by Dr. Dror Paley, MD, FRSC
International Center for Limb Lengthening (ICLL)
Note from PFFDvsg Webmaster: This article is from the final section of Dr. Paley's Lengthening Reconstruction Surgery for Congenital Femoral Deficiency. I'm presenting this section here as a separate article because it is the best discussion I've seen of lengthening vs prosthetic treatment. I recommend that you read the whole paper (even though it may get a little confusing for us non-medical types).
Strategies and methods of lengthening reconstruction surgery(LRS) at our
center is described above. Prosthetic replacement surgery(PRS) refers to
Symes amputation and Van Ness Rotationplasty followed by prosthetic fitting.
The latter two PRS have been used almost indiscriminately for all types of
CFD. The reason for this dates back to the disastrous experience with the
Wagner technique for LRS of these femurs (13). Many patients were worse off
after the reconstruction than if surgery had never been performed (treatment
worse than the disease). In our experience with the above strategies in children
and adults not a single patient thus far falls into that category. We have
not compared our LRS results with PRS results since we do not have a comparable
cohort. Nevertheless our results in 54 patients with congenital short femur
syndrome are: Type la (45 patients) Excellent-32, Good-10, Fair-3 and Poor-0
(result score is based on clinical subjective, clinical objective and
radiographic criteria); Type lb (2 patients) Good- 1, Fair-1; Type 2a (1
patient) Good-1; Type 2b (3 patients) Excellent-l, Good - 1, Fair-1; Type
3a (1 patient Good -1; Type 3b (2 patients) Good-2. Many of these patients
have completed only one lengthening while others have completed as many as
three lengthenings. In a separate study of 70 Ilizarov femoral lengthenings
clinical and radiographic results were compared between congenital,
post-traumatic and developmental cases undergoing lengthening. There was
no significant difference in results based on etiology.
While more authors are recommending LRS, pseudarthrosis and the status of
the hip is used as a primary deciding factor for LRS vs PRS. It should be
emphasized that the hip status does not change after PRS. We argue therefore
that the status of the hip should not be a major deciding factor for PRS
or not. In fact hip procedures used for LRS are useful to stabilize the hip
and improve gait even if PRS is chosen. The status of the knee for us is
the deciding factor to recommend LRS vs PRS. Therefore our absolute indications
for PRS are primarily in Type 3 cases. In Type 2 cases it should also be
considered depending on how good the knee is and how much predicted discrepancy
there is. Type la&b should rarely be considered for PRS, unless there
is a stiff knee associated with these types. Finally in Type 1 CFD, LRS is
so reliable in our hands that PRS should only be considered when psychologic
or socio-economic reasons prevail.
One of the arguments for PRS is the contention that LRS leads to psychologic
scarring and loss of childhood. In our experience LRS if properly conducted
with an appropriate rehabilitation program and surgeries strategically spaced
apart does not lead to obvious psychological scarring to the child. In fact
my experience (having written several letters of recommendation to college
for these children) is that most children develop stronger characters and
a more goal oriented approach to life. It can truly be a growing
experience. LRS is an investment. The child invests part of their childhood
in order to live the majority of their life as an adult with as near normal
an extremity as possible. We try to complete the LRS before the child enters
high school whenever possible so that the formative years of body image at
the time when the children are most self conscious are with both limbs of
equal length and near normal function. In this manner most go through a normal
adolescence. The psychological stress of wearing a prosthesis during adolescence
is not well quantified by psychological profiles performed on these individuals
as adults. Therefore it is difficult to compare LRS vs PRS.
Psych-socio-economic stresses can play a major role in the decision LRS vs PRS. Single parents, marital difficulties, financial difficulties, drug problems, behavioral problems, learning disabilities and mental capacity, etceteras may interfere in the compliance, maturity, and home stability required to undergo LRS. PRS is easier, more painless and requires far less of the family. In such situations where the family would find it difficult to comply, or too stressful for the other family members PRS is the preferable option. Distance may play a factor too. If the patient is unable to commute to a center that can provide successful LRS then PRS may be a preferable option. This also applies to the postoperative rehabilitation required which is an absolute prerequisite. This problem may be soluble by rehabilitation hospitals and free care hospitals for children. Finally successful LRS requires a team dedicated to this type of treatment. It is not a procedure that should be performed casually or by surgeons inexperienced in the treatment of these patients. Experience in limb lengthening for other conditions is not sufficient to know how to successfully lengthen children with CFD. It requires a long commitment of time on the surgeonís part and on the part of the surgeons team. It requires appropriate rehabilitation services. If all of these facilities are not available then LRS should not be considered at that venue. The latter is perhaps the main limiting factor today in the availability of LRS.
Note from PFFDvsg Webmaster: For more information
contact:
The International Center for Limb
Lengthening
Sinai Hospital of Baltimore
2411 West Belvedere Avenue
Baltimore, Maryland 21215 USA
410-601-8700 800-221-8425
fax: 410-601-9576
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