The mucopolysaccharidoses (MPS) are a group of
inherited lysosomal storage disorders with clinical manifestations relevant
to the orthopaedic surgeon. Our aim was to review the recent advances
in their management and the implications for surgical practice. The current literature about MPSs is summarised, emphasising
orthopaedic complications and their management. Recent advances in the diagnosis and management of MPSs include
the recognition of slowly progressive, late presenting subtypes,
developments in life-prolonging systemic treatment and potentially
new indications for surgical treatment. The outcomes of surgery
in these patients are not yet validated and some procedures have
a high rate of complications which differ from those in patients
who do not have a MPS. The diagnosis of a MPS should be considered in adolescents or
young adults with a previously unrecognised dysplasia of the hip.
Surgeons treating patients with a MPS should report their experience
and studies should include the assessment of function and quality
of life to guide treatment. Cite this article:
Nail patella syndrome (NPS) is a skeletal dysplasia with patellofemoral
dysfunction as a key symptom. We present the first in-depth radiological
evaluation of the knee in a large series of NPS patients and describe
the typical malformations. Conventional radiological examination of 95 skeletally mature
patients with NPS was performed. Patellar morphology was classified
according to the Wiberg classification as modified by Baumgartl
and Ficat criteria, and trochlear shape was classified according
to the Dejour classification.Aim
Patients and Methods
Clinical and radiological data were reviewed for all patients
with mucopolysaccharidoses (MPS) with thoracolumbar kyphosis managed
non-operatively or operatively in our institution. In all 16 patients were included (eight female: eight male; 50%
male), of whom nine had Hurler, five Morquio and two Hunter syndrome.
Six patients were treated non-operatively (mean age at presentation
of 6.3 years; 0.4 to 12.9); mean kyphotic progression +1.5o/year;
mean follow-up of 3.1 years (1 to 5.1) and ten patients operatively (mean
age at presentation of 4.7 years; 0.9 to 14.4); mean kyphotic progression
10.8o/year; mean follow-up of 8.2 years; 4.8 to 11.8)
by circumferential arthrodesis with posterior instrumentation in
patients with flexible deformities (n = 6).Aims
Methods
To present our experience of using a combination of intra-articular
osteotomy and external fixation to treat different deformities of
the knee. A total of six patients with a mean age of 26.5 years (15 to
50) with an abnormal hemi-joint line convergence angle (HJLCA) and
mechanical axis deviation (MAD) were included. Elevation of a tibial
hemiplateau or femoral condylar advancement was performed and limb
lengthening with correction of residual deformity using a circular
or monolateral Ilizarov frame.Aims
Patients and Methods
We present the results of the surgical correction of lower-limb deformities caused by metabolic bone disease. Our series consisted of 17 patients with a diagnosis of hypophosphataemic rickets and two with renal osteodystrophy; their mean age was 25.6 years (14 to 57). In all, 43 lower-limb segments (27 femora and 16 tibiae) were osteotomised and the deformity corrected using a monolateral external fixator. The segment was then stabilised with locked intramedullary nailing. In addition, six femora in three patients were subsequently lengthened by distraction osteogenesis. The mean follow-up was 60 months (18 to 120). The frontal alignment parameters (the mechanical axis deviation, the lateral distal femoral angle and the medial proximal tibial angle) and the sagittal alignment parameters (the posterior distal femoral angle and the posterior proximal tibial angle) improved post-operatively. The external fixator was removed either at the end of surgery or at the end of the lengthening period, allowing for early mobilisation and weight-bearing. We encountered five problems and four obstacles in the programme of treatment. The use of intramedullary nails prevented recurrence of deformity and refracture.
Internal lengthening devices in the femur lengthen
along the anatomical axis, potentially creating lateral shift of
the mechanical axis. We aimed to determine whether femoral lengthening
along the anatomical axis has an inadvertent effect on lower limb
alignment. Isolated femoral lengthening using the Intramedullary
Skeletal Kinetic Distractor was performed in 27 femora in 24 patients
(mean age 32 years (16 to 57)). Patients who underwent simultaneous realignment
procedures or concurrent tibial lengthening, or who developed mal-
or nonunion, were excluded. Pre-operative and six-month post-operative
radiographs were used to measure lower limb alignment. The mean lengthening
achieved was 4.4 cm (1.5 to 8.0). In 26 of 27 limbs, the mechanical
axis shifted laterally by a mean of 1.0 mm/cm of lengthening (0
to 3.5). In one femur that was initially in varus, a 3 mm medial
shift occurred during a lengthening of 2.2 cm. In a normally aligned limb, intramedullary lengthening along
the anatomical axis of the femur results in a lateral shift of the
mechanical axis by approximately 1 mm for each 1 cm of lengthening.
The Morquio syndrome is a rare disorder which presents with a number of musculoskeletal problems. The literature describing total knee replacement in these patients is sparse. We describe the management of a patient with bilateral instability and pain in the knees using bilateral constrained knee replacements, and followed up for five years with pre- and postoperative knee scores. We highlight the difficulties encountered and discuss the end results.
A flexed knee gait is common in patients with bilateral spastic
cerebral palsy and occurs with increased age. There is a risk for
the recurrence of a flexed knee gait when treated in childhood,
and the aim of this study was to investigate whether multilevel
procedures might also be undertaken in adulthood. At a mean of 22.9 months (standard deviation 12.9), after single
event multi level surgery, 3D gait analysis was undertaken pre-
and post-operatively for 37 adult patients with bilateral cerebral
palsy and a fixed knee gait.Aims
Patients and Methods
Patellar instability most frequently presents
during adolescence. Congenital and infantile dislocation of the
patella is a distinct entity from adolescent instability and measurable
abnormalities may be present at birth. In the normal patellofemoral
joint an increase in quadriceps angle and patellar height are matched
by an increase in trochlear depth as the joint matures. Adolescent
instability may herald a lifelong condition leading to chronic disability
and arthritis. Restoring normal anatomy by trochleoplasty, tibial tubercle transfer
or medial patellofemoral ligament (MPFL) reconstruction in the young
adult prevents further instability. Although these techniques are
proven in the young adult, they may cause growth arrest and deformity
where the physis is open. A vigorous non-operative strategy may
permit delay of surgery until growth is complete. Where non-operative
treatment has failed a modified MPFL reconstruction may be performed
to maintain stability until physeal closure permits anatomical reconstruction.
If significant growth remains an extraosseous reconstruction of
the MPFL may impart the lowest risk to the physis. If minor growth
remains image intensifier guided placement of femoral intraosseous
fixation may impart a small, but acceptable, risk to the physis. This paper presents and discusses the literature relating to
adolescent instability and provides a framework for management of
these patients. Cite this article:
Successful management of late presenting hip
dislocation in childhood is judged by the outcome not just at skeletal
maturity but well beyond into adulthood and late middle age. This
review considers different methods of treatment and looks critically
at the handful of studies reporting long-term follow-up after successful
reduction. Cite this article
Primary total knee arthroplasty (TKA) is a reliable
procedure with reproducible long-term results. Nevertheless, there
are conditions related to the type of patient or local conditions
of the knee that can make it a difficult procedure. The most common
scenarios that make it difficult are discussed in this review. These
include patients with many previous operations and incisions, and
those with severe coronal deformities, genu recurvatum, a stiff knee,
extra-articular deformities and those who have previously undergone
osteotomy around the knee and those with chronic dislocation of
the patella. Each condition is analysed according to the characteristics of
the patient, the pre-operative planning and the reported outcomes. When approaching the difficult primary TKA surgeons should use
a systematic approach, which begins with the review of the existing
literature for each specific clinical situation. Cite this article:
The significance of weight in the indications
for unicompartmental knee replacement (UKR) is unclear. Our hypothesis was
that weight does not affect the long-term rate of survival of UKRs. We undertook a retrospective study of 212 UKRs at a mean follow-up
of 12 years (7 to 22). The patients were distributed according to
body mass index (BMI; <
The ten-year rates of survival were similar in the two weight
subgroups (≥ 82 kg: 93.5% (95% confidence interval (CI) 66.5 to
96.3); <
82 kg: 92.5% (95% CI 82.5 to 94.1)) and also in the
two BMI subgroups (≥ 30 kg/m2: 92% (95% CI 82.5 to 95.3);
<
30 kg/m2: 94% (95% CI 78.4 to 95.9)). Multimodal
regression analysis revealed that weight plays a part in reducing
the risk of revision with a relative risk of 0.387, although this
did not reach statistical significance (p = 0.662). The results
relating weight and BMI to the clinical outcome were not statistically
significant. Thus, this study confirms that weight does not influence
the long-term rate of survival of UKR. Cite this article:
We analysed retrospectively the risk factors
leading to femoral overgrowth after flexible intramedullary nailing
in 43 children (mean age 7.1 years (3.6 to 12.0)) with fractures
of the shaft of the femur. We reviewed their demographic data, mechanism
of injury, associated injuries, the type and location of the fractures,
the nail–canal diameter (NCD) ratios and femoral overgrowth at a
mean follow-up of 40.7 months (25.2 to 92.7). At that time, the
children were divided into two groups, those with femoral overgrowth
of <
1 cm (Group 1), and those with overgrowth of ≥ 1 cm (Group
2). The mean femoral overgrowth of all patients was 0.6 cm at final
follow-up. Overgrowth of ≥ 1 cm was noted in 11 children (25.6%).
The NCD ratio was significantly lower in Group 2 than in Group 1,
with an odds ratio of 30.0 (p = 0.003). We believe that a low NCD ratio is an indicator of an unstable
configuration with flexible intramedullary nailing, and have identified
an association between a low NCD ratio and femoral overgrowth resulting
in leg-length discrepancy after flexible intramedullary nailing
in paediatric femoral shaft fractures. Cite this article:
The aim of this prospective single-centre study
was to assess the difference in clinical outcome between total knee replacement
(TKR) using computerised navigation and that of conventional TKR.
We hypothesised that navigation would give a better result at every
stage within the first five years. A total of 195 patients (195
knees) with a mean age of 70.0 years (39 to 89) were allocated alternately
into two treatment groups, which used either conventional instrumentation
(group A, 97 knees) or a navigation system (group B, 98 knees).
After five years, complete clinical scores were available for 121
patients (62%). A total of 18 patients were lost to follow-up. Compared
with conventional surgery, navigated TKR resulted in a better mean
Knee Society score (p = 0.008). The difference in mean Knee Society
scores over time between the two groups was not constant (p = 0.006),
which suggests that these groups differed in their response to surgery
with time. No significant difference in the frequency of malalignment
was seen between the two groups. In summary, computerised navigation resulted in a better functional
outcome at five years than conventional techniques. Given the similarity
in mechanical alignment between the two groups, rotational alignment
may prove to be a better method of identifying differences in clinical
outcome after navigated surgery.
Fractures of the femoral neck in children are
rare, high-energy injuries with high complication rates. Their treatment has
become more interventional but evidence of the efficacy of such
measures is limited. We performed a systematic review of studies
examining different types of treatment and their outcomes, including
avascular necrosis (AVN), nonunion, coxa vara, premature physeal
closure (PPC), and Ratliff’s clinical criteria. A total of 30 studies
were included, comprising 935 patients. Operative treatment and
open reduction were associated with higher rates of AVN. Delbet
types I and II fractures were most likely to undergo open reduction
and internal fixation. Coxa vara was reduced in the operative group,
whereas nonunion and PPC were not related to surgical intervention. Nonunion
and coxa vara were unaffected by the method of reduction. Capsular
decompression had no effect on AVN. Although surgery allows a more
anatomical union, it is uncertain whether operative treatment or
the type of reduction affects the rate of AVN, nonunion or PPC,
because more severe fractures were operated upon more frequently.
A delay in treatment beyond 24 hours was associated with a higher
incidence of AVN. Cite this article:
Ollier’s disease is characterised by severe deformity of the extremities and retarded growth because of multiple enchondromas. For correction of deformity, the Ilizarov method has been used although it has many complications. A 17-year-old boy with Ollier’s disease had a limb-length discrepancy of 17.4 cm, with a valgus deformity of the right knee and recurvatum of the femur of 23°. He had undergone three unsuccessful attempts to correct the deformities by using external fixators. We used a fully implantable, motorised, lengthening and correction nail (Fitbone) to achieve full correction of all the deformities without complications. We decided to carry out the procedure in three stages. First, we lengthened the femur by 3.6 cm and the tibia by 4 cm. We then exchanged the femoral nail for a longer implant and achieved a further 6 cm of length. This reduced the shortening to 3.8 cm. When the boy has finished secondary school we will adjust the remaining discrepancy.
Guiding growth by harnessing the ability of growing bone to undergo plastic deformation is one of the oldest orthopaedic principles. Correction of deformity remains a major part of the workload for paediatric orthopaedic surgeons and recently, along with developments in limb reconstruction and computer-directed frame correction, there has been renewed interest in surgical methods of physeal manipulation or ‘guided growth’. Manipulating natural bone growth to correct a deformity is appealing, as it allows gradual correction by non- or minimally invasive methods. This paper reviews the techniques employed for guided growth in current orthopaedic practice, including the basic science and recent advances underlying mechanical physeal manipulation of both healthy and pathological physes.
We describe our experience with the ‘four-in-one’ procedure for habitual dislocation of the patella in five children (six knees). All the patients presented with severe generalised ligamentous laxity and aplasia of the trochlear groove. All had a lateral release, proximal ‘tube’ realignment of the patella, semitendinosus tenodesis and transfer of the patellar tendon. The mean age at the time of the operation was 6.1 years (4.9 to 6.9), and the patients were followed up for a mean of 54.5 months (31 to 66). The clinical results were evaluated using the Kujala score. There has been no recurrence of dislocation. All the patients have returned to full activities and the parents and children were satisfied with the clinical results. The mean Kujala score was 95.3 (88 to 98). Two patients had marginal skin necrosis which healed after debridement and secondary closure. These early results in this small group have shown that the ‘four-in-one’ procedure is effective in the treatment of obligatory dislocation of the patella in children with severe ligamentous laxity and trochlear aplasia.
Trochlear dysplasia is an important anatomical abnormality in symptomatic patellar instability. Our study assessed the mismatch between the bony and cartilaginous morphology in patients with a dysplastic trochlea compared with a control group. MRI scans of 25 knees in 23 patients with trochlear dysplasia and in 11 patients in a randomly selected control group were reviewed retrospectively in order to assess the morphology of the cartilaginous and bony trochlea. Inter- and intra-observer error was assessed. In the dysplastic group there were 15 women and eight men with a mean age of 20.4 years (14 to 30). The mean bony sulcus angle was 167.9° (141° to 203°), whereas the mean cartilaginous sulcus angle was 186.5° (152° to 214°; p <
0.001). In 74 of 75 axial images (98.7%) the cartilaginous contour was different from the osseous contour on subjective assessment, the cartilage exacerbated the abnormality. Our study shows that the morphology of the cartilaginous trochlea differs markedly from that of the underlying bony trochlea in patients with trochlear dysplasia. MRI is necessary in order to demonstrate the pathology and to facilitate surgical planning.