Hereditary multiple exostoses with ulnar distraction osteogenesis

Case contributed by Matt Skalski
Diagnosis certain

Presentation

Arm deformity.

Patient Data

Age: 15 years
Gender: Male
Frontal
Oblique
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Multiple exostotic deformities involving the metacarpals, radius, and ulna, with subjacent medullary continuity. These are predominately metaphyseal in location and oriented away from the joint. A large sessile exostosis is noted along the radial aspect of the ulnar diaphysis which extends beyond the field of view. The radius is bowed, with an increased unlar inclination of the distal articular surface. The distal ulna is markedly short, terminating approximately 1.5 cm proximal to the radius.

Lateral
Frontal
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Mid-diaphyseal osteotomy of the ulna, with a distracting external fixator. 

Frontal
Lateral
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The external fixator has been removed and the ulnar length is increased, now meeting the carpus. 

Case Discussion

The Ilizarov technique for bone lengthening effectively increases bone length without using a bone graft or internal hardware fixation, as demonstrated in this case of hereditary multiple exostoses (HME). In this technique, progressive distraction across an osteotomy site with preserved periosteum leads to osteogenesis across the intervening gap, thus lengthening the bone. With the distracted gap actively increased by 1 mm/day, the physician can accurately achieve the desired bone length.

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