Artikel
Dysplasia Epiphysealis Hemimelica (Trevor’s Disease) of the scaphoid in a 9-year-old boy
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Veröffentlicht: | 6. Februar 2020 |
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Objectives/Interrogation: Dysplasia Epiphysealis Hemimelica (Trevor's Disease) is a rare disorder of the epiphysis in children, characterized by asymmetrical epiphyseal growth, considered a variant of osteochondromas. Its presentation in the upper limb is extremely rare.
There is no malignancy reported. We demonstrate in this report a patient who underwent surgical treatment with preoperative programming through 3D-printing based on the CT scan images.
Methods: A 7-year-old boy entered the service with a complain of deformity in the left wrist associated with radial border pain without a history of trauma in February 2016. His orthopedic examination showed generalized restriction of left wrist range of motion. The wrist radiographs showed an enlarged, bipartite scaphoid with its extremities joined through a radiolucent halo with the distal portion oriented with a pronounced palmar tilt and more sclerotic than the remaining carpal bones. There were no adjacent joint changes. Magnetic resonance imaging demonstrating pericartilaginous hypersignal in the T2-weighted sequence. At first, clinical observation was chosen (Figure 1 [Fig. 1]).
Results and Conclusions: The new exams showed an increase in the scaphoid with a pronounced worsening of the deformity, increase in the area of sclerosis and involvement of the articular surface of the distal radius. Tomography ratified such changes. With these findings, surgical treatment with corrective osteotomy of the affected bone for joint rescue was chosen. For surgical program, biomodel reconstruction was used in 3D printing of the structure to be submitted to the intervention using DICOM® images. Process carried out without costs through the Information Technology Center Renato Archer, Campinas, São Paulo, Brazil (Figure 2 [Fig. 2]).