Rotationplasty: A unique option for patients with osteosarcoma
Rotationplasty: A unique option for patients with osteosarcoma
Osteosarcoma is the most common type of bone cancer among children, adolescents and young adults. Treatments vary and typically involve surgical intervention, radiation and possibly chemotherapy. The following article will discuss a five year old male patient who was diagnosed with osteosarcoma and underwent rotationplasty, a unique and relatively rare surgical procedure to treat his condition.
Introduction
Osteosarcoma is a bone cancer that most often occurs in the long bones that make up the arms or legs. Osteosarcoma develops in the osteoblasts or the bone growing cells of the body. The most common symptom of osteosarcoma is pain in the affected bone, which is usually the upper arm or leg. If the tumor progresses, eventually swelling and motion limitations will occur. For example, patients with osteosarcoma of the lower extremity may develop a limp due to tumor growth. In some cases a pathologic fracture will occur due to the tumor causing bone weakness resulting in a fracture of the bone during routine, daily movements.
Osteosarcoma develops in the osteoblasts or the bone growing cells of the body.
Causes:
Although there is no known cause, researchers have found some factors that put individuals at risk for developing osteosarcoma. Osteosarcoma is the most common malignant bone tumor in youth. In the United States, there are around 400 children diagnosed with osteosarcoma every year. It typically occurs after the age of 10, and it is extremely rare before the age of 5. Males are more likely to be affected than females. African American children have a slightly higher risk of developing osteosarcomas than Caucasians.
Osteosarcoma is the most common malignant bone tumor in youth. Males are more likely to be affected than females. African American children have a slightly higher risk of developing osteosarcomas.
People with rare, inherited cancer syndromes, such as Li-Fraumeni Syndrome and retinoblastoma, have a higher risk of developing osteosarcoma. Li-Fraumeni Syndrome is a cancer predisposition disorder that has a mutation of a certain tumor suppressor gene; this allows tumors to grow and gives a person a higher risk of developing cancer. Individuals diagnosed with this Syndrome have been linked to an increased risk of osteosarcoma. Researchers have also found that children with retinoblastoma, a rare childhood malignant cancer of the eye, have a higher risk of developing osteosarcomas. Also, studies have shown that individuals who were treated with previous radiation treatments have shown to have an increased risk of developing osteosarcoma.
Diagnoses:
X-rays are the initial imaging test for osteosarcoma. Typically patients will present to their physician’s office with pain in a particular extremity, thus indicating investigation with x-ray imaging. If the results suggest that there is a tumor, CT, MR or PET imaging will be done to show the tumor characteristics and staging. A biopsy will need to be collected to identify the type of tumor In order to determine if the cancer has spread or not, bone scans and blood tests will also be done.
Case Study:
The patient in this case is a healthy, active five-year old boy who presented to his pediatrician’s office with his mother. The patient had fallen a few weeks prior while playing outside. He had since complained of knee, ankle and generalized leg pain. His parents were not immediately concerned. However, as his pain persisted, they had decided to bring him to his pediatrician for evaluation. Upon examination of the patient, the pediatrician order a radiograph of the patient’s knee. This imaging study did reveal the presence of a large aggressive tumor of the knee. After biopsy, this tumor was found to be osteosarcoma.
The typical radiographic imaging appearances of osteosarcoma include medullary and cortical bone destruction, moth-eaten appearance, aggressive periosteal reaction, soft-tissue masses, and tumor matrix calcification.
Periosteal reaction refers to the formation of new bone after trauma or some other pathology. There are many different types of periosteal reactions including; single layer, multilayered, solid, spiculated, disorganized/complex and Codman triangle.
In this patient’s case, additional CT and Nuclear Medicine studies were done to assess the progression of the disease and assist with biopsy localization. The PET study revealed a hypermetabolic mass of the distal left femur. Luckily, in this case, there was no indication of metastasis.
Treatment:
Treatment options for osteosarcoma include chemotherapy, surgery and sometimes radiation therapy. The treatment plan depends on where the tumor is, how fast it is growing and whether or not it has spread. Age and overall health are also factored into the decision. Most individuals diagnosed with osteosarcomas need both chemotherapy and surgery. Chemotherapy is typically given before and after surgery. Surgery involves removing the cancerous cells or tumor and is accomplished by either limb-salvage (also referred to as limb-sparing) or amputation. Not often but in some cases, where the tumor has formed in places such as the pelvis, jaw, or spine it may be harder for the surgeons to remove the tumor, and radiation therapy may be the treatment option for the patient. In this particular case, the patient, underwent chemotherapy and rotationplasty surgery to treat osteosarcoma.
What is Rotationplasty?
Rotationplasty is a functional surgical procedure that can be used to treat malignant bone tumors that occur near the knee. This unique procedure is not very commonly performed but has gained some prominence in recent years for its effectiveness in treating certain congenital and acquired conditions affecting the lower extremities. This innovative technique involves the rotation of a limb segment to address functional deficits, offering a viable solution for patients facing challenges with mobility due to various limb abnormalities.
Figure: What is Rotationplasty? (2023). Boston Children’s Hospital. [Source]
Indications for Rotationplasty:
Rotationplasty is primarily indicated for patients with specific limb abnormalities that affect function and quality of life. The procedure is particularly valuable in cases where traditional surgical interventions may be limited in their ability to restore optimal limb function. Rotationplasty is typically indicated for children under the age of 12. This is because of the rapid rate of bone growth at this age. The bone will continue to grow with the child and the prosthetic can easily be adjusted to lengthen with the growth of the child. Children are also very resilient and have great success in re-learning how to walk with the ankle now acting as the knee.
Surgical Techniques:
The surgical techniques employed in rotationplasty vary based on the nature of the deformity and the specific goals of the procedure. In a typical procedure the tumor is removed from the patient. In this case, the tumor was located in the distal femur. The distal femur and proximal tibia and fibula, including the entire knee joint were removed. Next, the lower leg is rotated 180 degrees before reattaching the tibia to the femur. Since it is now backwards, the ankle is able to function as a knee joint.
During the procedure, careful attention is paid to preserving neurovascular structures to ensure postoperative limb viability. The choice of fixation method depends on factors such as the patient’s age, the extent of the deformity/tumor, and the surgeon’s preference.
Figure: [Source]
Rehabilitation following rotationplasty is a crucial component of the overall treatment plan. Physical therapy is typically initiated early in the postoperative period to optimize joint mobility, muscle strength, and overall functional outcomes. The collaborative effort between the surgical team and rehabilitation specialists is integral to achieving the best possible results. The patient is fitted with a prosthetic where the foot slides into the prosthesis. This prosthesis will be continually adjusted as the child grows and develops.
Outcomes and Complications:
The success of rotationplasty is often measured by improvements in limb function, and overall quality of life. In this particular case, there was a favorable outcome for the patient who is now cancer free and continues to grow as a normal, healthy five-year old boy. However, like any surgical procedure, rotationplasty is not without risks and potential complications.
Complications may include infection at the surgical site, delayed or non-union of the osteotomy site, neurovascular injury, and malrotation of the limb. Thorough preoperative assessment, meticulous surgical technique, and close postoperative monitoring are essential components of minimizing these risks.
Conclusion
Rotationplasty represents a valuable surgical option for young children receiving a difficult diagnosis of Osteosarcoma. In this case, the patient’s mother was grateful for the option of this surgical procedure to give her son a future with optimal mobility in which other surgical options such as amputation or limb-salvage surgery would not.
References
- Osteosarcoma. (2023). St. Jude Children’s Research Hospital. Retrieved from https://www.stjude.org
- Osteosarcoma: What Causes Osteosarcoma? (2020, October 8). American Cancer
- Society. Retrieved from https://www.cancer.org
- Gersten, T. (2010, October 16). Osteosarcoma. University of Florida Health. Retreived from https://ufhealth.org
- What is Rotation Plasty? (2023). Boston Children’s Hospital. https://www.childrenshospital.org
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