To The Editor:
We read with interest the article "Fibular Hemimelia:
Comparison of Outcome Measurements After Amputation and Lengthening" (2000;82:1732-5),
by McCarthy et al. The conclusion, "children who undergo
early amputation for the treatment of fibular hemimelia are more
active, have less pain, are more satisfied with the result of the
treatment, have fewer complications, undergo fewer procedures, and
incur less cost than those who undergo lengthening. . . . even though
good results can be obtained with lengthening procedures," warrants
closer scrutiny.
First, the number of patients who underwent limb-lengthening
in this study is very small (eleven limbs in ten patients). Only
six patients were treated with the Ilizarov method, and five were treated
with the older Wagner method, which is associated with more complications
and poorer results1. The mean
age at the time of amputation was 1.2 years (range, seven months to
2.3 years) compared with a mean age of 9.7 years (range, 5.5 to
18.3 years) at the time of the initial lengthening procedure. The
children in the lengthening group were followed until an average age
of 16.8 years in comparison with the children in the amputation
group, who were followed until an average age of 8.1 years. The
patients in the lengthening group were therefore treated later,
making treatment more difficult2,
especially that involving large leg-length discrepancies that require
as many as three lengthening procedures or two such procedures and
one epiphysiodesis. It is more difficult to obtain and maintain
correction of foot deformities that are not treated at an early
age2. The two groups in the study
by McCarthy et al. are therefore disparate; use of a case-matched
control format would have produced a more scientifically valid study.
When the children in the amputation group, who were followed until
an average age of only 8.1 years, grow to be larger and heavier adolescents
and, later, adults, their oxygen demands will progressively rise, and
they will tire easily. This phenomenon as well as a total dependency
on their prostheses and the long-term difficulties of stump care
may adversely affect their satisfaction with the procedure in their
later years.
A table detailing patient age and gender, severity of the deformity,
number and nature of surgeries performed, and associated complications
would have allowed the reader to more easily scrutinize the data.
In our experience, the number of rays in the foot and the percentage
of fibular shortening compared with the length of the ipsilateral
tibia have little correlation to the degree of severity of the hemimelia
or the final result of the surgery.
The authors place great emphasis on patient satisfaction without
going into any detail as to how this was measured. Standard outcomes
instruments such as the Short Form-36 or the Children Health Information
Service Rand Scale3 are more reliable
measures than the scale used in this study.
A very important issue not discussed by the authors is that of
the surgeon’s clinical experience with limb-lengthening and
reconstruction procedures. Of the twenty-one complications in the
limb-lengthening group, five were pin-site infections, which are
easily treatable with antibiotics and/or pin exchange. Bone-graft
dislodgment, another complication listed, is unique to the Wagner technique.
Tibial angulation has been observed to occur in association with fractures,
amputation stumps, and the tethering effect of the fibular anlage,
as has been described by Cozin2.
We minimize its occurrence by excising the anlage and overcorrecting
the bone regenerated by distraction toward varus alignment2. Similarly, anlage excision with
soft-tissue release and Achilles-tendon lengthening, along with
a supramalleolar varus and extension osteotomy and/or a
subtalar osteotomy, allows for lasting correction of the ankle valgus and
equinus2. This correction is made
during the first lengthening procedure, which can be performed when
the patient is as young as twelve to eighteen months of age. Daily
intensive physical therapy that focuses on knee extension helps
to prevent flexion contractures, as does the injection of botox
(botulinum toxin) into the gastrocnemius and soleus muscle complex
and the hamstring muscles. The tibial frame can be extended to the femur
with use of a knee hinge and an anterior extension bar, which allows
the knee to be locked in extension after physical therapy and at
night. If a contracture develops, a limited soft-tissue release
is required. We faced similar problems with limb-lengthening procedures
at our center in the earlier days; our results have improved as
our methods have evolved over time. We anticipate problems and obstacles
and manage complications with early intervention4.
McCarthy et al. raised the issue of cost, stating that amputation
for the management of fibular hemimelia requires fewer surgical
procedures and therefore seems to be less expensive than limb-lengthening
and reconstruction. In the Discussion section, they candidly admit that "the
cost analysis did not include the cost of prosthetics, which can
be quite high throughout a patient’s lifetime." The
accurate figure for prosthetic management after amputation (US $30,000
for the surgery alone) is in the range of US $403,000 over
the lifetime of the patient2,5.
This estimate does not take into account postoperative problems
relating to stump breakdown, phantom pain, spurs, and stump overgrowth.
The cost per procedure over the course of the limb-lengthening treatment
ranges from approximately US $50,000 to $59,0002,5 and is lower if epiphysiodesis
of the contralateral limb is used to avoid additional lengthening
surgery.
Finally, the very young age of the children in the amputation
group (the oldest child being 2.3 years of age) effectively precludes
their involvement in the decision-making process related to choice of
treatment. The irreversible and destructive nature of amputation
surgery raises many ethical issues and makes many parents uneasy
about deciding in favor of treatment with amputation6. If given an opportunity to participate in
the decision-making process at an appropriate age and given the
option of undergoing limb reconstruction with reproducible results,
would the child have made the same decision in favor of amputation?
Many older children resent irreversible surgical decisions made
on their behalf7.
We are of the opinion that, if a limb can be safely and reliably
lengthened, reconstructed, and preserved, amputation should be the
surgical treatment of last resort. Whenever feasible, efforts should
be made to involve the child in the decision-making process, especially when
an ablative procedure such as amputation is being considered.
J.J. McCarthy, G.L. Glancy, F.M. Chang, and R.E. Eilert
reply:
We thank Drs. Patel, Paley, and Herzenberg for their insightful
comments, and we would like to address each of them in order.
First, we agree that the number of patients in our study is quite
small, although there are only two other studies that directly compare
lengthening with amputation (both with a similar number of patients)8,9. Our study is unique because we
focus on clinical outcomes.
We recognize that, although the duration of follow-up was similar
for both groups, the age at follow-up was different. This is primarily
because the amputations were performed in patients at a much earlier
age. Our patients who have undergone amputation have not experienced
clinical deterioration as they have become older, and this observation
is supported in the literature10-12.
Additionally, patients are not "totally" dependent
upon their prostheses, as they can ambulate without them.
Second, Drs. Patel, Paley, and Herzenberg request a table detailing
patient age and gender, severity of deformity, number and nature
of surgeries performed, and associated complications. Although not
presented in the form of a table, this information is included in
the text of the article. The authors of the letter find that, in
their experience, the number of rays in the foot and the percentage
of the fibular shortening when compared with the length of the ipsilateral
tibia have little correlation to the degree of severity of the hemimelia
or to the final clinical result. We agree that other factors (discussed
in the body of our article) may play a role in determining the degree
of severity. We chose criteria from published classification systems13.
Third, Dr. Patel and colleagues suggest that we use the Children
Health Information Service Rand Scale3;
this does not directly assess patient satisfaction. In our article,
we used a patient self-reporting system (involving "yes" or "no" answers)
to determine if the patients were satisfied with the results of
treatment. Assessments of activity level and level of pain were determined
with use of a Likert-type scale as shown in Table I.
Fourth, all procedures were performed by the senior authors,
who have a great deal of clinical experience both with limb-lengthening
procedures and in the area of pediatric orthopaedics. As stated
in our article, the patients in the lengthening group actually did
quite well. In fact, if the complications in our patients were reviewed
and classified according to the system of Paley4,
in which complications were categorized as "problems," "obstacles," or "complications," we
would have had no true major complications. We appreciate the diligence
of Dr. Patel and colleagues in anticipating problems and obstacles
in the treatment of hemimelia, and we certainly make an effort to
do the same.
Fifth, as stated in the article, "we primarily used
the cost analysis as a measure of the duration and complexity of the
treatment and do not believe that cost should play a deciding role
in determining the treatment plan." The article that was
cited by Dr. Patel and colleagues in regard to the projected cost
per treatment dealt with procedures undertaken for conditions with
many different etiologies5; although
we agree that the costs associated with treatment after an amputation
can be extremely high, many problems secondary to amputation, such
as stump overgrowth, spurs, and stump breakdown, were uncommon in our
patients who had either a Syme or a Boyd amputation.
Lastly, we would like to address the comment about the benefit
of including children in the decision-making process related to
choice of treatment. Deciding whether a child should have surgery
is difficult for parents, and, understandably, they would like to
have the child involved in the decision. As the authors discussed,
treatment for correction of the foot begins when the patient is
quite young, at approximately the same age as that of patients having
conversion for a standard prosthesis by a Syme or Boyd amputation.
Involving a two or three-year-old child in such a decision-making
process is unreasonable. The counterargument might be to wait until the
child is an adult before allowing him or her to make the choice,
in which case, care could certainly be compromised, as was already
noted2. The reference cited by
Dr. Patel and colleagues regarding children resenting surgical decisions
made on their behalf (As Nature Made Him: The Boy Who Was
Raised As a Girl7) is
not hypothesis-based or peer-reviewed, nor can it be appropriately applied
to children undergoing amputation.
We thank Drs. Patel, Paley, and Herzenberg for their comments,
and we appreciate the opportunity to reply. We agree that a limb
can be safely and reliably lengthened, and, in fact, our article
supports this fully. On the other hand, early amputation of the
forefoot and prosthetic fitting is a reasonable alternative for
a functional long-term result, and parents should be fully informed
of all of the treatment options for their child.