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An unusual pterygopalatine meningocele associated with neurofibromatosis type 1. Case report
P H Chapman1, H D Curtin, M J Cunningham
1Division of Neurosurgery, Massachusetts General Hospital and Harvard Medical School, Boston 02114, USA.
Abstract:
The authors describe an unusual meningocele of the lateral wall of the cavernous sinus and the anterior skull base in a young patient with typical stigmata of neurofibromatosis Type 1 (NF1). This lesion was discovered during evaluation for recurrent meningitis. It represented an anterior continuation of Meckel's cave into a large cerebrospinal fluid space within the lateral wall of the cavernous sinus, extending extracranially through an enlarged superior orbital fissure into the pterygopalatine fossa adjacent to the nasal cavity. It was successfully obliterated, via an intradural middle fossa approach, with fat packing and fenestration into the subarachnoid space. This meningocele most likely represents a variant of cranial nerve dural ectasia occasionally seen in individuals with NF1. It has as its basis the same mesodermal defect responsible for the more common sphenoid wing dysplasia and spinal dural ectasias identified with this condition. Involvement of the trigeminal nerve with expansion of the lateral wall of cavernous sinus has not been reported previously. The authors surmise, however, that it may be present in some cases of orbital meningocele associated with sphenoid wing dysplasia.
Insights
A rare meningocele in the cavernous sinus was found in a patient with neurofibromatosis Type 1 (NF1). This cerebrospinal fluid leak was successfully treated, offering insights into NF1-related cranial nerve abnormalities.
Area of Science:
- Neurosurgery
- Radiology
- Genetics
Background:
- Neurofibromatosis Type 1 (NF1) is a genetic disorder associated with various tumors and skeletal abnormalities.
- Cranial nerve dural ectasia, including sphenoid wing dysplasia and spinal dural ectasia, are known manifestations of NF1.
- Recurrent meningitis can be a presenting symptom of underlying structural abnormalities in the skull base.
Observation:
- An unusual meningocele involving the lateral wall of the cavernous sinus and anterior skull base was identified in a young patient with NF1.
- The meningocele represented an anterior extension of Meckel's cave, creating a cerebrospinal fluid space that traversed the superior orbital fissure.
- The lesion extended extracranially into the pterygopalatine fossa, adjacent to the nasal cavity.
Findings:
- The meningocele was successfully obliterated using an intradural middle fossa approach with fat packing and fenestration into the subarachnoid space.
- This case highlights a potential variant of cranial nerve dural ectasia in NF1, linked to mesodermal defects.
- Expansion of the cavernous sinus lateral wall involving the trigeminal nerve, as seen in this meningocele, has not been previously reported.
Implications:
- This finding expands the spectrum of cranial manifestations associated with NF1.
- Understanding such dural ectasias is crucial for diagnosing and managing complex skull base pathologies in NF1 patients.
- The successful surgical obliteration demonstrates a viable treatment option for this rare condition.