Proximal Focal Femoral Deficiency Unconventional Treatment

DOUGLAS W. KIBURZ, MD AND RAE R. JACOBS, MD**Kansas City, Kansas


Through the leadership of Drs. Amstutz, Aitken, Kiman and associates and others, Proximal Femoral Focal Deficiency (PFFD) as a unique entity has become identified and classified and treatment has been organized. Three cases demonstrate the importance of tailoring the treatment program for each individual. The first case is that of a 4-year-old male with Class D PFFD treated with an ilio-femoral fusion for problems relating to his horizontally oriented spike of residual distal femur. The fusion achieved union, the knee was converted to a hip joint andprosthetic fitting was improved. The second case involves a girl born with Class D PFFD, fibular hemimelia and four rays on her right side. She was treated with a Van Nes rotational osteotomy at 18 months of age and grew to become a socially and psychologically well-adjusted, skeletally mature young woman whose rotation through growth had been controlled with a hip hinge and customized prostheses. The last case is a woman who presented at 24 years of age after bilateral Syme's amputations for her left Class B and right Class A PFFDs. She had discarded her above-knee prostheses and had been walking on her amputation limbs for many years with recent complaints of trochanteric bursitis secondary to pelvic obliquity brought on by leg-length discrepancy. She was successfully equipped with minimum-height limb-equalizing prostheses and experienced relief of her symptoms.

Conclusions

  1. Treatment principles have been improved with the shared experience regarding PFFD, yet each case must be individualized in light of the established treatment principles.
  2. Unstable painful joints are better eliminated.
  3. Below-knee prosthetic fitting is more functional than above-knee fitting.
  4. Leg-length equalization is more important than normal height restoration.

** University of Kansas Medical Center, 39th and Rainbow Boulevard, Kansas City, KS 66103