Identify the lesion given below in the image?
Facial nerve palsy
Masticator space abscess
Answer- B. Plexiform Neurofibromatosis
- The diagnosis of the given patient in the image is plexiform neurofibromatosis.
- Plexiform neurofibromas represent an uncommon variant (30%) of neurofibromatosis type 1 (NF-1) in which neurofibromas arise from multiple nerves as bulging and deforming masses involving also connective tissue and skin folds. Hence the clinical description of lesions as “bags of worms.”
- These lesions manifest early in life and tend to transform into malignant peripheral nerve sheath tumors (MPNST). Malignant progression is generally considered the main cause of mortality, occurring in 2% to 16% of cases.
- The main symptoms are ocular motility disturbances from eye involvement, dyspnea and respiratory failure from upper airway compression, neurological deficits from cranial nerve compression, and social deprivation, depression and other mood disorders resulting from facial disfigurement.