Hereditary multiple exostoses with Madelung deformity and ulnar distraction osteogenesis

Case contributed by Dr Matt Skalski

Presentation

Arm deformity.

Patient Data

Age: 15
Gender: Male

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 1.5cm proximal to the radius. 

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

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

Case Discussion

The Ilizarov technique for bone lengthening is an effective way to achieve an increase in bone length without the use of 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 1mm/day, the physician can accurately achieve the desired bone length. 

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Case Information

rID: 41862
Case created: 23rd Dec 2015
Last edited: 23rd Dec 2015
Inclusion in quiz mode: Included

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