J Korean Soc Radiol.  2015 Nov;73(5):307-311. 10.3348/jksr.2015.73.5.307.

Congenital Osteofibrous Dysplasia, Involving the Tibia of a Neonate

• Kim SY
• Lee SH

• KMID: 2079565
• DOI: http://doi.org/10.3348/jksr.2015.73.5.307

Abstract

Osteofibrous dysplasia (OFD) is a benign fibro-osseous lesion found in long bones, and congenital OFD in neonates is very rare. The diagnosis of OFD in neonates is difficult, and it is sometimes misidentified as any of a number of other congenital tumors or tumor-like lesions, in which case biopsies are often necessary. After a histological confirmation of OFD, non-surgical or delayed surgical treatment is generally recommended. We present image findings from the radiographs and magnetic resonance images in the case of a 7-day-old female infant with pathologically confirmed congenital OFD.

MeSH Terms

Biopsy
Diagnosis
Female
Humans
Infant
Infant, Newborn*
Tibia*

Figure

• 

  Fig. 1 Radiographs. A, B. The initial AP and lateral image demonstrate an expansile osteolytic lesion (black arrow) with inner irregular sclerosis, involving the cortex and medullary cavity of the proximal meta-diaphysis of the right tibia. The margin of the lesion is irregular, multi-lobulated, but relatively well-defined. The cortical disruption of the proximal tibia suggests a pathologic fracture (white arrow), and a thin linear periosteal reaction at its distal part is noted. C, D. The follow-up AP and lateral image (after 3 weeks) show a callus formation at the pathologic fracture, and a bowing deformity of the proximal tibia. An osteolytic lesion (arrowhead), not detected on the initial radiograph, is seen in the distal dia-metaphysis of the fibula.

• 

  Fig. 2 MRI of the right lower leg. A. A sagittal T2-weighted MR image shows an expansile mass replacing the entire medullary space, which reveals heterogeneous hyperintense signal intensity (white arrow). A disrupted periosteal reaction, continuous to the pathologic fracture, is noted (black arrow). B-D. The axial T2-weighted (B), T1-weighted (C), and enhanced T1-weighted (D) MR images show prominent signal change with heterogeneous enhancement in extraosseous soft tissue (star). The transition between the extraosseous component and the normal muscles is wide and ill-defined.

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  Fig. 3 The histological result shows fibrous stromas (star) with immature bony trabeculae (white arrow) and osteoblastic rim (black arrow) (hematoxylin and eosin, × 20).

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