72 year old female presented for review with right hip pain. She is nearly wheelchair bound. She has progressive shepherd's crook deformity of the right hip with cutting out of the previously central internal fixation.
She had previously presented at age 55 with an intertrochanteric  R hip fracture which went on non-union. This was unusual enough for us to investigate her for metabolic bone disease. She was found to have a familial form of hypophosphatasia. She had bilateral Zickel nail fixation of her proximal femurs. Subsequently she had a fracture at the nail tip on the left and was revised in 1991 to a gamma nail (elsewhere). Now she presents with hip pain, shepherds crook deformity of the proximal right femur and the nail has cut out of the trochanteric region and perhaps out of the femoral head. Her sister who likely had the same condition had three unsuccessful total hip replacements with early loosening. The sister eventually died with the problem unresolved after a fall and head injury. My patient is terrified of having a hip replacement.

If this was a simple situation I would offer her sub-trochanteric osteotomy to realign the femoral neck then fixation with a plate. Unfortunately neither she nor I have any confidence she will heal after that unless the fixation is total bone contact (i.e. intramedullary). So I am considering subtrochanteric (dome) osteotomy and exchange nailing. She has seen metabolic bone disease experts as recently as a year ago with no suggestions as to covering the surgical situtation with any medical treatment.

Questions -

1. What do you think of the surgical plan? Any other suggestions?
2. Would it be a good idea to infuse pamidronate pre and post op?
3. Any tips or experience with adult hypophosphatasia?

See detailed history of this case for more information.