Upper limb reduction deficiency (ULRD)
is a rare condition that has been known ever since the 6th century B.C. This is
a lifelong deficiency which in an afflicted child can lead to practical
limitations, social restrictions and physical problems.
The overall aim of this research was to increase the knowledge about children
with upper limb reduction deficiencies from three perspectives: the deficiencies
themselves, the use of prostheses and the well-being of the affected children.
To validate information regarding upper limb deficiency in the Swedish Register
for Congenital Malformations (SRCM), all infants reported to this register
during 1973-1987 were re-classified according to a more detailed classification.
The result was compared with a clinic-based register at the Limb Deficiency and
Arm Prostheses Centre in Örebro, Sweden. The findings indicate that SRCM, with
its calculated underestimation of 6%, can be used for studying the prevalence of
ULRD in Sweden. However, as SRCM is a surveillance register, the quality of some
information seems to be low, making detailed description of cases difficult. Use
of the population register data for clinical purposes could therefore result in
lower validity. Additional information and follow-up of specific cases are
therefore recommended.
The presence of scoliosis and trunk asymmetry was studied in 60 persons with
transverse ULRD. Nineteen persons (31%) had a scoliosis of between 10 and 19º
and 30 persons had minor curves of between 5 and 10º. There was a significant
correlation between leg length inequality and side of the convexity, with the
convexity directed towards the side of the shorter leg in 21 of 28 persons. This
indicates that children with transverse ULRD may have a transient scoliosis of
postural origin of no clinical significance.
A new observation-based test, the Assessment of Capacity for Myoelectric Control
(ACMC), which measures a persons capacity to control a myoelectric prosthetic
hand during the performance of ordinary daily tasks, was developed. Occupational
therapists completed 210 assessments of 75 persons. Rasch rating scale analysis
was used for validation and reliability estimations. The results demonstrate
internal scale and person response validity.
The external reliability of ACMC was established by scorings from three raters
with different degrees of experience on 27 videotapes of client performance. The
major finding in this study was that in order to obtain reliable measures from
the ACMC the raters have to have some experience of this group of clients. Until
the ACMC can adjust for rater severity, the same rater should perform the ACMC
when it is used for follow-up or clinical trials.
In a study of 62 children we found that, overall, children with ULRD who have
been fitted with a myoelectric prosthetic hand are just as well adjusted
psychosocially as their able-bodied peers. There are indications, however, of
social stigmata related to the deficiency which have to be considered
differently in boys and girls. Most children who have been provided with a
myoelectric prosthesis at an early age continue to use the prosthesis.
Keywords: children, upper limb, deficiency, register validation, scoliosis, arm
prosthesis, measurement, occupational therapy, psychopathology, depression.
ISBN: 91 7140 091 5