COPYRIGHT 2004

BY

THE JOURNAL

OF

BONE

AND JOINT

SURGERY, INCORPORATED

Total Hip Arthroplasty in

Patients with Dwarfism
BY JOHN B. CHIAVETTA, MD, JAVAD PARVIZI, MD, FRCS,
WILLIAM J. SHAUGHNESSY, MD, AND MIGUEL E. CABANELA, MD
Investigation performed at the Department of Orthopedics, Mayo Clinic, Rochester, Minnesota

Background: Patients with short stature and osseous deformities resulting from osteochondrodysplasia frequently
have premature development of advanced degenerative disease of the hip and thus may require total hip arthroplasty. The outcome of total hip arthroplasty in this group of young patients is unknown. In this study, we evaluated
the long-term clinical and radiographic outcomes of total hip arthroplasty in patients with osteochondrodysplasia.
Methods: Between 1971 and 1997, sixty-two total hip arthroplasties were performed at our institution in thirty-seven
patients with severe osteoarthritis secondary to osteochondrodysplasia. There were seventeen female patients and
twenty male patients. Their average height was 142 cm, and their average age at the time of the index arthroplasty
was thirty-seven years. The patients were followed clinically with use of the Harris hip score for a mean of 12.8 years,
and they were followed radiographically for a mean of 11.5 years.
Results: The mean Harris hip score improved significantly (p < 0.0001), from 57 points preoperatively to 87 points
at the time of the latest follow-up. Of the sixty hips available for follow-up, eighteen (30%) had required revision arthroplasty: fourteen required it because of aseptic loosening of one or both components; two, because of deep infection; one, because of periprosthetic fracture; and one, because of extensive osteolysis. There were two additional
periprosthetic femoral fractures, which were treated with open reduction and internal fixation with retention of the
components. The majority of patients had other functionally limiting conditions, such as spinal deformities, in addition to the degenerative arthritis.
Conclusions: There was a high prevalence of complications, periprosthetic fractures, and mechanical failure in these
patients with osteochondrodysplasia who underwent total hip arthroplasty. Young age, severe deformity, and multiple
joint involvement may in part explain these findings. Nonetheless, total hip arthroplasty proved to be reliable for alleviating pain and improving function in patients with advanced symptomatic arthritis of the hip secondary to osteochondrodysplasia.
Level of Evidence: Therapeutic study, Level IV (case series [no, or historical, control group]). See Instructions to Authors for a complete description of levels of evidence.

here are a variety of skeletal dysplasias that may result

in short stature, joint deformities, and early osteoarthritis of the weight-bearing joints1. In patients with
such dysplasias, alteration of the biomechanics of the joint
and/or abnormal articular cartilage are thought to contribute
to the initiation and progression of degenerative arthritis2-8. In
addition, these patients often have other skeletal deformities
that compound their disability.
While a few publications have described acceptable shortterm and mid-term outcomes following hip arthroplasty in osteochondrodysplastic patients9-13, the longer-term results of hip
arthroplasty in this patient population are unknown. The objective of this study was to report the longer-term outcome of
total hip arthroplasty in patients with skeletal dysplasia and
dwarfism who were treated at a single institution. In particular,
we tried to identify technical aspects of hip arthroplasty in these

patients that might explain the high prevalence of complications. We also report the outcome of total hip arthroplasty in
patients with conditions for which hip arthroplasty has not
been previously reported, to our knowledge.
Materials and Methods
Demographic Data
ll patients with osteochondrodysplasia who had undergone total hip arthroplasty between 1971 and 1997 were
identified in an institutional computerized database. Of
19,829 primary total hip arthroplasties performed at our institution during the years of the study, sixty-two were done in
thirty-seven patients with short stature. There were twenty
male patients and seventeen female patients, who had a mean
age of thirty-seven years (range, seventeen to eighty years).
The average height was 142 cm (range, 93 to 175 cm), and the


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Preoperative Data
All patients had a radiographically or clinically confirmed diagnosis of skeletal dysplasia. Nineteen patients (thirty-three
hips) had formal genetic evaluations performed at our institution, and a specific diagnosis of osteochondrodysplasia was
confirmed. Fourteen patients (twenty-one hips) had no record
of formal genetic evaluations at our institution, but a diagnosis
of a specific osteochondrodysplasia had been confirmed at a
different institution. Four patients of short stature (eight hips),
with a mean height of 140 cm, did not have a specific diagnosis
of an osteochondrodysplastic condition in their record. The
underlying diagnoses, for the hips for which it had been established, included spondyloepiphyseal dysplasia (twenty-eight
hips), Morquio syndrome (seven hips), multiple epiphyseal
dysplasia (seven hips), achondroplasia (four hips), diastrophic
dysplasia (two hips), pseudoachondroplasia (two hips), trisomy-21 (two hips), type-VI mucopolysaccharidosis (one hip),
and trisomy-13 (one hip); no etiology could be established for
four patients (eight hips). All patients had severe degenerative
disease of the hip joint. In addition, evidence of developmental
dysplasia of the hip was noted in eighteen hips.
Prior Operations
Fourteen hips had undergone a prior operation. These included proximal femoral osteotomy (ten hips), cup arthroplasty (two hips), combined proximal femoral osteotomy and
Judet hemiarthroplasty (one hip), and joint dbridement with
excision of osteophytes (one hip).
Fig. 1-A

Preoperative anteroposterior pelvic radiograph of a

twenty-three-year-old man with achondroplasia and
severe femoral deformity.

average weight was 52 kg (range, 25 to 86 kg). There was one

early death and one patient was lost to follow-up, leaving sixty
hips in thirty-five patients to be evaluated after a minimum
two-year follow-up.

Follow-up
Institutional approval and the consent of all patients were obtained prior to the initiation of this investigation; no patient
refused to participate in the study. Clinical and radiographic
data on all patients were collected prospectively. Patients were
contacted on a regular basis, which usually included examinations at three months, one year, two years, five years, and every
five years thereafter. Some follow-up contacts were conducted
with use of questionnaires or letters. The duration of clinical

Fig. 1-B

Postoperative lateral radiograph showing the custom-made prosthesis that

was used because of the complexity of
the left femoral deformity.


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cent line of >1 mm in width at the bone-cement interface or

any progressive migration or tilting of the component16. Uncemented femoral components showing progressive subsidence
or divergent radiodense lines about the stem were defined as
loose17. Uncemented acetabular components showing progressive migration or a change in position were defined as loose18.
Functional Evaluation
The clinical outcome was assessed with use of the Harris hip
score19.
Statistical Analysis
The changes in the Harris hip scores were evaluated with the
Wilcoxon signed-rank test. The chi-square test was used to
compare the rate of periprosthetic fractures in this population
with that after total hip arthroplasty in patients of normal stature. Significance was determined with use of a 95% confidence
level. A survivorship curve that included all hips in the study
was calculated, with revision for any reason as the end point.
Fig. 1-C

Postoperative anteroposterior pelvic radiograph showing the custommade femoral prosthesis on the left and the standard prosthesis on
the right.

follow-up averaged 12.8 years (range, two to twenty-five

years), and the duration of radiographic follow-up averaged
11.5 years (range, two to twenty-five years). All patients were
followed for a minimum of two years or until death or failure
of the prosthesis. One patient (one hip) was lost to follow-up
and one patient (one hip) died four weeks after surgery, leaving sixty hips in thirty-five patients followed for a minimum
of two years. There were four deaths (six hips), unrelated to
the hip arthroplasty, during the study period.
Surgical Data
Severe femoral shaft deformity was present in nineteen extremities, six of which required a custom-made femoral prosthesis to accommodate the deformity. Both components were
cemented in thirty-two hips, the femoral component was cemented and the acetabular component was uncemented in
twelve hips, and both components were uncemented in eighteen hips. Structural bone graft was used for reconstruction of
a shallow acetabulum in one hip. Morselized bone graft was
used to fill acetabular cavitary defects related to degenerative
cysts in five hips. No osteotomies were performed for correction of deformity.
Radiographic Evaluation
Serial anteroposterior and lateral radiographs of the treated
joint were reviewed by three of us (J.B.C., J.P., and M.E.C.) for
signs of loosening and evaluation of component position. A
cemented femoral component was defined as loose when
there was a complete radiolucent line at the bone-cement interface, subsidence of the stem or the mantle of cement, or a
cracked cement mantle14,15. A cemented acetabular component was defined as loose when there was a complete radiolu-

Results
Functional Outcome
he Harris hip score improved significantly (p < 0.0001),
from a mean of 57 points (range, 35 to 75 points) preoperatively to a mean of 87 points (range, 45 to 100 points) at the
time of final follow-up. Twenty-seven patients (forty-two hips)
had retained the original prosthetic components at the time of
the latest follow-up. The outcome was considered excellent or
good (a Harris hip score of >80 points, no use of walking aids,
and no pain in the hip) in sixteen patients (twenty-four hips)
and fair in twelve patients (eighteen hips). One patient had an
excellent outcome on the left side and a fair outcome on the
right. Of the twelve patients (eighteen hips) with a fair outcome,
nine (fourteen hips) had confounding factors that had adverse
effects on their hip score. These factors included deformity or
stenosis affecting the spine or degenerative disease of other
joints, including the knees or the contralateral hip.

Radiographic Findings
Prior to the hip arthroplasties, there was severe deformity of
the femoral head in thirty-seven hips, severe deformity of the
femoral neck in forty, and severe deformity of the femoral
shaft in nineteen (Figs. 1-A, 1-B, and 1-C).
At the latest follow-up examination of the unrevised
hips, progressive circumferential radiolucency was noted
around one cemented femoral component. Seven cups (five
cemented and two uncemented) were defined as loose, with
either progressive circumferential radiolucency or migration.
No other retained component was loose. The femoral component was in >5 degrees of varus in five hips and >5 of valgus
in seven hips. There was radiographic evidence of heterotopic
ossification, all Brooker grade II20, in five hips.
Complications and Mortality
Intraoperative complications included two linear proximal
femoral fractures that occurred during insertion of press-fit


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stems. The stems bypassed the fractures, both of which were

treated with cerclage wiring and healed uneventfully. Three
patients (three hips) had a Vancouver B1-type periprosthetic
fracture21, at an average of 5.5 years (range, 3.1 to 7.5 years) after the index arthroplasty. Two fractures were treated effectively with open reduction and internal fixation, with
retention of the components (Figs. 2-A and 2-B). The third
was treated with removal of a well-fixed stem and replacement
with a long-stemmed component. Dislocation occurred in
three hips despite what appeared to be satisfactory position
and fixation of the components. Other postoperative complications included transient sciatic nerve palsy (one hip) and

Fig. 2-A

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superficial wound infection (one hip).

Pneumonia due to prolonged airway support developed
in one patient with type-IV mucopolysaccharidosis (Maroteaux-Lamy syndrome), and the patient died from septicemia
four weeks following surgery. There were no other deaths
within the first two years after the total hip arthroplasty.
Revisions
Eighteen (29%) of the sixty hips required revision arthroplasty.
Three hips were revised within four years after the index operation. The reasons for revision included aseptic loosening of
both the cup and the stem (five hips), loosening of the cup with

Fig. 2-B

Fig. 2-A Anteroposterior radiograph made 7.5 years after the index arthroplasty, showing a periprosthetic fracture at the tip of the cement
mantle in a bowed femur. There was a delay in treatment, and callus is seen at the fracture site. The two staples seen in the distal part
of the femur are from a prior supracondylar osteotomy. Fig. 2-B The fracture was internally fixed with a plate and screws, and the component was retained.


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Fig. 3

Survivorship curve, including all hips, with revision for any reason as the end point. The dashed lines represent the 95% confidence interval.

a loose stem found intraoperatively (one hip), loosening of the

stem with a loose cup found intraoperatively (one hip), loosening of the cup with a well-fixed stem (five hips), loosening of the
stem with a well-fixed cup (two hips), periprosthetic fracture
with a well-fixed femoral component and a loose cup (one hip),
deep infection (two hips), and extensive osteolysis (one hip).
There was aseptic loosening leading to revision of nine cemented cups, five cemented stems, two uncemented cups, and
three uncemented stems. A survivorship curve was calculated
for all hips in the study (Fig. 3). A log-rank test comparing the
arthroplasties done from 1971 through 1985 with those done
after 1985 showed no significant difference in survivorship (p =
0.11), although there was a trend for better survivorship in the
hips treated prior to 1986.
Discussion
steochondrodysplasia may be caused by a genetic mutation affecting enzymatic function22, receptor function23,24,
proteoglycan storage25,26, or protein synthesis27.
A majority of patients with osteochondrodysplasia have
multiple joint involvement, often with severe deformities. The
alteration in biological, mechanical, and structural properties
of the joints accelerates the degenerative process, leading to
substantial disability in some patients. The longer-term results
of total hip arthroplasty in patients with skeletal dysplasia are
largely unknown. In a study of uncemented total hip replacements in patients with diastrophic dysplasia followed for an
average of five years, Peltonen et al.28 found that two of fifteen

hips required revision because of clinical and radiographic evidence of loosening of the acetabular component.
Our longer-term follow-up study of a relatively large
group of patients with osteochondrodysplasia revealed some
important findings. It confirmed that the severe articular or
periarticular deformity that is present in this group of patients
may cause technical difficulties in the performance of the hip
arthroplasty. In particular, component selection and positioning were found to be challenging in most of these patients. Use
of custom-designed prostheses and judicious use of intraoperative radiographs were both necessary for optimal component positioning in some patients. It is therefore imperative
that thorough preoperative planning be carried out prior to
the arthroplasty to ensure the availability of appropriate components during surgery.
The rate of postoperative periprosthetic femoral fracture in our study population (5%; three of sixty-two) was significantly greater (p = 0.0116) than the rate after all other
primary hip arthroplasties done at our institution over the
same time-period (1.3%; 251 of 19,767). All three fractures
were at the tip of the prosthesis or cement mantle, where a
stress riser was created in an already deformed femur. Although the rate of periprosthetic fracture is high in this population, we do not routinely perform realignment osteotomies
in bowed femora at the time of hip arthroplasty. It is difficult
to predict which femora are at risk for fracture, and the majority of the patients in this study had some degree of bowing of
the femora but did not sustain a periprosthetic fracture. We


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do, however, counsel patients in this population preoperatively

regarding this complication.
All patients in this series had multiple joint involvement, and the majority had spinal deformities as well. It is of
utmost importance to investigate the cause of hip pain in
these patients because, despite degenerative changes in the hip
joint, the pain may be referred from the lumbar spine. We often inject a local anesthetic agent into the hip joint preoperatively to evaluate the sources of the pain.
The presence of spinal deformities adds more complexity to the management of these patients. In some types of skeletal dysplasia, such as Morquio disease, odontoid hypoplasia
may result in cervical instability, which if unrecognized during
induction of anesthesia and positioning can have disastrous
consequences. The anesthesia team should be aware of the
multisystem involvement in this population and, in particular,
the possibility of cervical spine instability.
The multiple joint and spine deformities found in most
of these patients may result in alterations in gait and higher
loads on the prosthetic joints. In addition, we found that the
femoral components were in a suboptimal position in almost
one-quarter of the patients in this study. These observations
may explain in part the high revision rate that was encountered. We now routinely use intraoperative radiographs in this
patient population to avoid malpositioning.
The majority of the revisions and retained loose components in our series were in patients treated with a cemented acetabular cup, which is known to be associated with an inferior
outcome, particularly in young patients15. One other factor that
may have played a role in the higher failure rate was the fact that
the majority of our patients were young and fairly active and resumed unrestricted activity following the arthroplasty.
Interestingly, there was a trend for better survival of the
replacements in the patients treated between 1971 and 1985
compared with those treated after 1985. This difference in survival was not significant, however. Although there was no dif-

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ference in the average ages in the two groups, the patients in

the latter group were generally more active and had fewer
other comorbidities. This could explain this trend for a higher
rate of failure in the more recently treated patients.
There are limitations to this study. First, it was retrospective, with all the inherent limitations of such a study design. Second, the variety of underlying diagnoses and the use
of multiple prosthetic designs over a long time span both may
have been confounding factors. The refinements in surgical
techniques and prosthetic design that have occurred recently
may promise better outcomes for this patient group. In particular, the trend away from cementing acetabular components
in all age-groups, and particularly in young patients, could
potentially lead to better outcomes.
Despite these limitations, this study confirms that total
hip arthroplasty is an effective and reliable treatment option
for end-stage premature arthritis of the hip in short-stature
patients with osteochondrodysplasia.

John B. Chiavetta, MD
William J. Shaughnessy, MD
Miguel E. Cabanela, MD
Mayo Clinic, 200 First Street S.W., Rochester, MN 55905. E-mail address
for M.E. Cabanela: [email protected]
Javad Parvizi, MD, FRCS
Rothman Institute at Thomas Jefferson University, 925 Chestnut Street,
Philadelphia, PA 19107
The authors did not receive grants or outside funding in support of their
research or preparation of this manuscript. They did not receive payments or other benefits or a commitment or agreement to provide such
benefits from a commercial entity. No commercial entity paid or
directed, or agreed to pay or direct, any benefits to any research fund,
foundation, educational institution, or other charitable or nonprofit
organization with which the authors are affiliated or associated.

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