Fibular hemimelia

Therapy

Treatment varies depending upon the severity of the disorder. The first step is to reduce the malposition of the foot. In babies, this is achieved with a cast or splint. Later, the cast can be replaced by orthoses. Depending on the stability of the knee joint, this will be a lower leg orthosis or a thigh brace.

At the age of approximately 12 months, the young patient should have received sufficient care (heel lift or orthosis) to allow him/her to start walking freely, as appropriate for his/her age.

If a major leg length discrepancy is to be expected at the start of puberty, then leg lengthening by means of external fixation should be considered. The decision for such an operation should be made as early as possible, since this is of psychological advantage for both the parents and the child. Treatment with a ring fixator permits the simultaneous correction of the complex leg and foot deformities that are associated with fibular hemimelia. Depending on the severity of the disorder, this operation may have to be repeated at a later time. If the difference in leg length and the axial deformity are not particularly pronounced, epiphysiodesis (surgical blocking of the growth plate) may be considered for temporarily directing growth.

At about the age of 6, problems resulting from knee instability need to be discussed in order to avoid an early occurrence of degenerative joint damage in these children.

Although treatment with an external fixator always involves a high level of psychological stress, for both the parents and the child, it should be taken into account that the impairments experienced with this treatment method are only temporary. Amputation, on the other hand, is final.

Sample treatment schedule

0–10 months Consultation, casts, radiographs if necessary, transection of the fibula

7 mon –1 Year Treatment with orthosis,
MRI for imaging pathological changes (fibula, bone malformation, interior knee)

1–3 years If necessary, resection of the fibula/heel lift/correction of the distal misalignment with wedge osteotomy and K-wire fixation, and thus a 1st axial correction

3–5 years 2nd axial correction and, if applicable, lengthening with external fixator; if needed also temporary epiphysiodesis after the age of 5.

Starting from age 7 If applicable, anterior cruciate ligament plasty; if needed also temporary epiphysiodesis

8–11 years If applicable, further axial correction and lengthening; if needed also temporary epiphysiodesis

12–15 years If necessary, growth stop on opposite side

  After conclusion of growth (> 14–16 years): final axial correction and lengthening
   

Reports of experience