This study evaluated the effect of limb lengthening
on longitudinal growth in patients with
We carried out an MRI study of the lumbar spine in 15 patients with
Lengthening of the humerus is now an established
technique. We compared the complications of humeral lengthening
with those of femoral lengthening and investigated whether or not
the callus formation in the humerus proceeds at a higher rate than
that in the femur. A total of 24 humeral and 24 femoral lengthenings
were performed on 12 patients with
Genu varum in the achondroplastic patient has a complex and multifactorial aetiology. There is little mention in the literature of the role of fibular overgrowth. Using the ratio of fibular to tibial length as a measurement of possible fibular overgrowth, we have related it to the development of genu varum. Full-length standing anteroposterior radiographs of 53 patients with
Forty-eight patients with
Recent studies of the fibroblast growth factor receptor 3 (FGFR3) gene have established that
Two types of fracture, early and late, have been
reported following limb lengthening in patients with
A thoracolumbar gibbus is an uncommon but potentially dangerous feature of
Lack of full extension of the elbow is a common abnormality in patients with
Extensive limb lengthening may be indicated in achondroplastic patients who wish to achieve a height within the normal range for their population. However, increasing the magnitude of lengthening is associated with further complications particularly adjacent joint stiffness and fractures. We studied the relationship between the magnitude of femoral lengthening and callus pattern, adjacent joint stiffness and fracture of the regenerate bone in 40 femoral lengthenings in 20 achondroplastic patients. They were divided into two groups; group A had lengthening of less than 50% and group B of more than 50% of their initial femoral length. The patterns of radiological callus formation were classified according to shape, type and features. The incidence of callus features, knee stiffness and regenerate bone fracture were analysed in the two groups. Group B was associated with an increased incidence of concave, lateral and central callus shapes, adjacent joint and stiffness and fracture. Statistically, the incidence of stiffness in adjacent joints and regenerate bone fracture was significantly associated with the magnitude of lengthening. We suggest that careful radiological assessment of the patterns of callus formation is a useful method for the evaluation and monitoring of regenerate bone.
A retrospective study was performed in 18 patients
with
The aim of this study was to compare outcomes after growth-friendly treatment for early-onset scoliosis (EOS) between patients with skeletal dysplasias versus those with other syndromes. We retrospectively identified 20 patients with skeletal dysplasias and 292 with other syndromes (control group) who had completed surgical growth-friendly EOS treatment between 1 January 2000 and 31 December 2018. We compared radiological parameters, complications, and health-related quality of life (HRQoL) at mean follow-up of 8.6 years (SD 3.3) in the dysplasia group and 6.6 years (SD 2.6) in the control group.Aims
Methods
The aim of this retrospective cohort study was to assess and investigate the safety and efficacy of using a distal tibial osteotomy compared to proximal osteotomy for limb lengthening in children. In this study, there were 59 consecutive tibial lengthening and deformity corrections in 57 children using a circular frame. All were performed or supervised by the senior author between January 2013 and June 2019. A total of 25 who underwent a distal tibial osteotomy were analyzed and compared to a group of 34 who had a standard proximal tibial osteotomy. For each patient, the primary diagnosis, time in frame, complications, and lengthening achieved were recorded. From these data, the frame index was calculated (days/cm) and analyzed.Aims
Methods
Temporary hemiepiphysiodesis (HED) is applied to children and adolescents to correct angular deformities (ADs) in long bones through guided growth. Traditional Blount staples or two-hole plates are mainly used for this indication. Despite precise surgical techniques and attentive postoperative follow-up, implant-associated complications are frequently described. To address these pitfalls, a flexible staple was developed to combine the advantages of the established implants. This study provides the first results of guided growth using the new implant and compares these with the established two-hole plates and Blount staples. Between January 2013 and December 2016, 138 patients (22 children, 116 adolescents) with genu valgum or genu varum were treated with 285 flexible staples. The minimum follow-up was 24 months. These results were compared with 98 patients treated with 205 two-hole plates and 92 patients treated with 535 Blount staples. In long-standing anteroposterior radiographs, mechanical axis deviations (MADs) were measured before and during treatment to analyze treatment efficiency. The evaluation of the new flexible staple was performed according to the idea, development, evaluation, assessment, long-term (IDEAL) study framework (Stage 2a).Aims
Methods
As an alternative to external fixators, intramedullary lengthening nails (ILNs) can be employed for distraction osteogenesis. While previous studies have demonstrated that typical complications of external devices, such as soft-tissue tethering, and pin site infection can be avoided with ILNs, there is a lack of studies that exclusively investigated tibial distraction osteogenesis with motorized ILNs inserted via an antegrade approach. A total of 58 patients (median age 17 years (interquartile range (IQR) 15 to 21)) treated by unilateral tibial distraction osteogenesis for a median leg length discrepancy of 41 mm (IQR 34 to 53), and nine patients with disproportionate short stature treated by bilateral simultaneous tibial distraction osteogenesis, with magnetically controlled motorized ILNs inserted via an antegrade approach, were retrospectively analyzed. The median follow-up was 37 months (IQR 30 to 51). Outcome measurements were accuracy, precision, reliability, bone healing, complications, and patient-reported outcome assessed by the Limb Deformity-Scoliosis Research Society Score (LD-SRS-30).Aims
Methods
Accurate skeletal age and final adult height prediction methods in paediatric orthopaedics are crucial for determining optimal timing of growth-guiding interventions and minimizing complications in treatments of various conditions. This study aimed to evaluate the accuracy of final adult height predictions using the central peak height (CPH) method with long leg X-rays and four different multiplier tables. This study included 31 patients who underwent temporary hemiepiphysiodesis for varus or valgus deformity of the leg between 2014 and 2020. The skeletal age at surgical intervention was evaluated using the CPH method with long leg radiographs. The true final adult height (FHTRUE) was determined when the growth plates were closed. The final height prediction accuracy of four different multiplier tables (1. Bayley and Pinneau; 2. Paley et al; 3. Sanders – Greulich and Pyle (SGP); and 4. Sanders – peak height velocity (PHV)) was then compared using either skeletal age or chronological age.Aims
Methods
Radiological and histological examinations are described in a full term stillborn infant with punctate epiphysial dysplasia diagnosed in utero as
We describe a technique for slow, progressive, symmetrical distraction of the growth plate using a lightweight dynamic axial fixation system. Results are given for the elongation of 40 bony segments in children with limb-length discrepancies and 60 segments in children with
The STRYDE nail is an evolution of the PRECICE Intramedullary Limb Lengthening System, with unique features regarding its composition. It is designed for load bearing throughout treatment in order to improve patient experience and outcomes and allow for simultaneous bilateral lower limb lengthening. The literature published to date is limited regarding outcomes and potential problems. We report on our early experience and raise awareness for the potential of adverse effects from this device. This is a retrospective review of prospective data collected on all patients treated in our institution using this implant. We report the demographics, nail accuracy, reliability, consolidation index, and cases where concerning clinical and radiological findings were encountered. There were 14 STRYDE nails implanted in nine patients (three male and six female) between June 2019 and September 2020. Mean age at surgery was 33 years (14 to 65). Five patients underwent bilateral lengthening (two femoral and three tibial) and four patients unilateral femoral lengthening for multiple aetiologies.Aims
Methods
Three elderly sibling dwarfs are reported from a large and otherwise normal family. Their condition is an unusual and irregular form of cartilaginous defect, combined with club-feet, and bearing some resemblance to the Morquio type. A brief consideration of the literature of the generahised developmental bony syndromes shows confusion of thought and nomenclature. A plea is therefore made for simplification and a rational and simple grouping of these conditions is attempted. It seems particularly desirable that many confusing names for particular syndromes should be scrapped (for example, dyschondroplasia, chondro-osteodystrophy), although quite evidently the terms
The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management. A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation.Aims
Patients and Methods
The aim of this study was to present the first retrieval analysis findings of PRECICE STRYDE intermedullary nails removed from patients, providing useful information in the post-market surveillance of these recently introduced devices. We collected ten nails removed from six patients, together with patient clinical data and plain radiograph imaging. We performed macro- and microscopic analysis of all surfaces and graded the presence of corrosion using validated semiquantitative scoring methods. We determined the elemental composition of surface debris using energy dispersive x-ray spectroscopy (EDS) and used metrology analysis to characterize the surface adjacent to the extendable junctions.Aims
Methods
Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service. Descriptive study of prospectively collected data. Clinical data of all patients undergoing surgery for spinal deformity between 2005 and 2018 was collected, compared to the demographics of the national population, and analyzed by underlying aetiology.Aims
Methods
We compared the complications and outcome of tibial lengthening using the Ilizarov method with and without the use of a supplementary intramedullary nail. In a retrospective case-matched series assembled from 176 patients with tibial lengthening, we matched 52 patients (26 pairs, group A with nail and group B without) according to the following criteria in order of importance: 1) difference in amount of lengthening (± 2 cm); 2) percentage difference in lengthening (± 5%); 3) difference in patient’s age (± seven years); 4) aetiology of the shortening, and 5) level of difficulty in obtaining the correction. The outcome was evaluated using the external fixator index, the healing index and an outcome score according to the criteria of Paley. It was found that some complications were specific to group A or B respectively, but others were common to both groups. The outcome was generally better in lengthenings with a nail, although there was a higher incidence of rectifiable equinus deformity in these patients.
Acute angulation at the thoracolumbar junction
with segmental subluxation of the spine occurring at the level above
an anteriorly hypoplastic vertebra in otherwise normal children
is a rare condition described as infantile developmental thoracolumbar
kyphosis. Three patient series with total of 18 children have been
reported in the literature. We report five children who presented
with thoracolumbar kyphosis and discuss the treatment algorithm. We
reviewed the medical records and spinal imaging at initial clinical
presentation and at minimum two-year follow-up. The mean age at
presentation was eight months (two to 12). All five children had
L2 anterior vertebral body hypoplasia. The kyphosis improved spontaneously
in three children kept under monitoring. In contrast, the deformity
was progressive in two patients who were treated with bracing. The
kyphosis and segmental subluxation corrected at latest follow-up
(mean age 52 months; 48 to 60) in all patients with near complete
reconstitution of the anomalous vertebra. The deformity and radiological
imaging on a young child can cause anxiety to both parents and treating
physicians. Diagnostic workup and treatment algorithm in the management
of infantile developmental thoracolumbar kyphosis is proposed. Observation
is indicated for non-progressive kyphosis and bracing if there is evidence
of kyphosis and segmental subluxation deterioration beyond walking
age. Surgical stabilisation of the spine can be reserved for severe
progressive deformities unresponsive to conservative treatment. Cite this article:
External fixation is widely used in orthopaedic
and trauma surgery. Infections around pin or wire sites, which are usually
localised, non-invasive, and are easily managed, are common. Occasionally,
more serious invasive complications such as necrotising fasciitis
(NF) and toxic shock syndrome (TSS) may occur. We retrospectively reviewed all patients who underwent external
fixation between 1997 and 2012 in our limb lengthening and reconstruction
programme. A total of eight patients (seven female and one male)
with a mean age of 20 years (5 to 45) in which pin/wire track infections
became limb- or life-threatening were identified. Of these, four
were due to TSS and four to NF. Their management is described. A
satisfactory outcome was obtained with early diagnosis and aggressive
medical and surgical treatment. Clinicians caring for patients who have external fixation and
in whom infection has developed should be aware of the possibility
of these more serious complications. Early diagnosis and aggressive
treatment are required in order to obtain a satisfactory outcome. Cite this article:
We undertook a retrospective analysis of 306
procedures on 233 patients, with a mean age of 12 years (1 to 21),
in order to evaluate the use of somatosensory evoked potential (SSEP)
monitoring for the early detection of nerve compromise during external
fixation procedures for limb lengthening and correction of deformity.
Significant SSEP changes were identified during 58 procedures (19%).
In 32 instances (10.5%) the changes were transient, and resolved
once the surgical cause had been removed. The remaining 26 (8.5%)
were analysed in two groups, depending on whether or not corrective
action had been performed in response to critical changes in the
SSEP recordings. In 16 cases in which no corrective action was taken,
13 (81.2%, 4.2% overall) developed a post-operative neurological
deficit, six of which were permanent and seven temporary, persisting
for five to 18 months. In the ten procedures in which corrective
action was taken, four patients (40%, 1.3% overall) had a temporary
(one to eight months) post-operative neuropathy and six had no deficit. After appropriate intervention in response to SSEP changes, the
incidence and severity of neurological deficits were significantly
reduced, with no cases of permanent neuropathy. SSEP monitoring
showed 100% sensitivity and 91% specificity for the detection of
nerve injury during external fixation. It is an excellent diagnostic
technique for identifying nerve lesions when they are still highly
reversible.
Computer hexapod assisted orthopaedic surgery (CHAOS), is a method
to achieve the intra-operative correction of long bone deformities
using a hexapod external fixator before definitive internal fixation
with minimally invasive stabilisation techniques. The aims of this study were to determine the reliability of this
method in a consecutive case series of patients undergoing femoral
deformity correction, with a minimum six-month follow-up, to assess
the complications and to define the ideal group of patients for
whom this treatment is appropriate. The medical records and radiographs of all patients who underwent
CHAOS for femoral deformity at our institution between 2005 and
2011 were retrospectively reviewed. Records were available for all
55 consecutive procedures undertaken in 49 patients with a mean
age of 35.6 years (10.9 to 75.3) at the time of surgery.Aims
Patients and Methods
Patients with skeletal dysplasia are prone to
developing advanced osteoarthritis of the knee requiring total knee replacement
(TKR) at a younger age than the general population. TKR in this
unique group of patients is a technically demanding procedure owing
to the deformity, flexion contracture, generalised hypotonia and ligamentous
laxity. We retrospectively reviewed the outcome of 11 TKRs performed
in eight patients with skeletal dysplasia at our institution using
the Stanmore Modular Individualised Lower Extremity System (SMILES)
custom-made rotating-hinge TKR. There were three men and five women
with mean age of 57 years (41 to 79). Patients were followed clinically
and radiologically for a mean of seven years (3 to 11.5). The mean
Knee Society clinical and function scores improved from 24 (14 to
36) and 20 (5 to 40) pre-operatively, respectively, to 68 (28 to
80) and 50 (22 to 74), respectively, at final follow-up. Four complications
were recorded, including a patellar fracture following a fall, a
tibial peri-prosthetic fracture, persistent anterior knee pain,
and aseptic loosening of a femoral component requiring revision.
Our results demonstrate that custom primary rotating-hinge TKR in
patients with skeletal dysplasia is effective at relieving pain,
with a satisfactory range of movement and improved function. It compensates
for bony deformity and ligament deficiency and reduces the likelihood
of corrective osteotomy. Patellofemoral joint complications are
frequent and functional outcome is worse than with primary TKR in
the general population.
Between October 2001 and September 2009 we lengthened 242 lower-limb segments in 180 patients using the Intramedullary Skeletal Kinetic Distractor (ISKD). Mechanical failure was defined either as breakage of the ISKD or failure of the internal mechanism to activate. Retrieved nails which failed mechanically were examined by the manufacturer for defects. In all, 15 ISKDs in 12 patients (13 limbs) failed mechanically representing an overall failure rate of 6.2%, with fracture of the device occurring in ten of the 15 failures. Two nails in one patient failed to lengthen and had to be replaced. The manufacturer detected an error in the assembly of the nail, which prompted a wide recall. One nail jammed after being forcefully inserted, and two nails failed to lengthen fully. Lengthening was achieved in all 12 patients, although three required a second operation to exchange a defective nail for a new, functioning device. The ISKD is a complex mechanical device which lengthens by the oscillation of two telescopic sections connected by a threaded rod. The junction between these sections is surrounded by a keyring collar. This keyring collar is the weakest part of the device.
The purpose of this study was to compare the results and complications of tibial lengthening over an intramedullary nail with treatment using the traditional Ilizarov method. In this matched case study, 16 adult patients underwent 19 tibial lengthening over nails (LON) procedures. For the matched case group, 17 patients who underwent 19 Ilizarov tibial lengthenings were retrospectively matched to the LON group.Objectives
Methods
Nonunion of the humerus with bone loss and shortening due to osteomyelitis is rare but difficult to treat. We describe our experience with a callus distraction technique using a monolateral external fixator for the treatment of this condition. Between October 1994 and January 2004, 11 patients were treated. There were seven males and four females, with a mean age of 14 years (10 to 17). The mean bone loss was 1.9 cm (1 to 2.7) and the mean length discrepancy in the upper limb was 5.6 cm (3.5 to 8.0). The mean follow-up was for 106 months (54 to 166). The mean external fixation index was 34.8 days/cm (29.8 to 40.5). The mean lengthening was 9.5 cm (5.5 to 13.4). There were seven excellent results, three good and one poor. There were nine excellent functional results and two good. The treatment of humeral nonunion with bone loss and shortening due to osteomyelitis by callus distraction is a safe and effective means of improving function and cosmesis.
This retrospective study compared post-operative
epidural analgesia (E), continuous peripheral nerve blocks (CPNB) and
morphine infusion (M) in 68 children undergoing limb reconstruction
with circular frames. The data collected included episodes of severe
pain, post-operative duration of analgesia, requirement for top-up
analgesia, number of osteotomies, side effects and complications.
There was a significant difference between the number of episodes
of severe pain in patients receiving a morphine infusion and those
receiving epidurals or CPNB (M
Between 1987 and 2006 we performed a modified Thompson’s quadricepsplasty on 40 fracture-related stiff knees and followed the patients for a mean of 7.9 years (2 to 11.1). The factors affecting the final gain of movement were investigated. A total of 15 knees required lengthening of the rectus femoris. The mean flexion gain was 70.2° (42.3° to 112.5°). According to Judet’s criteria, the results were excellent in 30 knees, good in seven, and fair in three. The range of movement which was achieved intra-operatively was related to the gain of knee flexion on univariate analysis. Five patients had complications: deep infection in one, recurrent patellar dislocation in one, and rupture of the extensor mechanism in three. This modified technique gives satisfactory results. Achieving maximum knee flexion intra-operatively seems to be the most important factor in enhancing the outcome in patients with stiffness of the knee following fracture.
We report five children who presented at the mean age of 1.5 years (1.1 to 1.9) with a progressive thoracolumbar kyphosis associated with segmental instability and subluxation of the spine at the level above an anteriorly-wedged hypoplastic vertebra at L1 or L2. The spinal deformity appeared to be developmental and not congenital in origin. The anterior wedging of the vertebra may have been secondary to localised segmental instability and subsequent kyphotic deformity. We suggest the term ‘infantile developmental thoracolumbar kyphosis with segmental subluxation of the spine’ to differentiate this type of deformity from congenital displacement of the spine in which the congenital vertebral anomaly does not resolve. Infantile developmental kyphosis with segmental subluxation of the spine, if progressive, may carry the risk of neurological compromise. In all of our patients the kyphotic deformity progressed over a period of three months and all were treated by localised posterior spinal fusion. At a mean follow-up of 6.6 years (5.0 to 9.0), gradual correction of the kyphosis was seen on serial radiographs as well as reconstitution of the hypoplastic wedged vertebra to normality. Exploration of the arthrodesis was necessary at nine months in one patient who developed a pseudarthrosis.
The hip joint is commonly involved in multiple epiphyseal dysplasia and patients may require total hip replacement before the age of 30 years. We retrospectively reviewed nine patients (16 hips) from four families. The diagnosis of multiple epiphyseal dysplasia was based on a family history, genetic counselling, clinical features and radiological findings. The mean age at surgery was 32 years (17 to 63), with a mean follow-up of 15.9 years (5.5 to 24). Of the 16 hips, ten required revision at a mean of 12.5 years (5 to 15) consisting of complete revision of the acetabular component in three hips and isolated exchange of the liner in seven. No femoral component has loosened or required revision during the period of follow-up. With revision for any reason, the 15-year survival was only 11.4% (95% confidence interval 1.4 to 21.4). However, when considering revision of the acetabular shell in isolation the survival at ten years was 93.7% (95% confidence interval 87.7 to 99.7), reducing to 76.7% (95% confidence interval 87.7 to 98.7) at 15 and 20 years, respectively.
We present a retrospective study of 27 patients treated by callus distraction using a unilateral external fixator of our own design for nonunion with bone loss and shortening of the femur caused by suppurative osteomyelitis. The unilateral external fixator was used either alone or in combination with an intramedullary nail. The mean age of the patients was 13.6 years (8 to 18). The fixator was used alone in 13 patients and with an intramedullary nail in 14. The bone results at a mean follow-up of 88 months (37 to 144) were excellent in 16 patients and good in 11. The functional results were excellent in 18 patients and good in nine. However, four patients still had draining sinuses at the latest follow-up. A residual deformity greater than 7° was present in seven femora, but this did not adversely affect function or require further treatment.