Supracondylar fractures are the most frequently occurring paediatric
fractures about the elbow and may be associated with a neurovascular
injury. The British Orthopaedic Association Standards for Trauma
11 (BOAST 11) guidelines describe best practice for supracondylar
fracture management. This study aimed to assess whether emergency
departments in the United Kingdom adhere to BOAST 11 standard 1:
a documented assessment, performed on presentation, must include
the status of the radial pulse, digital capillary refill time, and
the individual function of the radial, median (including the anterior
interosseous), and ulnar nerves. Stage 1: We conducted a multicentre, retrospective audit of adherence
to BOAST 11 standard 1. Data were collected from eight hospitals
in the United Kingdom. A total of 433 children with Gartland type
2 or 3 supracondylar fractures were eligible for inclusion. A centrally
created data collection sheet was used to guide objective analysis
of whether BOAST 11 standard 1 was adhered to. Stage 2: We created
a quality improvement proforma for use in emergency departments.
This was piloted in one of the hospitals used in the primary audit
and was re-audited using equivalent methodology. In all, 102 patients
presenting between January 2016 and July 2017 were eligible for inclusion
in the re-audit.Aims
Materials and Methods
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
This review explores recent advances in fixator design and used in contemporary orthopaedic practice including the management of bone loss, complex deformity and severe isolated limb injury.
The aim of this study is to use a defined population of patients with meningococcal septicaemia to calculate the incidence of orthopaedic complications. Medical records and radiographs were analyzed retrospectively for all patients admitted to the Paediatric Intensive Care Unit (PICU) of the Bristol Royal Hospital for Children from 01/01/2001 to 31/12/2012 with meningococcal septicaemia. Of the 130 patients with meningococcal septicaemia alive at discharge, 10 developed orthopaedic sequelae, representing an overall incidence in this patient population of 7.7%. 9 patients required an amputation, mostly in the lower limb, 16/22 (72.7%). 48 growth plate abnormalities were identified in 8 patients. 39 (81.3%) The most commonly affected was the distal tibia (38.5%). 10 ankles were identified as having a varus malalignment. 6 patients had documented leg length discrepancy Using a clearly defined denominator this study has identified an incidence of orthopaedic sequelae following meningococcal septicaemia of 7.7%. The National Institute for Clinical Excellence (NICE) suggested that the incidence of growth disturbance is approximately 3%. This study highlights the underestimation of orthopaedic complications following meningococcal septicaemia. Close follow up of at risk patients should be considered to reduce the potential impact of these debilitating injuries.
Paley et al has developed the multiplier method for predicting leg length. It is a tool that is used clinically to predict leg length discrepancy. The method is also a way of comparing different populations, to identify differences in growth trajectory. This has been done by identifying the differing multipliers for girls and boys. However it has not been used to identify trends in populations separated by time. Tanner showed that in the first half of the twentieth century girls went from an average age of menarche of 15 in 1900, to 13 in 1970, how this has affected growth trajectory over the last 50 years has not been studied. The multiplier method is based on data collected in the 1950's by Anderson and Green, we aim to assess whether there has been a change in growth trajectory between this historical cohort and a contemporary European based cohort.Background:
Purpose:
Paley et al has developed a multiplier method for calculating both leg length and total height. In the development of this algorithm, they evaluated the effect of factors including bone age and sex. They established that sex had a significant impact, but adjusting for bone age did not improve accuracy. Bone age and menarche have been shown to improve other height prediction models. We used a large prospective cohort to evaluate if the multiplier is independent of physiological age using menarche as a proxy.Introduction:
Purpose:
Predictions of lower limb growth are based upon historical data, collected from patients who had coexistent poliomyelitis. By utilising standardised longitudinal prospective European data, our objective was to generate superior estimates for the age and rate at which lower limb skeletal maturity is reached; thus improving the timing of epiphysiodesis, for the management of leg length discrepancy. The Avon Longitudinal Study of Parents and Children of the 90s (ALSPAC) is a longitudinal cohort study of children recruited antenatally 2. Using a previously validated Multiplier Method, a sequence of leg length multipliers were calculated for each child. 15,458 individuals were recruited to the ALSPAC study; and of those whose growth was measured, 52% were boys and 48% girls, each with an average of eight recording episodes. 25,828 leg length multiplier (LLM) values were calculated with final recordings taken at a mean age of 15.5 years. From this data, the age at which girls reach skeletal maturity (LLM=1) is 11 months later than previously calculated and for boys nearly 9 months earlier. With nearly 4000 more children recruited in this cohort than preceding studies, this study brings increased power to future leg length calculations.
Paley et al developed a mathematical model to predict height, using age, sex and current height. His predictions were based on growth charts from epidemiological databases, and then validated using 52 children. We looked at a recent large, local database, to assess whether the height multiplier is a reliable tool that can be used in clinical practice. The Avon Longitudinal Study of Parents and Children of the 90s (ALSPAC) is a population based cohort study of 14, 000 contemporary British families. 5363 children had final height measured with an average of 10.5 additional height measurements. The height multiplier equation was defined as height at specific age divided by height at skeletal maturity. No significant difference was observed between the mean results from Paley et al and the ALSPAC data. There was a significant range of results in the ALSPAC data, with a standard deviation of the multiplier of 0.08 for ages 7–15. This large population study shows no significant difference between the historical databases Paley used and the more current European databases. The large range of results shown by the ALSPAC cast doubt on the clinical usefulness of individual results.
Meningococcal infection is the most common infective cause of death in children and causes significant morbidity in survivors. Patients admitted to the Paediatric Intensive Care Unit (PICU) of the Bristol Royal Hospital for Children from 01/01/2001 to 31/12/2012 with a primary diagnosis of meningococcal septicaemia were reviewed. A total of 10 (7.7%) of 130 patients developed orthopaedic complications. Those affected were significantly younger (p < 0.05), remained on PICU for longer (p < 0.001) and boys had a greater risk of developing orthopaedic complications (risk ratio: 3.1; 95% CI: 0.69–14.14). 9 patients required an amputation, 16/22 (72.7%) in the lower limb. Patient requiring amputation had multiple limb involvement. 48 growth plate abnormalities were identified in 8 patients, 39 (81.3%) in the lower limb, most commonly in the distal tibia. This study has identified a high incidence of musculoskeletal morbidity. Close surveillance of these patients is recommended to identify growth arrest before the onset of clinically significant deformity. It identifies a defined population of patients with meningococcal septicaemia using admission to PICU as an entry criterion allowing accurate determination of the incidence and characteristics of the skeletal consequences of this condition.
Survivors of infantile meningococcal septicaemia often develop progressive skeletal deformity as a consequence of physeal damage at multiple sites, particularly in the lower limb. Distal tibial physeal arrest typically occurs with sparing of the distal fibular physis leading to a rapidly progressive varus deformity. Isolated case reports include this deformity, but to our knowledge there is no previous literature that specifically reports the development of this deformity and potential treatment options. We report our experience of 6 patients (7ankles) with this deformity, managed with corrective osteotomy using a programmable circular fixator.
We investigated the effect of adjuvant and neoadjuvant chemotherapy regimens on the tibial regenerate after removal of the external fixator in a rabbit model of distraction osteogenesis using New Zealand white rabbits. Forty rabbits were randomly distributed into two groups. In the neoadjuvant group, half of the rabbits received 1mg/kg cisplatinum & 2mg/kg adriamycin at eight weeks of age followed by 1mg/kg cisplatinum & 4mg/kg adriamycin at ten weeks of age. The remaining ten received an identical volume of normal saline using the same regimen. The adjuvant group differed only in the timing of the chemotherapy infusion. Half received the initial infusion ten days prior to the osteotomy, with the second infusion four days following the osteotomy. Again, the remaining ten rabbits received an identical volume of normal saline using the same regimen. This produced an identical interval between infusions and identical age at osteotomy in both groups. All rabbits underwent a tibial osteotomy at 12 weeks of age. Distraction started 24hours after osteotomy at a rate of 0.75mm a day for 10 days, followed by 18 days without correction to allow for consolidation of the regenerate. At week 16 there was no difference in Bone Mineral Density (BMD), Bone Mineral Content (BMC) or volumetric Bone Mineral Density (vBMD) in the adjuvant group. Neoadjuvant chemotherapy appears to have a significant detrimental effect on BMD, vBMD and BMC. Despite this there were no significant alterations in the mechanical properties of the regenerate. Histologically there was a trend for increased cortical thickness in the control groups compared to intervention however this did not prove statistically significant. In conclusion, adjuvant chemotherapy may be more beneficial for cases where distraction osteogenesis is being considered to replace segmental bone loss after tumour excision.
This study addresses the evolution of the orthopaedic management of patients with hypophosphatemic rickets, with the aim of providing skeletal mature aligned lower limbs, with minimal surgical insult. We describe a case series of 8 patients with hypophosphatemic rickets that highlight an evolution in practice over the last 8 years. Our initial treatment involved external fixation with circular frames, addressing both axial deformity and length. Two contralateral long bones were addressed simultaneously and surgery was conducted in early adolescence. Problems encountered were poor quality regenerate, requiring prolonged periods in external fixation, and often recurrence of deformity following frame removal. Minor deformity recurrence made planning for the often inevitable knee replacement difficulty. Our current management is that patients only start surgical correction once skeletal maturity has been reached, two contralateral axial long bone corrections are performed using CHAOS (computer hexapod-assisted orthopaedic surgery) procedures with IM nails and multiple osteotomies (occasionally locking plates are required). Patients are able to fully weight bear immediately post operatively, after a suitable recovery period the remaining bones can be corrected. Then lengthening can be considered once the osteotomies have fully consolidated by exchange nailing of the femur, for an IM lengthening nail. At this stage the patient is able to fully consider the risks and benefits of this final and potentially unnecessary procedure. Hypophosphotaemic rickets is a rare condition, both the disease and its management can have severe effects on patients both physically and psychologically, at Bristol we have developed an treatment pathway that we feel helps to minimize the physical and psychological effects of treatment, with the end result of aligned lower limbs, that should provide a good basis for arthroplasty surgery if required in adulthood.Methods
Conclusions
Lower limb mal-alignment due to deformity is a significant cause of early degenerative change and dysfunction. Standard techniques are available to determine the centre of rotation of angulation (CORA) and extent of the majority of deformities, however distal femoral deformity is difficult to assess because of the difference between anatomic and mechanical axes. We found the described technique involving constructing a line perpendicular to a line from the tip of the greater trochanter to the centre of the femoral head inaccurate, particularly if the trochanter is abnormal. We devised a novel technique which accurately determines the CORA and extent of distal femoral deformity, allowing accurate correction. Using standard leg alignment views of the normal femur, the distal femoral metaphysis and joint line are stylized as a block. A line bisecting the axis of the proximal femur is then extended distally to intersect the joint. The angle (θ) between the joint and the proximal femoral axis and the position (p) where the extended proximal femoral axis intersects the joint line are calculated. These measurements can then be reproduced on the abnormal distal femur in order to calculate the CORA and extent of the deformity, permitting accurate correction. We examined the utility and reproducibility of the new method using 100 normal femora. θ = 81 ± sd 2.5°. As expected, θ correlated with femoral length (r=0.74). P (expressed as the percentage of the distance from the lateral edge of the joint block to the intersection) = 61% ± sd 8%. P was not correlated with θ. Intra-and inter-observer errors for these measurements are within acceptable limits and observations of 30-paired normal femora demonstrate similar values for θ and p on the two sides. We have found this technique to be universally applicable and reliable in a variety of distal femoral deformities.
Lower limb mal-alignment due to deformity is a significant cause of early degenerative change and dysfunction. Standard techniques are available to determine the centre of rotation of angulation (CORA) and extent of the majority of deformities, however distal femoral deformity is difficult to assess because of the difference between anatomic and mechanical axes. We found the described technique involving constructing a line perpendicular to a line from the tip of the greater trochanter to the centre of the femoral head inaccurate, particularly if the trochanter is abnormal. We devised a novel technique which accurately determines the CORA and extent of distal femoral deformity, allowing accurate correction. Using standard leg alignment views of the normal femur, the distal femoral metaphysis and joint line are stylized as a block. A line bisecting the axis of the proximal femur is then extended distally to intersect the joint. The angle (Θ) between the joint and the proximal femoral axis and the position (p) where the extended proximal femoral axis intersects the joint line are calculated. These measurements can then be reproduced on the abnormal distal femur in order to calculate the CORA and extent of the deformity, permitting accurate correction. We examined the utility and reproducibility of the new method using 100 normal femora. Θ = 81 ± sd 2.5. As expected, Θ correlated with femoral length (r=0.74). P (expressed as the percentage of the distance from the lateral edge of the joint block to the intersection) = 61% ± sd 8%. P was not correlated with Θ. Intra-and inter-observer errors for these measurements are within acceptable limits and observations of 30-paired normal femora demonstrate similar values for Θ and p on the two sides. We have found this technique to be universally applicable and reliable in a variety of distal femoral deformities.
Lower limb mal-alignment due to deformity is a significant cause of early degenerative change and dysfunction. Standard techniques are available to determine the centre of rotation of angulation (CORA) and extent of the deformities. However, distal femoral deformity is difficult to assess because of the difference between anatomic and mechanical axes. We describe a novel technique which accurately determines the CORA and extent of distal femoral deformity. Using standard leg alignment views of the normal femur, the distal femoral metaphysis and joint line are stylised as a block. A line bisecting the anatomical axis of the proximal femur is then extended distally to intersect the joint. The angle (?) between the joint and the proximal femoral axis, and the position (p) where the extended proximal femoral axis intersects the joint line are calculated. These measurements can then be reproduced on the abnormal distal femur in order to calculate the CORA and extent of deformity, permitting accurate correction. We examined the utility and reproducibility of the new method using 100 normal femora. We found this technique to be universally robust in a variety of distal femoral deformities.
Lower limb mal-alignment due to deformity is a significant cause of early degenerative change and limb dysfunction. Standard techniques are available to determine the centre of rotation of angulation (CORA) and extent of the majority of deformities, however distal femoral deformity is difficult to assess because of the difference between the anatomic and mechanical axes. We have found the described technique involving constructing a line perpendicular to a line from the tip of the greater trochanter to the centre of the femoral head inaccurate, particularly if the trochanter is abnormal. We have devised a novel technique which accurately determines the CORA and extent of distal femoral deformity, allowing accurate correction. Using standard leg alignment views of the normal femur, the distal femoral metaphysis and joint line are stylised as a block. A line bisecting the axis of the proximal femur is then extended distally to intersect the joint. The angle (𝛉) between the joint and the proximal femoral axis and the position (p) where the extended proximal femoral axis intersects the joint line are calculated. These measurements can then be reproduced on the abnormal distal femur in order to calculate the CORA and extent of the deformity, permitting accurate correction. We have examined the utility and reproducibility of the new method using one hundred normal femurs. Θ=81+/− sd 2.5°. As expected, 𝛉 correlated with femoral length (r=0.74). P (expressed as the percentage of the distal from the medial edge of the joint block to the intersection) = 61% +/− sd 8%. P was not correlated with 𝛉. Intra-and inter-observer errors for these measurements are within acceptable limits and observations of twenty paired normal femora demonstrate similar values for 𝛉 and p on the two sides. We have employed this technique in a variety of distal femoral deformities, including vitamin D resistant rickets, growth arrest, fibula hemimelia, post-traumatic deformity and Ellis-van Creveld syndrome. We find the system universally applicable and reliable.
A Ring Fixator (Taylor Spatial Frame (TSF); Smith &
Nephew, Memphis, TN), was used in the treatment of 5 patients (ages 11 to 16 years) with proximal tibial growth arrest following trauma. The mean corrections were 14.20 (max 280, min 00) in the saggital plane and 140 (max 380, min 20) in the coronal plane. Leg length discrepancy was also corrected (max 1 cm). The average time in frame was 17.8 weeks, with an average correction time of 29.8 days. Knee Society Clinical Rating System (KSCRS) scores post operatively ranged from 95 to 100. All patients returned to full activity, and would accept the same treatment if offered again. The circular fixator is an effective, minimally invasive method of treatment for post-traumatic proximal tibial deformity. Patients remain active during treatment encouraging a rapid return to school/work activities.
Survivors of meningococcal septicaemia often develop progressive skeletal deformity secondary to physeal damage at multiple sites, particularly in the lower limb. Distal tibial physeal arrest typically occurs with sparing of the distal fibular physis leading to a rapidly progressive varus ankle deformity. There is no previous literature reporting this ankle deformity following meningococcal septicaemia. We report the management of this deformity in 13 ankles in 10 consecutive patients 36 months after meningococcal septicaemia. Plain radiographs and MRI were used to define the deformity and the extent of growth plate involvement. The Taylor Spatial Frame (TSF) with a distal tibial metaphyseal osteotomy was used to restore the distal tibio-fibular joint. Distal fibular epiphysiodesis was performed in all ankles at the initial procedure. Distal tibial epiphysiodesis was performed at the time of fixator removal. The age at operation ranged from 3–14 years (mean 8). The preoperative ankle varus deformity ranged from 9–29 degrees (mean 19). The differential shortening of the tibia with respect to fibula was on average 1.2 cms. The mean time in frame was 136 days. After a mean follow-up of 1.7 years results were excellent in all patients with complete correction of deformity and shortening. Mechanincal axis was corrected in all patients. Complications included, 4 superficial pin site infections, 1 lateral peroneal nerve palsy which recovered completely. There were no major nerve or vascular complications. We consider that this approach provides a powerful method of correction for this difficult group of patients.
The results of a functional, clinical and radiological study of 30 children (60 hips) with whole body cerebral palsy are presented with a mean follow-up of ten years. Bilateral simultaneous combined soft-tissue and bony surgery was performed at a mean age of 7.7 years (3.1–12.2). Evaluation involved interviews with patient/carers and clinical examination. Plain radiographs of the pelvis assessed migration percentage and centre-edge angle. Twenty two patients were recalled. Five had died of unrelated causes and three were lost to follow-up. Pain was uncommon, present in 1 patient (4.5%). Improved handling was reported in 18 of 22 patients (82%). Carer handling problems were attributed to growth of the patients. All patients/carers considered the procedure worthwhile. The range of hip movements improved, with a mean windsweep index of 36 (50 pre-operatively) Radiological containment improved, with mean migration percentage of 20 degrees (50 preoperatively) and mean centre-edge angle of 29 degrees (−5 preoperatively) No statistical difference was noted between the three year and ten year follow-up results demonstrating maintained clinical and radiological outcome improvement. In conclusion, we consider that bilateral simultaneous combined hip reconstruction in whole body cerebral palsy provides painless, mobile and anatomically competent hips in the long term.
Patients were reviewed clinically and completed questionnaires documenting pain, function and satisfaction before and after treatment at a mean follow-up of 44 months (range 14–131). All patient’s notes and radiographs were examined.
Complications included almost universal minor pin-site infections, flexion contractures of the toes in 5 feet and skin ulceration in 2 feet, 1 requiring a muscle flap.
17 patients have undergone 20 microdrilling procedures to stimulate bone union in cases of established non-union. This occurred at the docking site following completion of bone transport using a stacked Taylor Spatial Frame, non-union following arthrodesis or non-union in long bone fracture. Additional bone grafting was performed in only one patient. Further stimulation of union via injection of Bone Morphogenetic Protein was undertaken with 3 microdrilling procedures. Of the 20 microdrilling procedures, 8 were considered fully successful in terms of stimulation of union, 7 were partially successful and 5 were not felt to have been successful. The mean time to fully successful union following microdrilling was 11.4 weeks, ranging from 6 to 19 weeks. There were 2 complications, both acute infections at the microdrilling site. Both of these were in patients with previous significant pin site infections. We present the use of a microdrilling technique as a safe and effective minimally invasive technique that promotes union in cases of refractory non-union, whilst avoiding the donor site morbidity associated with open bone grafting. We present, as a pilot study, our experience in the use of this technique in patients treated with circular frames for acute fractures, at the docking site in cases of bone transport and in cases of non-union following arthrodesis.
The majority of the available literature considers the short term outcome of surgical reconstruction of the hips in this condition. This paper demonstrates that the initial improvements in structure and function are maintained in the longer term.
The results of a functional, clinical and radiological study of 30 children (60 hips) with whole-body cerebral palsy were reviewed at a mean follow-up of 10.2 years (9.5 to 11). Correction of windsweep deformity of the hips was performed by bilateral simultaneous combined soft-tissue and bony surgery at a mean age of 7.7 years (3.1 to 12.2). We were able to recall 22 patients; five had died of unrelated causes and three were lost to follow-up. Evaluation involved interviews with patients/carers and clinical and radiological examination. The gross motor functional classification system was used to assess overall motor function and showed improvement in seven patients. Of the 12 patients thought to have pain pre-operatively, only one had pain post-operatively. Improved handling was reported in 18 of 22 patients (82%). Those with handling problems were attributed by the carers to growth of the patients. All patients/carers considered the procedure worthwhile. The range of hip movements improved, and the mean windsweep index improved from 50 pre-operatively to 36 at follow-up. The migration percentage and centre-edge angle were assessed on plain radiographs. Radiological containment improved, the mean migration percentage improved from 50 pre-operatively to 20 at follow-up and the mean centre-edge angle improved from −5° to 29°. No statistical difference was noted between the three-year and ten-year follow-up results, indicating that the improvements in clinical and radiological outcome had been maintained.
We have treated 17 patients with bone defects of the tibia by internal bone transport using a stacked Taylor Spatial Frame. There were 12 cases of infected non unions, 2 cases of osteomyelitis, 1 case of acute traumatic bone loss, 1 case of non union in a patient with neurofibromatosis, and 1 case of pseudoarthrosis of the tibia. The mean bone defect was 51.8mm (range 10–100mm). Leg length has been restored to within 10mm in 16 cases and to within 15mm in one case. All patients have united. Residual deformity at the docking site or regenerate was negligible in 4 patients and less than 5 degrees in any plane in the remaining 13 patients. There have been two cases of re-fracture which have united with conservative treatment and 1 case of partial peroneal nerve palsy which is recovering. The use of a stacked Taylor Spatial Frame system is effective in mediating bone transport resulting in predictable regenerate, accurate docking and minimal induced bone deformity.
Osteofibrous dysplasia is an unusual developmental condition of childhood, which almost exclusively affects the tibia. It is thought to follow a slowly progressive course and to stabilise after skeletal maturity. The possible link with adamantinoma is controversial and some authors believe that they are part of one histological process. We retrospectively reviewed 16 patients who were diagnosed as having osteofibrous dysplasia initially or on the final histological examination. Their management was diverse, depending on the severity of symptoms and the extent of the lesion. Definitive (extraperiosteal) surgery was localised ‘shark-bite’ excision for small lesions in five patients. Extensive lesions were treated by segmental excision and fibular autograft in six patients, external fixation and bone transport in four and proximal tibial replacement in one. One patient who had a fibular autograft required further excision and bone transport for recurrence. Six initially underwent curettage and all had recurrence. There were no recurrences after localised extraperiosteal excision or bone transport. There were three confirmed cases of adamantinoma. The relevant literature is reviewed. We recommend extraperiosteal excision in all cases of osteofibrous dysplasia, with segmental excision and reconstruction in more extensive lesions.
The purpose of this study is to demonstrate that definitive surgery (extraperiosteal excision) is required in patients with osteofibrous dysplasia (OFD) due to the risk of recurrence and co-existent adamantinoma OFD is an unusual childhood condition, which almost exclusively affects the tibia. It is thought to follow a slowly progressive course and to stabilise after skeletal maturity. The possible link with adamantinoma is controversial with some authors believing that they are part of one histological process. This therefore provides difficulty in recommending treatment options A retrospective review of OFD was conducted. Using the Stanmore Bone Tumour Unit database 22 cases were identified who were initially diagnosed with OFD or were diagnosed on final histology. All cases were tibial except one lesion in the ulna and one in the fibula Management was diverse depending on the severity of symptoms and the extent of the lesions encountered. Definitive (extraperiosteal) surgery in the majority of our patients was localized excision for small lesions (less than 50% of the bony circumference) and segmental excision followed by reconstructive surgery for more extensive ones. Seven patients had a sharkbite excision and a further seven were treated with fibula autografting. Of the latter group, one required further excision and bone transport due to recurrence of OFD. An additional five underwent bone transport &
distraction osteogenesis using the Ilizarov technique and one had a proximal tibial replacement. Nine initially underwent curettage, but eight recurred (recurrence rate 88.9%). No recurrences occurred following localized extraperiosteal excisions and bone transport. There were three confirmed cases of adamantinoma. In view of the risk of association of OFD with adamantinoma, and to some extent the continuous morbidity of OFD if left untreated, we believe that radical extraperiosteal excision is indicated in most if not all cases of OFD
The aim of the study was to assess the outcome of treatment of recurrent clubfoot deformity using the Ilizarov frame.
Objective: To review of an uncommon deformity arising in four patients. Method: A clinical and radiological review. Conclusions: heightened awareness and early treatment with monitoring is required. We describe four cases of distal radial epiphyseal dysplasia associated with a localised area of cutis aplasia congenita (CAC) over the dorsum of the distal forearm. The cutis aplasia was diagnosed at birth in all cases, but the radial dysplasia was not recognised until presentation to our orthopaedic department between the ages of 5 and 10 years. Radial dysplasia describes a spectrum of osseous, musculotendinous, and neurovascular dysplasias of the pre-axial border of the upper limb, and is the most common form of longitudinal deficiency. Cutis aplasia congenita involves an ulcerated area lacking in normal skin formation, present at birth. The most common site is on the scalp, but it has been described on the extremities, and overlying embryological malformations. This association, the long-term implications and the requirement for follow-up until skeletal maturity have not previously been described. We emphasise the importance of continued monitoring of these patients as the effects of radial dysplasia did not become obvious for several years, and the potential benefit from achieving early skin cover with grafting rather than allowing healing by secondary intention is discussed. It is important to increase awareness of this condition so that early orthopaedic and plastic surgical opinions can be sought, in order to reduce the disabling effect on the underlying radius.
The Proteus syndrome involves asymmetrical gigantism, verrucous epidermal naevi, vascular malformations, hamartomas and hyperostosis. The clinical features have frequently been described, but the radiological features have not been studied in detail. This paper describes the radiological features of a group of 18 patients (12 male, 6 female) that presented to the Department of Dermatology and Orthopaedic Surgery of this institution. Plain radiographs of each affected area were obtained to assist in the diagnosis and subsequent management of each patient. These radiographs were evaluated in an attempt to define the radiological anatomy of the osseous lesions. The abnormalities were classified as involving abnormal ossification, hyperostotic overgrowth or ectopic calcification. The individual features of each group will be presented. We attempted to define radiological parameters that were specific to this condition and therefore useful in diagnosis. We considered a number of radiological measurements and found a consistent alteration in bony architecture of the upper and lower limbs of affected individuals. This association was not detected in other overgrowth symptoms. This is the first objective radiological parameter that assists in the diagnosis of this rare condition.
Hip pain in cerebral palsy is regarded to be underreported. Management of these patients at home is difficult as the patients mature. In the ‘non walker’ category, the aims of surgery are to relieve pain and to allow sitting and transfer. Neuromuscular hips may have variable acetabular deficiencies ie) anterior /posterior / lateral. Many forms of surgical management, of varying complexity, have been described to address these problems. To describe a new technique with multidirectional coverage that achieves pain free hips, 15 patients were reviewed over a 4 year period. Inclusion criteria :- 1 Subluxated / dislocated hips with hip pain. 2 Patients who have failed conservative management. 3. Those not suitable for redirectional osteotomies. A standardised technique was performed by one surgeon, at one institution. In summary, the technique involves initially a standard derotation varus osteotomy. Via an anterior approach, a lateral iliac unicortical graft and strips of cancellous graft are harvested. The cancellous graft is laid on top of the intact capsule, in the areas of deficiency. The cancellous graft is held by the unicortical graft with a single screw. 15 patients were reviewed. Patients were categorised as ‘walkers’ (3) and ‘non- walkers’ (12.) The mean age was 13.2 years. All patients were pain free after recovery. This was defined as not requiring analgesia and parental satisfaction. The radiological appearances showed that all the shelves had incorporated, with satisfactory cover of the femoral head. This technique addresses multidirectional cover of femoral head. The technique is relatively easy to perform. All the patients have achieved a pain free outcome to date.