Objectives/hypothesis: Multiple spontaneous nasal meningoencephaloceles in the same patient are rare lesions. Although many skull base defects occur after prior trauma or surgery, otolaryngologists must be aware of the potential for spontaneous encephaloceles. We present our experience with this unusual condition and discuss its pathophysiology and unique management issues.
Study design: Retrospective.
Methods: Review of medical records, radiographic images, and cerebrospinal fluid pressures.
Results: We identified 5 patients with multiple, simultaneous, spontaneous encephaloceles: 4 patients with 2 encephaloceles and 1 patient with 3 encephaloceles (11 in all). Locations of the 11 encephaloceles were sphenoid lateral recess (6), frontal sinus with supraorbital ethmoid extension (2), ethmoid roof (1), frontal sinus (1), and central sphenoid (1). Three patients had bilateral sphenoid lateral recess encephaloceles, accounting for all six in that location. All four patients with available radiographic studies demonstrated empty sella turcica. Surgical approaches included endoscopic transpterygoid approach to the lateral sphenoid recess (3), endoscopic approach to ethmoid and central sphenoid (3), and osteoplastic flap with frontal sinus obliteration (2). We had 100% success at latest endoscopic follow-up (mean period, 17 mo). Three patients had postoperative lumbar punctures with mean cerebrospinal fluid pressure of 28.3 cm water (range, 19-34 cm; normal range, 0-15 cm).
Conclusions: Multiple spontaneous encephaloceles can be managed safely and successfully using endoscopic and extracranial approaches. A high index of suspicion for this diagnosis must be maintained, especially in patients with radiographic evidence of laterally pneumatized sphenoid sinuses or empty sella. Spontaneous encephaloceles and cerebrospinal fluid leaks represent a form of intracranial hypertension.