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ORIGINAL ARTICLE

Multilevel Guided Growth for Hip and Knee Varus


Secondary to Chondrodysplasia
Peter M. Stevens, MD and Eduardo N. Novais, MD

Key Words: coxa vara, genu varum, Schmid-type chon-


Background: Young children with chondrodysplasia may de- drodysplasia, guided growth, 8-plate
velop multilevel varus deformities that compromise comfort and
gait. The classic treatment of performing corrective, staged os- (J Pediatr Orthop 2012;32:626–630)
teotomies, at each level of deformity, is a daunting prospect that
is fraught with potential complications. To avoid this scenario,
we have adopted single-event, multilevel surgery, using guided
growth to simultaneously address bilateral varus deformities of
the knee and hip, with good results.
C hildren with chondrodysplasia often present as tod-
dlers with parental concerns focusing upon bowed
legs, limping, and decreased walking tolerance due to
Methods: Three cousins with Schmid-type metaphyseal dyspla- fatigue and pain. These symptoms and findings are non-
sia, presented for treatment of progressive varus deformities. In physiological in nature and usually progress without
lieu of osteotomies, we used simultaneous guided growth of the treatment. In addition to the obvious genu varum and
proximal and distal lateral femora and proximal lateral tibiae, intoeing, coxa vara and ankle varus contribute to the
while ignoring the distal tibial deformity. The pan-genu 8-plates complex clinical and radiographic picture. The traditional
served to neutralize the mechanical axis while preserving a means of managing these deformities is to perform re-
horizontal knee. The rationale for applying the trochanteric alignment osteotomies.1–3 If one strictly adheres to the
8-plate was to stabilize the proximal femoral chondroepiphysis, center of rotational axis of deformity principles, this
hoping to postpone or avert intertrochanteric osteotomy. The would require 12 osteotomies (proximal and distal fem-
average age at surgery was 28 months, with a range of 19 to 33 ora, proximal and distal tibiae, and fibulae) to address all
months, and follow-up has ranged from 28 to 59 months of the deformities. They would have to be staged, re-
(average 48 mo). The pan-genu 8-plates were removed after an quiring several hospitalizations and serial cast immobili-
average of 12 months, leaving the trochanteric implants in situ, zation. Even with optimal realignment, recurrent deformity
pending further growth. may occur in 30% to 70% of the cases.4,5
Results: Neutralization of the mechanical axis resolved lateral There has been increasing acceptance of guided
knee thrust and intoeing. As the femur is effectively adducted by growth for pediatric knee deformity correction.6,7 Cor-
knee realignment, the greater trochanteric impingement on the rection of genu varum effectively adducts the femora and
ilium is alleviated. Lateral tethering of the trochanteric apophysis indirectly improves the Hilgenreiner angle,8 while allevi-
served to increase the femoral neck-shaft angle, improving the ating greater trochanteric abutment against the ileum. In
abductor lever arm. Each patient experienced complete resolution an analogous manner, the added benefit of rendering the
of the fatigue hip pain and Trendelenburg gait. As knee alignment tibia vertical, through knee correction, is to mitigate the
was restored, the ankle varus resolved spontaneously, requiring apparent varus of the ankle.
no direct treatment. The clinical improvement was reflected in We reasoned that the same technique and principles
trending of the radiographic angles and axes toward normal. of guided growth should benefit the hip and further im-
Conclusions: These children have benefited from outpatient prove abductor efficiency by preventing trochanteric
guided growth, rather than the anticipated osteotomies, to “overgrowth.” Mindful that the proximal femur is capped
correct multilevel varus deformities. Our goal is to exclusively by a common chondroepiphyses, we have recently
use guided growth, repeatedly as needed, to avoid osteotomies switched from intertrochanteric osteotomy to guided
altogether. Annual follow-up until skeletal maturity is planned. growth, tethering the trochanteric apophysis laterally
Level of Evidence Level IV—retrospective case series. with a tension band 8-plate. For chondrodysplasia man-
agement, our intent is to stabilize the proximal femur,
allowing the femoral neck stress fracture (“triangular
From the Department of Orthopaedics, University of Utah, Salt Lake
City, UT.
metaphyseal defect”) to heal. This alleviates the fatigue
The authors received no financial support for this study. pain and Trendelenburg gait, while improving the range
Dr Stevens receives royalties form Orthofix, Inc on the 8-plate. The other of hip abduction. In this report, we review the rationale
author declares no conflict of interest. and initial results of this minimally invasive technique of
Reprints: Peter M. Stevens, MD, Department of Orthopaedics, Uni- single-event, multilevel guided growth. We recognize that
versity of Utah, P.O. #58246, Salt Lake City, UT 84158. E-mail:
peter.stevens@hsc.utah.edu. longer follow-up will be required before we can determine
Copyright r 2012 by Lippincott Williams & Wilkins whether this represents a definitive approach.

626 | www.pedorthopaedics.com J Pediatr Orthop  Volume 32, Number 6, September 2012


J Pediatr Orthop  Volume 32, Number 6, September 2012 Multi-level Guided Growth for Hip and Knee Varus

METHODS
This series includes 2 boys and 1 girl (all cousins) HILGENRIENER’S
who underwent hip and knee guided growth for the ANGLE H LINE
treatment of bilateral and symmetrical, multilevel varus
deformities resulting from Schmid-type metaphyseal
chondrodysplasia. Each subject presented with fatigue
pain and gait disturbance due to symmetrical and pro-
gressive varus deformities of the hips, knees, and ankles.
Interestingly, an affected parent of each child had pre- FEMORAL
viously been treated by the senior author (P.M.S.) with PHYSIS/PHYSIS - ZONES +
staged, bilateral, multilevel, valgus-producing osteoto- ANGLE
mies of the proximal and distal femora, as well as the MECHANICAL AXIS
proximal tibia/fibula. This had required multiple hospi- ZONE-3
talizations with the requisite cast immobilization and 3 2 1 1 2 3
delayed weight bearing, after the osteotomies. These pa-
rents were exceptionally well informed and fully suppor-
TIBAL
tive of the strategy of guided growth as a means of PHYSIS/PHYSIS
forestalling, or perhaps preventing, osteotomies. The ANGLE
possibility of rebound deformity was fully discussed,
along with the options of repeating guided growth versus
eventual osteotomy.
The medical records were retrospectively reviewed
for collection of clinical data before and after surgical
intervention including assessment of gait pattern,
Trendelenburg sign, range of hip abduction (standing),
standing femoral intercondylar distance, and doc- FIGURE 1. Radiographic measurements: Coxa vara meas-
urements included Hilgenreiner’s angle (H-A) = verticality of
umentation of ligamentous laxity and torsional profile. the femoral neck physis relative to a horizontal H line and
We reviewed the preoperative and follow-up ante- physis/physis angle (P/P-/A). The latter measurement proved
roposterior standing radiographs of the lower limbs to to be the most specific and reliable. The mechanical axis de-
measure the mechanical axis deviation and displacement viation is depicted on the left leg. See table for pre to post-
by zones (Fig. 1) and the orientation of the proximal/ operative comparisons.
distal femoral physes and the proximal/distal tibial
physes, relative to each other. We felt that in this age and guide pins inserted into the cartilaginous trochanter
group, these measurements were more reproducible and and metaphysis of the femur. Although it may be coun-
relevant than assessing anatomic angles such as the lateral terintuitive to place a screw in cartilage, indeed it had
distal femoral angle, proximal medial tibial angle, and good purchase and none have failed to date. Two fully
lateral distal tibial angle. We also reviewed preoperative threaded, cannulated 4.5-mm screws are used to secure
and follow-up anteroposterior pelvic radiographs, with
the legs rotated inward 15 degrees, to demonstrate the
metaphyseal defect that is indicative of a stress fracture,
presumably exacerbated by vertical shear (Fig. 2). The
Hilgenreiner-epiphyseal angle, the epiphyseal-diaphyseal
angle, head-shaft angle, and the neck-shaft angle were
recorded.
TROCHANTERIC
IMPINGEMENT
Surgical Technique
The optimal age for tethering the proximal femoral
chondroepiphysis is between 2 and 3 years old. This
promotes healing of the medial “triangular defect” (stress
fracture) of the femoral neck. The hip portion of the
surgery is described herein. With the patient supine, a
bump was placed under the sacrum, allowing them to be STRESS FRACTURE

rolled toward the opposite side and elevating the tro- A.I.R. VIEW
chanter off the surgical table. This reveals the proximal
femur in profile, including the true neck-shaft angle and
the metaphyseal defect. A 3 to 4 cm lateral incision is FIGURE 2. On an abduction, inward rotation view (AIR) the
centered at the base of the trochanter, and the iliotibial stress factures on the medial femoral neck metaphyses are
band is split. A Keith needle is inserted into the physis, readily apparent. Much of the greater trochanter is unossified
parallel to its plane. The plate is centered on the needle (dotted line).

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Stevens and Novais J Pediatr Orthop  Volume 32, Number 6, September 2012

vara improved in all patients (Table 1). With respect to


the knees, all 3 patients have demonstrated complete
correction of genu varum and with neutralization of the
mechanical axis, accompanied by resolution of lateral
thrust and inward torsion. In each patient, the ankle
varus has resolved, without need for intervention. They
are now being seen on an annual basis and this will be
continued until skeletal maturity.
Each patient had the pan-genu plates removed when
the mechanical axis was rendered neutral. All of the tro-
chanteric 8-plates remain in situ and intact; they will be
removed only if and when they become symptomatic.
After eventual trochanteric 8-plate removal, the children
will be observed for recurrent varus and treated
accordingly–either with guided growth or with inter-
trochanteric osteotomy. The parents, who had experi-
enced the traditional approach of multiple osteotomies,
hospitalizations, and casts, are very enthusiastic about the
changing paradigm of treatment for their progeny.

Case #1
T.F. presented at the age of 19 months with a
waddling gait, fatigue hip pain, genu varum, lateral
thrust, and intoeing. His father had undergone bilateral
osteotomies of the distal femora and tibias, as well as
intertrochanteric osteotomies. Reasoning that he was too
young for the intertrochanteric osteotomies but in need of
knee correction, T.F. underwent pan-genu guided growth
with 8-plates in the lateral distal femora and lateral
FIGURE 3. Intraoperative fluoroscopic view showing proper
placement of the 8-plate. The proximal screw purchase in proximal tibias. His angular and rotational deformities
cartilage +/ bone is secure. corrected to neutral, and he was fully active, without
complaints. However, the proximal femoral varus re-
the plate to the femur, confirming this with the fluoro- mained unchanged; rather than subject him to inter-
scope (Fig. 3). The wound is closed in layers and a soft trochanteric osteotomies at the time of pan-genu plate
dressing applied. In a similar manner, under sterile removal, 8-plates were applied to the trochanteric
tourniquet control, we applied 8-plates to the distal lat- apophyses. Nearly 6 years later, he has no symptoms, no
eral femur and the proximal lateral tibia as previously limp, and no functional limitations. The plates will be left
described.6 We found it unnecessary to perform a fibular in situ pending further growth.
epiphysiodesis. We did not address the ankle varus. The
triple level guided growth was performed on an out-
Case #2
patient basis, applying an 8-plate (Orthofix, Verona, O.G. presented at the age of 2 and ½ with the same
Italy). No cast immobilization or restriction of activities clinical and radiographic findings. Simultaneous guided
was required. growth was undertaken, applying bilateral 8-plates to the
proximal and distal lateral femora, as well as to the
proximal lateral tibiae. At 18 months of follow-up (age 4),
RESULTS the mechanical axis and torsional profile were neutral and
The 3 patients included in this series were followed the pan-genu plates removed. His Trendelenburg gait has
for an average of 4 years (range, 28 to 59 mo). The first resolved and he has no complaints of pain or fatigue. He
patient had plates applied to the greater trochanter at the is now at 4 and ½ years status post–guided growth for
time of pan-genu plate removal. The other 2 underwent coxa vara, and the trochanteric plates remain in situ. It is
simultaneous guided growth of the proximal femur at the noteworthy that his mother had had staged, pan-genu
same time as pan-genu plate insertion. All patients healed osteotomies but never had her hips treated. At the age of
without complications. The fatigue hip pain resolved 30, she is now quite symptomatic with progressive hip
within 3 months and the waddling gait by 6 months. At discomfort, limited abduction, and Trendelenburg gait
follow-up, each patient has a normal gait, improved hip and is seeking treatment (Fig. 4).
abduction, negative Trendelenburg sign, alleviation of hip
pain, and no functional limitations. The femoral neck Case #3
“triangular defect” (presumed stress fracture) healed in all D.F. first presented at the age of 11 months for
patients. Similarly, the radiographic parameters of coxa evaluation, already manifesting the stigmata of Schmid

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J Pediatr Orthop  Volume 32, Number 6, September 2012 Multi-level Guided Growth for Hip and Knee Varus

TABLE 1. Radiographic Measurements Pre- and Postoperative


HEA (deg.) ESA (deg.) HSA (deg.) NSA (deg.) MAD (mm) Femur Physes-Physes (deg.) Tibia Physes-Physes (deg.)
Age FUP
Sex (y) (y) Side Pre Post Pre Post Pre Post Pre Post Pre Post Pre Post Pre Post
M 2.7 4.8 R 59 41 30 34 116 121 98 106 49 0 56 42 22 1
L 61 39 32 35 116 123 99 105 48 6 58 41 25 1
M 2.7 4.4 R 70 45 38 44 122 129 104 112 48 0 63 46 23 3
L 71 46 42 45 123 132 105 113 50 2 58 45 27 4
F 1.5 2 R 61 42 36 52 120 134 98 115 34 3 67 40 20 2
L 58 41 40 56 126 136 104 115 47 0 57 42 29 3
ESA indicates epiphysis-shaft angle; FUP, follow-up period; HAS, head-shaft angle; HEA, Hilgenreiner-epiphyseal angle; MAD, mechanical axis deviation (from
center of knee); NSA, neck-shaft angle.

dysplasia, including the characteristic metaphyseal defects DISCUSSION


in the femoral neck and genu varum. At the age of 19 We report a series of 3 consecutive patients who
months, she underwent combined pan-genu and proximal underwent guided growth for the treatment of coxa vara
femoral guided growth, without cast immobilization or and genu varum secondary to Schmid-type metaphyseal
hospitalization. At 12 months of follow-up, she had chondrodysplasia. At an average of 4 years of follow-up,
neutral mechanical axes and the pan-genu plates were we observed normal alignment of the lower extremities on
recently removed, again leaving the hip plates in situ all patients and continuous improvement of the varus
(Fig. 5B). She continues to do well at the age of 3 years, deformity of the proximal femur.
11 months. Her mother had been treated by means of Schmid-type metaphyseal chondrodysplasia is the
multiple osteotomies and was therefore expecting the most common form of metaphyseal dysplasia.9 The family
same for the daughter. history, combined with obvious genu varum and restricted
hip abduction typically suggest the diagnosis in infancy. By
the time the child commences weight bearing, the com-
bined effects of gravity and muscle loading across joints
serve to accelerate varus deformity at all levels. In the
ambulatory child, complaints of fatigue pain and intoeing
often precipitate medical consultation. Serial standing
radiographs will reveal the severity and progression of
deformities noted at the hip, knee, and ankle. The classic
teaching for treatment of multilevel varus secondary to
chondrodysplasia involves surgical correction by means of
femoral intertrochanteric osteotomt.2,4,5,10,11 Despite well-
done osteotomies, however, recurrence of coxa vara is cited
in the literature as ranging from 30% to 70%.2,10,12 Sur-
gery in very young child may offer the advantages of op-
timizing abductor efficiency and avoiding progression of
acetabular dysplasia. In contrast, in a very young child, the
proximal femur is mainly cartilaginous that may difficult
fixation and increase the risk of recurrence.5
Guided growth is based upon the concept that a
tension band plate, applied to one side of the physis, will
reversibly inhibit growth on that side while permitting the
opposite side to grow in a normal or possibly accelerated
manner.6 This technique has been well documented in the
literature for correcting knee deformity associated with
+3 cm. osteochondrodysplasias.8,13 Heretofore, there have been
no reports of its use in the proximal femur. Knowing the
risks and vagaries of valgus intertrochanteric osteotomies,
including recurrent varus,5 we were reticent to subject our
FIGURE 4. The concept of 3 level guided growth is to increase
the effective neck shaft angle while adducting the leg and patients to this procedure at such a young age. At the
correcting the genu varum, without the need for osteotomy or same time, we felt that stabilization of the upper femur
immobilization. This is not a “growth arrest”; note the 3 cm. would alleviate pain and allow the fatigue fracture to
gain in limb length (dotted arrow) and improvement in the heal. Guided growth of the proximal femur serves as an
abductor lever arm. adjunct, optimizing medial growth and reducing the shear

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Stevens and Novais J Pediatr Orthop  Volume 32, Number 6, September 2012

A B C D

19 mos.

AGE
31 mos.
OUT PATIENT
NO SPICA CAST PANGENU Age 4
IMMEDIATE MOBILIZATION PLATES
REMOVED

FIGURE 5. A, B, Pre- and postoperative comparison (patient 2) showing the interval change over 12 months. The pan-genu plates
were removed and the trochanteric plates were left in situ. C, Age 4 correection maintained. D, Clinical abduction – asympto-
matic/Trendelenburg negative gait.

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