Frequently Asked questions

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Congenital Femoral Deficiency (CFD), also known as Proximal Femoral Focal Deficiency (PFFD) is a rare birth defect that is characterized by a short femur (thigh bone), associated with hip and knee deformity, deficiency, or instability. CFD occurs in about one in 200,000 births and the cause is unknown.

CFD is characterised by a progressive limb length discrepancy. The affected limb does not grow properly, so as Henry ages, the discrepancy between the two limbs will continue to increase. As the expected discrepancy at skeletal maturity increases, the number of lengthenings required to equalise length will increase. Henry’s predicted limb-length difference at full maturity is 23cm.

Most children with CFD are offered above-the-knee (Symes) amputation. This is not something we have ever considered. CFD is very rare and its surgical treatment relatively new. For this reason, few surgeons are experienced and trained to treat CFD. For Henry, he requires multiple surgeries. The first surgery Henry needs is a SUPERhip procedure. In Australia, there are no surgeons with experience who can offer this. 

We had the pleasure of virtually meeting Dr. Dror Paley (Orthopaedic Surgeon) who is known as the world's most experienced limb lengthening surgeon. Dr. Paley has extensive experience with deformity reconstruction and limb lengthening. After reviewing Henry’s scans, Dr. Paley informed us that Henry will require a SUPERhip procedure and 3 leg lengthenings. Dr. Paley will usually perform the first surgery around age two or three. Prior to surgery, shoe lifts, orthoses (braces), and prostheses can all be used to manage the limb length discrepancy. Henry wears an Articulated Ankle-Foot Orthosis (AFO) and has a built-up component which enables him to walk.

Dr. Paley invented the SUPERhip procedure in 1997 and it is designed to comprehensively reconstruct severe bone and soft tissue deformities. It is recommended the SUPERhip procedure be done between the ages of two and three. The SUPERhip is performed six to twelve months before the first lengthening. Henry is predicted to require 3 leg lengthening procedures.

When bone breaks, it naturally regenerates to fix the fracture. Lengthening works by separating the bone and distracting (pulling apart) the bone segments very slowly so that new bone continues to form in the gap. As the bone segments are slowly distracted, the bone regenerates, resulting in increased length.