Which of the following is the MOST common skeletal manifestation in Type-1 Neurofibromatosis?
Coical thinning of long bones
Pseudoahrosis
Sphenoid dysplasia
Scoliosis
Von Recklinghausen's Disease or NF-1 - skeletal manifestations Scoliosis is the most common skeletal manifestation of NF-1, affecting 10%-30% of patients. More Info: There are two other bony lesions distinctive enough to be included in the diagnostic criteria for NF-1. 1. Dysplasia of the wing of the sphenoid bone, results in poor formation of the wall and/or floor of the orbit. This leads to proptosis (from herniation of meninges or brain into the orbit) or enophthalmos. 2. Dysplasia of a long bone, characterized by congenital thinning and bowing, affects approximately 2% of children with NF-1. Although the tibia is most commonly affected, the femur, humerus and other long bones may also be involved. Failure of primary union following a fracture results in a "false joint" or pseudahrosis. Ref: Listernick R., Charrow J. (2012). Chapter 141. The Neurofibromatoses. In L.A. Goldsmith, S.I. Katz, B.A. Gilchrest, A.S. Paller, D.J. Leffell, N.A. Dallas (Eds), Fitzpatrick's Dermatology in General Medicine, 8e.