Background: Limb-length discrepancy or angular
deformities as a result of altered bone growth may lead to a decreased
range of motion and impaired function as well as premature osteoarthritis
in patients with multiple hereditary osteochondromatosis. The purpose
of this study was to describe the function of the forearm in untreated
patients in order to facilitate comparison with studies of the results
of treatment of this condition.
Methods: The medical records of fifty-one pediatric
patients were identified and served as the basis for identifying
a cohort of adult relatives with the disorder. Participants were
asked about pain and limitations in vocational or recreational activities
and about concerns with regard to cosmetic appearance. Radiographs
of the forearm and wrist were made to quantify the deformity. Functional
outcome was assessed on the basis of a comparison with the norms
for grip and pinch strength and for scores on the hand function
test of Jebsen et al. Limitations in the range of motion of the
upper extremities were converted to standard impairment ratings.
Results: Participants included twenty-two men and
seventeen women with an average age of forty-two years (range, twenty
to eighty years). Most of the patients were employed in careers of
their choice, with only five (13%) indicating that they
were limited in any way in the performance of their jobs. Twenty-six
subjects (67%) participated in recreational activities,
and sixty-eight arms (88%) were reported to be free of
pain. Objective measurement of function demonstrated greater disability
than that found from subjective reporting. Fourteen arms had an
impairment rating of >10%, while twenty had decreased
pinch strength and sixteen had decreased grip strength. Ten arms
(13%) had decreased hand function according to the hand
test of Jebsen et al. Radiographic evaluation demonstrated osteoarthritic
changes in three limbs.
Conclusions: Affected individuals had definite decreases
in hand and wrist function, yet these did not result in major increases
in pain or in limitations in daily work and recreation. This cohort
provides a basis for comparison with the results of operative treatment
in affected individuals with multiple hereditary osteochondromatosis.