To report on the physiotherapy intervention provided to a school-aged child with bilateral PFFD by Limb/pelvis-hypoplasia-aplasia syndrome (LPHA) and the progress made toward independent ambulation.
The subject was a 10-year-old boy born with bilateral PFFD by LPHA, with no history of surgical intervention and mainly using a wheelchair for mobility. Both hips exhibited joint contractures (flexion 60°/70°, extension -25°/-25°, adduction 0°/-10°). The left lower limb PFFD was classified as Aitken Type C, and was associated with fibular aplasia, missing 4th and 5th toes, and knee joint contractures (flexion 70°, extension -50°). The right PFFD was classified as Aitken Type B with fibular and tibia aplasia and severe foot dysplasia. The child had a prosthesis for the right and an ankle-foot orthosis for the left. However, the insufficient length of the lower extremities resulted in an inadequate stride length, making ambulation challenging. Furthermore, hip joint contractures were compensated by pelvic rotation during ambulation, worsened the lumbar lordosis and left knee pain. To address the above issues, new prostheses were fabricated and a tailored physiotherapy program was implemented. We targeted to optimize physical function and increase the ability to ambulate. To expand the base of support, we introduced a pair of Lofstrand crutches. Since the child had never used Lofstrand crutches before, there were concerns about the potential for excessive dependence on the crutches during ambulation and the possibility of upper limb joints pain. Consequently, the initial focus was on strengthening exercises for the upper limbs and trunk. This allowed improvements in strength and balance, enabling the child to perform push-ups with Lofstrand crutches. Additionally, due to an increase in pelvic rotation and instability during reciprocal gait, we aimed to achieve the swing-through gait using push-ups. We monitored for any pain after each physiotherapy session. By gradually increasing the length of prostheses, the stride length was also increased.
Ten months of physiotherapy resulted in the stabilization of the swing-through gait with Lofstrand crutches. The 10-meter walking test improved from being difficult to measure to 8.86 seconds, and no new symptoms developed. The ability to ambulate with reduced pelvic rotation and movement of the center of mass was achieved.
To enhance the ambulation ability of children with bilateral PFFD by LPHA, it is important to consider not only the residual function and morphology of the affected limb but also those of the adjacent joints and upper limbs.
Tailored physiotherapy programs are vital for addressing individual needs in children with Bilateral PFFD by LPHA.
prosthesis
ambulation
