|Patient: 1 year 6 month old|
|History: 1 year 6 month old patient with nasal mass presents for surgical resection. A sinus tract is seen just below the nose and is noted to drain a non-malodorous white substance.|
Ill-defined soft tissue mass with some central areas of fluid. CT shows some bone destruction.
Location: predominantly anterior to the right frontal bone, extending towards the lower margin of the fonticulus nasofrontalis.
Frontal, extra nasal portion of the mass measured on the order of about 4.3 x 1.7 x 1.8 cm in cephalocaudad, AP and transverse dimensions respective.
Post contrast administration-peripheral enhancement with again some slight ill-definition to margins which could be related to superinfection or trauma. This mass demonstrated restricted effusion on diffusion weighted images.
|Diagnosis: Nasal dermoid|
Congential midline nasal masses- 1:20,000 to 40,000 births. Although rare, these disorders are clinically important because of their potential for connection to the central nervous system. Biopsy of a lesion with an intracranial connection can lead to meningitis or cerebrospinal fluid leak. The treatment of these masses is surgical excision. Preoperative knowledge of an intracranial connection allows for neurosurgical consultation and planning for craniotomy.
The differential of a midline nasal mass includes inflammatory lesions, traumatic deformity, benign neoplasms, malignant neoplasms, and congenital masses.
Dermoid sinus cysts present as a mass on the dorsum of the nose or intranasally, with a pit or sinus tract opening on the nasal dorsum, hair around the external opening, and discharge of pus or sebaceous material.
-They can occur as an isolated cyst or with a sinus tract opening to the skin.
-They constitute 1-3% of all dermoids but 3-12% of dermoids of the head and neck.
-According to the prenasal theory, during normal development a projection of dura protrudes through the fonticulus frontalis or inferiorly into the prenasal space. This projection normally regresses but if it does not the dura can remain attached to the epidermis and result in trapped ectodermal elements.
Nasal gliomas are firm masses which are nonpulsatile, present on the nasal dorsum and/or arise from the lateral nasal wall, do not increase in size with crying, do not transilluminate, have telangiectasias of the overlying skin, and do not enlarge with bilateral compression of the internal jugular veins (Furstenberg test). They can be associated with a widened nose or with hypertelorism secondary to growth of the mass. Sixty percent are extranasal, 30% intranasal, and 10% are both.
-The embryological development of nasal gliomas is similar to nasal dermoids. Abnormal closure of the fonticulus frontalis can lead to an ectopic rest of glial tissue being left extracranially. This is similar to the mechanism for the formation of encephaloceles, however there is not always an intracranial connection to a glioma and there is by definition an intracranial connection to an encephalocele.
Encephaloceles may present as nasal broadening and/or as a blue, pulsatile, compressible mass near the nasal bridge which transilluminates, enlarges with crying or with bilateral compression of the internal jugular veins, or as an intranasal mass arising from the cribriform plate.
-Encephaloceles are extracranial herniations of the meninges and/or brain which maintain a subarachnoid connection. If it contains only meninges it is termed a meningocele, when it also contains brain tissue it is called a meningoencephalocele.
-Ingraham and Matson divided encephaloceles into three categories: occipital, sincipital, and basal. Occipital are the most common at 75%. Sincipital are frontonasal lesions which present as a mass over the nose, glabella, or forehead. The intracranial connection is usually anterior to the cribriform plate. Basal lesions make up about 10% of lesions and present as an intranasal or nasopharyngeal mass. Basal lesions herniate either through the cribriform plate or posterior to it which explains their presentation in the nose instead of externally.
Treatment of nasal dermoids, gliomas, and encephaloceles is by complete surgical excision. Early surgical intervention is recommended to avoid further distortion of the nose or bony atrophy caused by growth of the mass or recurrent inflammation. Other complications are abscess formation, osteomyelitis, and meningitis with those lesions with an intracranial connection. The entire lesion along with any fistulous tract must be excised in order to prevent recurrence. Denoyelle et al. reported a recurrence rate of 5.5%(two of thirty-six patients) for nasal dermoids in their series, both with an external rhinoplasty approach(6).
-The key information necessary for surgical planning is the presence of an intracranial connection to the mass.
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Case Number: 49128313Last Updated: 01-12-2011 The reader is fully responsible for confirming the accuracy of this content.