Aims. The aim of this study was to quantify the risk of developing cancer from the exposure to radiation associated with surgery to correct limb deformities in children. Patients and Methods. A total of 35 children were studied. There were 19 girls and 16 boys. Their mean age was 11.9 years (2 to 18) at the time of surgery. Details of the radiological examinations were recorded during gradual correction using a Taylor Spatial Frame. The dose area product for each radiograph was obtained from the Computerised Radiology Information System database. The effective dose in millisieverts (mSv) was calculated using conversion coefficients for the anatomical area. The lifetime risk of developing cancer was calculated using government-approved Health Protection Agency reports, accounting for the age and gender of the child. Results. Correction was undertaken in five femurs, 18 tibiae, and 12 feet. The median duration of treatment was 45 months (11 to 118). The mean effective dose was 0.31 mSv (0.05 to 0.64) for the femur, 0.29 mSv (0.01 to 0.97) for the tibia, and 0.027 mSv (0.001 to 0.161) for the foot. The cumulative exposure gave ‘negligible’ risk in 26 children and ‘minimal’ risk in nine children, according to Public Health England categories. These results are below the mean annual background radiation in the United Kingdom. Conclusion. The lifetime attributable risk of developing cancer from repeated exposure to radiation was negligible or minimal in all children. This is the first study to quantify the exposure to radiation from serial radiographs in children with limb deformities who are treated surgically using circular external fixation, linking this to the risk of developing cancer. Cite this article: Bone Joint J 2019;101-B:241–245

We describe two siblings with congenital growth defects of the distal limb bones and their progress over four years. They bear some resemblances to a boy described in 1958 by Solonen and Sulamaa and to a father and his three sons described in 1944 by Nievergelt.

Aims

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.

Aims. Alternative alignment concepts, including kinematic and restricted kinematic, have been introduced to help improve clinical outcomes following total knee arthroplasty (TKA). The purpose of this study was to evaluate the clinical results, along with patient satisfaction, following TKA using the concept of restricted kinematic alignment. Methods. A total of 121 consecutive TKAs performed between 11 February 2018 to 11 June 2019 with preoperative varus deformity were reviewed at minimum one-year follow-up. Three knees were excluded due to severe preoperative varus deformity greater than 15°, and a further three due to requiring revision surgery, leaving 109 patients and 115 knees to undergo primary TKA using the concept of restricted kinematic alignment with advanced technology. Patients were stratified into three groups based on the preoperative limb varus deformity: Group A with 1° to 5° varus (43 knees); Group B between 6° and 10° varus (56 knees); and Group C with varus greater than 10° (16 knees). This study group was compared with a matched cohort of 115 TKAs and 115 patients using a neutral mechanical alignment target with manual instruments performed from 24 October 2016 to 14 January 2019. Results. Mean overall patient satisfaction for the entire cohort was 4.7 (SE 0.1) on a 5-point Likert scale, with 93% being either very satisfied or satisfied compared with a Likert of 4.3 and patient satisfaction of 81% in the mechanical alignment group (p < 0.001 and p < 0.006 respectively). At mean follow-up of 17 months (11 to 27), the mean overall Likert, Knee Injury and Osteoarthritis Outcome Score for Joint Replacement, Western Ontario and McMaster Universities Osteoarthritis Index, Forgotten Joint Score, and Knee Society Knee and Function Scores were significantly better in the kinematic group than in the neutral mechanical alignment group. The most common complication in both groups was contracture requiring manipulation under anaesthesia, involving seven knees (6.1%) in the kinematic group and nine knees (7.8%) in the mechanical alignment group. Conclusion. With the advent of advanced technology, and the ability to obtain accurate bone cuts, the target limb alignment, and soft-tissue balance within millimetres, using a restricted kinematic alignment concept demonstrated excellent patient satisfaction following primary TKA. Longer-term analysis is required as to the durability of this method. Cite this article: Bone Joint J 2021;103-B(6 Supple A):59–66

Aims. This study reviews the use of a titanium mesh cage (TMC) as an adjunct to intramedullary nail or plate reconstruction of an extra-articular segmental long bone defect. Patients and Methods. A total of 17 patients (aged 17 to 61 years) treated for a segmental long bone defect by nail or plate fixation and an adjunctive TMC were included. The bone defects treated were in the tibia (nine), femur (six), radius (one), and humerus (one). The mean length of the segmental bone defect was 8.4 cm (2.2 to 13); the mean length of the titanium mesh cage was 8.3 cm (2.6 to 13). The clinical and radiological records of the patients were analyzed retrospectively. Results. The mean time to follow-up was 55 months (12 to 126). Overall, 16 (94%) of the patients achieved radiological filling of their bony defect and united to the native bone ends proximally and distally, resulting in a functioning limb. Complications included device failure in two patients (12%), infection in two (12%), and wound dehiscence in one (6%). Four patients (24%) required secondary surgery, four (24%) had a residual limb-length discrepancy, and one (6%) had a residual angular limb deformity. Conclusion. A titanium mesh cage is a useful adjunct in the treatment of an extra-articular segmental defect in a long bone. Cite this article: Bone Joint J 2018;100-B:646–51

Aims. Guided growth using eight-plates is commonly used for correction of angular limb deformities in growing children. The principle is of tethering at the physeal periphery while enabling growth in the rest of the physis. The method is also applied for epiphysiodesis to correct limb-length discrepancy (LLD). Concerns have been raised regarding the potential of this method to create an epiphyseal deformity. However, this has not been investigated. The purpose of this study was to detect and quantify the occurrence of deformities in the proximal tibial epiphysis following treatment with eight-plates. Patients and Methods. A retrospective study was performed including 42 children at a mean age of 10.8 years (3.7 to 15.7) undergoing eight-plate insertion in the proximal tibia for correction of coronal plane deformities or LLD between 2007 and 2015. A total of 64 plates were inserted; 48 plates (34 patients) were inserted to correct angular deformities and 16 plates (8 patients) for LLD. Medical records, Picture Archive and Communication System images, and conventional radiographs were reviewed. Measurements included interscrew angle, lateral and medial plateau slope angles measured between the plateau surface and the line between the ends of the physis, and tibial plateau roof angle defined as 180° minus the sum of both plateau angles. Measurements were compared between radiographs performed adjacent to surgery and those at latest follow-up, and between operated and non-operated plateaus. Statistical analysis was performed using BMDP Statistical Software. Results. Slope angle increased in 31 (49.2%) of operated epiphyses by a mean of 5° (1° to 23°) compared with 29 (31.9%) in non-operated epiphyses (p = 0.043). Roof angle decreased in 29 (46.0%) of operated tibias and in 25 (27.5%) of non-operated ones by a mean of 5° (1° to 18°) (p = 0.028). Slope angle change frequency was similar in patients with LLD, varus and valgus correction (p = 0.37) but roof angle changes were slightly more frequent in LLD (p = 0.059) and correlated with the change in inter screw angles (r = 0.74, p = 0.001). Conclusion. The use of eight-plates in the proximal tibia for deformity correction and limb-length equalization causes a change in the bony morphology of the tibial plateau in a significant number of patients and the effect is more pronounced in the correction of LLD. Cite this article: Bone Joint J 2018;100-B:1112–16

Congenital Talipes Equinovarus (CTEV) is one of the most common congenital limb deformities. We reviewed the records of infants who had received treatment for structural CTEV between 1 January 2007 and 30 November 2012. This was cross-referenced with the prenatal scans of mothers over a corresponding period of time. We investigated the sensitivity, specificity, and positive and negative predictive values of the fetal anomaly scan for the detection of CTEV and explored whether the publication of Fetal Anomaly Screening Programme guidelines in 2010 affected the rate of detection. During the study period there were 95 532 prenatal scans and 34 373 live births at our hospital. A total of 37 fetuses with findings suggestive of CTEV were included in the study, of whom 30 were found to have structural CTEV at birth. The sensitivity of screening for CTEV was 71.4% and the positive predictive value was 81.1%. The negative predictive value and specificity were more than 99.5%. There was no significant difference between the rates of detection before and after publication of the guidelines (p = 0.5). We conclude that a prenatal fetal anomaly ultrasound screening diagnosis of CTEV has a good positive predictive value enabling prenatal counselling. The change in screening guidance has not affected the proportion of missed cases. This information will aid counselling parents about the effectiveness and accuracy of prenatal ultrasound in diagnosing CTEV. Cite this article: Bone Joint J 2014;96-B:984–8

A girl aged ten, of Cape Coloured stock, with typical features of parastremmatic dwarfism has been investigated. The clinical manifestations included disproportionate dwarfism, limb deformity, a short stiff neck, and marked thoracic kyphosis. The radiographic changes were dramatic, the skeleton having a "flocky" appearance due to patches of radiolucency in an irregular lattice of sclerosis. The metaphyses and epiphyses of the long bones were grossly expanded, and the vertebrae were flattened and distorted. The clinical and radiographic features of five other previously reported individuals with parastremmatic dwarfism were very similar to those of our patient. The differential diagnosis of this condition includes metatrophic dwarfism, diatrophic dwarfism and the spondylo-epiphysial dysplasias. However, the unique "flocky" radiographic appearance of the bones permits diagnostic precision. There is some evidence to indicate that parastremmatic dwarfism might be transmitted as an autosomal dominant, although this is by no means certain. The fact that our patient had seven normal siblings and unaffected parents would be compatible woth autosomal recessive inheritance

Aims

The health-related quality of life (HRQoL) of paediatric patients with orthopaedic conditions and spinal deformity is important, but existing generic tools have their shortcomings. We aim to evaluate the use of Paediatric Quality of Life Inventory (PedsQL) 4.0 generic core scales in the paediatric population with specific comparisons between those with spinal and limb pathologies, and to explore the feasibility of using PedsQL for studying scoliosis patients’ HRQoL.

1. Fifteen patients who had had osteomyelitis of the lower femur or upper tibia during infancy have been studied. The cases of four patients showing particular features of the disease are presented in detail and the remainder are summarised. 2. It is suggested that damage to the epiphysis may be due to an abscess, or to ischaemia following occlusion of the blood supply. In the latter instance the prognosis may be better. 3. In the early stages the radiographic appearances may be deceptive, suggesting that damage to the epiphysis is irreparable. 4. Significant recovery of the epiphysis may occur in some patients after a delay of several years. This, together with the often good function which is preserved at the joint, should deter the surgeon from early destructive operation on the limb. 5. Deformity, which was present to some extent in all cases, appeared early and was not well controlled by conservative treatment. Operations were performed in twelve patients. 6. Shortening of the limb occurred in all patients and in some it was severe

Survivors of infantile meningococcal septicaemia often develop progressive skeletal deformity as a result of physeal damage at many 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. There have been reports of isolated cases of this deformity, but to our knowledge there have been no papers which specifically describe the development of the deformity and the options for treatment.

Surgery to correct this deformity is complex because of the patient’s age, previous scarring and the multiplanar nature of the deformity. The surgical goal is to restore leg-length equality and the mechanical axis at the end of growth. Surgery should be planned and staged throughout growth in order to achieve the best functional results.

We report our experience in six patients (seven ankles) with this deformity, who were managed by corrective osteotomy using a programmable circular fixator.

The use of joint-preserving surgery of the hip has been largely abandoned since the introduction of total hip replacement. However, with the modification of such techniques as pelvic osteotomy, and the introduction of intracapsular procedures such as surgical hip dislocation and arthroscopy, previously unexpected options for the surgical treatment of sequelae of childhood conditions, including developmental dysplasia of the hip, slipped upper femoral epiphysis and Perthes’ disease, have become available. Moreover, femoroacetabular impingement has been identified as a significant aetiological factor in the development of osteoarthritis in many hips previously considered to suffer from primary osteoarthritis.

As mechanical causes of degenerative joint disease are now recognised earlier in the disease process, these techniques may be used to decelerate or even prevent progression to osteoarthritis. We review the recent development of these concepts and the associated surgical techniques.

Cite this article: Bone Joint J 2014;96-B:5–18.

Septicaemia resulting from meningococcal infection is a devastating illness affecting children. Those who survive can develop late orthopaedic sequelae from growth plate arrests, with resultant complex deformities. Our aim in this study was to review the case histories of a series of patients with late orthopaedic sequelae, all treated by the senior author (CFB). We also describe a treatment strategy to address the multiple deformities that may occur in these patients.

Between 1997 and 2009, ten patients (seven girls and three boys) were treated for late orthopaedic sequelae following meningococcal septicaemia. All had involvement of the lower limbs, and one also had involvement of the upper limbs. Each patient had a median of three operations (one to nine). Methods of treatment included a combination of angular deformity correction, limb lengthening and epiphysiodesis. All patients were skeletally mature at the final follow-up. One patient with bilateral below-knee amputations had satisfactory correction of her right amputation stump deformity, and has complete ablation of both her proximal tibial growth plates. In eight patients length discrepancy in the lower limb was corrected to within 1 cm, with normalisation of the mechanical axis of the lower limb.

Meningococcal septicaemia can lead to late orthopaedic sequelae due to growth plate arrests. Central growth plate arrests lead to limb-length discrepancy and the need for lengthening procedures, and peripheral growth plate arrests lead to angular deformities requiring corrective osteotomies and ablation of the damaged physis. In addition, limb amputations may be necessary and there may be altered growth of the stump requiring further surgery. Long-term follow-up of these patients is essential to recognise and treat any recurrence of deformity.

We report the outcome of 28 patients with spina bifida who between 1989 and 2006 underwent 43 lower extremity deformity corrections using the Ilizarov technique. The indications were a flexion deformity of the knee in 13 limbs, tibial rotational deformity in 11 and foot deformity in 19. The mean age at operation was 12.3 years (5.2 to 20.6). Patients had a mean of 1.6 previous operations (0 to 5) on the affected limb. The mean duration of treatment with a frame was 9.4 weeks (3 to 26) and the mean follow-up was 4.4 years (1 to 9). There were 12 problems (27.9%), five obstacles (11.6%) and 13 complications (30.2%) in the 43 procedures. Further operations were needed in seven patients. Three knees had significant recurrence of deformity. Two tibiae required further surgery for recurrence. All feet were plantigrade and braceable.

We conclude that the Ilizarov technique offers a refreshing approach to the complex lower-limb deformity in spina bifida.

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.

We present a retrospective study of patients suffering from a variety of benign tumours in whom external fixators were used to treat deformity and limb-length discrepancy, and for the reconstruction of bone defects. A total of 43 limbs in 31 patients (12 male and 19 female) with a mean age of 14 years (2 to 54) were treated.

The diagnosis was Ollier’s disease in 12 limbs, fibrous dysplasia in 11, osteochondroma in eight, giant cell tumour in five, osteofibrous dysplasia in five and non-ossifying fibroma in two. The lesions were treated in the tibia in 19 limbs, in the femur in 16, and in the forearm in eight. The Ilizarov frame was used in 25 limbs, the Taylor Spatial Frame in seven, the Orthofix fixator in six, the Monotube in four and the Heidelberg fixator in one. The mean follow-up was 72 months (22 to 221).

The mean external fixation period was 168 days (71 to 352). The mean external fixation index was 42 days/cm (22.2 to 102.0) in the 22 patients who required limb lengthening. The mean correction angle for those with angular deformity was 23° (7° to 45°).

At final follow-up all patients had returned to normal activities. Four patients required a second operation for recurrent deformity of further limb lengthening. Local recurrence occurred in one patient, requiring further surgery.

Limb lengthening by callus distraction and external fixation has a high rate of complications. We describe our experience using an intramedullary nail (Fitbone) which contains a motorised and programmable sliding mechanism for limb lengthening and bone transport. Between 2001 and 2004 we lengthened 13 femora and 11 tibiae in ten patients (seven men and three women) with a mean age of 32 years (21 to 47) using this nail. The indications for operation were short stature in six patients and developmental or acquired disorders in the rest.

The mean lengthening achieved was 40 mm (27 to 60). The mean length of stay in hospital was seven days (5 to 9). The mean healing index was 35 days/cm (18.8 to 70.9). There were no cases of implant-related infection or malunion.