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Congenital Pseudarthrosis of the Tibia in Pediatric Patients

MR Imaging

Andreas H. Mahnken1, Gundula Staatz1, Benita Hermanns2, Rolf W. Gunther1 and Michael Weber3

1 Department of Diagnostic Radiology, University Hospital, University of Technology Aachen, Pauwelsstr. 30, 52074 Aachen, Germany.
2 Institute of Pathology, University Hospital, University of Technology Aachen, 52074 Aachen, Germany.
3 Department of Orthopedics, University Hospital, University of Technology Aachen, 52074 Aachen, Germany.



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Fig. 1. Drawings illustrate Crawford's [6] classification of congenital pseudarthrosis of tibia. Patients with all types present with anterolateral bowing of tibia. In type I, the medullary canal is preserved. Cortical thickening might be observed. Type II is defined by presence of thinned medullary canal, cortical thickening, and tabulation defect. The dominant finding in type III is a cystic lesion, which may be fractured. In type IV, pseudarthrosis is present with tibial and possibly fibular nonunion.

 


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Fig. 2A. 7-month-old boy with neurofibromatosis and congenital pseudarthrosis of the tibia (Crawford [6] type I). Short tau inversion recovery image (TR/TE, 1400/30; inversion time, 110) shows congenital pseudarthrosis of the tibia as hyperintense lesion (arrowheads). Note diffuse edema (arrows) of soft tissue ventral to the tibia.

 


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Fig. 2B. 7-month-old boy with neurofibromatosis and congenital pseudarthrosis of the tibia (Crawford [6] type I). T1-weighted spin-echo MR image (550/20) depicts anterolateral bowing and circumscribed cortical thickening of the tibia (arrows). Medullary canal is preserved. Bone marrow in region of anterolateral bowing of tibia shows slight hypointensity.

 


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Fig. 3A. 1-month-old male neonate with congenital pseudarthrosis of tibia (Crawford [6] type III) without neurofibromatosis. Lateral radiograph of calf depicts characteristic cystic lesion in distal part of tibia.

 


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Fig. 3B. 1-month-old male neonate with congenital pseudarthrosis of tibia (Crawford [6] type III) without neurofibromatosis. Lesion (arrows) appears isointense on T1-weighted spin-echo MR image (TR/TE, 550/20).

 


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Fig. 3C. 1-month-old male neonate with congenital pseudarthrosis of tibia (Crawford [6] type III) without neurofibromatosis. On T2-weighted turbo spin-echo image (3800/120), area of pseudarthrosis (arrow) appears hyperintense with thickened hyperintense periosteum (arrowheads).

 


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Fig. 3D. 1-month-old male neonate with congenital pseudarthrosis of tibia (Crawford [6] type III) without neurofibromatosis. Similar findings—hyperintense pseudarthrosis (arrow) with thickened hyperintense periosteum (arrowheads)—can be seen on contrast-enhanced fat-suppressed T1-weighted MR image (550/20). Histologic finding was that lesion was chondroid tissue.

 


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Fig. 4A. 5-year-old boy with neurofibromatosis and congenital tibial pseudarthrosis (Crawford [6] type IV). Lateral radiograph of right leg depicts congenital tibial pseudarthrosis type IV with frank pseudarthrosis of distal tibia.

 


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Fig. 4B. 5-year-old boy with neurofibromatosis and congenital tibial pseudarthrosis (Crawford [6] type IV). On T2-weighted fat-suppressed selective presaturation inversion recovery image (TR/TE, 4100/20), pseudarthrosis is revealed as tibial nonunion with increased signal intensity (arrowheads) and hyperintense tissue ventral to nonunion (arrows).

 


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Fig. 4C. 5-year-old boy with neurofibromatosis and congenital tibial pseudarthrosis (Crawford [6] type IV). T1-weighted MR image (550/20) show a hypointense pseudarthrosis area with hypointense soft tissue ventral to pseudarthrosis (arrows).

 


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Fig. 4D. 5-year-old boy with neurofibromatosis and congenital tibial pseudarthrosis (Crawford [6] type IV). Pseudarthrosis and soft tissue (arrows) show marked contrast enhancement after administration of gadolinium, corresponding to hypercellular periosteum found at histologic examination.

 

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