Neurofibromatosis type 1: ulnar pseudoarthrosis
Progressive forearm deformity.
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There is resorption of a portion of the ulnar shaft at the junction of the middle and distal thirds. There is a narrow sharpened pencil-like appearance of the ulnar distal to the absent section with cupping of the ulnar proximal to this, mimicking the appearance of a joint.
This abnormality is rare and has been termed ulnar pseudoarthrosis and is usually only seen in patients with neurofibromatosis type 1 (NF1) which was true in this case. Involvement of the ulna is uncommon with tibial pseudoarthrosis being many times more prevalent in patients with NF1.
- Durga Nagaraju K, Vidyadhara S, Raja D et-al. Congenital pseudarthrosis of the ulna. J Pediatr Orthop B. 2007;16 (2): 150-2. doi:10.1097/BPB.0b013e328010b13f - Pubmed citation
- Ramelli GP, Slongo T, TschäPpeler H et-al. Congenital pseudarthrosis of the ulna and radius in two cases of neurofibromatosis type 1. Pediatr. Surg. Int. 2001;17 (2-3): 239-41. Pubmed citation
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