Aims. The Fassier Duval (FD) rod is a third-generation telescopic implant for children with osteogenesis imperfecta (OI). Threaded fixation enables proximal insertion without opening the knee or ankle joint. We have reviewed our combined two-centre experience with this implant. Methods. In total, 34 children with a mean age of five years (1 to 14) with severe OI have undergone rodding of 72 lower limb long bones (27 tibial, 45 femoral) for recurrent fractures with progressive deformity despite optimized bone health and bisphosphonate therapy. Data were collected prospectively, with 1.5 to 11 years follow-up. Results. A total of 24 patients (33%) required exchange of implants (14 femora and ten tibiae) including 11 rods bending with refracture. Four (5%) required reoperation with implant retention. Loss of proximal fixation in the femur and distal fixation in the tibia were common. Four patients developed coxa vara requiring surgical correction. In total, 13 patients experienced further fractures without rod bending; eight required implant revision. There was one deep infection. The five-year survival rate, with rod revision as the endpoint, was 63% (95% confidence interval (CI) 44% to 77%) for femoral rods, with a mean age at implantation of 4.8 years (1.3 to 14.8), and 64% (95% CI 36% to 82%) for tibial rods, with a mean age at implantation of 5.2 years (2.0 to 13.8). Conclusion. FD rods are easier to implant but do not improve on the revision rates reported for second generation T-piece rods. Proximal femoral fixation is problematic in younger children with a partially ossified greater trochanter. Distal tibial fixation typically fails after two years. Future generation implants should address proximal femoral and distal tibial fixation to avoid the majority of complications in this series. Cite this article: Bone Joint J 2020;102-B(8):1048–1055

Aim. A retrospective study was undertaken to evaluate and compare the results of telescoping (group I) versus non-telescoping rods (group II) in the treatment of osteogenesis imperfecta. Materials and Methods. Thirty-three lower limb segments in ten patients were studied (14 segments in group I and 19 segments in group II). The surgical techniques of Sofield and Miller (1959) and Lang-Stevenson and Sharrard (1984) for rod insertion were used. All cases were assessed clinically regarding mobility status, growth and limb-length, refracture, and infection. They were also assessed radiologically regarding rod migration, bone outgrowing the rod, incorrect T-piece placement, breakage and bending of rods. Results. The average duration of follow-up was 86.2 months (range : 6 to 8 years). Mobility status and bone growth were better in group I than in group II patients. The overall implant related complication rate was 28.6% in group I in comparison to 68.4% in group II. Rod migration was twice more common in group II than in group I. Bone outgrowing the rod and breakage of rods with fracture was seen in group II only. The three-year survival rate for telescoping rods was 92.9% in contrast to 68.4% for non-telescoping rods. The reoperation rate was 7.2% in group I and 31.6% in group II. Conclusions. From this comparative study it was clear that the results were significantly better after Sheffield rods with regard to mobility status, longevity of the rod, and the frequency of complications requiring reoperations. Also most of the complications were related to the technique of rod insertion and the type of rod

The Sheffield Expanding Intramedullary Rod System was developed after experiencing problems with existing rod systems in the management of osteogenesis imperfecta. Between 1986 and 1996 we treated 74 bones in the lower limb in 28 children at a median follow-up of 5.25 years. We have reviewed 24 children with a total of 60 rods. Before surgery, all children had had multiple fractures of the lower limb. At review eight patients had experienced no further fractures, but three had suffered five or more subsequently. Before initial stabilisation, 15 children had never walked, and only three (13%) used walking as their main means of mobility. After surgery, half of those who showed motor arrest were able to walk (p = 0.016). The number of patients able to walk, with or without aids, increased to 17 (p = 0.0001). We have experienced no evidence of epiphyseal damage after the procedure, and complication rates requiring rod exchange have been low (7%)

A retrospective review of patients with spinal growing rods in a single institution. Demographic data including age at first surgery, diagnosis, pre- and post-operative cobb angles from erect standardised radiographs were collected. The type of construct used i.e. spine to rib or spine to spine was noted along with the type of growing mechanism used (magnetic or cassette). Any complications were collated for each technique. Our results include 26 patients who had growing rod insertion, 12 in the spine - spine group and 14 in rib - spine group. Pre-operative cobb angles of 71 and 78 degrees respectively with a correction to 36 and 35 degrees. Mean age at surgery was 63 months in spine to spine group and 67 months in rib to spine group. Spine to spine group had 2 proximal pull out of hooks and the rib spine group had one pull out of hook. The correction achieved by the new technique is comparable to the spine – spine constructs. Complications are seen in both groups. The perceived benefit of the new technique is the proximal spine is not violated so there is a reduced risk of mass fusion. The canal and pedicles are not included proximally, so there will be no effect on the growing diameter of the canal. Biomechanically the construct is more robust and should allow greater control of the curve. Further follow up and analysis of this new technique is warranted

Aims. Congenital pseudarthrosis of the tibia (CPT) has traditionally been a difficult condition to treat, with high complication rates, including nonunion, refractures, malalignment, and leg length discrepancy. Surgical approaches to treatment of CPT include intramedullary rodding, external fixation, combined intramedullary rodding and external fixation, vascularized fibular graft, and most recently cross-union. The current study aims to compare the outcomes and complication rates of cross-union versus other surgical approaches as an index surgery for the management of CPT. Our hypothesis was that a good index surgery for CPT achieves union and minimizes complications such as refractures and limb length discrepancy. Methods. A multicentre study was conducted involving two institutions in Singapore and China. All patients with CPT who were surgically managed between January 2009 and December 2021 were included. The patients were divided based on their index surgery. Group 1 included patients who underwent excision of hamartoma, cross-union of the tibia and fibula, autogenic iliac bone grafting, and internal fixation for their index surgery. Group 2 included patients who underwent all other surgical procedures for their index surgery, including excision of hamartoma, intramedullary rodding, and/or external fixation, without cross-union of the tibia and fibula. Comparisons of the rates of union, refracture, limb length discrepancy, reoperations, and other complications were performed between the two groups. Results. A total of 36 patients were included in the study. Group 1 comprised 13 patients, while Group 2 comprised 23 patients. The mean age at index surgery was four years (1 to 13). The mean duration of follow-up was 4.85 years (1.75 to 14). All patients in Group 1 achieved bony union at a mean of three months (1.5 to 4), but ten of 23 patients in Group 2 had nonunion of the pseudarthrosis (p = 0.006). None of the patients in Group 1 had a refracture, while seven of 13 patients who achieved bony union in Group 2 suffered a refracture (p = 0.005). None of the patients in Group 1 had a limb length discrepancy of more than 2 cm, while ten of 23 patients in Group 2 have a limb length discrepancy of more than 2 cm (p = 0.006). In Group 1, four of 13 patients had a complication, while 16 of 23 patients in Group 2 had a complication (p = 0.004). Excluding removal of implants, four of 13 patients in Group 1 had to undergo additional surgery, while 18 of 23 patients in Group 2 had to undergo additional surgery following the index surgery (p = 0.011). Conclusion. A good index surgery of excision of hamartoma, cross-union of the tibia and fibula, autogenic iliac bone grafting, and internal fixation for CPT achieves union and minimizes complications such as refractures, limb length discrepancy, and need for additional surgeries

The aim was to assess the long-term impact of humeral and forearm rodding on functional ability, grip strength, joint range of motion and angular deformity in children with osteogenesis imperfecta. A retrospective chart review was conducted on 57 children with osteogenesis imperfecta who underwent humeral rodding or forearm rodding at our institution between 1996 and 2013. Functional ability was assessed using the self-care and mobility domains of the Pediatric Evaluation and Disability Inventory (PEDI). Grip strength was measured using a dynamometer and joint range of motion with a goniometer. Deformity was measured on radiographs of the humerus or forearm. Outcomes were assessed pre-operatively and every year post-operatively. Differences between pre-operative and 1-year post-operative outcomes were compared using paired T-tests. In 44 patients with a minimum of 2 years follow-up, outcome measures at 1-year post-surgery were compared to those at the latest clinic visit (mean follow-up = 8.0 years). Humeral and forearm rodding resulted in a significant improvement in PEDI self-care score (mean change =5.75, p=0.028 for the humerus, mean change = 6.77, p=0.0017 for the forearm) and mobility score (mean change =3.59, p=0.008 for the humerus, mean change =7.21, p=0.020 for the forearm) at 1 year post-surgery. Grip strength improved following forearm rodding (mean change = +6.13N, p=0.015) but not humeral rodding. Joint range of movement improved following humeral rodding but not forearm rodding. There was a significant improvement in radiographic angular deformity of the forearm and humerus following surgery (p<0.0001). Over 80% of improvements were maintained in the long-term. Humeral and forearm rodding in children with osteogenesis imperfecta leads to long-term improvement in functional ability and angular deformity

Aims. 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. Patients and Methods. 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. Results. Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10). Conclusion. Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563–1569

Aim. Since 2008 we have been using third generation Fassier Duval (FD) telescopic rods for children with complex and severe osteogenesis imperfecta (sOI). We present our experience with the technique. Method. 8 children with sOI have undergone rodding of 16 lower limb long bones; 6 tibial, 10 femoral. 3 were revision procedures, exchanging previous Sheffield rods that had been implanted with the female portion only. Indications were recurrent fracture and/or progressive deformity. Age range and follow up are 2–12 and 0.4–4.75 years respectively. All but one of the procedures were performed by a single surgeon. Results. There have been 3 re-fractures at 3–5 years with bending of the implant at the rod-tip interface and one re-fracture around a well-positioned implant without bending, all requiring revision. Some female femoral rods have migrated distally (3/10) leading in one case to re-fracture above the implant, which was also revised. All rods have initially telescoped well but we have experienced high pull out rates of the threads in the distal tibia (3/6) at an average 18 months post implantation (range 14–20 months). None has yet required revision for this. Proximal migration of the femoral female component has not occurred except in one revision case, which responded well to interference wire fixation. We have one case of coxa vara that developed in a child whose femur was rodded at 2 years of age. Conclusion. The FD implants have functioned well and enabled us to avoid opening the ankle joint for tibial roddings. Nonetheless the re-fracture rates remain high in this group of severely affected children and are comparable to older generation implants. Proximal femoral and distal tibial anchoring points remain common sites of failure though rarely require early re-operation

The aim of this retrospective study was to review the outcome of patients treated with Fassier-Duval (FD) rods and highlight some of the complications found during treatment. Between April 2006 and August 2010 we inserted 24 FD rods in 13 patients. 17 rods for osteogenesis imperfecta (OI), 2 for fractures and deformity associated with cerebral palsy, 1 for fracture associated with muscular dystrophy, 1 for fibrous dysplasia and 3 for centralisation of single bone forearms. In the upper limb one patient required revision for proximal migration of the male component and another patient is waiting for revision for the same problem. In the lower limb, a tibial nail was revised because of proximal migration of the male component. A femoral nail was adjusted because of loss of the proximal fixation. One of the OI patients fell, fractured the femur and bent a femoral nail. This awaits revision at a later date. A second OI patient fell on 2 separate occasions bending both a tibial and a femoral nail respectively. These were both revised to trigen intramedullary nails. In all the other cases there were no complications. In summary the Fassier Duval system provides a versatile way of providing intramedullary stabilisation for growing bones through a single entry point. However in our experience we have a 33% complication rate most notably bending of the rods. We advocate careful patient selection and using as high a diameter nail as is feasible

Aims

Given the possible radiation damage and inaccuracy of radiological investigations, particularly in children, ultrasound and superb microvascular imaging (SMI) may offer alternative methods of evaluating new bone formation when limb lengthening is undertaken in paediatric patients. The aim of this study was to assess the use of ultrasound combined with SMI in monitoring new bone formation during limb lengthening in children.

The aim of this study was to determine the effectiveness of intra-operative tranexamic acid in children with osteogenesis imperfecta, who have been shown to have increased bleeding tendency, in deformity correction surgery. We retrospectively reviewed all cases of lower limb intramedullary rodding in patients with osteogenesis imperfecta treated in our unit from 2000–2013 in whom a pre and post- operative full blood count was available (n=69). Case notes were reviewed and patients were grouped according to the use of tranexamic acid. Peri-operative change in haemoglobin (Hb), haematocrit (HCT) and requirement for blood transfusion was assessed. Of 69 operations performed, 62.3% were in female patients, 78.3% were femoral, and 43.5% were bilateral cases (19 femur, 11 tibia). In the non-tranexamic acid group there was a mean drop in Hb of 28.9 g/L (range 0–62), mean HCT drop of 8.8 (range 2.2–19.4) and 3 patients required red cell transfusion. In the tranexamic acid group there was a mean Hb drop of 22.5 g/L (range 1–49), mean HCT drop of 7.35 (range −0.8–16.7) and one patient required red cell transfusion. There was a significant decrease in Hb drop (p=0.0287) in the tranexamic acid group. Tranexamic acid seems to decrease the drop in haemoglobin during lower limb intramedullary rodding in patients with osteogenesis imperfecta, with little associated risk. Protocols should be established for future use a further review undertaken

PURPOSE. To report the experience and evolution of treatment of Congenital Pseudarthrosis of the Tibia in a single surgeon, consecutive series of 19 patients. METHODS. A retrospective case series review, with radiological assessment and functional outcome scores including the Activity Scale for Kids (ASK) and the Oxford Ankle Foot Questionnaire (OAFQ) Child & Teenager version. Patients were classified as per Boyd & Andersen. Alteration in the Tibial mechanical axis was compared pre-treatment and at last review. RESULTS. The average follow up from presentation was 6.3 years, 7 patients had reached skeletal maturity. Union was obtained in 79% at the time of review with a mean time to union of an established pseudarthrosis of 15.2 months. 9 of 19 had an established diagnosis of Neurofibromatosis. 8 had a frank pseudarthrosis at presentation, 2 of which were present at birth. There were 3 re-fractures following treatment. One of the 19 required amputation after failed surgical treatment. Each patient had an average of 2.4 surgical procedures. 14 patients had either fixed or elongating rods, 10 had Ilizarov treatment. 2 have outstanding surgery planned. CONCLUSIONS. A philosophy of treatment has evolved which produces acceptable results. Splintage is used for antero-lateral bowing with corrective osteotomies and rodding, without extensive bone or periosteal resection, for significant or progressive deformity or fracture. Ilizarov treatment is used for recalcitrant cases over the age of 6 years. The technique has evolved over time to include bone graft wraps and the use of Bone Morphogenic protein in combination with the Ilizarov technique. Patients have below population and age matched average scores for both the ASK and OAFQ

We report a postal survey of 59 families of children with osteogenesis imperfecta. From the 51 replies we collected data on developmental milestones and walking ability and related them to the Sillence and the Shapiro classifications of osteogenesis imperfecta. Twenty-four of the patients had been treated by intramedullary rodding. Both classifications helped to predict eventual walking ability. We found that independent sitting by the age of ten months was a predictor for the use of walking as the main means of mobility with 76% attaining this. Of the patients who did not achieve sitting by ten months, walking became the main means of mobility in only 18%. The developmental pattern of mobility was similar in the rodded and non-rodded patients

The use of recombinant human bone morphogenetic protein-2 (rhBMP-2) for the treatment of congenital pseudarthrosis of the tibia has been investigated in only one previous study, with promising results. The aim of this study was to determine whether rhBMP-2 might improve the outcome of this disorder. We reviewed the medical records of five patients with a mean age of 7.4 years (2.3 to 21) with congenital pseudarthrosis of the tibia who had been treated with rhBMP-2 and intramedullary rodding. Ilizarov external fixation was also used in four of these patients. Radiological union of the pseudarthrosis was evident in all of them at a mean of 3.5 months (3.2 to 4) post-operatively. The Ilizarov device was removed after a mean of 4.2 months (3.0 to 5.3). These results indicate that treatment of congenital pseudarthrosis of the tibia using rhBMP-2 in combination with intramedullary stabilisation and Ilizarov external fixation may improve the initial rate of union and reduce the time to union. Further studies with more patients and longer follow-up are necessary to determine whether this surgial procedure may significantly enhance the outcome of congenital pseudarthrosis of the tibia, considering the refracture rate (two of five patients) in this small case series

The aim of the study was to review the effectiveness of rigid IM nailing in stabilisation and deformity correction of lower limb long bones in adolescents with metabolic bone disease which to our knowledge has not been studied before. Medical records and radiographs were retrospectively reviewed looking at indications, deformity correction, number of osteotomies-if needed, bone healing, time to healing and incidence of complications. Between Aug 2010 and Mar 2015 fifteen patients (24 segments) had rigid IM nailing. Ten patients had Osteogenesis Imperfecta, four with McCune Albright syndrome and one with hypophosphatemic rickets. 22 femora and two tibiae were IM nailed. The mean age of the patients was 13.1 (9.6–16.75 years). Eleven out 24 segments were previously rodded. Eight segments were for acute fractures. 13 bones had significant deformities requiring corrective osteotomies. One patient had previous fracture non union. All patients were allowed to partial weight bear immediately postoperatively and were fully mobile six weeks following surgery. Mean follow up was 24 months (3–51 months) post-operatively. All deformities were corrected. All fractures and osteotomies radiologically united. Mean radiological union time was 5.5 months (6 weeks – 11 months). Patients with acute fractures had mean radiological union time of 4 months. Patients who had osteotomies had a mean radiological union time of 7.1 months. The patient with previous non union had BMP at the same time and radiologically healed in 10 months. Two patients had persistent bisphosphonate osteotomy lines but were asymptomatic. One patient had removal of a prominent distal locking screw and one had persistent Trendelenburg gait. Rigid intramedullary nailing is effective in stabilisation and deformity correction of long bones in adolescent patients with brittle bone disease. The technique has a low complication rate. We recommend the use of this technique in paediatric limb reconstruction in managing metabolic bone conditions

Aims

The objective of this study was to evaluate the clinical and radiological outcomes of patients younger than six months of age with developmental dysplasia of the hip (DDH) managed by either a Pavlik harness or Tübingen hip flexion splint.

We have reviewed the results of the Sofield-Millar operation on 58 long bones in ten patients. If more than three osteotomies were undertaken the time to union of the bone was significantly prolonged (p< 0.001) with significant thinning of the bone (p< 0.02). We have used a modified technique in order to minimise surgical trauma and devascularisation of the bone. The rod is introduced under the control of an image-intensifier. Small surgical exposures are made only at the sites of corrective wedge osteotomies. The number of osteotomies is kept to the minimum

We have used the Ilizarov technique for severe flexion deformity of the knee in 11 patients (13 knees) between 1986 and 1994 and have followed them up for an average of 4.1 years. The age of the patients at operation ranged from 1.7 to 18.8 years. The femoral and tibial components were connected by two anterior hinges, medial and lateral, and two posterior distraction rods. The deformity was corrected to a femorotibial lateral shaft angle of less than 20°. A permanent orthosis was applied after removal of the fixator. Fractures occurred in four patients and paralysis of the common peroneal nerve in another. There was a recurrence of the deformity in four patients. At the last review all patients were able to walk on their operated leg with or without an orthosis. We have found the Ilizarov method to be helpful in correcting severe fixed flexion deformity of the knee, with relatively few complications, but the basic principles of the method must be carefully followed

We performed limb lengthening and correction of deformity of nine long bones of the lower limb in six children (mean age, 14.7 years) with osteogenesis imperfecta (OI). All had femoral lengthening and three also had ipsilateral tibial lengthening. Angular deformities were corrected simultaneously. Five limb segments were treated using a monolateral external fixator and four with the Ilizarov frame. In three children, lengthening was done over previously inserted femoral intramedullary rods. The mean lengthening achieved was 6.26 cm (mean healing index, 33.25 days/cm). Significant complications included one deep infection, one fracture of the femur and one anterior angulation deformity of the tibia. The abnormal bone of OI tolerated the external fixators throughout the period of lengthening without any episodes of migration of wires or pins through the soft bone. The regenerate bone formed within the time which is normally expected in limb-lengthening procedures performed for other conditions. We conclude that despite the abnormal bone characteristics, distraction osteogenesis to correct limb-length discrepancy and angular deformity can be performed safely in children with OI

Aims

The treatment of tibial aplasia is controversial. Amputation represents the gold standard with good functional results, but is frequently refused by the families. In these patients, treatment with reconstructive limb salvage can be considered. Due to the complexity of the deformity, this remains challenging and should be staged. The present study evaluated the role of femoro-pedal distraction using a circular external fixator in reconstructive treatment of tibial aplasia. The purpose of femoro-pedal distraction is to realign the limb and achieve soft tissue lengthening to allow subsequent reconstructive surgery.