Orbitofascial plexiform neurofibromatosis

Case contributed by Bahman Rasuli
Diagnosis certain

Presentation

Progressive swelling of the right hemifacial and globe since 2 years old.

Patient Data

Age: 10 years
Gender: Female

A preseptal and septal soft tissue infiltrating masses involving the right orbit with intraorbital extraconal extension along the superior and lateral aspects of the orbit

Dysplasia of the right sphenoid wing

Similar infiltrated soft tissue masses along with the right hemifacial and cervical subcutaneous region cause swelling and asymmetry.

Case Discussion

The patient has manifestations of neurofibromatosis type 1, which is a known diagnosis in this patient, as evident by sphenoid wing dysplasia as well as plexiform neurofibromata involving the orbit, hemifacial, and neck on the right side.

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