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Special Section: Giant Congenital Nevus

Giant Mucocele of the Frontal Sinus

Galiè, Manlio MD, DMD, PhD; Mandrioli, Stefano MD; Tieghi, Riccardo MD; Clauser, Luigi MD, DMD, PhD

Author Information
Journal of Craniofacial Surgery: September 2005 - Volume 16 - Issue 5 - p 933-935
doi: 10.1097/01.scs.0000168999.20258.ca
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Abstract

Mucoceles of the frontal sinus are relatively uncommon lesions of benign entity that occur with approximately equal frequencies in adult males and females, with the highest incidence in the third and fourth decades. They are uncommon in pediatric subjects.1,2

Mucoceles are slow growing and locally aggressive lesions that occur as a result of accumulation and retention of mucous secretions in the sinus caused by the loss of draining properties of the mucous epithelium of the sinus.2-6 The fluid content progressively obliterates the sinus, and the pressure so generated leads to gradual erosion and distortion of the anterior and posterior bone walls.7-10 The lesion may extend to the orbital and intracranial structures and lead to meningitis, brain abscess, or cerebrospinal fluid (CSF) fistulas.9,11,12

Because of the proximity of mucoceles to the brain, progress in volume may cause morbidity and potential mortality.13

The etiology may be multifactorial: trauma, allergy, inflammation, anatomic abnormality, previous surgery, osteoma, fibrous dysplasia, or ossifying fibroma.2-4,9,6

The main symptoms of orbital involvement are pain, swelling, exophthalmos, diplopia, and loss of vision. Proptosis is usually the main complaint. Oculomotor nerve palsy with ptosis is rare, but it can be seen in patients with frontal mucocele.14

Diagnosis is based on a clinical investigation conducted with the aid of computed tomography (CT) scans and magnetic resonance imaging (MRI). The CT scan is the main diagnostic assessment tool used for determining regional anatomy and extent of the lesion, in particular the intracranial expansion and the scope of bone erosion.7 MRI is helpful in making a definitive diagnosis because gadolinium enhancement on MRI differentiates mucoceles from neoplasms.15 Surgery is the only effective treatment and may range from functional endoscopic sinus surgery to craniotomy and craniofacial exposure with or without obliteration of the sinus.13,16-22

CASE REPORT

A 72-year-old man presenting with a large right frontal lesion that caused proptosis and diplopia was referred to the Department of Cranio-Maxillo-Facial Surgery of St. Anna University Hospital. The mass had slowly grown over the course of 2 years, restraining the globe downward and causing limitations in ocular movements (Fig 1). It was not painful and CSF leak was noted. CT and MRI scan showed a lesion arising from the right frontal sinus with erosion of the anterior and posterior walls, orbital roof, and ethmoid with intracranial extension and dura involvement (Fig 2).

F1-39
Fig 1:
A 72-year-old man with a large right frontal mass causing dystopia, proptosis, and diplopia.
F2-39
Fig 2:
Computed tomography and magnetic resonance imaging scan showing erosion of the anterior, posterior, orbit, and ethmoid with downward displacement of the globe.

A combined one-stage maxillofacial-neurosurgical approach was then performed. Intravenous antibiotics were administrated perioperatively and continued until the fifth postoperative day. Coronal incision with subperiosteal dissection and right fronto-orbital craniotomy was carried out. The mucocele consisted of mucopurulent fluid, and the ostium of the sinus was blocked by secretions. The anterior and posterior walls, the orbital roof, the ethmoid, and intracranial portion of the dura were eroded.

The sinus mucosa was completely removed, and the sinus and nasofrontal duct were obliterated with pericranial galeal flap, fibrin glue, and temporal muscle patch. The bony repair was carried out with bone grafts harvested from calvaria. The bone grafts were fixed to the residual frontal walls by microplates. Finally, the dura was repaired with temporalis muscle fascia sealed with fibrin glue. The postoperative phase was normal, and all preoperative signs and symptoms resolved after surgery.

A CT scan performed at 6 months postoperatively showed no evidence of recurrence or resorption of bone grafts. Two years after surgery, the result is satisfactory both morphologically and functionally (Fig 3).

F3-39
Fig 3:
The patient at 2 years after surgery. Morphologic and functional results have been obtained. Some right periorbital refinements are planned in the future.

DISCUSSION

Frontal sinus mucoceles represent a relatively rare, slow-growing pathology, usually clinically silent, caused by the loss of drainage properties of the mucosa of the sinus. They can involve the orbit, the anterior cranial fossa, and intracranial content. Some authors23,24 describe the role of cytokines as osteolytic factors involved in the disruption of the surrounding structures.

Patients with extensive cranio-orbital mucoceles with intracranial involvement require major surgery with restoration of the normal cranio-orbital anatomy and function. The surgical procedure should be performed in one stage through a coronal subperiosteal approach with craniotomy and wide exposure of the fronto-orbito-ethmoid region. Management of the frontal sinus should be followed by cranialization and accurate removal of the mucosa, obliteration of the nasofrontal duct with vascularized pericranial-galeal flap and temporal muscle, and immediate dura repair. Reconstruction of the missing bones can be achieved by using cranial bone. Endoscopic sinus surgery should be the treatment of choice for noninvasive frontal mucocele.

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Keywords:

Giant mucocele; frontal sinus; bone grafting; craniofacial surgery

© 2005 Mutaz B. Habal, MD