14 Original article

The role of arthrography in selecting an osteotomy

for the correction of genu varum in pediatric patients
with achondroplasia
Shannon C. Fraser, Philip R. Neubauer and Michael C. Ain
Genu varum is a common manifestation of achondroplasia.
In the pediatric population, determination of the
appropriate corrective osteotomy can be challenging
because of a large cartilage envelope. To determine
whether osteotomy based on radiographs alone
corresponded to osteotomy based on arthrography and
radiography, we retrospectively studied 41 patients (75
limbs) and identified the appropriate osteotomy based on
(i) radiographs alone and (ii) radiographs and arthrography.
We found that the osteotomy choice changed in
45 limbs (60.8%) overall and in 34 limbs (94.44%) of
patients aged below 8 years.

c 2011 Wolters Kluwer Health

J Pediatr Orthop B 20:1416
| Lippincott Williams & Wilkins.

Introduction

of additional clinical modalities to improve accuracy [7].

In a study comparing supine fluoroscopy with full-length
standing radiographs for the assessment of lower limb
alignment, Sabharwal and Zhao [8] found that fluoroscopy
was useful in certain populations, but noted a trend toward
greater variability between the modalities in terms of
estimation of mechanical axis deviation between the two
modalities in younger patients, although the difference was
not statistically significant. The authors suggested that this
trend was a result of incomplete ossification with greater
soft-tissue laxity in younger patients.

Achondroplasia, the most common type of skeletal

dysplasia, is caused by an autosomal-dominant mutation
in the fibroblast growth factor receptor 3, which impairs
endochondral bone formation [1]. Clinical features include rhizomelic shortening of the limbs, short stature,
midface hypoplasia, and, commonly, genu varum [1].
Kopits [2] reported that approximately half of the patients with achondroplasia had substantial tibial bowing.
The cause of genu varum in achondroplasia is currently
unknown, but it results in pain, joint instability, and limitation of function [3]. Genu varum may be more likely to
occur in boys than in girls [4].
A subset of patients with achondroplasia and genu varum
requires osteotomies to eliminate or reduce clinically
significant pain and fibular thrust. Although osteotomy
can provide relief for such symptoms, it is not without
complications, including compartment syndrome, peroneal palsy, recurrence of the angular deformity, nonunion,
malunion, infection, growth-plate damage, and vascular
injury [5].
Traditionally, radiographs are used to determine the
center of rotation of angulation and, thereby, the appropriate site for the osteotomy procedure [6]. However,
because of the large cartilage envelope in young people,
a radiograph alone may not be sufficient for locating the
deformity site. Inan et al. [7] found poor interobserver
agreement using the mechanical axis deviation method
to measure the medial proximal tibial angle and lateral
distal tibial angle in children aged below 6 years. They
determined that significant measurement errors in the
proximal and distal tibial joints can occur, especially in
young children with achondroplasia, and suggested the use
c 2011 Wolters Kluwer Health | Lippincott Williams & Wilkins
1060-152X

Journal of Pediatric Orthopaedics B 2011, 20:1416

Keywords: achondroplasia, arthrogram, genu varum, osteotomy,
rab osteotomy
Department of Orthopaedic Surgery, The Johns Hopkins University,
Baltimore, Maryland, USA
Correspondence to Dr Michael C. Ain, MD, c/o Elaine P. Henze,
BJ, ELS, Medical Editor and Director, Editorial Services, Department of
Orthopaedic Surgery, Johns Hopkins Bayview Medical Center,
4940 Eastern Avenue, #A665, Baltimore, MD 21224-2780
Tel: + 1 410 550 5400; fax: + 1 410 550 2899;
e-mail: ehenze1@jhmi.edu

We postulated that using arthrography to visualize the

epiphyseal cartilage, especially in children, might change
the preoperative interpretation of the center of rotation
of angulation, thus affecting the type of osteotomy performed. Our goals were to determine: (i) whether the choice
of osteotomy based on radiographic findings alone would
have differed from that based on combined radiographic
and arthrographic findings, and (ii) whether the difference was more prevalent in one age group than another.

Methods
Patient population

Of approximately 700 patients with achondroplasia treated

by the senior author (M.C.A.) between January 1996 and
January 2006, we identified 41 pediatric patients (74 limbs)
who had undergone a corrective osteotomy for genu varum;
who had preoperative hip, knee, and ankle joint radiographs
and arthrograms; and who had a minimum of 2 years
of follow-up. Indications for surgery included clinically
significant pain secondary to malalignment and/or fibular
thrust. The average age of the 27 men and 14 women was
8.85 years (range: 317 years) (Fig. 1).
DOI: 10.1097/BPB.0b013e328340a7cf

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Arthrography, genu varum, achondroplasia Fraser et al. 15

Procedures

We retrospectively reviewed the standing anteroposterior

and lateral radiographs of each patient to determine the
center of rotation of angulation, and the senior author
(M.C.A.) identified the appropriate proximal osteotomy
location based on those radiographic findings alone. For
each patient, the lateral distal femoral angle, proximal
medial tibial angle, and distal tibial angle were drawn to
determine the most appropriate site for the osteotomy.
On a separate occasion, the senior author (M.C.A.) blindly
reviewed each patients arthrograms using the same
method as described above. The arthrograms had been
obtained a few days or immediately before surgery (stance
had been reproduced by applying pressure to the bottom
of the feet of the anesthetized patient in an attempt to
obtain realistic images). Each anteroposterior radiograph
and arthrogram had been obtained with the patients
patella forward, thereby providing the same view of rotation
in both studies. We noted the type of osteotomy that had
been used to correct the deformity and compared it with
the choice based on radiographic findings alone. All
osteotomies were in the metaphyseal region of the distal
femur, the proximal tibia, or the distal tibia. A change in
procedure was defined as a change in the site of osteotomy
between these three locations.
Procedures performed were closing wedge, opening wedge,
dome, and modified Rab (oblique) proximal tibial osteotomies. Our analysis included 55 proximal tibial osteotomies (26 of which used a modified Rab procedure), 32
distal tibial osteotomies, 10 distal femoral osteotomies, and
2 proximal femoral osteotomies. For the modified Rab
procedure, a dual screw technique was used in place of the
traditional single screw with a wedging cast [9]. With the
large degree of laxity often present in the knee ligaments
of these patients, the correction afforded with wedging cast
may occur at the ligaments of the knee rather than at the
Fig. 1

Number of patients

7
6
5
4
3
2
1
0

9 10 11 12 13 14 15 16 17
Patient age

Age distribution of the 41 patients in the study (average: 8.85 years;

range: 317 years).

site of the osteotomy. The dual screw technique provides

a more precise correction that can be locked into place,
potentially improving healing by reducing the mobility
allowed by the wedging cast.
Statistical analyses

We used a w2 test of association to determine whether the

patients age was associated with a change of osteotomy
procedure based on the addition of arthrographic findings. We compared the younger half of the patient
population (21 patients aged below 8 years) to the older
half (20 patients aged above 8 years) with respect to the
proportion of patients for whom the inclusion of arthrographic findings in the decision-making process changed
the procedure that would have been performed based on
only radiographic findings. The level of significance was
set at a P value of less than 0.05.

Results
We found that, in 26 out of 41 patients (63.4%; 45 of 74
limbs, 60.8%), inclusion of arthrographic findings in the
decision-making process changed the procedure that
would have been performed if only radiographic findings
were available.
When patients were grouped by age at the time of
surgery, we found that the inclusion of arthrographic findings changed the surgical procedure significantly more
often in the younger patients (i.e. aged below 8 years; 20
out of 21 patients, 95.3%; 34 of 36 limbs, 94.44%) than
in the older patients (i.e. aged above 8 years; six of 20
patients, 30.0%; 11 of 38 limbs; 28.95%).

Discussion
In answer to our first investigational question (did the choice
of osteotomy based on radiographic findings alone differ from
that based on combined radiographic and arthrographic
findings?), we found that a different procedure would have
been performed in 60.8% of limbs if the arthrogram had not
been performed. In most patients, the inclusion of the
arthrogram led to a change in the site at which the
osteotomy was performed. We recognize that internal
rotation of tibiae can also be an important component of
achondroplastic deformity of the lower extremity. Malrotation was addressed in each patient individually, depending
on where the osteotomy was performed. For example, in
cases where malalignment occurred in the femur and
malrotation was identified in the tibia, two osteotomies
were performed to address both deformities.
We believe that our claim about the inclusion of arthrography in the decision-making process contributes to the
selection of the most appropriate choice for each patient
is supported by our high rates of excellent (Fig. 2) and
good outcomes (as determined by each patients postoperative alignment, lessening of pain, and improvement
in function; by the lack of any cases of recurrence to date

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16 Journal of Pediatric Orthopaedics B 2011, Vol 20 No 1

Fig. 2

Radiographs for a 4-year-old patient with achondroplasia who underwent bilateral distal tibial and distal femoral osteotomies. (a) Preoperative standing
radiograph; (b) immediate postoperative radiograph; (c) radiograph of fully healed osteotomies 3 years postoperatively (patient age: 7 years).

at a minimum per-patient follow-up of 2 years; and by

only one malunion). For example, on the basis of standard
radiography alone, the patient shown in Fig. 2 would
likely have undergone a classical proximal tibial osteotomy using standard radiographs; however, with the addition of the arthrogram, the choice of procedure was
changed, resulting in the excellent outcome shown.
Historically, there is a generally acknowledged recurrence
rate of 30 to 50%. In a study evaluating osteotomy for the
correction of genu varum in patients with achondroplasia,
Beals and Stanley [10] reported 16 cases of recurrence in
39 procedures, two of which required reoperation. In our
study, we had no recurrences and no reoperations, which
we believe reflects improved decision-making and subsequent patient outcomes as a result of the inclusion
of the arthrogram. To our knowledge, there are no other
publication studies assessing the surgical correction of
genu varum in patients with achondroplasia; therefore,
our study represents the largest series to date.
In answer to our second investigational question (was the
choice related to patient age?), we found a significant
relationship between these parameters: most patients for
whom the choice would have changed were aged below
8 years, but the choice also would have changed for a notable
proportion of older patients (six out of 20, 30.0%). In a study
by Inan et al. [7], a greater degree of inconsistency in
measurements of lower extremity alignment in younger (r 6
years old) than in older patients was observed. Our study has
provided a potential method for reducing that variability.
Our investigation had a few limitations. First, not all patients were followed to skeletal maturity (to date, some are
still not skeletally mature). However, all patients did have
a minimum follow-up of 2 years. Second, a more ideal
study design might have been to have had two separate
preoperative evaluation groups, one in which radiographs
alone were used and one in which both radiographs
and arthrograms were used, which would have allowed for

a direct comparison of outcomes between the two

methods. However, this comparison was not an option
because of the retrospective nature of our study.
Despite these limitations, it could be argued that, based on
our results of no recurrence to date, one factor of such an
outcome is the accurate assessment of a patients center of
rotation of angulation. Therefore, our outcomes suggest
that the use of arthrography in conjunction with radiography, especially in the pediatric population, appears to
afford more complete visualization of the cartilage of the
hip, knee, and ankle joints than radiographs alone; the most
accurate interpretation of each patients center of rotation
of angulation; and the most appropriate choice of corrective
osteotomy, leading to a good postoperative outcome.

Acknowledgement
This study was supported in part by the Johns Hopkins
School of Medicine Deans Fund.

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