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AimsTemporary 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.MethodsBetween 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).ResultsOverall, 79% (109/138) of patients treated with flexible staples achieved sufficient deformity correction. The median treatment duration was 16 months (interquartile range (IQR) 8 to 21). The flexible staples achieved a median MAD correction of 1.2 mm/month/HED site (IQR 0.6 to 2.0) in valgus deformities and 0.6 mm/month/HED site (IQR 0.2 to 1.5) in varus deformities. Wound infections occurred in 1%, haematomas and joint effusions in 4%, and implant-associated complications in 1% of patients treated with flexible staples. Valgus AD were corrected faster using flexible staples than two-hole plates and Blount staples. Furthermore, the median MAD after treatment was lower in varus and valgus AD, fewer implant-associated complications were detected, and reduced implantation times were recorded using flexible staples.ConclusionThe flexible staple seems to be a viable option for guided growth, showing comparable or possibly better results regarding correction speed and reducing implant-associated complications. Further comparative studies are required to substantiate these findings.Cite this article: Bone Joint J 2023;105-B(3):331–340.

Aims Distraction osteogenesis with intramedullary lengthening devices has undergone rapid development in the past decade with implant enhancement. In this first single-centre matched-pair analysis we focus on the comparison of treatment with the PRECICE and STRYDE intramedullary lengthening devices and aim to clarify any clinical and radiological differences. Methods A single-centre 2:1 matched-pair retrospective analysis of 42 patients treated with the STRYDE and 82 patients treated with the PRECICE nail between May 2013 and November 2020 was conducted. Clinical and lengthening parameters were compared while focusing radiological assessment on osseous alterations related to the nail’s telescopic junction and locking bolts at four different stages. Results Osteolysis next to the telescopic junction was observed in 31/48 segments (65%) lengthened with the STRYDE nail before implant removal compared to 1/91 segment (1%) in the PRECICE cohort. In the STRYDE cohort, osteolysis initially increased, but decreased or resolved in almost all lengthened segments (86%) after implant removal. Implant failure was observed in 9/48 STRYDE (19%) and in 8/92 PRECICE nails (9%). Breakage of the distal locking bolts was found in 5/48 STRYDE nails (10%) compared to none in the PRECICE cohort. Treatment-associated pain was generally recorded as mild and found in 30/48 patients (63%) and 39/92 (42%) in the STRYDE and PRECICE cohorts, respectively. Temporary range of motion (ROM) limitations under distraction were registered in 17/48 (35%) segments treated with the STRYDE and 35/92 segments (38%) treated with the PRECICE nail. Conclusion Osteolysis and periosteal reaction, implant breakage, and pain during lengthening and consolidation is more likely in patients treated with the STRYDE nail compared to the PRECICE nail. Temporary ROM limitations during lengthening occurred independent of the applied device. Implant-related osseous alterations seem to remodel after implant removal. Cite this article: Bone Joint J 2023;105-B(1):88–96.

Leg length discrepancies (LLD) are frequently associated with coronal malalignment. Temporary hemiepiphysiodesis (HED) is a well-established procedure for the correction of limb malalignment in skeletally immature patients. For treatment of LLD > 2 cm, lengthening with intramedullary devices gains increasing popularity. However, no studies have investigated the combined application of HED and intramedullary lengthening in skeletally immature patients. This retrospective single-center study evaluated the clinical and radiological outcomes of femoral lengthening with an antegrade intramedullary lengthening nail combined with temporary HED performed in 25 patients (14 females) between 2014 and 2019. Temporary HED through the implantation of flexible staples of the distal femur and/or proximal tibia was either performed prior (n = 11), simultaneously (n = 10) or subsequently (n = 4) to femoral lengthening. The mean follow-up period was 3.7 years (±1.4). The median initial LLD was 39.0 mm (35.0–45.0). Twenty-one patients (84%) presented valgus and four (16%) showed varus malalignment. Leg length equalization was achieved in 13 of the skeletally mature patients (62%). The median LLD of the eight patients with residual LLD > 10 mm at skeletal maturity was 15.5 mm (12.8–21.8). Limb realignment was observed in nine of seventeen skeletally mature patients (53%) in the valgus group, and in one of four patients (25%) in the varus group. Combining antegrade femoral lengthening and temporary HED is a viable option to correct LLD and coronal limb malalignment in skeletally immature patients; however, achieving limb length equalization and realignment may be difficult in cases of severe LLD and angular deformity, in particular.

Background Aneurysmal bone cyst (ABC) is a benign, locally aggressive tumor that occurs in childhood and early adulthood. It usually affects the metaphysis of long bones but can also occur in the foot. Methods This single-center study is a retrospective review of ten patients with primary ABCs of the foot which underwent an intralesional curettage or a polidocanol instillation. Results After intralesional curettage a local recurrence was observed in 3/5 cases. The instillation of polidocanol showed a significant reduction of the initial cyst volume (p = 0.0267). In the instillation subgroup a primary complete healing was achieved in three cases. Due to a persisting disease two cases were converted to intralesional curettage without local recurrence in the follow-up. Conclusions Percutaneous instillation of polidocanol is a minimally invasive treatment option for ABCs of the foot and especially for small bones. Nevertheless, several sequential instillations and a conversion to intralesional curettage can be necessary.

Background Limb lengthening with magnetically driven intramedullary lengthening nails is a fast-developing field and represents an alternative to external fixators. Although previous studies have assessed the application of magnetically driven intramedullary lengthening nails, these studies have been heterogenous regarding the nailing approach, the bone treated, and the implant type; they also have analyzed relatively small patient groups at short follow-up durations. Questions/purposes (1) Is femoral lengthening with magnetically driven antegrade intramedullary lengthening nails accurate and precise? (2) What are the most common complications of treatment? (3) What factors are associated with unplanned additional surgery? Methods We retrospectively analyzed the longitudinally maintained database of our orthopaedic teaching hospital to identify all patients who underwent surgery for leg length discrepancy (LLD) between October 2014 and April 2019. In total, we surgically treated 323 patients for LLD of 2 cm or more. Of those 55% (177 of 323) were treated with distraction osteogenesis with magnetically driven intramedullary lengthening nails, 18% (59 of 323) with external fixation, and 27% (87 of 323) with epiphysiodesis around the knee. Based on that, 29% (93 of 323) of patients underwent unilateral femoral distraction osteogenesis with magnetically driven antegrade femoral lengthening nails and were eligible for analysis. No patient was excluded, and 3% (3 of 93) were lost before the minimum study follow-up of 2 years, leaving 97% (90 of 93) for analysis. Patients with a distal femoral deformity were treated via a retrograde femoral approach (10% [33 of 323]) or with external fixators (3% [10 of 323]) and were not included in this study. Distraction osteogenesis with magnetically driven intramedullary lengthening nails was not considered for patients with deep tissue infection, those with bone dimensions considered to be too small in relation to the available implants, and for patients younger than 8 years. This study included 90 patients (44 females, 43 left femora) treated for a median (interquartile range) preoperative LLD of 39 mm (32 to 52) at a median age of 15 years (14 to 17). The same limb lengthening system was applied in all patients. The median (IQR) follow-up was 35 months (24 to 78). Data were acquired through a chart review performed by someone not involved in the surgical care of the included patients. Data acquisition was supervised and curated by two of the involved surgeons. Accuracy was calculated as 100 - [(achieved distraction in mm - planned distraction in mm) / (planned distraction in mm) x 100] and precision as 100 - (relative standard deviation of accuracy). Treatment-associated complications were summarized descriptively and characterized as complications resulting in unplanned additional surgery or those not resulting in unplanned surgery. To analyze the risk of unplanned additional surgery by entity, we calculated odds ratios (ORs) comparing the incidence of unplanned additional surgery in the different entity cohorts with the idiopathic LLD cohort as a reference. By calculating ORs, we analyzed the risk for unplanned additional surgery depending on sex, age, surgery time, and previous lengthening. Due to the lack of long-term evidence about motorized lengthening nails remaining in situ and concerns about potential implant-related adverse effects, removal was routinely scheduled 1 year after consolidation. For implant removal, 92% (83 of 90) of patients underwent planned additional surgery, which was not recorded as an adverse event of the treatment. Ninety-seven percent (87 of 90) of patients completed lengthening with the implant remaining in situ until the end of distraction. The median (IQR) distraction length was 37 mm (30 to 45) with a median distraction index of 0.9 mm/day (0.7 to 1.0) and median consolidation index of 31 days/cm (25 to 42). Results The calculated accuracy and precision were 94% and 90%, respectively. In total, 76% (68 of 90) of our patients experienced complications, which resulted in 20% (18 of 90) of patients undergoing unplanned additional surgery. The most common complication overall was adjustment of the distraction rate in 27% (24 of 90) of patients (faster: 16% [14 of 90]; slower: 11% [10 of 90]) and temporary restriction of knee motion, which occurred in 20% (18 of 90) of our patients and resolved in all patients who experienced it. The most serious complications were bacterial osteomyelitis and knee subluxation, which occurred in 3% (3 of 90) and 1% (1 of 90) of our patients, respectively. With the numbers available, we found only one factor associated with an increased likelihood of unplanned additional surgery: Patients with postinfectious LLD had higher odds of unplanned additional surgery than patients with idiopathic LLD (7% [1 of 15] versus 50% [3 of 6], OR 14.0 [95% CI 1.06 to 185.49]; p = 0.02). However, we caution readers this finding is fragile, and the confidence interval suggests that the effect size estimate is likely to be imprecise. Conclusion Femoral distraction osteogenesis with magnetically driven antegrade intramedullary lengthening nails appears to be an accurate and reliable treatment for femoral lengthening. However, depending on the etiology, a high risk of unplanned additional surgery should be anticipated, and a high proportion of patients will experience temporary joint stiffness. We recommend close orthopaedic follow-up and physiotherapy during treatment. This treatment of LLD can be considered alongside other nails, external fixators, and epiphysiodesis. Multicenter studies comparing this with other approaches are needed. Level of evidence Level IV, therapeutic study.

Background: Symptomatic patients with severe brachymetatarsia are commonly treated with callus distraction using external mini-fixator (EF) or internal device (ID). This study points out advantages and limitations of both methods comparing clinical and radiographical parameters. Methods: Retrospective analysis of 21 metatarsal bones in twelve patients. Twelve metatarsals were treated with ID (Genos Mini), nine with EF (MiniRail). Results: Mean lengthening distance was 17.3 mm using EF and 11.7 mm using ID (p = 0.016). Adverse results were observed in 89% of metatarsals treated with EF and in 33% treated with ID (p = 0.011). Postoperative surgical intervention was required in 33% using EF compared to 0% using ID (p = 0.031). Mean total German Foot Function Index (FFI-T) improved from 49 to 33 using EF and from 47 to 22 using ID (p < 0.001). Conclusion: CD is a reliable surgical treatment for BMT. Surgeons should be aware of implant-related advantages and complications when counseling patients about treatment options.

(1) Purpose: Lower limb deformities can have a severe impact on health-related quality of life (HRQL). LIMB-Q Kids is a new patient-reported outcome measure (PROM) aiming to elucidate the experience of 8–18-year-old patients before, during and after treatment, and to measure the different aspects of HRQL. The aim of this study was to translate and culturally adapt LIMB-Q Kids to German. (2) Methods: The International Society for Pharmacoeconomics and Outcomes Research (ISPOR) guidelines were followed. Three forward translations, a backward translation, an expert panel meeting with eight participants, and twenty cognitive debriefing interviews led to the final German version of LIMB-Q Kids. (3) Results: In the forward translations, 4/159 items were difficult to translate, and 2/159 items in the backward translation differed from the original English version. Cognitive debriefing interviews with 20 patients identified 7/159 items that were difficult to comprehend/answer, and 2 of these items were changed. (4) Conclusions: Lower limb deformities can have a great impact on children, and it is important to measure and consider the impact on HRQL. In order to be able to use PROMs in different countries, conceptually equivalent translations and cultural adaptations should be performed in order to ensure comprehensibility. The final German version of LIMB-Q Kids is ready for use in an international field test.

BackgroundMany patients with achondroplasia experience functional impairments because of rhizomelic upper extremities (proximal limb shortening). Bilateral humeral lengthening may overcome these functional limitations, but it is associated with several risks, such as radial nerve palsy and insufficient bone regeneration. Only a few studies have reported on patient satisfaction and functional outcome after humeral lengthening in patients with achondroplasia. Furthermore, the reported numbers of adverse events associated with lengthening procedures using external fixators vary widely.Questions/purposes(1) Does bilateral humeral lengthening with a monolateral external fixator in patients with achondroplasia reliably improve patient function and autonomy, and what proportion of patients achieved at least 8 cm of humeral lengthening? (2) What adverse events occur after bilateral humeral lengthening with monolateral external fixators?MethodsBetween 2011 and 2019, 44 patients underwent humeral lengthening at our institution. Humeral lengthening was performed in patients with severe shortening of the upper extremities and functional impairments. In humeri in which intramedullary devices were not applicable, lengthening was performed with monolateral external fixators in 40 patients. Eight patients were excluded because they underwent unilateral lengthening for etiologies other than achondroplasia, and another four patients did not fulfill the minimum study follow-up period of 2 years, leaving 28 patients with bilateral humeral lengthening to treat achondroplasia available for analysis in this retrospective study. The patients had a median (interquartile range) age of 8 years (8 to 10), and 50% (14 of 28) were girls. The median follow-up time was 6 years (4 to 8). The median humeral lengthening was 9 cm (9 to 10) with a median elongation of 73% (67% to 78%) from an initial median length of 12 cm (11 to 13). To determine whether this treatment reliably improved patient function and autonomy, surgeons retrospectively evaluated patient charts. An unvalidated retrospective patient-reported outcome measure questionnaire consisting of nine items (with answers of "yes" or "no" or a 5-point Likert scale) was administered to assess the patient's functional improvement in activities of daily living, physical appearance, and overall satisfaction, such that 45 points was the highest possible score. The radiographic outcome was assessed on calibrated radiographs of the humerus. To ascertain the proportion of adverse events, study surgeons performed a chart review and telephone interviews. Major complications were defined as events that resulted in unplanned revision surgery, nerve injury (either temporary or permanent), refracture of the bone regenerate, or permanent functional sequelae. Minor complications were characterized as events that resolved without further surgical interventions.ResultsOn our unvalidated assessment of patient function and independence, all patients reported improvement at their most recent follow-up compared with scores obtained before treatment (median [IQR] 24 [16 to 28] before surgery versus 44 [42 to 45] at latest follow-up, difference of medians 20 points, p < 0.001). A total of 89% (25 of 28) of patients achieved the desired 8 cm of lengthening in both arms. A total of 50% (14 of 28) of our patients experienced a major complication. Specifically, 39% (11 of 28) had an unplanned reoperation, 39% (11 of 28) had a radial nerve palsy, 18% (5 of 28) had a refracture of the regenerate, and 4% (1 of 28) concluded treatment with a severe limb length discrepancy. In addition, 82% (23 of 28) of our patients experienced minor complications that resolved without further surgery and did not involve radial nerve symptoms. Radial nerve palsy was observed immediately postoperatively in eight of 13 segments, and 1 to 7 days postoperatively in five of 13 segments. The treatment goal was not achieved because of radial nerve palsy in 5% (3 of 56) of lengthened segments, which occurred in 7% (2 of 28) of patients. Full functional recovery of the radial nerve was observed in all patients after a median (IQR) of 3 months (2 to 5). Refractures of bone regenerates were observed in 11% (6 of 56) of humeri in 18% (5 of 28) of patients. Of those refractures, 1 of 6 patients was treated nonsurgically with a hanging cast, while 5 of 6 patients underwent revision surgery with intramedullary rodding.ConclusionMost patients with achondroplasia who underwent humeral lengthening achieved the treatment goal without permanent sequelae; nonetheless, complications of treatment were common, and the road to recovery was long and often complicated, with many patients experiencing problems that were either painful (such as refracture) or bothersome (such as temporary radial nerve palsy). However, using a subjective scale, patients seemed improved after treatment; nevertheless, robust outcomes tools are not available for this condition, and so we must interpret that finding with caution. Considering our discoveries, bilateral humeral lengthening with a monolateral external fixator should only be considered in patients with severe functional impairments because of rhizomelic shortening of the upper extremities. If feasible, internal lengthening devices might be preferable, as these are generally associated with higher patient comfort and decreased complication rates compared with external fixators.Level of EvidenceLevel IV, therapeutic study.

Background: Patient-reported outcome measures are gaining increasing importance in clinical research and quality control. Clinical impairment through limb deformities can appear in various forms. This study aimed at translating and culturally adaptating the Limb Deformity-Scoliosis Research Society (LD-SRS) patient-reported outcome measure (PROM) into German by following the scientific rigor of the cross-cultural adaptation process as well as ensuring the reliability of the translated version. The LD-SRS is applicable in children and adults. Methods: The translation was performed in accordance with the creators of the LD-SRS following the Professional Society for Health Economics and Outcomes Research (ISPOR) guidelines for translation and cultural adaptation. Two forward translations were performed, and after a consensus meeting, a professional translator translated the PROM back to English. The creators reviewed the back translation of the preliminary German version. Thirty patients with upper and lower limb deformities participated in cognitive debriefing interviews. The version was proofread and, finally, the test-retest reliability was estimated. Results: The mean age was 19 years (range 6–61). Twenty-six patients (87%) completed the retest after 6 days (range 3–26). The internal consistency was estimated with a Cronbach’s alpha of 0.96 (range 0.94–0.97), and the intraclass correlation was 0.92 (range 0.89–0.94), indicating an excellent reliability. The scores were normally distributed. Thereafter, the German version was proofread and finalized. Conclusions: The German translation and cross-cultural adaptation of the LD-SRS score resulted in a high reliability and internal consistency. The German LD-SRS score is readily usable and may be applied in future studies of German-speaking limb deformity patients.

Aims Temporary epiphysiodesis (ED) is commonly applied in children and adolescents to treat leg length discrepancies (LLDs) and tall stature. Traditional Blount staples or modern two-hole plates are used in clinical practice. However, they require accurate planning, precise surgical techniques, and attentive follow-up to achieve the desired outcome without complications. This study reports the results of ED using a novel rigid staple (RigidTack) incorporating safety, as well as technical and procedural success according to the idea, development, evaluation, assessment, long-term (IDEAL) study framework. Methods A cohort of 56 patients, including 45 unilateral EDs for LLD and 11 bilateral EDs for tall stature, were prospectively analyzed. ED was performed with 222 rigid staples with a mean follow-up of 24.4 months (8 to 49). Patients with a predicted LLD of ≥ 2 cm at skeletal maturity were included. Mean age at surgery was 12.1 years (8 to 14). Correction and complication rates including implant-associated problems, and secondary deformities as well as perioperative parameters, were recorded (IDEAL stage 2a). These results were compared to historical cohorts treated for correction of LLD with two-hole plates or Blount staples. Results The mean LLD was reduced from 25.2 mm (15 to 45) before surgery to 9.3 mm (6 to 25) at skeletal maturity. Implant-associated complications occurred in 4/56 treatments (7%), and secondary frontal plane deformities were detected in 5/45 legs (11%) of the LLD cohort. Including tall stature patients, the rate increased to 12/67 legs (18%). Sagittal plane deformities were observed during 1/45 LLD treatments (2%). Compared to two-hole plates and Blount staples, similar correction rates were observed in all devices. Lower rates of frontal and sagittal plane deformities were observed using rigid staples. Conclusion Treatment of LLD using novel rigid staples appears a feasible and promising strategy. Secondary frontal and sagittal plane deformities remain a potential complication, although the rate seems to be lower in patients treated with rigid staples. Further comparative studies are needed to investigate this issue. Cite this article: Bone Joint J 2021;103-B(8):1428–1437.

ZusammenfassungArthrotische Gelenkveränderungen weisen eine Vielzahl an Ursachen auf. Ist eine eindeutige Zuordnung nicht möglich, spricht man von primären Arthrosen, hingegen kann die Entwicklung sekundärer Arthrosen auf konkrete Risikofaktoren zurückgeführt werden. Einen Großteil davon bilden angeborene und idiopathische Fehlstellungen, die auch als präarthrotische Deformitäten bezeichnet werden. Eine sekundäre Arthrose manifestiert sich in der Regel bereits in deutlich jüngerem Lebensalter als ein primärer Gelenkverschleiß, gerade deshalb ist die rechtzeitige Diagnosestellung von entscheidender Bedeutung. Angeborene und idiopathische Deformitäten bleiben meist lange asymptomatisch, was die Diagnostik deutlich erschwert. In den letzten Jahren hat die Früherkennung häufiger Deformitäten jedoch stark an Bedeutung gewonnen, wie man am Beispiel der Sonografie zur Beurteilung der Hüftreifung im Rahmen der kindlichen Vorsorgeuntersuchung erkennen kann. Je nach Art der Deformität ist sowohl eine konservative, als auch eine operative Therapie möglich. Dabei spielt der richtige Zeitpunkt für den Therapiebeginn eine wichtige Rolle, denn sowohl ein Abwarten des Spontanverlaufs wie bei auffälligen frontalen Beinachsen, aber auch eine sofortige Behandlung wie bei der Hüftdysplasie können lege artis sein. Zu den häufigsten operativen Maßnahmen zählen die Wachstumslenkung und die Umstellungsosteotomie. Ziel der Therapie ist die Reduktion des Arthroserisikos auf das der Durchschnittsbevölkerung durch Wiederherstellung anatomisch und biomechanisch korrekter Stellungsverhältnisse. Folglich handelt es sich bei der Behandlung von präarthrotischen Deformitäten um präventive, gelenkerhaltende Maßnahmen, die in der Regel schonender und komplikationsärmer sind, als ein späterer endoprothetischer Gelenkersatz. Die Gefahr eines verspäteten Behandlungsbeginns ist die Notwendigkeit (aufwendigerer) operativer Interventionen.

Subtalar arthroereises (STA) is a minimally invasive and reversible surgery to correct symptomatic flexible flatfoot (FFF) in children. Various techniques were described either applying expandable sinus tarsi implants or lateral calcaneus stop screws. Studies comparing the outcome of STA with different devices are rare. This retrospective single-center cohort study analyzes the results of STA using three different implants. 113 STA were performed in 73 consecutive patients (28 females). Mean age at surgery was 10.8 years (range 5–16). Mean follow-up was 29.0 months (range 1–111). In 21 feet the non-absorbable Kalix® endorthesis and in 56 feet the absorbable Giannini endorthesis were applied. Subtalar extraarticular screw arthroereises (SESA) was conducted in 36 feet. Clinical, radiographic and pedobarographic parameters were analyzed. No intraoperative complications were observed. All three procedures achieved comparable improvements of the clinical, radiographic and pedobarographic parameters. The mean foot function index (FFI) improved from 36.4 (range 12–63) to 22.8 (range 2–55). The mean preoperative calcaneal inclination angle and the lateral talocalcaneal angle improved from 9.5° (range 0–22) and 42.3° (range 21–62) to 12.8° (range 0–26) and 37.6° (range 15–56), respectively. Pedobarographically determined values of the arch index, the medial midfoot contact area and the medial forefoot peak pressure decreased. In contrast to SESA (1/36, 3%), a higher incidence of implant-related complications was observed using Kalix® (6/21, 29%) and Giannini (10/56, 8%) sinus tarsi implants. Peroneal muscle contractures only occurred in the SESA group (4/36, 11%). Premature removal due to treatment-related complications was necessary in 6/21 Kalix® implants (29%), 4/56 Giannini implants (7%) and 4/36 SESA implants (11%). Implant choice for treatment of painful FFF in children with STA seems to play a subordinate role. Clinical, radiographic and pedobarographic outcomes are comparable between the applied implants. Surgeons and patients should be aware of the different spectrum of implant-related complications. Treatment can be reliably monitored by radiation-free pedobarography providing dynamic information about the deformity.

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. Methods This was a retrospective study involving ten patients (12 limbs) with tibial aplasia, who underwent staged reconstruction. During the first operation a circular hexapod external fixator was applied and femoro-pedal distraction was undertaken over several months. Subsequent surgery included reconstruction of the knee joint and alignment of the foot. Results The mean follow-up was 7.1 years (2 to 10). The mean age of the patients at the time of the application of the fixator was 2.3 years (1.1 to 5.0). The mean time under distraction was 139.7 days (81.0 to 177.0). A mean fibular distalization of 38.7 mm (14.0 to 67.0) was achieved. Pin infections occurred in four limbs (33.3%) and osteitis in one. A femoral fracture occurred in one patient. Premature removal of the frame was not required in any patient. Sufficient realignment of the leg as well as soft tissue lengthening was achieved in all patients, allowing subsequent reconstruction. All patients were able to mobilize fully weight bearing after reconstruction. Functional outcome was limited in all limbs, and five patients (50.0%) required additional reconstructive operations. Conclusion Regarding the functional results in the treatment of tibial aplasia, amputation remains superior to limb salvage. The latter procedure should only be performed in patients whose parents refuse amputation. Femoro-pedal distraction efficiently prepares the limb by realigning the leg and soft tissue lengthening. Minor complications are frequent, but usually do not hinder the continuation of distraction. Even though a fully weight-bearing limb is achieved, the functional outcome of reconstructive treatment remains limited. Recurrent deformities frequently occur and may require further operations. Cite this article: Bone Joint J 2020;102-B(9):1248–1255.

It is widely accepted that the endocannabinoid system (ECS) is a modulator of neuroinflammation associated with neurodegenerative disorders, including Alzheimer's disease (AD). Thus, expression of the cannabinoid receptor 2 (CB2) is induced in plaque-associated microglia and astrocytes in brain tissues from AD patients and in genetic mouse models expressing pathogenic variants of the amyloid precursor protein (APP). However, the exact mechanism of CB2 signaling in this mouse model remains elusive, because the genetic deletion of CB2 and the pharmacological activation of CB2 both reduced neuroinflammation. Here, we demonstrate that CB2 deletion also improved cognitive and learning deficits in APP/PS1*CB2-/- mice. This was accompanied by reduced neuronal loss and decreased plaque levels and coincided with increased expression of Aβ degrading enzymes. Interestingly, plaque-associated microglia in APP/PS1*CB2-/- mice showed a less activated morphology, while plaques were smaller and more condensed than in APP/PS1 mice. Taken together, these results indicate a beneficial effect of CB2-deficiency in APP transgenic mice. CB2 appears to be part of a protective system that may be detrimental when engaged continuously.