Abstract: Treatment of congenital pseudarthrosis of the tibia remains a major challenge in pediatric orthopedics. Ideal timing and preference of surgical procedures are discussed controversially. A variety of reconstructive treatment strategies have been described in literature, but so far none has proven its superiority. The aim of treatment is to obtain long-term bone union, to prevent refracture, and to correct angular deformities and leg length discrepancies. This study retrospectively evaluates the outcome of different reconstructive strategies. Sixty-nine patients were identified who presented to our outpatient department between 1997 and 2019. Twenty-six of these patients underwent reconstructive surgical treatment and were included in this study. The study cohort was divided into three groups. Excision of the pseudarthrosis was performed in all patients in Group A and B, and in two patients of Group C. Group A (six/26 patients) received subsequent bone transport through external fixation maintaining original length. In Group B (15/26 patients), patients underwent either previous, simultaneous, or subsequent extrafocal lengthening through external fixation to reconstitute length. In Group C (five/26 patients), internal fixation with intramedullary nails was applied. Radiological and clinical evaluation was performed to assess bone union and complication rates. Results varied considerably between the study groups. Overall, the primary bone fusion rate was 69.2%. There were four refractures, all occurring in Group B. The long-term bone union rate without refracture was 53.8%. The overall complication rate was 53.8% and 23.1% showed persistent pseudarthrosis. Two secondary amputations were performed due to failed bone fusion. In conclusion, excision of the pseudarthrosis and extrafocal lengthening achieves a satisfying bone union rate and limb reconstruction, while bone transport does not offer significant advantages but shows higher complication rates. Intramedullary stabilization should be applied to maintain bone union, but shows lower bone union rates when used as a stand-alone treatment regimen. Regardless of the primary bone fusion rates, the probability of long-term bone union remains unpredictable.

Abstract: 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.

Abstract: Many 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? Methods Between 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. Results On 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. Conclusion Most 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 Evidence Level IV, therapeutic study.

Abstract: 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.

Abstract: Background and purpose: For correction of leg-length discrepancy or angular deformity of the lower limb in skeletally immature patients temporary or permanent (hemi-)epiphysiodesis can be employed. These are reliable treatments with few complications. Recently, radiographic analysis of treatment-related alterations of the central knee anatomy gained interest among pediatric orthopedic surgeons. To date the comparison and adequate interpretation of potential changes of the central knee anatomy is limited due to the lack of defined standardized radiographic references. We aimed to establish new reference values of the central knee anatomy.Patients and methods: A retrospective analysis of calibrated longstanding anteroposterior radiographs of 254 skeletally immature patients with a chronological age ranging from 8 to 16 years was conducted. The following radiographic parameters were assessed: (1) femoral floor angle, (2) tibial roof angle, (3) width at femoral physis, and (4) femoral notch–intercondylar distance.Results: All observed radiographic parameters were normally distributed with a mean age of 12.4 years (standard deviation [SD] 2, 95% confidence interval [CI] 12.2–12.6). Mean femoral floor angle was 142° (SD 6, CI 141.8–142.9), mean tibial roof angle was 144° (SD 5, CI 143.7–144.1), mean width at femoral physis was 73 mm (SD 6, CI 72.8–73.9), and mean femoral notch–intercondylar distance was 8 mm (SD 1, CI 7.5–7.7). The estimated intraclass correlation coefficient values were excellent for all measurements.Conclusion: This study provides new radiographic reference values of the central knee anatomy for children between 8 and 16 years and we suggest considering values within the range of 2 SD as the physiological range.

Abstract: Temporary hemiepiphysiodesis (HED) is applied to children and adolescents to correct angular deformities (ADs) in long bones through guided growth. Traditional Blount staples or two-hole plates are mainly used for this indication. Despite precise surgical techniques and attentive postoperative follow-up, implant-associated complications are frequently described. To address these pitfalls, a flexible staple was developed to combine the advantages of the established implants. This study provides the first results of guided growth using the new implant and compares these with the established two-hole plates and Blount staples. Methods Between January 2013 and December 2016, 138 patients (22 children, 116 adolescents) with genu valgum or genu varum were treated with 285 flexible staples. The minimum follow-up was 24 months. These results were compared with 98 patients treated with 205 two-hole plates and 92 patients treated with 535 Blount staples. In long-standing anteroposterior radiographs, mechanical axis deviations (MADs) were measured before and during treatment to analyze treatment efficiency. The evaluation of the new flexible staple was performed according to the idea, development, evaluation, assessment, long-term (IDEAL) study framework (Stage 2a). Results Overall, 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. Conclusion The 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.

Abstract: The need for concomitant proximal fibular epiphysiodesis (PFE) when correcting leg length discrepancy (LLD) with temporary proximal tibial epiphysiodesis (PTE) in children is controversially discussed. This single center, retrospective cohort study analyzes proximal fibular growth in patients treated by PTE with and without concomitant PFE. Radiographic measurements were conducted before implantation and at implant removal. The position of the fibular head in relation to the tibia was assessed with recently established radiographic reference values. All patients (n = 58, 19 females) received PTE to treat LLD at a mean age of 12.2 years (range 7 to 15). In 27/58 (47%) concomitant PFE was performed. Mean follow-up was 36.2 months (range 14.2 to 78.0). The position of the proximal fibula at implant removal was within physiological range in 21/26 patients (81%) with PFE and in 21/30 patients (70%) without PFE. Proximal fibular overgrowth newly developed in 2/26 patients (8%) treated with PFE and in 5/30 patients (17%) treated without PFE (p = 0.431). Peroneal nerve injury or discomfort due to proximal fibular overlength was not reported. The position of the proximal fibula should be critically assessed preoperatively under consideration of reference values before PTE. In consequence of this study, the authors do not routinely perform PFE concomitantly with PTE for correction of moderate LLD in children if the proximal fibula is localized within physiological radiographic margins determined by the established reference values.