Combined bony and soft tissue reconstruction for hip joint stabilisation in proximal focal femoral deficiency (PFFD) (interventional procedures consultation)
NATIONAL INSTITUTE FOR HEALTH AND CLINICAL EXCELLENCE
Interventional procedure consultation document
Combined bony and soft tissue reconstruction for hip joint stabilisation in proximal focal femoral deficiency (PFFD)
|Children are sometimes born with defects of the hip joint and upper thigh bone, called proximal femoral focal deficiency, often abbreviated to PFFD. Surgical treatment, aiming to produce as functional a leg as possible, may be appropriate. This may also involve a corrective operation on the hip joint (sometimes referred to as the ‘superhip' procedure). The initial operation may be combined with other surgery to lengthen the leg and treat other problems such as poor knee function at the same time or at a later date.|
The National Institute for Health and Clinical Excellence (NICE) is examining combined bony and soft tissue reconstruction for hip joint stabilisation in proximal focal femoral deficiency (PFFD) and will publish guidance on its safety and efficacy to the NHS in England, Wales, Scotland and Northern Ireland. NICE's Interventional Procedures Advisory Committee has considered the available evidence and the views of Specialist Advisers, who are consultants with knowledge of the procedure. The Advisory Committee has made provisional recommendations about combined bony and soft tissue reconstruction for hip joint stabilisation in proximal focal femoral deficiency (PFFD).
This document summarises the procedure and sets out the provisional recommendations made by the Advisory Committee. It has been prepared for public consultation. The Advisory Committee particularly welcomes:
Note that this document is not NICE's formal guidance on this procedure. The recommendations are provisional and may change after consultation.
The process that NICE will follow after the consultation period ends is as follows.
For further details, see the Interventional Procedures Programme manual, which is available from the NICE website (www.nice.org.uk/ipprogrammemanual).
NICE is committed to promoting through its guidance race and disability equality and equality between men and women, and to eliminating all forms of discrimination. One of the ways we do this is by trying to involve as wide a range of people and interest groups as possible in the development of our guidance on interventional procedures. In particular, we aim to encourage people and organisations from groups in the population who might not normally comment on our guidance to do so. We also ask consultees to highlight any ways in which draft guidance fails to promote equality or tackle discrimination and give suggestions for how it might be improved. NICE reserves the right to summarise and edit comments received during consultations, or not to publish them at all, where in the reasonable opinion of NICE, the comments are voluminous, publication would be unlawful or publication would otherwise be inappropriate.
Closing date for comments: 27 January 2009
Target date for publication of guidance: April 2009
|1.1||Current evidence on the safety and efficacy of combined bony and soft tissue reconstruction for hip joint stabilisation in proximal focal femoral deficiency (PFFD) is inadequate in quantity, quality and consistency. Therefore this procedure should only be used with special arrangements for clinical governance, consent and audit or research.|
Clinicians wishing to undertake combined bony and soft tissue reconstruction for hip joint stabilisation in PFFD should take the following actions.
|1.3||The procedure should only be carried out in units which specialise in limb reconstruction, by surgeons with specialist knowledge of neonatal dysplasias and expertise in limb lengthening procedures.|
|2.1||Indications and current treatments|
|2.1.1||Proximal focal femoral deficiency is a congenital syndrome typically characterised by poor hip joint development and femoral shortening. The severity of the syndrome is variable. In severe cases, there may be no hip joint and the femur may be very short. In addition, PFFD may be associated with other lower limb abnormalities, such as an abnormal knee joint, lower limb malrotation, inadequacy of the proximal musculature and limb length discrepancy.|
|2.1.2||Treatment options depend on the extent of the PFFD. In patients with severe forms of PFFD, it may not be possible to produce a leg which is functional and of the correct length, so partial limb amputation and fitting of a prosthesis may be the preferred management. In patients with relatively mild PFFD, an attempt can be made to correct the abnormalities of the hip joint and the upper femur.|
|2.2||Outline of the procedure|
|2.2.1||The procedure is carried out with the patient under general anaesthesia. There are several variations on the procedure. Hip stabilisation involves a long incision on the outer side of the thigh. With soft tissues retracted or released, the upper femur deformity is corrected by division and fixation. If needed, the pelvic bone may also be divided and moved to help reconstruct the hip joint. After surgery, the joint may need to be immobilised in a plaster cast. If the hip joint cannot be salvaged, the upper femur may be stabilised against the pelvis using a pelvic support osteotomy, and this may be combined with leg lengthening procedures.|
|2.2.2||Several additional procedures may be required to achieve reconstruction or to enable prosthetic attachment, either at the same time or afterwards as separate procedures. These may include leg lengthening, epiphysiodesis of the normal (opposite) femur, knee reconstruction, Van Nes rotationplasty, and ‘above knee' amputation.|
|Sections 2.3 and 2.4 describe efficacy and safety outcomes from the published literature that the Committee considered as part of the evidence about this procedure. For more detailed information on the evidence, see the overview, available at www.nice.org.uk/IP711overview.|
|2.3.1||A case series of 14 patients reported that 64% (9/14) of patients had a good clinical outcome after hip stabilisation, leg lengthening and external fixation (based on a composite measure of gait, range of movement, degree of dislocation and residual shortening), at a mean follow-up of 17 years. At final follow-up, the mean difference in patients' limb length was 11.6 cm (range 1–20 cm) (duration of follow-up not stated).|
|2.3.2||A case report of three patients who had plaster casts for 3 months then valgus osteotomy described successful reorientation and stabilisation of the hip and straightening of the femur. Femoral lengthening was undertaken in one patient and planned in two others at a follow-up of 2.3–8 years.|
|2.3.3||The Specialist Advisers considered key efficacy outcomes to be overall limb function and a reduced need for repeat operations. The Specialist Advisers also stated that for some patients the overall result may not be as good as would have been achieved by amputation and fitting of a prosthesis.|
|2.4.1||The case series of 14 patients reported that 43% (6/14) needed more than one lengthening procedure. Osteitis occurred in 43% (6/14) of patients, angular deformity in 21% (3/14) of patients, fracture (not otherwise described) in 7% (1/14) of patients, and pseudoarthritis in 7% (1/14) of patients (mean follow-up 17 years).|
|2.4.2||The Specialist Advisers stated that adverse events relating to the procedure, known from the literature or anecdotally, included significant hip and knee stiffness as a result of excessive lengthening, hip dislocation and recurrent deformity. The Specialist Advisers also considered theoretical adverse events to include avascular necrosis, bone non-union, infection, nerve or vascular injury, poor limb function, recurrence of contractures and wound dehiscence.|
|3.1||This guidance requires that clinicians undertaking the procedure make special arrangements for audit. NICE has identified relevant audit criteria and is developing audit support (which is for use at local discretion), which will be available when the guidance is published.|
Chairman, Interventional Procedures Advisory Committee
This page was last updated: 30 March 2010