Multiple neurofibromatosis (NF) or von Recklinghausen's disease is an autosomal dominant disorder affecting the growth of neural tissues, caused due to the mutation of the gene located at 17q11.2 chromosome, known as neurofibromatosis type I (NF1) gene. The gene product neurofibromin serves as a tumor suppressor; hence, decreased production of this protein results in a myriad of clinical features, which include café au lait spots, multiple skin tumors, axillary and inguinal freckling, optic glioma, and Lisch nodules (pigmented hamartomas of the iris). Besides the functional troubles, it is an esthetically devastating disease. Like other genetic diseases, it has no definitive treatment and surgical corrections have a bleak prospect in improving cosmesis and only help in creating a lesser monster.
In this article, we discuss the etiopathogenesis, clinical features, and the intricacy of the surgical management of the craniofacial involvement of von Recklinghausen's disease along with two case reports.
How to cite this article
Mistry TA, Natarajan S, Deshpande G, Mistry Y. Craniofacial Plexiform Neurofibromatosis. J Contemp Dent 2018;8(1):50-56.
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