This is an enhanced PDF from The Journal of Bone and Joint Surgery The PDF of the article you requested follows this cover page. Adult Outcomes Following Amputation or Lengthening for Fibular Deficiency Janet L. Walker, Dwana Knapp, Christin Minter, Jennette L. Boakes, Juan Carlos Salazar, James O. Sanders, John P. Lubicky, David M. Drvaric and Jon R. Davids J Bone Joint Surg Am. 2009;91:797-804. doi:10.2106/JBJS.G.01297 This information is current as of April 7, 2009 Supplementary material Commentary and Perspective, data tables, additional images, video clips and/or translated abstracts are available for this article. This information can be accessed at http://www.ejbjs.org/cgi/content/full/91/4/797/DC1 Reprints and Permissions Click here to order reprints or request permission to use material from this article, or locate the article citation on jbjs.org and click on the [Reprints and Permissions] link. Publisher Information The Journal of Bone and Joint Surgery 20 Pickering Street, Needham, MA 02492-3157 www.jbjs.org 797 C OPYRIGHT Ó 2009 BY T HE J OURNAL OF B ONE AND J OINT S URGERY, I NCORPORATED Adult Outcomes Following Amputation or Lengthening for Fibular Deﬁciency By Janet L. Walker, MD, Dwana Knapp, MSW, Christin Minter, MA, Jennette L. Boakes, MD, Juan Carlos Salazar, PhD, James O. Sanders, MD, John P. Lubicky, MD, David M. Drvaric, MD, and Jon R. Davids, MD Investigation performed at Shriners Hospitals for Children (Lexington, Kentucky; Northern California [Sacramento, California]; Erie, Pennsylvania; Chicago, Illinois; Springﬁeld, Massachusetts; and Greenville, South Carolina) Background: Fibular deﬁciency results in a small, unstable foot and ankle as well as a limb-length discrepancy. The purpose of this study was to assess outcomes in adults who, as children, had had amputation or limb-lengthening, commonly used treatments for ﬁbular deﬁciency. Methods: Retrospective review of existing data collected since 1950 at six pediatric orthopaedic centers identiﬁed 248 patients with ﬁbular deﬁciency who were twenty-one years of age or older at the time of the review. Excluding patients with other anomalies and other treatments (with the excluded group including six who had had lengthening and then amputation), we identiﬁed ninety-eight patients who had had amputation or limb-lengthening for the treatment of isolated unilateral ﬁbular deﬁciency. Sixty-two patients (with thirty-six amputations and twenty-six lengthening procedures) completed several questionnaires, including one asking general demographic questions, the Beck Depression Inventory- II, the Quality of Life Questionnaire, and the American Academy of Orthopaedic Surgeons Lower Limb Questionnaire including the Short Form-36. A group of twenty-eight control subjects completed the Beck Depression Inventory-II and the Quality of Life Questionnaire. Results: There were forty men and twenty-two women. The average age at the time of the interview was thirty-three years. There were more amputations in those with fewer rays and less ﬁbular preservation. Lengthening resulted in more surgical procedures (6.3 compared with 2.4 in patients treated with amputation) and more days in the hospital (184 compared with sixty-three) (both p < 0.0001). However, when we compared treatment outcomes we did not ﬁnd differences between groups with regard to education, employment, income, public assistance or disability payments, pain or use of pain medicine, sports participation, activity restriction, comfort wearing shorts, dislike of limb appearance, or satisfaction with treatment. No patient who had been treated for ﬁbular deﬁciency reported signs of depression. The only signiﬁcant difference between treatment groups shown by the Quality of Life Questionnaire was in the scores on the Job Satisﬁers content scale, with the amputees scoring better than the patients treated with lengthening (p = 0.015). The American Academy of Orthopaedic Surgeons Lower Limb Module did not demonstrate differences in health-related quality of life or physical function. Conclusions: The patients who were treated with lengthening had started out with more residual foot rays and more ﬁbular preservation than the amputees. They also required more surgical intervention than did those with an amputation. While patients with an amputation spent less of their childhood undergoing treatment, they were found to have a better outcome in terms of only one of seventeen quality-of-life parameters. Both groups of patients who had had treatment of ﬁbular deﬁciency were functioning at high levels, with an average to above-average quality of life compared with that of the normal adult population. Level of Evidence: Therapeutic Level III. See Instructions to Authors for a complete description of levels of evidence. Disclosure: In support of their research for or preparation of this work, one or more of the authors received, in any one year, outside funding or grants in excess of $10,000 from Shriners Hospitals for Children and Kosair Charities, Inc. Neither they nor a member of their immediate families received payments or other beneﬁts or a commitment or agreement to provide such beneﬁts from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any beneﬁts to any research fund, foundation, division, center, clinical practice, or other charitable or nonproﬁt organization with which the authors, or a member of their immediate families, are afﬁliated or associated. J Bone Joint Surg Am. 2009;91:797-804 d doi:10.2106/JBJS.G.01297 798 T H E J O U R N A L O F B O N E & J O I N T S U R G E RY J B J S . O R G d A D U LT O U T C O M E S F O L L O W I N G A M P U TAT I O N OR LENGTHENING V O L U M E 9 1-A N U M B E R 4 A P R I L 2 009 d d FOR FIBULAR DEFICIENCY F ibular deﬁciency is the most common congenital deﬁ- identiﬁed through the hospital diagnosis-based databases and ciency of the long bones, with a reported prevalence of from a list of all patients who had been seen at each hospital seven to twenty per million live births1,2. It is theorized that from 1950 to the present. Existing records and radiographs of all a defect in the femoral-ﬁbular-ulnar developmental ﬁeld can patients, twenty-one years of age or older, who had been treated result in a clinical spectrum ranging from complete absence for ﬁbular deﬁciency at six centers were reviewed for informa- of the limb or proximal femoral focal deﬁciency to a missing tion regarding the ﬁbular deﬁciency, resultant deformities, and toe and simple tarsal coalition3,4. The more common clinical treatments. If a physical ﬁnding was recorded in the chart as problems attributed to isolated deﬁciency of the ﬁbula include a being present it was considered to be present. If a physical ﬁnding small foot, poor ankle stability, and leg-length discrepancy. was not mentioned, it was considered to be absent unless existing Syme or Boyd amputation or limb-lengthening during child- photographs or radiographs showed the abnormality. hood are accepted treatments for these problems. Genu valgum, To conﬁrm the diagnosis, all charts and radiographs re- knee instability, and patellofemoral problems may also have to trieved from the database search were reviewed by two investi- be corrected, but they rarely affect the choice of deﬁnitive gators (J.L.W. and J.L.B.). The diagnosis was conﬁrmed in 248 treatment. Amputation allows the application of a prosthesis, patients, on the basis of documentation to the effect that ﬁbular which can be adjusted for length differences and enables the deﬁciency was present. Sixty patients with bilateral ﬁbular deﬁ- individual to experience a normal level of physical functioning5. ciency, twelve with contralateral lower-limb anomalies, thirty- Limb-lengthening is a complex treatment involving multiple seven with ipsilateral proximal femoral focal deﬁciency or a surgical procedures over a prolonged period of time, and the congenitally short femur (femoral discrepancy that was greater limb may have residual problems such as limited joint motion, than tibial discrepancy), and one with Charcot-Marie-Tooth muscle weakness, and bone fragility or deformity6. However, disease were excluded from the study. Since mild femoral hy- lengthening and reconstruction allow the individual to retain poplasia, genu valgum, patellofemoral problems, tibial bowing, the small foot and avoid the lifelong need for a prosthesis7. foot deformities, and tarsal coalition are so common in patients We are aware of few studies comparing the outcomes of with ﬁbular deﬁciency, these diagnoses were not used as exclu- these two common treatments for ﬁbular deﬁciency. Naudie sion criteria. Because questionnaires were valid only when used et al.8 found that patients treated with lengthening had more by English-speaking persons, non-English-speaking patients (n = complications and needed more surgery than did those treated 7) were excluded. One patient was known to have died and was with amputation. Many of their patients treated with length- excluded. Only those patients who had had lengthening or a ening still required braces or shoe-lifts. Choi et al.9 reported an Syme or Boyd amputation (a through-the-ankle joint amputation 88% rate of satisfactory results after amputation compared that preserved the heel pad), but not those who had had both, with a 55% rate after lengthening. McCarthy et al.10 found that, were included in the comparison of treatments. Six patients who at a mean of seven years postoperatively, children who had had had had initial attempts at lengthening and, for reasons not early amputation were more active, had less pain, and were more completely clear in the record, later had amputation and twenty- satisﬁed than were those who had had lengthening. six who had had other types of surgery or no surgery at all were Information on the long-term outcomes of these two also excluded. During the time frame in which these children had treatments is scarce. Birch et al.11 reported on ten adults who surgical treatment, there were no uniform protocols in place at had had amputation when they were children. The patients any of the six centers. Decisions about treatment were made by the found that amputation did not limit their ability to pursue or parents and surgeons. Because of the retrospective nature of this achieve personal goals. Dutoit et al.12 reviewed the cases of study, the criteria for treatment decisions could not be discerned. twenty-six adults who had had lengthening and found radio- After the above exclusions, the study population included graphic deterioration of the knee and ankle. Twenty-two of sixty-one patients treated with amputation and thirty-seven these patients considered their limb to be unaesthetic and treated with lengthening. Using last known addresses, tele- twenty continued to have problems with their shoes. phone numbers, parents’ names, Social Security numbers, and Since the ultimate goal of treatment for children with Internet searches, we located sixty-two former patients (thirty- ﬁbular deﬁciency is a high-level quality of life throughout their six treated with amputation and twenty-six, with lengthening), lifetime, the purpose of this study was to compare these two and all agreed to complete the questionnaires listed below, in treatment regimens with regard to their outcomes in adult- person or by telephone, administered by one of the investigators hood. This was accomplished with use of validated outcomes (D.K.). Because of the time required to answer all of these instruments to assess depression, quality of life, general health, questions, not all patients chose to complete all questionnaires. and physical functioning in a follow-up study of adults in General questionnaire: This nonvalidated form was de- whom ﬁbular deﬁciency had been previously treated with signed locally to collect basic demographic and socioeconomic amputation or lengthening. data (based on the U.S. Census format), to perform the evalu- ations of knee symptoms and comfort with physical appear- Materials and Methods ance, and to evaluate patient satisfaction (with the questions T his retrospective comparative study was approved by the medical institutional review board at each of the six pedi- atric orthopaedic centers. Patients with ﬁbular deﬁciency were used in the study by McCarthy et al.10). Beck Depression Inventory-II13: This twenty-one-question validated screening tool is used to detect possible symptoms of 799 T H E J O U R N A L O F B O N E & J O I N T S U R G E RY J B J S . O R G d A D U LT O U T C O M E S F O L L O W I N G A M P U TAT I O N OR LENGTHENING V O L U M E 9 1-A N U M B E R 4 A P R I L 2 009 d d FOR FIBULAR DEFICIENCY depression that may have an impact on responses to other older, were in good health, had normal cognition, and had no questions regarding quality of life and physical functioning. known orthopaedic problems consented to complete these two Quality of Life Questionnaire 14 : The Quality of Life questionnaires. The characteristics of these volunteers were Questionnaire is used to examine ﬁve major quality-of-life not otherwise matched to those of the patients who had been domains with use of ﬁfteen content scales, a social desirability treated for ﬁbular deﬁciency. T tests, chi-square tests, analysis scale, and a total quality-of-life score. It is a validated outcome of variance with Bonferroni post hoc analyses, and correlation tool with established norms. coefﬁcients were performed with SAS19 and StatView 20 software American Academy of Orthopaedic Surgeons Lower Limb to carry out comparisons among the different treatment groups Module15: This is a validated outcomes questionnaire consisting and the control group. Power analyses and sample-size calcu- of the Short Form-36 16, a general health-based survey of lations were performed with use of web-based software21. quality of life, and questions regarding lower-limb function. General population norms are reported. Source of Funding American Academy of Orthopaedic Surgeons Foot and The external funding sources for this study played no role in the Ankle Module17: This is a validated outcome tool similar to the study design, implementation, data interpretation, or prepa- American Academy of Orthopaedic Surgeons Lower Limb ration of this report. They provided funds for staff salary sup- Questionnaire that contains a Short Form-36 and questions port and supplies. regarding foot and ankle function and shoe comfort. All patients completed the Short Form-36 as part of the American Academy Results of Orthopaedic Surgeons Lower Limb Module. The foot and ankle function questions from this Foot and Ankle Module were completed only by the patients treated with lengthening. Both F orty men and twenty-two women who had been treated for ﬁbular deﬁciency were interviewed. Their average age was thirty-three years, with a range of twenty-one to ﬁfty-ﬁve years. groups completed the shoe-comfort questions. No differences in terms of age or sex distribution, side involved, Prosthesis Evaluation Questionnaire18: This is a validated number of residual rays, extent of ﬁbular deﬁciency, tibial visual analogue questionnaire used to measure patients’ satis- bowing, or the procedures performed were found between the faction with their prosthesis. When this questionnaire was sixty-two patients who were interviewed and the thirty-six used in a telephone interview, we asked subjects to answer the patients who were not located (p = 0.23 to 0.99). questions on a scale of 0 to 10, with 0 indicating very dissat- A comparison of the characteristics of the interviewed isﬁed and 10 indicating very satisﬁed. The total scores were patients who had an amputation with those of the patients who calculated in the same fashion as those for the questionnaire on were treated with lengthening as well as of the deformities of the which the patient marked their response. While this methodol- two groups is presented in Table I. The extent of ﬁbular absence ogy has not been validated, it gives some measure of the ampu- was classiﬁed with the system described by Achterman and tees’ prosthesis-related quality of life. This questionnaire was Kalamchi22. Type I indicates that some of the ﬁbula is present administered only to the patients who had had an amputation. and is subdivided into Type IA and Type IB. In Type IA, the As recommended by a consulting biostatistician, the distal ﬁbular physis is proximal to the dome of the talus and the Quality of Life Questionnaire and the Beck Depression proximal ﬁbular epiphysis is distal to the proximal tibial physis. Inventory-II were also administered to a control group to In Type IB, 30% to 50% of the ﬁbula remains and, although it is account for any temporal societal stresses that might have present distally, it does not support the ankle laterally. Type II occurred during the time frame of our interviews but that indicates that there is no ﬁbula or only a vestigial remnant of the could not be measured or controlled for. Examples are feelings ﬁbula, and it is the most common form. The number of residual about the economy, the war in Iraq, or the terrorist attacks on metatarsals, the presence of tibial bowing, and the extent of the September 11, 2001. By introducing a control group to com- ﬁbular deﬁciency differed signiﬁcantly between the treatment plete these questionnaires, we increased, if not eliminated, the groups, with greater degrees of deﬁciency and deformity seen in likelihood that the psychosocial results were related to our the amputation group (Table I). Lengthening resulted in sig- patients’ physical impairment rather than to any salient global niﬁcantly more surgical procedures and a higher total number stressors. The normative data provided with the Quality of Life of days in the hospital. The number of surgical procedures and Questionnaire have a standard deviation of 10. Because of the total number of days in the hospital refer to all operations uncertainty about the variability in a smaller control group, we and hospital days due to the treatment of the ﬁbular deﬁciency estimated a standard deviation of between 10 and 25. It was and related lower-extremity and prosthetic problems and in- estimated that, in order to have an 80% chance of detecting a clude the days spent in inpatient rehabilitation for that treat- difference in the means of 20 between the control group and ment. The total number of days in the hospital did not correlate either the amputation or limb-lengthening group, a sample with the scores on the Beck Depression Inventory-II, Quality of size of between ﬁve and twenty-six control subjects was re- Life Questionnaire, Short Form-36, or American Academy of quired. Control subjects were recruited by posting ﬂyers at Orthopaedic Surgeons Lower Limb Module (R2 range = 0.0004 local grocery and department stores, libraries, hospitals, and to 0.161). university student centers in the community of the main in- The twenty-six patients treated with lengthening had, in vestigators. Twenty-eight adults who were twenty-one years or total, forty-eight limb segments (forty-one tibiae and seven 800 T H E J O U R N A L O F B O N E & J O I N T S U R G E RY J B J S . O R G d A D U LT O U T C O M E S F O L L O W I N G A M P U TAT I O N OR LENGTHENING V O L U M E 9 1-A N U M B E R 4 A P R I L 2 009 d d FOR FIBULAR DEFICIENCY TABLE I Comparisons of the Amputation and Limb-Lengthening Groups Amputation* Lengthening* Difference of the Means (N = 36) (N = 26) P Value (95% Conﬁdence Interval) Power Value Sex (male/female) 21/15 19/7 0.231 0.224 Mean age at follow-up (yr) 32.5 32.8 0.917 0.051 Race (white/Black/Asian/unknown) 33/2/0/1 24/1/1/0 0.482 0.227 Side (right/left) 22/14 15/11 0.787 0.058 22 Classiﬁcation ([IA 1 IB]/II) (0 1 1)/35 (4 1 5)/17 0.0008 0.320 (0.13, 0.51) Presence/absence of ankle equinus 15/21 9/17 0.574 0.087 Presence/absence of ankle valgus 22/14 12/14 0.243 0.215 Presence/absence of tibial bowing 25/11 10/16 0.015 0.310 (0.07, 0.55) Mean no. of foot rays 3.18 4.25 <0.0001 21.070 (21.56, 20.58) Presence/absence of tarsal coalition 14/22 12/14 0.567 0.088 Mean no. of procedures 2.42 6.31 <0.0001 23.890 (25.05, 22.73) Mean no. of days in hospital 63.4 184.3 <0.0001 2120.9 (2171.9, 269.9) *The values are given as the number of patients unless otherwise indicated. femora) lengthened. Two patients had simultaneous lengthening ening. Three patients had ankle fusions and three had osteo- of the tibia and femur, and the remainder of the operations were tomies for the treatment of foot deformities following lengthening. done as separate procedures. One limb segment was lengthened Final foot deformities could not be fully assessed by telephone in eight patients; two, in fourteen patients; and three, in four interview. Of the eleven patients treated with lengthening who patients. Fourteen lengthening procedures were reportedly done completed the American Academy of Orthopaedic Surgeons with the Wagner technique23; fourteen, with the Ilizarov tech- shoe-comfort questions, seven often or sometimes wore shoes nique24; ten, with the Anderson technique25; ﬁve, with an EBI with a lift or orthotic modiﬁcation. Orthoﬁx device26; one, with the Abbott technique27; two, with The average age of the thirty-six patients who had a Syme traction over a Rush rod; and two, with an unknown method. or Boyd amputation was 4.1 years (range, six months to 17.3 The average amount of length gained by the forty lengthening years) at the time of the amputation. The age at amputation did procedures for which the amount was recorded was 4.9 cm, with not correlate with the scores on the Beck Depression Inventory- a range of 2 to 10.8 cm. The number of limb segments that had II, Quality of Life Questionnaire, Short Form-36, or American been lengthened did not correlate with the scores on the Beck Academy of Orthopaedic Surgeons Lower Limb Questionnaire Depression Inventory-II, Quality of Life Questionnaire, Short (R2 range = 0.00006 to 0.173). The group as a whole had an Form-36, or American Academy of Orthopaedic Surgeons Lower average of 1.2 procedures before or after the amputation. Limb Questionnaire (R2 range = 0.00002 to 0.326). In addition Twelve of the thirty-six patients had an average of two surgical to the lengthening procedures, a contralateral epiphysiodesis to procedures prior to the amputation, most often in an attempt adjust limb length was used in the proximal part of the tibia of to correct the foot/ankle deformity. Eighteen had an average of two patients, the distal part of the femur of one, and both the 3.3 surgical procedures after the amputation. These included proximal part of the tibia and the distal part of the femur of six. six patients who had a total of twelve operations for stump- One patient also had a contralateral femoral shortening. The related problems, ﬁve patients who had a total of eight oper- reasons for surgery to address limb malalignment were more ations for treatment of femoral valgus, and four patients who difﬁcult to assess in the patients treated with lengthening than had patellar realignment procedures. Eight patients also re- they were in the amputees. Preexisting deformities were often quired osteotomy of the tibia to treat anterior bowing and/or addressed during the lengthening procedures. However, limb valgus deformities. The tibial osteotomy was performed prior malalignment is also a frequent complication of lengthening or to the amputation in one patient, concomitant with the am- a result of subsequent fracture or bending of the regenerated putation in two, and following it in ﬁve. bone in the lengthening gap. The twenty-six patients treated with General questionnaire (see Appendix): There were no lengthening had a total of eighteen tibial and seven femoral oste- signiﬁcant differences between treatment groups with regard otomies as procedures separate from the lengthening procedures. to educational achievement, employment, income level, public Because foot and ankle deformities were not considered assistance, or disability payments. We also did not ﬁnd sig- to be exclusion criteria, patients with lengthening had frequent niﬁcant differences in reported limb pain, use of pain medi- surgical procedures for these problems. Eight patients had a cine, sports participation, reported activity restriction, dislike of total of nine soft-tissue procedures prior to their ﬁrst length- limb appearance, or satisfaction with treatment. Finally, we did 801 T H E J O U R N A L O F B O N E & J O I N T S U R G E RY J B J S . O R G d A D U LT O U T C O M E S F O L L O W I N G A M P U TAT I O N OR LENGTHENING V O L U M E 9 1-A N U M B E R 4 A P R I L 2 009 d d FOR FIBULAR DEFICIENCY not ﬁnd differences in comfort wearing shorts as rated on a scale dissatisﬁed to 100 for very satisﬁed. Younger patients were more of 0 for ‘‘so uncomfortable as to not wear shorts in public’’ to 10 satisﬁed with the prosthesis (p = 0.043), and this satisfaction was for being ‘‘fully comfortable wearing shorts in public without correlated with a higher level of comfort with wearing shorts in concern about appearance.’’ public (p = 0.0065). Beck Depression Inventory-II (see Appendix): We found no signiﬁcant difference in the scores on the Beck Depression Discussion Inventory-II between the amputees and the patients treated with lengthening. The controls had a signiﬁcantly higher total score than the amputees, suggesting that the control participants had T he purpose of this study was to compare long-term func- tional outcomes after amputation and lengthening for the treatment of unilateral ﬁbular deﬁciency. A large population of more depression. However, the mean scores for both groups patients with a diagnosis of ﬁbular deﬁciency was initially iden- were within the scale of ‘‘no indicators of depression.’’ Ac- tiﬁed, but many were excluded in order to achieve subject groups cording to the Beck Depression Inventory-II, no subject who with appropriate indications for either treatment. Of those who had had treatment of ﬁbular deﬁciency demonstrated indica- met our very strict criteria and had had a Syme or Boyd ampu- tors of depression. One control subject scored in the range of tation or a limb-lengthening, 63% were interviewed. Analysis of moderate depression. preoperative variables demonstrated that the patients who were Quality of Life Questionnaire (see Appendix): The only interviewed were, overall, eight years younger than, but were signiﬁcant difference between the treatment groups was on the otherwise representative of, the entire group treated with am- Job Satisﬁers content scale, with the amputees scoring better putation or lengthening. However, the patients who were not than the patients treated with lengthening. The Quality of Life found may have had other differences at the time of follow-up Questionnaire Manual14 indicates that those who achieve high that made them more difﬁcult to locate. scores on this scale report that they have good salary, beneﬁts, Because of the retrospective nature of this study and the chances for promotion, opportunities for involvement in job clinical variability of ﬁbular deﬁciency, it was not feasible to activities, reinforcement, and opportunities for training. Those have matched groups. Both procedures were performed at all who achieve low scores report that their pay and beneﬁts are centers, but treatment decisions were made by parents and poor, that they have little chance for involvement in job activ- surgeons without any established protocols. While some am- ities, that promotion is unlikely, and that they would change putees had one or two residual metatarsals at birth, all patients jobs if they could. For a number of the Quality of Life Ques- who were treated with lengthening had three or more. There tionnaire parameters, the ﬁbular deﬁciency group scored higher appeared to be a treatment bias for amputation in persons with than the controls. In all groups, the mean scores for all pa- fewer residual rays. This would seem to be appropriate by rameters were within one standard deviation of the reported today’s standards, as lengthening of a limb with one or two general population means of 50. residual rays would, at best, result in a limb of adequate length Short Form-36 (see Appendix): A comparison of the nor- with a foot so small it would be ineffective for push-off and mative scores for the different scales of the Short Form-36 did not cause problems with shoe wear. indicate any differences in health-related quality of life between Existing radiographs allowed classiﬁcation only on the the amputation and limb-lengthening treatment groups. Both basis of the amount of ﬁbular absence. There was a treatment groups had mean scores that were within one standard deviation bias toward amputation for patients with a larger degree of of the reported general population means of 50. ﬁbular absence or the presence of tibial bowing. This might be American Academy of Orthopaedic Surgeons Lower Limb expected since greater deﬁciency of the distal portion of the Module (see Appendix): There were no signiﬁcant differences ﬁbula results in difﬁculty with stabilizing and maintaining between the amputation and limb-lengthening groups with functional ankle alignment. Tibial bowing is also associated regard to lower-limb physical functioning or their reported with a greater degree of ﬁbular deﬁciency. As a result of the shoe-comfort scores. The mean score for both groups was retrospective nature of this study, there was insufﬁcient doc- within one standard deviation of the reported general popu- umentation of the amount of foot and ankle deformity and lation means of 50. overall limb-length discrepancy prior to treatment to allow American Academy of Orthopaedic Surgeons Foot and classiﬁcation on the basis of the more recent criteria used to Ankle Module: As assessed with the American Academy of Or- grade ﬁbular deﬁciency and for surgical planning28,29. However, thopaedic Surgeons Foot and Ankle Module, the foot and ankle the prevalence of ankle deformity and tarsal coalition appeared physical functioning of eleven patients treated with lengthening to be the same in the two groups. Retrospective chart and had a mean normative score of 45.4, with a range of 29.5 to 55.6. radiographic review may have led to underreporting of the This mean score was within one standard deviation of the re- frequency of these abnormalities since tarsal coalitions may not ported general population mean of 50. Two patients scored more be seen on radiographs of young children or be reported in than one standard deviation below the mean. operative reports on amputation specimens. Prosthesis Evaluation Questionnaire: All of the amputees As expected, patients treated with lengthening had more who were surveyed had a prosthesis, and a Prosthesis Evalua- frequent surgical intervention than did those with amputation. tion Questionnaire was completed for thirty-one of them. The Because of the time frame in which these adults were treated as mean score was 76.9 (range, 49 to 92) on a scale of 0 for very children, this review includes patients who were managed with 802 T H E J O U R N A L O F B O N E & J O I N T S U R G E RY J B J S . O R G d A D U LT O U T C O M E S F O L L O W I N G A M P U TAT I O N OR LENGTHENING V O L U M E 9 1-A N U M B E R 4 A P R I L 2 009 d d FOR FIBULAR DEFICIENCY techniques that are no longer used. Also, because many ad- ham Health Proﬁle, they assessed adult patients who had had vances in medical care and hospitalization practices have oc- major limb amputations. They found that a young age at the curred, the patients in both groups would have spent fewer days time of the amputation was associated with a better health- in the hospital and perhaps had fewer surgical procedures had related quality of life in the categories of physical disability, they been treated today. Because of these advances, the technical energy level, emotional reactions, and social isolation. The fact outcomes of limb-lengthening procedures done today might be that our patients had the amputation in childhood may explain better than those experienced by the former patients in this the high level of health and function that they were experi- study. The same can be said of the results of the amputations encing compared with general population norms. However, we because of advances in prosthetic design, suspension, and re- could not correlate outcomes to patient age at the time of the habilitation. Other authors have speculated that, despite the amputation in this study. higher initial costs of limb-lengthening due to greater medical In our control group of adults without general health or and surgical intervention, lengthening is less expensive than orthopaedic problems, the scores on the Beck Depression amputation if the lifetime costs of the prostheses are included7,30. Inventory-II and for a number of the Quality of Life Question- Unlike McCarthy et al.10, who performed a short-term naire parameters were signiﬁcantly below those for the patients follow-up study, we did not ﬁnd differences in the reported who had been treated for ﬁbular deﬁciency. While signiﬁcant, pain level or satisfaction with treatment between patients the mean differences between the groups were small. There is treated with lengthening and those treated with amputation. often a general assumption that people who have undergone Because our patients were not examined at the time of follow- major medical treatment for physical disabilities have a di- up, residual problems such as foot and ankle deformity, limb- minished quality of life. However, several studies have dem- length inequality, limb malalignment, and knee dysfunction onstrated that this is not the case. Livingston et al.34 reported could not be assessed. The responses to the American Academy that there is no consistent correlation between physical func- of Orthopaedic Surgeons Lower Limb Module did not reﬂect tion and psychosocial well-being. Studies have shown that the differences in levels of physical functioning, although it should quality of life of cancer survivors35 and patients with spinal cord be noted that, because of the time required for the interviews, injury 36 was equal to or better than those found in the general this questionnaire was completed by fewer patients than the population. Gerhards et al. suggested that psychological adap- other questionnaires. This presents a possible selection bias tation is a likely reason for these results31. Another reason for the based on subject fatigue. quality-of-life results found in our study may be that traumatic The Quality of Life Questionnaire showed that all patients events have a greater impact on quality of life than do congenital who had been treated for ﬁbular deﬁciency were experiencing a anomalies. A study investigating injury in adolescence showed quality of life in a range of slightly below to slightly above re- that major trauma in this age group is associated with signiﬁ- ported norms. Statistically, the only difference between the cantly lower quality-of-life outcomes compared with those in a treatment groups was in the scores on the Job Satisﬁers scale, healthy control group (p < 0.0001)37. In a series of studies of the with the amputation group scoring better than the limb- prospective Copenhagen Birth Cohort in Denmark, Ventegodt lengthening group. We found no signiﬁcant difference between et al.38,39 concluded ‘‘that our quality of life, health and ability as the amputation and control groups with regard to their scores adults are primarily determined by what we ourselves choose to on the Job Satisﬁers scale or the Occupational Relations scale on do with our lives as young people and as adults—and only to a the Quality of Life Questionnaire. However, the Job Charac- marginal degree determined by factors related to our back- teristics subscore for the controls was signiﬁcantly poorer than ground.’’ These reports corroborate the ﬁndings in the present that for the amputees. Despite these statistical differences, the study that the quality of life of adults who had been treated for mean scores on the Quality of Life Questionnaire for both of ﬁbular deﬁciency is not diminished, and is somewhat better, our treatment groups and for our controls were within one compared with that of nondisabled peers. standard deviation of reported general population means. In summary, we demonstrated that adults who had been Gerhards et al.31 demonstrated that adults with an acquired treated for ﬁbular deﬁciency in childhood function well, with an above-the-knee amputation reported greater job satisfaction average to above-average quality of life regardless of whether the than controls despite the amputees having a lower occupational treatment consisted of amputation or limb-lengthening. This status than the controls. Schoppen et al.32 showed that adults study did not show a clear preference for one treatment or the with an acquired lower-limb amputation had greater job satis- other. Amputation had a better outcome only in terms of the faction than able-bodied controls despite having worse physical score on the Job Satisﬁers content scale, while all other measures health scores on the Short Form-36 than the control group. of depression, quality of life, general health, and physical We also did not ﬁnd differences between our amputation functioning were not signiﬁcantly different between the two and limb-lengthening groups with regard to the scores on the groups. More sophisticated and disease-speciﬁc outcome tools Short Form-36. In both of our patient groups who had been may be able to identify differences. This study was not designed treated for ﬁbular deﬁciency, the score for general health to detect a clear superiority of one treatment over the other since outcome on the Short Form-36 was within one standard de- the two cohorts of patients differed with regard to the extent of viation of general population norms. Demet et al.33 reported on the deformity and the decision-making regarding the chosen health-related quality of life of amputees. Using the Notting- surgical treatment. At this point, recommendations to patients 803 T H E J O U R N A L O F B O N E & J O I N T S U R G E RY J B J S . O R G d A D U LT O U T C O M E S F O L L O W I N G A M P U TAT I O N OR LENGTHENING V O L U M E 9 1-A N U M B E R 4 A P R I L 2 009 d d FOR FIBULAR DEFICIENCY still have to be individualized and involve long discussions of 2425 Stockton Boulevard, the pros and cons of the available treatments. Sacramento, CA 95817 Juan Carlos Salazar, PhD Appendix ´ Escuela de Estadıstica, Universidad Nacional de Colombia, Tables showing details of the outcomes comparisons ´ Sede Medellın, Calle 59A, #63-020, Bloque 43, among the groups are available with the electronic ver- ´ Oﬁcina 109, Medellın, Colombia sions of this article, on our web site at jbjs.org (go to the article citation and click on ‘‘Supplementary Material’’) and on our James O. Sanders, MD quarterly CD/DVD (call our subscription department, at 781- Shriners Hospitals for Children, 1645 West 8th Street, Erie, PA 16505 449-9780, to order the CD or DVD). n John P. Lubicky, MD Indiana University School of Medicine, Riley Hospital for Children, 702 Barnhill Drive, ROC 4250, Indianapolis, IN 46202 Janet L. Walker, MD Dwana Knapp, MSW David M. Drvaric, MD Christin Minter, MA Shriners Hospitals for Children, 516 Carew Street, Shriners Hospitals for Children, 1900 Richmond Road, Springﬁeld, MA 01104 Lexington, KY 40502 Jon R. Davids, MD Jennette L. Boakes, MD Shriners Hospitals for Children, 950 West Faris Road, Shriners Hospitals for Children, Greenville, SC 29605 References 1. Froster UG, Baird PA. Congenital defects of lower limbs and associated mal- 17. American Academy of Orthopaedic Surgeons. Foot and ankle outcomes formations: a population based study. Am J Med Genet. 1993;45:60-4. questionnaire. Version 2.0. Revised 2005. http://www.aaos.org/research/ outcomes/Foot_Ankle.pdf. Accessed 2008 Dec 24. 2. Rogala EJ, Wynne-Davies R, Littlejohn A, Gormley J. Congenital limb anomalies: frequency and aetiological factors. Data from the Edinburgh Register of the New- 18. Legro MW, Reiber GD, Smith DG, del Aguila M, Larsen J, Boone D. born (1964-68). J Med Genet. 1974;11:221-33. Prosthesis evaluation questionnaire for persons with lower limb amputations: assessing prosthesis-related quality of life. Arch Phys Med Rehabil. 1998;79: 3. Duraiswami PK. 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