Book contents
- Frontmatter
- Contents
- List of contributors
- Foreword
- Preface
- Section 1 Shoulder
- Section 2 Arm
- Section 3 Elbow
- Section 4 Forearm
- Section 5 Wrist
- Section 6 Hand
- Section 7 Hip and Pelvis
- Section 8 Thigh
- Section 9 Leg
- Section 10 Ankle
- Section 11 Foot
- Section 12 Tumors/Miscellaneous
- Case 75 Intraosseous lipoma
- Case 76 Lipoma arborescens
- Case 77 Liposarcoma
- Case 78 Mazabraud syndrome
- Case 79 Neurofibromatosis type I (NF I)
- Case 80 Benign peripheral nerve sheath tumors (PNSTs)
- Case 81 Malignant peripheral nerve sheath tumors (MPNSTs)
- Case 82 Synovial sarcoma
- Case 83 Aggressive fibromatosis (desmoid tumor)
- Case 84 Chondrosarcoma
- Case 85 Pigmented villonodular synovitis (PVNS)
- Case 86 Synovial chondromatosis (osteochondromatosis)
- Case 87 Myositis ossificans
- Case 88 Aneurysmal bone cyst (ABC)
- Case 89 Soft tissue hemangioma
- Case 90 Giant cell tumor (GCT)
- Case 91 Ganglion cyst
- Case 92 Chondroblastoma
- Case 93 Hypertrophic osteoarthropathy
- Case 94 SAPHO syndrome
- Index
- References
Case 79 - Neurofibromatosis type I (NF I)
from Section 12 - Tumors/Miscellaneous
Published online by Cambridge University Press: 05 July 2013
- Frontmatter
- Contents
- List of contributors
- Foreword
- Preface
- Section 1 Shoulder
- Section 2 Arm
- Section 3 Elbow
- Section 4 Forearm
- Section 5 Wrist
- Section 6 Hand
- Section 7 Hip and Pelvis
- Section 8 Thigh
- Section 9 Leg
- Section 10 Ankle
- Section 11 Foot
- Section 12 Tumors/Miscellaneous
- Case 75 Intraosseous lipoma
- Case 76 Lipoma arborescens
- Case 77 Liposarcoma
- Case 78 Mazabraud syndrome
- Case 79 Neurofibromatosis type I (NF I)
- Case 80 Benign peripheral nerve sheath tumors (PNSTs)
- Case 81 Malignant peripheral nerve sheath tumors (MPNSTs)
- Case 82 Synovial sarcoma
- Case 83 Aggressive fibromatosis (desmoid tumor)
- Case 84 Chondrosarcoma
- Case 85 Pigmented villonodular synovitis (PVNS)
- Case 86 Synovial chondromatosis (osteochondromatosis)
- Case 87 Myositis ossificans
- Case 88 Aneurysmal bone cyst (ABC)
- Case 89 Soft tissue hemangioma
- Case 90 Giant cell tumor (GCT)
- Case 91 Ganglion cyst
- Case 92 Chondroblastoma
- Case 93 Hypertrophic osteoarthropathy
- Case 94 SAPHO syndrome
- Index
- References
Summary
Imaging description
Skeletal abnormalities occur in approximately 25–40% of NF I cases. Scoliosis is the most common skeletal manifestation in NF I. The vertebrae are often abnormal showing posterior scalloping and enlarged intervertebral foramina due to dural ectasia or neurofibromas of the nerve roots. Posterior scalloping is believed to be mainly due to dural ectasia whereas anterior and lateral scalloping are the result of mesodermal dysplasia (Figure 79.1). In the rib cage the underlying mesenchymal abnormality combined with the erosions by intercostal neurofibromas result in ribbon-like deformities of the ribs. Bowing in the long bones in patients with NF I should suggest the possibility of early or ensuing congenital pseudoarthrosis of the tibia (Figure 79.2). Non-ossifying fibromas of the long bones in the lower extremities are also a feature of NF I.
Lateral thoracic meningoceles are the result of protrusion of the dura and arachnoid through the neural foramina (Figure 79.3). This is most common at the apex of the scoliotic curve and the majority of meningoceles are right sided. Chest films of patients with thoracic meningoceles exhibit a short focal scoliosis of the upper thoracic spine convex toward the lesion. The neural foramina appear enlarged and the pedicles show thinning with increased interpediculate distance. The distance between some adjacent ribs may increase.
- Type
- Chapter
- Information
- Pearls and Pitfalls in Musculoskeletal ImagingVariants and Other Difficult Diagnoses, pp. 176 - 181Publisher: Cambridge University PressPrint publication year: 2013