interpretation of the integrity of the skull base requires knowledge of the normal ossification pattern and timing of the anterior skull base ( Fig. 190.3 ).

      Fig. 190.3, Computed tomography scan of the skull base in a child with a congenital nasal mass. Arrow points to the region of the anterior skull base that is not ossified.

    • Magnetic resonance imaging of the face and brain: Magnetic resonance imaging (MRI) is the imaging modality of choice for frontonasal congenital masses. MRI differentiates tissue type better than CT, can more likely identify a tract, and can assess for concomitant intracranial anomalies.

Indications

  • NLDC: Once diagnosed, surgery is recommended.

  • Nasal dermoid cyst: nasal mass that causes deformity and may carry the risk of meningitis if there is an intracranial connection

  • Nasal glioma: nasal mass

  • Nasal encephalocele: nasal mass carrying the risk of meningitis and progressive craniofacial deformity

Contraindications

  • Severe medical comorbidities that prevent general anesthesia

  • Active infection with/without meningitis for glioma, dermoid, or encephalocele

Preoperative Preparation

  • NLDC: Consultation with an Ophthalmologist is recommended. This will allow concurrent probing of the lacrimal duct and the placement and management of lacrimal duct stents.

  • Midline nasal mass: Preoperative imaging is mandatory, and this allows proper surgical planning. The preoperative imaging guides the approach and the extent of surgery. As such, consider consultation with neurosurgery and craniofacial surgery as indicated.

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