Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Congenital Midline Nasal Masses Congenital midline nasal masses are rare anomalies. Although usually detected at birth, they may not be recognized until well into adulthood. Nasal dermal sinus cysts, nasal gliomas, and encephaloceles constitute the great majority of these lesions. Complications associated with these lesions may be severe, particularly if intracranial extension exists. Local abscess formation, cellulitis, and cosmetic deformity are frequent. CSF leakage, frontal lobe abscess formation, and meningitis are not uncommon. Nasal dermoids are by far the most common lesion, followed by gliomas and encephaloceles. Since the process occurs early in embryogenesis, the pathogenesis of these lesions remains obscure. The "prenasal theory" cites alterations in embryogenesis as responsible for the development of these lesions. Faulty closure of the anterior neuropore can result in a defect in the fonticulus frontalis, foramen cecum, cribriform plate, sphenoid and ethmoid bones. Development of nasal dermal sinus cysts is understood to result from faulty involution of the dural tract. If brain tissue is isolated extracranially by fusion of the cranial sutures, a glioma results. When a bony defect allows herniation of dura mater and brain tissue extracranially, an encephalocele results. The differential diagnosis includes developmental anomalies, inflammatory lesions and benign or malignant neoplasms. Nasal gliomas are CNS masses which have lost their intracranial connection. First described in 1952 by Reid, nearly 150 cases have been reported. They occur with a male-to-female ratio of three-to-two. No familial predisposition or association with anomalies has been reported. Nasal gliomas tend to grow in proportion to the child and malignant degeneration has not occurred. Twenty percent of nasal gliomas are in communication with the intracranial space via a fibrous stalk. Clinically they occur extranasally in 56% of cases, intranasally in 33%, and combined in 11% of cases. On examination, extranasal gliomas are firm, lobular, and noncompressible, ranging in size from 1 cm to 5 cm, usually off midline over the root of the nose. The overlying skin may be normal, red or bluish, with or without telangiectasias. Intranasal gliomas may present as a mass protruding from the nostril, or as a pale, firm, glistening polypoid mass within the nasal cavity or nasopharynx. Symptoms include nasal obstruction, epistaxis, or CSF rhinorrhea. The base most often arises from the lateral nasal wall. Encephaloceles were first described in the sixteenth century. Both sexes are affected equally, and malignant degeneration does not occur. Twenty-five percent of all cranial encephaloceles occur anteriorly, of which 15% are sincipital and 10% are basal. Sincipital lesions are extracranial and are further divided into nasofrontal, nasoethmoidal, and naso-orbital. Basal lesions are divided into spheno-orbital, sphenomaxillary, and sphenopharyngeal. Clinically, encephaloceles are soft, compressible, and appear somewhat bluish. They vary in size and may change with crying or head position. The Forsterburg test is usually positive and spontaneous pulsations may be seen. Encephaloceles may leak CSF spontaneously and a history of recurrent meningitis may be obtained. Extranasal encephaloceles extend through the fonticulus frontalis, whereas intranasal encephaloceles extend through the cribriform plate. On examination an intranasal encephalocele always arises medially. Nasal dermal sinus cysts account for 10% of all dermal cysts of the head and neck and 1% to 3% overall. The sex ratio is equal. In rare cases an autosomal dominant mode of inheritance has been suggested. Malignant degeneration has not been described and the incidence of associated anomalies is quite low. Extradural communication occurs in 10% of cases. Clinically, a sinus tract exists which opens to the skin of the nose anywhere between the base of the columella and the glabella, with the distal one-third of the nasal dorsum the most common site. Nasal dermal sinus cysts are firm, noncompressible, nonpulsatile masses which do not transilluminate. A cheesy material may be expressed from the cyst and small hairs may protrude from the dermal opening. Preoperative evaluation requires imaging studies to delineate any intracranial extension. Reported findings which suggestintracranial extension include a soft tissue mass, widened nasal septum, bifid septum, bifid perpendicular plate, bifid crista galli, interorbital widening, and defects in the cribriform plate. Polytomography and CAT of the skull base with coronal plane scanning is the most accurate method of defining bony defects of the skull. The role of MRI has been established, citing superior soft tissue contrast, noninvasiveness, lack of ionizing radiation, and easy multidimensional reconstruction. MRI is also effective in delineating intracranial extension of congenital midline nasal masses. Regardless of preoperative evaluations, the decision regarding the surgical resection should observe the known embryologic development of these lesions with respect to possible intracranial extension . All explorations should take place in the operating room under general anesthesia and neurosurgical backup should be arranged in the event that intracranial extension is identified at surgery. If intracranial extension is identified preoperatively, the stage procedure should be craniotomy with resection of intracranial cysts and repair of dural defects. Early excision is advocated by all authors to avoid craniofacial deformation, infection, and potential intracranial complications. Several incisions have been described, with midline and open rhinoplasty approaches the most popular. Incomplete excision attributes to the 20% recurrence rate. Thus several surgeons advocate the use of microsurgical instruments to aid in total excision. Case Presentation A 5-year-old African American male presented to the Emergency Room with a three-day history of fever and progressive irritability. On initial examination the child was febrile and nuchal rigidity was detected. A meningitis work-up was undertaken. Lumbar puncture yielded polymorphonuclear cells, an elevated fluid protein and low fluid glucose. Intravenous antibiotics therapy (Cefuroxime) was instituted and a CAT scan of the brain was obtained to evaluate a ventriculoperitoneal shunt which had been placed in early infancy for congenital hydrocephalus. Axial images revealed incidental ethmoid sinus and right maxillary sinus opacification and suggested the presence of a right ethmoidal mucocele. Endoscopic evaluation in the operating room revealed a large, smooth, slightly compressible, bluish mass arising from the roof of the nasal cavity. Coronal MR imaging demonstrated a large, homogeneous mass extending into the right nasal cavity in continuity with the brain, consistent with a basilar encephalocele. The child subsequently underwent a transcranial resection with reconstruction of the frontal floor with a pericranial flap, and has done remarkably well postoperatively. Bibliography Badrawy R. 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