Adult Outcomes Following Amputation or Lengthening for Fibular by mikesanye


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Adult Outcomes Following Amputation or Lengthening for Fibular
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

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Publisher Information           The Journal of Bone and Joint Surgery
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                                   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
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       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
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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
                 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
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   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
   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
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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
               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
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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
                  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
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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 (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

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