Abstract
Objective
There are many options for restoration of function in treating lower extremity sarcomas in young children. The trend has moved towards the use of expandable prosthetics for treatment; however, this has been fraught with complications and expense. The aim of this study was to assess long-term functional outcome scores and emotional satisfaction of rotationplasty and megaprosthesis patients compared to the general population.
Methods
Surveys querying surviving members of a rotationplasty cohort from our institution were sent out including MSTS, TESS and Rand SF-36 questionnaires. Demographic information, surgical intervention, disease status and length of follow-up were collected.
Results
The average MSTS score of the eight respondents available and who agreed to participate in the study was 65.4%. The average TESS was 90.0%. The results of our eight respondents showed Rand SF-36 results with norm-based scoring averages of 46.4 for physical health and 55.6 for mental health. In this study, the patients who were alive with no evidence of disease averaged greater than eighteen-years of follow-up.
Conclusion
Patients functioned well relative to their peers when in an appropriate prosthesis. Patients were emotionally satisfied with rotationplasty and functioned in-line with the general population physically and mentally.
Keywords: Rotationplasty, Megaprosthesis, Sarcomas
1. Introduction
Osteosarcoma in a child is a rare and devastating illness. With an annual occurrence of approximately 560 children and adolescents (or six in one million children) in the USA alone, it is the most common bone sarcoma.1 The mainstay of treatment of these tumors has been consistently chemotherapy with wide surgical resection of the tumor. Important surgical questions still revolve around limb function postoperatively, complications, emotional impact of surgery, and cancer related outcomes.2 Studies have shown that no survival advantage exists between amputated and salvaged limbs in the treatment of osteosarcoma.1,3,4 With improvements in imaging, chemotherapeutic modalities, and operative techniques, limb salvage and functional preservation of limbs have become paramount in terms of optimal surgical treatment. The goal in all cases is to maximize postoperative function while minimizing the chances of local tumor recurrence. Despite the strides made in terms of operative technique, indications and implant design, the local recurrence of osteosarcoma after surgery still remains between 4% and 6%.3,5 With the advent and advancements in extendible endoprosthetics, allograft-prosthetic composites, and osteoarticular allograft, limb salvage without amputation has become increasingly a popular option for patients and surgeons.
Rotationplasty remains a viable option for skeletally immature patients with disease involving the distal femur or proximal tibia (Fig. 1).1,6 The procedure involves removal of a diseased portion of bone, turning the shortened leg bone through 180°, and reattachment of the limb to the proximal osteotomy.1 This procedure represents an elegant method of all autologous reconstruction and straddles the realm of part amputation, part limb salvage. As one might expect, the resultant limb appearance may lead the patient and family to seek alternative methods of limb preservation. Despite the cosmetic appearance of rotationplasty, studies have shown that patients with this procedure maintain relatively high emotional and psychosocial functioning as well as the ability to maintain weight bearing and gait similar to that of an able-bodied peer.7,8
Fig. 1.
Clinical photograph of rotationplasty patient at long-term follow-up visit.
1.1. Rationale
Options for functional reconstruction of lower extremity in children with sarcomas include: expandable endoprosthetics, osteoarticular or intercalary allografts, allograft prosthetic composite, arthrodesis using either vascularised fibular grafting or nail cement spacer, amputation with traditional prosthetics and rotationplasty with custom below-knee prosthetics. Each of the aforementioned options have unique advantages and disadvantages.9, 10, 11, 12, 13 Loss of the distal femoral or proximal tibial growth plate in a child four or more years from skeletal maturity presents distinct challenges to both the patient and clinician. The goal of treatment should be to preserve the highest level of functionality with the most minimal potential complications.
Currently, there exists a trend toward the use of expandable megaprosthesis in the skeletally immature patient.9,11 The potential for complete limb salvage is an aesthetically pleasing option to both the patient and family.11 However, several potential long-term issues (leg-length discrepancy and durability of expandable endoprostheses, etc.) may lend credence to the consideration of other reconstructive options in younger children. Many of these children require multiple additional surgeries for: early mechanical failure, limb lengthening, infection, aseptic loosening and other additional complications.9,11, 12, 13, 14, 15, 16 As the surgical management in treating these patients have yet to be perfects, the authors desired to explore how rotationplasty patients, a limb salvage group familiar to our institution, fare after surgery in terms of overall functional status as well as quality of life and satisfaction after their diagnosis and management.10,17
2. Methods
This study was a retrospective, non-randomized, case study. The patients were members of a 24-patient rotationplasty cohort from, enrolled consecutively with each eligible patient from 1991 to 2004. Our primary indication for rotationplasty was a skeletally immature patient with a large bone sarcoma of the femur who also wished to reduce the likelihood of future operations associated with limb salvage reconstructions and wished to avoid high-thigh amputation or hip disarticulation. Demographic and treatment information was obtained from their medical charts and questionnaires were mailed out to obtain outcome scores.
All patients from this study were members of a rotationplasty cohort from 1991 to 2004. Using patient demographic information from the medical record, as well as publicly available information and the Social Security database, surveys were sent to these patients requesting their participation in this study. All patients who were alive and could be contacted were included in the study. Patients who were deceased at time of data collection were also excluded. Of the 24 patients in the cohort, seven were known to die of disease. Two were ineligible: one due to imprisonment and the other due to his participation in this study as an author. Seven patients were lost to follow-up and could not be contacted. The results presented are from the remaining eight respondents (Fig. 2). The average follow-up time from rotationplasty surgery for the participants was 18.4 years.
Fig. 2.
Patient inclusion criteria flowchart.
Description of experiment, treatment or surgery: All patients in the study underwent rotationplasty surgery with the same academic staff orthopaedic musculoskeletal oncologist and vascular surgeon. The surgical approach and operative management of these patients has been described previously.10,17
In this study, data collected included the age at rotationplasty, the age at follow-up questionnaire and the average length of time from surgery to follow-up questionnaire. Results of Musculoskeletal Tumor Society (MSTS) questionnaire score for the lower extremity, Toronto Extremity Salvage Score lower extremity questionnaire and Rand SF-36 scores were compared. The MSTS survey gives a functional assessment of six categories for patients following musculoskeletal tumor reconstruction of limbs: there is a different score for the upper and lower extremity. For the lower extremity the six categories are pain, function, emotional acceptance, supports, walking and gait. The scale is 0–5 with 0 being severe disabling pain and 5 being no pain for example. This gives a measure of impairment.18,19 The Toronto Extremity Salvage Score (TESS) allows for the functional assessment of patients following surgery for musculoskeletal tumors. Again, there is a survey for the upper and lower extremity, respectively. The survey asks activities of daily living and the patient responds on a scale of 1–5 with 1 being impossible and 5 not at all difficult. This questionnaire is a measure of disability.19,20 The Rand SF-36 is a measure of health status that gives results for eight concepts in regards to physical and mental health and the results are given in reference to norm-based scoring with norm-based scores below 45 being below the average range for the general population.21 Number of patients of this cohort that died of disease and alive with no evidence of disease was also recorded.
The author of this study who underwent rotationplasty declined participation to avoid any bias. The questionnaires were all voluntary and the participants received no monetary or personal benefit from their participation.
2.1. Results
The average age at time of rotationplasty was 11.6 years old, range 7–14 years. There were three male and five female respondents. The average age at time of follow-up questionnaires is 30.0 years old. The average time to follow-up is 18.4 years from rotationplasty surgery. The average MSTS score of the eight respondents available and who agreed to participate in the study was 65.4%, with an average (standard deviation) pain score of 3.6, function score of 3.0, emotional score of 3.4, support score of 3.0, walking score of 3.5 and gait score of 3.1. The average TESS was 90.0%. The most common patient complaints in the TESS were problems with kneeling, difficulty walking on uneven surfaces and the importance of a well-fitting prosthesis (Fig. 3).
Fig. 3.
Average MSTS results for pain, function, emotional acceptance, supports, walking and gait of our cohort.
The Rand SF-36 results for respondents are presented in reference to norm-based scoring (N): physical functioning (PF)-N 45.1, role limitations due to physical health (RP)-N 51.8, bodily pain (BP)-N 50.1, general health perceptions (GH)-N 48.3, vitality (V)-N 49.9, social functioning (SF)-N 53.1, role limitations due to emotional problems (RE)-N 55.3, general mental health (MH)-N 53.3, summary of physical health (PC) 46.4, and summary of mental health (MC) 55.6.
2.2. Discussion
Surgical selection for treating lower extremity sarcomas in younger children are designed to facilitate the highest level of function is clearly the goal.9, 10, 11 Currently, there exists a trend toward the use of expandable megaprostheses in the skeletally immature patient.9,11 In these cases, leg-length discrepancy and durability of expandable endoprosthetics may require reconsideration of reconstructive options in these younger children.11,14 Many children treated with endoprosthetics require multiple additional surgeries for: early mechanical failure, limb lengthening, infection, aseptic loosening, and other additional complications.9,11,14, 15, 16 This study reported rotationplasty patients function well with good-fitting prostheses and were generally happy with their outcome.
This study had a number of limitations. First, the overall cohort of rotationplasty patients is small due to the number of procedures performed and the incidence of osteosarcoma in children in this geographic area. Second, due to the fact that these patients were children when treated, the conversion of electronic medical records and the length of follow-up, two thirds of this cross-sectional study were lost to follow-up. The Social Security database was used in these cases to augment missing data. Further, there was no control group during this study period. All of these questionnaires were done by patients at home and returned in the mail and so if there were any questions about the forms, we were not alerted.
The study results show that our cohort of rotationplasty patients who were alive with no evidence of disease have favorable long–term functional outcome scores based upon MSTS and TESS questionnaires. Our cohort of rotationplasty patients are emotionally satisfied with their rotationplasties compared to the emotional satisfaction of the general population based upon SF-36 results. In this study, the patients who were alive with no evidence of disease were functioning well at an average of greater than eighteen-year follow-up. If the prosthesis fit right and the patient was initiated in it at a young age, patients functioned relatively well in comparison to their peers. Based upon the Rand SF-36 average scores, our rotationplasty cohort functions in line with the general population on all levels. To the knowledge of the writers, this study offers the longest follow-up of rotationplasty patients. The average age at time of rotationplasty was 11.6 years old and the average age at time of follow-up questionnaire was 30.0 years old. The average time of follow-up from rotationplasty surgery was 18.4 years. Future studies may evaluate the functional results of patients receiving rotationplasty, expandable endoprosthetics or intercalary allografts for the treatment of sarcomas in children in a prospective study. This would be difficult given the numbers of patients available and the comfort level of surgeons and would likely require a multi-institution study.
2.3. Conclusions
Patients functioned well relative to their peers in the general population when in an appropriate prosthesis. Patients were emotionally satisfied with rotationplasty and functioned in-line with the general population physically and mentally.
Conflicts of interest
One author is a beneficiary of this procedure. No other authors have any conflicts of interest to declare.
Ethical review Committee statement
Enclose copy of letter from ethical committee study approval.
All of the work on this project was performed at the University of Nebraska Medical Center.
Footnotes
Supplementary data to this article can be found online at https://doi.org/10.1016/j.jcot.2019.06.003.
Contributor Information
Courtney Grimsrud, Email: courtney.grimsrud@unmc.edu.
Cameron Killen, Email: cameron.killen@lumc.edu.
Michael Murphy, Email: mmurphy12@luc.edu.
Hongmei Wang, Email: hongmeiwang@unmc.edu.
Sean McGarry, Email: smcgarry@unmc.edu.
Appendix A. Supplementary data
The following is the Supplementary data to this article:
References
- 1.Grimer R.J. Surgical options for children with osteosarcoma. Lancet Oncol. 2005;6:85–92. doi: 10.1016/S1470-2045(05)01734-1. [DOI] [PubMed] [Google Scholar]
- 2.Messerschmitt P.J., Garcia R.M., Abdul-Karim F.W., Greenfield E.M., Getty P.J. Osteosarcome. J Am Acad Orthop Surg. 2009;17(8):515–527. doi: 10.5435/00124635-200908000-00005. [DOI] [PubMed] [Google Scholar]
- 3.Bacci G., Ferrari S., Lari S. Osteosarcoma of the limb. Amputation or limb salvage in patients treated by neoadjuvant chemotherapy. J Bone Joint Surg Br. 2002;84(1):88–92. doi: 10.1302/0301-620x.84b1.12211. [DOI] [PubMed] [Google Scholar]
- 4.Simon M.A., Aschliman M.A., Thomas N., Mankin H.J. Limb-salvage treatment versus amputation for osteosarcoma of the distal end of the femur. J Bone Joint Surg Am. 1986;68(9):1331–1337. [PubMed] [Google Scholar]
- 5.Ferrari S., Smeland S., Mercuri M. Neoadjuvant chemotherapy with high-dose ifosfamide, high-dose methotrexate, cisplatin, and doxorubicin for patients with localized osteosarcoma of the extremity: a joint study by the Italian and Scandinavian Sarcoma Groups. J Clin Oncol. 2005;23(34):8845–8852. doi: 10.1200/JCO.2004.00.5785. [DOI] [PubMed] [Google Scholar]
- 6.Nichter L.S., Menendez L.R. Reconstructive considerations for limb salvage surgery. Orthop Clin N Am. 1993;24(3):511–521. [PubMed] [Google Scholar]
- 7.Fuchs B., Kotajarvi B.R., Kaufman K.R., Sim F.H. Functional outcome of patients with rotationplasty about the knee. Clin Orthop Relat Res. 2003;415:52–58. doi: 10.1097/01.blo.0000093896.12372.c1. [DOI] [PubMed] [Google Scholar]
- 8.Veenstra K.M., Sprangers M.A., van der Eyken J.W., Taminiau A.H. Quality of life in survivors with a Van Ness-Borggreve rotationplasty after bone tumour resection. J Surg Oncol. 2000;73(4):192–197. doi: 10.1002/(sici)1096-9098(200004)73:4<192::aid-jso2>3.0.co;2-h. [DOI] [PubMed] [Google Scholar]
- 9.Cipriano C., Gruzinova I., Frank R., Gitelis S., Virkus W. Bone loss associated with the repiphysis expandable prosthesis for treatment of pediatric distal femoral malignancies. Clin Orthop Relat Res. 2015;473(3):81–88. doi: 10.1007/s11999-014-3564-3. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 10.Mahoney C.R., Hartman C.W., Simon P.J., Baxter B.T., Neff J.R. Vascular management in rotationplasty. Clin Orthop Relat Res. 2008;466(5):1210–1216. doi: 10.1007/s11999-008-0197-4. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 11.Saghieh S., Abboud M.R., Muwakkit S.A., Saab R., Rao B., Haidar R. Seven-year experience of using Repiphysis® expandable prosthesis in children with bone tumors. Pediatr Blood Canc. 2010;55(3):457–463. doi: 10.1002/pbc.22598. [DOI] [PubMed] [Google Scholar]
- 12.Campanacci D.A., Puccini S., Caff G. Vascularised fibular grafts as a salvage procedure in failed intercalary reconstructions after bone tumour resection of the femur. Injury. 2014;45(2):399–404. doi: 10.1016/j.injury.2013.10.012. [DOI] [PubMed] [Google Scholar]
- 13.Puri A., Gulia A., Pruthi M., Koushik S. Primary cement spacers: a cost-effective, durable limb salvage option for knee tumors. Knee. 2012;19(4):320–323. doi: 10.1016/j.knee.2011.06.012. [DOI] [PubMed] [Google Scholar]
- 14.Arteau A., Lewis V.O., Moon B.S., Satcher R.L., Bird J.E., Lin P.P. Tibial growth disturbance following distal femur resection with expandable endoprosthesis reconstruction. J Bone Joint Surg Am. 2015;97(22):e72. doi: 10.2106/JBJS.O.00060. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 15.Arteau A., Lin P.P., Moon B.S., Satcher R.L., Bird J.E., Lewis V.O. MSTS; 2013. High Mechanical Failure Rate of Repiphysis Expandable Endoprosthesis. [Google Scholar]
- 16.Mulkey P.D., Cummings J.E., Seidel M.J. MSTS; 2013. Consistent Tibial Fixation Failure Resolved with a New Generation Tibial Design for the Expandable Repiphysis Prosthesis. [Google Scholar]
- 17.Baxter B.T., Mahoney C., Johnson P.J. Concomitant arterial and venous reconstruction with resection of lower extremity sarcomas. Ann Vasc Surg. 2007;21(3):272–279. doi: 10.1016/j.avsg.2007.03.005. [DOI] [PubMed] [Google Scholar]
- 18.Enneking W.F., Dunham W., Gebhardt M.C., Malawer M., Pritchard D. A system for the functional evaluation of reconstructive procedures after surgical treatment of tumors of the musculoskeletal system. Clin Orthop Relat Res. 1993;286:241–246. [PubMed] [Google Scholar]
- 19.Janssen S.J., Paulino Pereira N.R., Raskin K.A. A comparison of questionnaires for assessing physical function in patients with lower extremity bone metastases. J Surg Oncol. 2016;114(6):691–696. doi: 10.1002/jso.24400. [DOI] [PubMed] [Google Scholar]
- 20.Davis A.M., Wright J.G., Williams J.I., Bombardier C., Griffin A., Bell R.S. Development of a measure of physical function for patients with bone and soft tissue sarcoma. Qual Life Res. 1996;5(5):508–516. doi: 10.1007/BF00540024. [DOI] [PubMed] [Google Scholar]
- 21.Hays R.D., Sherbourne C.D., Mazel R.M. The RAND 36-item health survey 1.0. Health Econ. 1993;2(3):217–227. doi: 10.1002/hec.4730020305. [DOI] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.