In-situ hypothermic preservation of the distal limb during thigh and femoral vessel resection and rotationplasty in a child with sarcoma: a case report

Abstract

Background:

The management of pediatric sarcomas of the upper thigh involving femoral vessels is challenging. While rotationplasty offers a valuable functional alternative to amputation, ischemia–reperfusion injury of the re-implanted distal limb may compromise the outcome after this procedure. Hypothermic in situ preservation of the distal limb may reduce ischemia–reperfusion–related complications.

Methods:

We report on the case of a 6-year-old girl presenting with a sarcoma of the thigh involving the femoral vessels. While performing en bloc resection of the upper thigh and femoral vessels, in-situ hypothermic preservation of the distal lower limb was used. Auto-transplantation of the distal lower limb was then completed with rotationplasty to facilitate further prosthesis adaptation and improve functional outcomes.

Results:

The postoperative course was uneventful. No ischemia–reperfusion–related complications occurred, and complete skin healing was achieved within 1 week. Histopathological examination of the resected tumor confirmed a good oncological response with clear margins. The patient is alive and well, in complete tumor remission 12 months after the operation, with a satisfactory functional outcome of the rotationplasty.

Conclusion:

In-situ hypothermic preservation is a simple technique that can reduce ischemia–reperfusion injury during complex thigh resection and auto-transplantation of the distal lower limb. It contributes to a straightforward post-operative course and avoids delays in oncological treatment.

Introduction

Management of femoral bone and soft tissue sarcomas of the thigh is challenging in pediatric patients, especially when major vessels are involved. When limb preservation is impossible, rotationplasty offers a functional alternative to femoral amputation. Originally described by Borggreve and Van Nes^[1,2^], the procedure consists of an autograft of the distal remaining part of the lower limb to which a prosthesis can be fitted. Winkelmann classified rotationplasty for the proximal femur into two main types: in type A, the ankle joint functions as a knee joint, whereas in type B, the knee joint functions as the hip joint and the ankle joint functions as the knee joint^[3^]. Depending on tumoral encasement of the femoral vessels, vascular supply to the distal limb must either be preserved and rotated or resected and re-anastomosed to achieve adequate oncologic margins^[4^]. Vascular compromise of the reconstructed limb constitutes one of the most serious potential complications^[5^]. Therefore, in cases of femoral vessel resection, avoiding warm ischemia of the distal extremity during resection of the mass represents a technical challenge. In organ transplantation surgery, hypothermic preservation of the graft is standard practice to reduce ischemia–reperfusion injuries, which significantly contribute to graft damage and impaired outcomes^[6^]. We report the case of a pediatric patient with a high-grade sarcoma of the proximal thigh involving the femoral vessels. The surgical approach consisted of a modified type B rotationplasty using in situ hypothermic preservation of the distal limb during the resection, ensuring optimal functional and oncological outcomes. This case report has been prepared in line with the SCARE checklist^[7^].

HIGHLIGHTS

• In situ hypothermia used during rotationplasty in pediatric thigh sarcoma.

• Cold perfusion with Custodiol^® reduces ischemia–reperfusion complications.

• Technique enables safe resection with vascular control and clear oncological margins.

• No postoperative vascular or healing complications were observed after surgery.

• Simple, transplant-inspired method improves functional and oncologic outcomes.

Case presentation

A 6-year-old girl presented with a progressively enlarging mass in the left thigh over 2 years. Diagnosis was delayed due to the family’s socio-economic situation. Her general condition remained preserved.

Computed tomography (CT) scan showed a large, heterogeneous, and highly vascularized soft tissue tumor centered in the anterior thigh compartment, extending from the femoral head to the distal femoral metaphysis. The lesion contained scattered calcifications with periosteal reaction. The femoral artery remained patent but was circumferentially encased by the tumor over 8 cm. The profunda femoris artery was occluded in its proximal portion, with no evident collateral circulation.

A magnetic resonance imaging characterized the mass as a 17 × 10 × 12 cm hypervascularized lesion, suggesting an alveolar or vascular sarcoma, with erosion of the anteromedial femur and no skip metastases.

Biopsy confirmed round cell sarcoma. RNA sequencing identified a BCOR gene tandem duplication (L1713_1742dup in exon 15), establishing the diagnosis of BCOR intratandem duplication (ITD) sarcoma^[8^].

No distant metastasis was detected, but positron emission tomography scan showed suspicious regional pelvic lymph node involvement.

She received neoadjuvant chemotherapy per the EuroEwing 2012 protocol^[9^], with four courses of vincristine, doxorubicin, and cyclophosphamide alternating with three courses of ifosfamide and etoposide. Pre-operative imaging showed a shrinkage to 15.4 × 9.1 × 7.1 cm (53% reduction). The femoral vessels remained encased inferiorly, but the sciatic nerve was spared (as shown in Fig. 1). The lesion extended from lateral to the inguinal canal down to the mid-diaphysis of the femur (123 mm from the articular surface of the medial femoral condyle). External iliac nodes were involved.

Figure 1.

(A and B) Contrast-enhanced CT scan of the thigh in sagittal and axial sections showing invasion of the left femoral pedicle vessels. (C) MRI in coronal section showing the tumor in contact with the femur. (D) MRI in axial section showing separation between the tumor and the sciatic nerve.

Multidisciplinary board recommended a two-stage surgery: (1) tumor resection, including encased femoral vessels and regional lymph nodes, plus type B rotationplasty; (2) delayed foot amputation to facilitate the fitting of a future prosthesis and improve the cosmetic aspect. This approach was chosen to avoid a hip disarticulation.

Operative technique

Dissection phase

With the patient supine, a longitudinal incision was made along the medial aspect of the thigh, extending to the knee, allowing for distal dissection to identify the distal part of the femoral vessels. The dissection continued proximally to the tumor’s inferior pole, with circumferential exposure of the distal femur and identification of the sciatic nerve.

Proximal dissection was extended toward the greater sciatic notch through a lateral thigh incision. A proximal thigh incision was made to resect the biopsy tract and access the femoral vessels in Scarpa’s triangle. The sartorius, rectus femoris, and tensor fasciae latae muscles were detached; the femoral nerve was transected. A posterior soft-tissue flap was raised, preserving the sciatic nerve. The ischium was freed inferiorly, and circumferential dissection of the femoral neck and acetabulum was performed. The distal femur was transected, and hip disarticulation was completed, leaving only the femoral vessels and sciatic nerve in continuity with the distal limb. The femoral vessels were prepared below the lower extremity of the tumor, and the femoral and external iliac lymph nodes were removed.

Cold ischemia phase

Distal femoral vessels below the tumor were transected, the artery was cannulated, and the distal limb was perfused with 4°C Custodiol^® solution (as shown in Fig. 2A and B). The venous outflow became progressively clear, and the distal limb was additionally cooled with slush ice. Femoral vessels were ligated and transected on either side of the tumor, completing en bloc resection (Fig. 2C).

Figure 2.

(A) Internal view of the thigh before resection of the femoral vessels, the tumor is completely dissected, the femoral pedicle clamped proximally and cut distally, with cannulation of the distal femoral artery for infusion of 4°C organ preservation solution (Custodiol^®). (B) View of the thigh after complete en bloc resection of the tumor and femoral vessels, the sciatic nerve is preserved. (C) View of the surgical specimen, the femoral vessels are clamped at their proximal end. (D) Post-operative X-ray 3 months after surgery. (E) Clinical result 4 months after surgery (before secondary distal amputation of the reversed distal limb).

The distal femur was recut, and the acetabulum was prepared for arthrodesis with the rotated distal femur, using a femoral segment as a bone graft (Fig. 2D and E). The cold ischemia phase lasted 85 minutes.

Anastomosis, unclamping, and closure

End-to-end vascular anastomosis was performed between the proximal and distal femoral vessels. The initial anastomosis had to be revised due to inadequate perfusion after the first attempt at unclamping; altogether, completion of the anastomosis lasted 160 minutes. After unclamping, rapid and homogeneous foot recoloration was confirmed, as well as the soft consistency of muscular compartments. Intra-operative Doppler confirmed vascular flows. No hemodynamic or metabolic disturbances occurred after unclamping.

The sciatic nerve was routed posteriorly along the femur and then positioned medially. Soft tissue flaps were adjusted for optimal closure, with the gluteus medius sutured to the pes anserinus, and the gluteus maximus, internal hamstrings, and adductors sutured to the patellar tendon.

Results

The postoperative course was uneventful. The distal limb did not present any post-reperfusion swelling or compartment syndrome. Skin sutures healed rapidly. Postoperative anemia required one transfusion, without further bleeding. Preventive anticoagulation consisted of intravenous unfractionated heparin 50 IU/kg bolus, then 100 IU/kg/day continuous infusion until postoperative day 7, followed by enoxaparin 50 IU/kg/day twice daily.

Histopathology confirmed BCOR-ITD sarcoma with a favorable response (1–2% residual tumor cells), clear margins, and no lymph node involvement.

Adjuvant treatment included chemotherapy and radiotherapy (after laparoscopic ovarian transposition). The second stage of the procedure for foot amputation will be carried out subsequently.

With 12 months of follow-up, the child remains in complete remission under treatment, with a good functional outcome of the rotationplasty.

Discussion

This is the first reported use of in-situ hypothermic preservation to reduce ischemia–reperfusion injury after thigh tumor resection and reimplantation of the distal limb. Initially developed for infectious and congenital conditions, rotationplasty was refined in the 1980s to improve function following limb resections for malignancies. When indicated, it offers a better alternative to amputations by preserving functional joints at the hip and knee. Furthermore, it may be a more functional and durable option than other reconstruction methods, such as resection arthrodesis, prosthesis reconstruction, or massive allografts^[10^].

Vascular management during rotationplasty remains debated: 180° rotation of the femoral vessels with the sciatic nerve may cause pseudoaneurysm and prolonged distal ischemia due to vascular kinking, while transection and reanastomosis may lead to anastomotic failure^[11^]. For femoral tumors, resection and anastomosis of femoral vessels allow safe margins and save time^[12^] but may prolong the warm dissection phase, increasing risks of healing delays or vascular compromise^[5^]. Temporary shunts can maintain perfusion during tumor resection^[4^], but they expose the limb to multiple ischemia–reperfusion events, potentially worsening tissue damage through oxidative stress and inflammation. Our approach, based on cold preservation, aims to reduce warm ischemia and minimize ischemia–reperfusion injury.

The principles guiding our technique are inspired by organ transplantation surgery, particularly the use of hypothermic preservation of grafts. Between aortic clamping in the donor and unclamping in the recipient, the graft is flushed and stored in an organ preservation solution between 4 and 8°C, based on the principle of static hypothermia. During this period, the metabolism of the organ is reduced, allowing better survival of cells in anaerobic mode^[13^], and a reduction in ischemia–reperfusion injury after revascularization of the organ in the recipient. As it is commonly used in organ transplantation, we anticipated it could similarly improve the outcome of the re-implanted distal limb after rotationplasty by reducing ischemia–reperfusion injury of tissues, minimizing post-reperfusion edema and the risk of compartment syndrome, and improving tissue healing.

Furthermore, the technical simplicity of this approach does not significantly extend the time needed to complete the procedure. The quick postoperative recovery, without surgical complications, is essential to respecting the entire oncological treatment plan.

Overall, rotationplasty plays a key role in the treatment of pediatric malignant limb tumors, offering an excellent balance between functional and oncological outcomes. Ischemia–reperfusion injury of the distal limb may cause postoperative complications and compromise the results of this technique, especially due to postoperative edema, compartment syndrome, and impaired tissue healing. In situ hypothermic preservation of the distal limb, inspired by organ transplantation techniques, appears to be a simple and efficient technique to improve outcomes. Future studies are needed to validate this approach and standardize the protocols.

Conclusion

In situ hypothermic preservation is a simple and reproducible technique that can effectively reduce ischemia–reperfusion injury during complex thigh resections requiring auto-transplantation of the distal lower limb. In our case, its use contributed to an uncomplicated postoperative course, without vascular or soft-tissue complications, and allowed timely continuation of oncologic therapy. This approach may, therefore, enhance both surgical safety and overall treatment flow in selected pediatric patients undergoing rotationplasty.

Ethical approval

According to the policies of our institution, this case report does not require ethical approval.

Consent

Written informed consent was obtained from the patient’s parent for the publication of this case report and accompanying images. A copy of the consent is available for review by the editor-in-chief upon request.

Sources of funding

The authors received no financial support for the research, authorship, or publication of this article.

Author contributions

L.K.: data collection, literature review, manuscript drafting, and manuscript revision. M.L.-I., S.H., Y.R.: participation in patient management and critical revision of the manuscript. E.M. and C.C.: surgical management, project supervision, manuscript supervision, and critical revision of the manuscript. All authors read and approved the final manuscript.

Conflicts of interest disclosure

The authors declare no conflicts of interest.

Research registration unique identifying number (UIN)

This case report does not qualify as a “First in Man” study and did not require registration.

Guarantor

Christophe Chardot.

Provenance and peer review

Not commissioned, externally peer-reviewed.

Data availability statement

Data supporting this case report are available from the corresponding author upon reasonable request.