HISTORY:
Sydney is a lovely young lady, 3 years old and otherwise
healthy, who was born with right postaxial hypoplasia. She presents today for
evaluation of lengthening versus Syme amputation. She has seen multiple doctors
here in Utah, as well as folks at Texas Scottish Rite, and has gotten a number
of opinions. She is going to start preschool this fall and the family would
like to go ahead with beginning treatment now.
PHYSICAL EXAMINATION:
On physical examination, Sydney is a beautiful 3-1/2-year-old
young lady. She is alert, oriented, and cooperative with the examination. She
walks well with mild genu valgum and a valgus thrust at the right knee. The
range of motion of the ankle shows she has a very tight Achilles and a rigid
hind foot consistent with probable coalitions. She has a 4-rayed foot, which is
plantigrade, but the ankle is already in fairly significant valgus; with stance
she already has impingement of the fibula on the foot, although she is not
having pain here. The tibia is short compared to the other side at about 2
centimeters. The knee is also smaller than the contralateral side and her ACL
(anterior cruciate ligament) is clearly out with a soft endpoint on anterior
drawer. The femur is also shorter compared to the left side and smaller,
roughly 1.5 to 2 centimeters. She has wide symmetric abduction and no sensation
of instability at the right hip. She denies any pain with ambulation.
RADIOGRAPHS:
Her longstanding film shows the right side to measure at 368
and the left at 406. She is in significant valgus on her shorter side secondary
to lateral femoral hypoplasia. Femur to femur, she is 210 and 225, giving us a
difference of 1.5, right shorter than left. Tibia to tibia, she is 148 to 168,
therefore a delta of 2 centimeters, right shorter than left.
IMPRESSION:
Postaxial hypoplasia with limb length inequality involving
both the femur and the tibia, and significant lateral femoral condyle
hypoplasia giving us valgus. She already has ankle valgus, coalitions in the
foot, a 4-rayed foot, and some very mild dysplasia of the right hip with some
valgus present.
PLAN:
We had a lengthy discussion today about lengthening versus
Syme amputation. With lengthening my concerns from the base up are as follows.
She has a 4-rayed foot, which is plantigrade and which is good; however, her
ankle is already in valgus, and she has already had previous treatment for
Achilles contracture. I am very concerned with lengthening of the tibia as much
we would have to in that we probably would need 1 to 2 lengthenings over time,
and the Achilles will become significantly problematic as would the ankle
valgus, leading us to having to do further procedures on the foot and the
Achilles to get her foot underneath her tibia. Certainly, the anterior cruciate
ligament being absent would also mean we would have to watch carefully the knee
with the tibial lengthening to make sure there is no subluxation. I am also
concerned about the very small nature of her femur, and the fact that I think
if we lengthen her tibia she would probably need a femoral lengthening in the
future, too, as her femur is about 93% of the contralateral side, and this
discrepancy would worsen over time. Certainly, she would need guided growth at
the distal medial femur, but I think she also might need a femoral lengthening
to better equate her knee height, especially in the face of a stiff ankle on
the right side. The other concern I have about the femoral lengthening is that
she does have some mild hip dysplasia, and we would have to make sure that her hip
was stable enough to undergo a femoral lengthening and not have further
subluxation. In addition, of course, the femoral lengthening would be
complicated by the fact that she does not have an anterior cruciate ligament at
the knee. Therefore, a number of procedures would have to be done, possibly
reconstruction of the right hip, femoral lengthening with concern about
possibly bypassing the need to stabilize the knee since there is a lack of
anterior cruciate ligament, or repeated guided growth which she would need for
either a Syme amputation or a lengthening. On the tibia, a tibial lengthening
concerns me not only because the anterior cruciate ligament is absent, but also
because of the ankle is stiff, as is the foot, and already in valgus. I would
worry that this would worsen significantly with tibial lengthening.
With regards to undertaking amputation, she is a little
older than we normally would amputate, but I think that she has a great,
supportive family and a terrific personality. Because she is not yet in
kindergarten, I think we still have youth on our side. With a Syme amputation,
she still will need guided growth in the future, and the family is aware of
this, at the right distal femur. She may also need an epiphysiodesis on the
contralateral femur if the knee height becomes problematic, although I find
that the knee height is not quite as symptomatic when there is a prosthesis as
when there is a stiff ankle as on the right.
The family at this point are leaning towards amputation, and
we filled out a booking sheet for that. I am also going to send them a copy of
this note so they can review the notes, and further discuss and think about
their options. If we do surgery here, I would plan for an inpatient stay of
probably 2 days for an epidural to minimize her phantom limb issues. We would
then undertake a prosthesis fitting at roughly 6 weeks out to hopefully have
her up in a prosthesis by 8 to 9 weeks from the time of her surgery. She would
need some physical therapy as well to help her with gait training with her
prosthesis. We had the orthotics and prosthetics staff meet her today, and they
are also going to walk her around the lab so she can see what is going on in
the lab and see some other prostheses. Angela helped me with the booking sheet
and will also get her in touch with some other families for a below-knee
amputation.
Hopefully this is helpful!!
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