Paediatric limb salvage · ankle converted to a knee after wide resection of a distal femoral or proximal tibial sarcoma
- Rotationplasty is a biological limb-salvage option for a skeletally immature child with a resectable sarcoma of the distal femur or proximal tibia in whom the sciatic nerve is free of tumour. The tumour-bearing segment and the knee are resected en bloc, the distal leg is rotated 180 degrees, and the tibia is fixed to the femoral stump so the ankle functions as a knee.
- Preserving the sciatic (tibial) nerve and the femoral/popliteal vessels is the whole game: an intact, sensate foot with a mobile, powered ankle is what makes the reconstructed limb work. The vessels are preserved and coiled where possible, or resected and reanastomosed where they are involved.
- The rotated ankle sits at the level of the contralateral knee — in a growing child, position the heel about 2 to 3 cm above the opposite knee so that growth brings the joints level at skeletal maturity.
- Plantarflexion (the gastrocnemius-soleus, now the quadriceps equivalent) extends the prosthetic knee; dorsiflexion (tibialis anterior, now the hamstring equivalent) flexes it. This gives an active, proprioceptive, energy-efficient gait superior to an above-knee amputation.
- Functional outcome is durable (high MSTS scores, the ability to run and play sport) but the cosmetic cost of a backwards-pointing foot is real. Mandatory psychological assessment, family counselling, and meeting a rotationplasty patient are prerequisites, not afterthoughts.
When & Why
Indication. A skeletally immature child (classically 6 to 14 years, with significant growth remaining) with a resectable high-grade malignant bone sarcoma — osteosarcoma or Ewing sarcoma — of the distal femur or proximal tibia, in whom wide margins are achievable, the sciatic nerve is free of tumour, the vessels are either uninvolved or reconstructable, there is no uncontrolled metastatic disease, and an adequate soft-tissue envelope remains. First described by Borggreve in 1930 for an ankylosed tuberculous knee, applied by Van Nes in 1950 to congenital femoral deficiency, and adapted to bone sarcomas by Salzer and Kotz in the 1980s. Contraindications. Sciatic nerve encasement; femoral/popliteal vessels unreconstructable; a contaminated field (a poorly placed biopsy tract or infection); inadequate soft tissue; a foot or ankle that cannot power a prosthesis; uncontrolled widespread metastases; and — critically — a patient or family who, after thorough counselling, cannot accept the cosmetic outcome. Very young age (under about 6 years) is a relative concern because of rotation drift with growth. The one decision that matters. For the same tumour a child can be reconstructed three ways. Each trades function, cosmesis, durability and revision burden differently:
A durable, biological, end-bearing reconstruction that grows with the child and gives below-knee-level gait. No implant to loosen, fail or infect, and no lifelong revisions. The cost is a visibly rotated, backwards-pointing foot.
Near-normal cosmesis and a quicker return to function, at the price of repeated lengthening procedures through childhood, and the long-term implant risks of infection, aseptic loosening and periprosthetic fracture.
The fastest, most reliable oncological option when limb salvage is not feasible, with a lower functional ceiling, higher energy cost of walking, and phantom-limb pain.
Counsel specifically for the cosmetic appearance of a rotated foot, the need for a lifelong below-knee-style prosthesis, the small risk of vascular failure (which can force a secondary amputation), progressive rotation drift during growth, nonunion at the femorotibial junction, and phantom sensation. Arrange for the family to meet a patient who has had the procedure and to see the prosthesis in use. Setup. Supine on a radiolucent table, the whole limb and the contralateral leg draped free so the rotated ankle can be matched to the opposite knee. General anaesthesia with a regional adjunct for post-operative pain, an arterial line and a urinary catheter, prophylactic antibiotics, a cell saver, and the image intensifier for the osteotomies and fixation. Have a vascular surgeon on standby and microvascular capability available. A tourniquet is rarely used — the vessels must be dissected and assessed throughout.
The Operation
The goal: resect the tumour-bearing distal femur (or proximal tibia) together with the knee with a wide margin, preserving a sensate, well-perfused distal leg; rotate that leg through 180 degrees; fix the tibia to the femoral stump with the ankle at the level of the contralateral knee; and place the preserved nerve and vessels without kink or tension. Done well, the patient leaves with a biological "knee" powered by their own gastrocnemius-soleus.
A clear anatomical/clinical diagram of a completed Winkelmann type A (distal femoral) rotationplasty: the distal femur and knee have been resected, the lower leg rotated 180 degrees so the foot points posteriorly, the tibia is fixed to the proximal femoral stump (intramedullary nail or plate), the ankle joint now lies at the level of the contralateral knee, and the coiled sciatic nerve and femoral/popliteal vessels are shown passing gently into the rotated segment. A paired panel showing the limb in a below-knee-style prosthesis would reinforce the ankle-as-knee concept.
Context: A verified image is being sourced.
Operative sequence
- Supine, radiolucent table, both legs free; cell saver, image intensifier, vascular standby.
- Confirm the planned proximal femoral and distal tibial osteotomy levels against the pre-operative MRI margin; mark the skin with the biopsy tract excised in continuity as an ellipse with the specimen.
- A long anteromedial utilitarian incision from the proximal thigh to the proximal leg, incorporating the biopsy tract.
- Identify the superficial femoral and popliteal artery and vein — proximally in the adductor canal, distally behind the knee. Dissect them cleanly off the tumour/specimen, ligating geniculate and perforator branches as needed to gain length.
- Decide now: preserve the vessels (leave them on a loop) if they are uninvolved and long enough, or plan resection with end-to-end anastomosis or an interposition vein graft if they are adherent or involved.
- Identify the sciatic nerve in the posterior thigh and trace its tibial and common-peroneal divisions distally.
- Preserve the nerve intact and mobilise it generously — it must coil into the rotated segment afterwards without tension, and an intact nerve is what keeps the foot sensate and the gastrocnemius-soleus (the future quadriceps) and tibialis anterior (the future hamstring) innervated and powerful.
- Divide the quadriceps and knee capsule, the cruciates and the menisci.
- Osteotomise the femur proximal to the tumour at the planned wide margin and the tibia distal to the tuberosity, preserving enough tibia for fixation; the patella and extensor mechanism are sacrificed with the knee (the new knee is the ankle).
- Remove the tumour-bearing distal femur and knee en bloc with the biopsy tract. Send marrow and soft-tissue margins for frozen section before proceeding.
- Externally rotate the distal segment (tibia, ankle and foot) through a full 180 degrees so the foot points posteriorly and the heel faces anteriorly.
- Check the neurovascular bundle is not kinked, tethered or under tension, and confirm a Doppler signal at the foot before committing to fixation.
- Place the rotated ankle joint at the level of the opposite knee; in a growing child position the heel about 2 to 3 cm above the contralateral knee so growth brings the joints level at maturity.
- Confirm length and rotation clinically against the draped opposite leg, and resect tibia or femur as needed to fine-tune.
- Rigidly fix the proximal tibia (now proximal) to the femoral stump: an intramedullary nail is preferred in larger/adolescent children for stability and early mobilisation; a locking compression plate suits smaller bones; external fixation is reserved for contaminated or poor-quality situations.
- Confirm alignment, rotation and fixation with the image intensifier.
- If preserved, lay the now-redundant vessel loop and the sciatic nerve gently into the soft tissues, coiled without kink; confirm flow once more.
- If resected, perform the end-to-end anastomosis or vein-graft reconstruction now and re-check the foot is warm, pink and pulsatile.
- Layered closure over drains without tension; the soft tissues must cover the fixation and the neurovascular bundle.
- Re-confirm foot perfusion (colour, warmth, capillary refill, Doppler) before leaving theatre; any concern mandates de-rotation and re-exploration.
- A well-padded bulky dressing or splint holds the ankle in neutral — the position that corresponds to the prosthetic knee in extension.
- Strict, hourly neurovascular observations for 48 hours; a low threshold to return to theatre for any vascular compromise.
- If there is any doubt about the anastomosis or flow, a CT or formal angiogram is obtained promptly.
- Coordinate wound timing with the chemotherapy cycle (most of these children receive neoadjuvant and adjuvant MAP-regimen chemotherapy).
The single most dangerous moment is immediately after the 180-degree rotation, when the preserved or reconstructed vessels are most prone to kinking, stretch or thrombosis. Before final fixation and again before closure, confirm a strong Doppler signal, warm pink skin and brisk capillary refill at the toes. If the foot is cool or pulseless, de-rotate, relieve any tension on the bundle, and reassess; persistent compromise means vascular re-exploration and revision of the anastomosis, and — if the limb cannot be salvaged — conversion to an above-knee amputation.
After rotation, the gastrocnemius-soleus (a plantarflexor) sits posteriorly and now acts across the prosthetic knee as the quadriceps equivalent — ankle plantarflexion drives knee extension for stance stability, while the tibialis anterior (a dorsiflexor) becomes the hamstring equivalent, dorsiflexion flexing the knee for swing. Because the sciatic nerve is intact, control is active and proprioceptive, which is why gait energy cost approaches that of a below-knee amputation and is far lower than an above-knee amputation.
In a skeletally immature child the distal tibial and foot physes keep growing, so the reconstructed limb lengthens over time. Place the heel about 2 to 3 cm above the contralateral knee at the index operation so the joints end up level at maturity. Set the ankle slightly long, and monitor length and rotation through growth — derotation osteotomy or length adjustment is sometimes needed later, particularly in the very young.
Aftercare & Complications
Rehabilitation | Phase | Timing | Activity and immobilisation | Milestone | |-------|--------|------------------------------|-----------| | 1 | 0 to 2 weeks | Bulky splint, ankle neutral; hourly neurovascular checks; non-weight-bearing; coordinate with chemotherapy | Wound healing, perfusion stable | | 2 | 2 to 6 weeks | Removable splint; gentle ankle range; bed-to-chair; ongoing vascular surveillance | Soft-tissue healing, drains out | | 3 | 6 to 12 weeks | Protected weight-bearing as union appears on radiograph; ankle and hip strengthening | Radiographic union at about 8 to 12 weeks | | 4 | 3 to 6 months | First then definitive below-knee-style prosthesis; gait training (plantarflexion equals knee extension) | Independent ambulation | | Ongoing | Years | Growth and rotation monitoring; prosthesis revisions; graded return to sport | Community ambulator, sport participation | Most children walk independently in their definitive prosthesis by 3 to 6 months and can run, cycle and swim; long-term series report durable function with high satisfaction once adaptation is complete. Ankle range translates into only modest prosthetic knee excursion, but it is active and well controlled. Complications
| Complication | Recognition | Prevention | Management |
|---|---|---|---|
| Vascular compromise / limb ischaemia | Cold, pulseless or mottled foot; poor capillary refill; rising pain | Meticulous vessel handling; intra-operative Doppler before and after rotation; avoid tension and kinking | Immediate de-rotation and re-exploration; revision anastomosis or graft; secondary amputation if unsalvageable |
| Rotation drift / progressive derotation | Foot gradually rotates forwards over years; prosthesis misfit and altered gait | Avoid in very young children; slight overcorrection; monitor rotation through growth | Derotation osteotomy if function or prosthetic fit is affected |
| Nonunion at the femorotibial junction | Pain and motion at the arthrodesis site; no bridging on radiograph | Rigid fixation; bone graft; time surgery around chemotherapy cycles | Bone graft and revision fixation; exchange or re-nailing |
| Wound breakdown / deep infection | Dehiscence, erythema, discharge; worse with chemotherapy and neutropenia | Meticulous soft-tissue handling; peri-operative antibiotics; timed around chemo | Debridement and antibiotics; vacuum dressing; skin graft or flap; retain or remove hardware |
| Limb-length discrepancy | Asymmetric gait; overgrowth or undergrowth of the reconstructed limb | Position the heel 2 to 3 cm above the contralateral knee for growth; monitor | Prosthesis adjustment; epiphysiodesis if marked |
| Local recurrence | New pain or mass at the resection site | Achieve and confirm wide margins intra-operatively | Wide re-resection or amputation plus systemic therapy |
| Phantom sensation / body-image distress | Altered or false sensation in the rotated foot; anxiety about appearance | Pre-emptive counselling and peer support; nerve coiling without tension | Desensitisation, pain team, psychology and peer-mentor support |
Viva & Exam Focus
ROTATIONROTATION — order of the operation
Clinical Decision Scenarios
Practise clinical reasoning and management decisions out loud
“A 10-year-old boy has an osteosarcoma of the distal femur. Staging is clear, the tumour is resectable, and the sciatic nerve and vessels are free. What reconstructive options do you discuss, and when would you favour rotationplasty?”
“Immediately after rotating the limb 180 degrees and applying the femorotibial fixation, the foot is cool and the Doppler signal at the ankle is lost. How do you manage this?”
Indication & decision
- Skeletally immature child (6 to 14 years) with a resectable distal femoral / proximal tibial osteosarcoma or Ewing sarcoma
- Sciatic nerve free; vessels uninvolved or reconstructable; no uncontrolled metastases
- Choose against expandable endoprosthesis and above-knee amputation — a values-based trade-off
- Mandatory: psychology, family counselling, meeting a rotationplasty patient
Exposure & resection
- Both legs free; cell saver, image intensifier, vascular standby
- Excise the biopsy tract in continuity
- Mobilise the femoral/popliteal vessels; preserve the sciatic nerve long
- Wide en bloc resection of tumour-bearing distal femur and knee; frozen-section margins
The rotation & fixation
- Rotate the distal limb a full 180 degrees — foot posterior, heel anterior
- Set the ankle at the contralateral knee; heel 2 to 3 cm above it for growth
- Fix tibia to femoral stump — IM nail (preferred) or locking plate
- Confirm Doppler flow before and after fixation; coil nerve and vessels without kink
Finish & pitfalls
- A cold foot after rotation is an emergency — de-rotate and explore
- Plantarflexion extends the prosthetic knee (gastrocnemius equals quadriceps)
- Monitor for rotation drift and limb-length discrepancy through growth
Outcomes
- Durable, high MSTS function; patients run, cycle, swim
- Gait energy cost near below-knee amputation, far below above-knee amputation
- Body-image concerns persist in a substantial minority despite good function
Background & Evidence
Epidemiology. Osteosarcoma is the commonest primary malignant bone tumour of childhood, with an incidence of roughly 3 to 4 cases per million children per year and a peak in the second decade; the distal femur and proximal tibia together account for the majority of lower-limb cases — precisely the rotationplasty territory. Ewing sarcoma is rarer (about 1 to 3 per million) with a male predominance. Because rotationplasty is reserved for selected skeletally immature patients in whom the sciatic nerve is spared, it represents only a small fraction of all sarcoma reconstructions and is concentrated in specialist paediatric sarcoma centres. Pathoanatomy. A high-grade sarcoma destroys the distal femoral (or proximal tibial) metaphysis and threatens the knee. Rotationplasty converts the problem into a solution: rather than discard the distal limb, the tumour-bearing segment and the knee are removed and the remaining leg is rotated 180 degrees so that the ankle joint, with its intact motor and sensory nerve supply, becomes the new knee. The gastrocnemius-soleus becomes the quadriceps equivalent; the tibialis anterior becomes the hamstring equivalent. The open distal tibial and foot physes continue to grow, so the reconstruction lengthens with the child.
| Winkelmann type | Tumour location | What is resected and reconstructed |
|---|---|---|
| Type A (the common type) | Distal femur | Resect the distal femur with the tumour and the knee; rotate the lower leg 180 degrees; fix the tibia to the remaining femur; the ankle becomes the new knee |
| Type B | Proximal tibia | Resect the proximal tibia with the tumour and the knee; rotate the distal segment; fix to the distal femur; the ankle becomes the new knee |
| Modified (proximal femur / hip) | Proximal femur or hip region | Larger resection with a modified (tibiopelvic) construct; reserved for selected proximal disease |
Key evidence. Rotationplasty is oncologically safe — survival is equivalent to amputation and to other limb-salvage options when margins are clear. Kotz and Salzer (1982) established the technique in four children with distal femoral osteosarcoma, with no local recurrence and no wound-healing failure despite chemotherapy. Long-term series confirm durable function: Gradl and colleagues (2015) reported a mean MSTS score of about 64 percent at a mean 14-year follow-up, with quality-of-life domains matching or exceeding population norms, and Veenstra and colleagues (2000) found psychosocial functioning and overall quality of life approaching those of healthy peers — while honestly recording that body-image and intimacy concerns persist in a substantial minority. The principal failure mode is vascular: Sawamura and colleagues (2008) found that secondary amputation for vascular compromise clustered in patients who needed a vascular anastomosis, had less than 95 percent chemotherapy-induced tumour necrosis, or presented with a pathological fracture.
References
Rotation-plasty for childhood osteosarcoma of the distal femur
- Four children with distal femoral osteosarcoma treated by rotation-plasty with wide en bloc excision of the distal femur, knee and proximal tibia
- No clinical or radiographic local recurrence over follow-up of 27 to 58 months
- No wound-healing problems despite high-dose methotrexate chemotherapy, allowing early prosthetic fitting
- Established rotationplasty as a standard limb-salvage alternative to amputation for this indication
Quality of life in survivors with a Van Nes-Borggreve rotationplasty after bone tumour resection
- 34 patients surveyed a mean of 6.3 years after rotationplasty
- Psychosocial functioning, overall quality of life and social support were comparable to healthy peers
- Physical functioning was poorer than healthy peers but better than chronically ill controls
- One-third to one-half reported negative effects on body image, social and intimate contact, and sexuality
- Two-thirds were actively engaged in sport and wore the prosthesis continuously
Long-term functional outcome and quality of life following rotationplasty for malignant tumours
- 12 patients reviewed at a mean follow-up of 14 years
- Mean MSTS score of about 64 percent and mean Tegner activity level of 4.1, consistent with recreational sport
- SF-36 physical functioning of 80, with vitality, social functioning and mental health at or above population norms
- Patients reported durable function and satisfaction for daily living and sport at long-term follow-up
Complications and risk factors for failure of rotationplasty — review of 25 patients
- Rotationplasty was successfully accomplished in 22 of 25 patients
- Three patients required secondary amputation for vascular compromise
- All three failures had undergone a vascular anastomosis and showed less than 95 percent chemotherapy-induced tumour necrosis
- Two of the three failures had a preoperative pathological fracture
- Vascular anastomosis, poor chemotherapy response and pathological fracture flag higher failure risk