Lower extremity length discrepancy solution by modified drilling epiphysiodesis and anthropological prediction methods: long-term results.

This study aimed to summarize our experience with modified drilling epiphysiodesis and prediction methods for accurate timing of surgery and to compare the results with those of recent work by other authors. The Macnicol and Gupta modified drilling technique was used. Distal femoral and/or proximal tibial permanent epiphysiodesis was performed in 42 children (21 boys, 21 girls; median age at surgery=12.6 years, age range=9.4-15.4 years) between 2004 and 2016. Based on the groundwork of previous studies, we developed an auxological prediction method. The limb length discrepancy (LLD) was assessed by clinical examination and verified by an x-ray of the hips when standing with blocks under the shorter leg. The predicted shortening was 2.7 cm ± 1.1 cm. The final discrepancy after finishing skeletal growth was 0.5 ± 0.5 cm. In 26 patients (61.9%), equalization of both legs was achieved (0-0.5 cm). In 4 patients (9.5%), the remaining shortening was more than 1.0 cm. Shortening of an initially longer leg occurred in two patients (0.5 cm and 0.6 cm). Failure of growth plate arrest was observed in 1 patient. In another 3 patients, the efficacy of tibial epiphysiodesis was unsatisfactory. Minor complications occurred in 5 cases, but there was no evidence of angular deformity in the frontal and sagittal planes or proximal fibula overgrowth. Three patients (7.1%) returned to the operating room. Compared to published data, more patients achieved complete equalization of leg length. The results of this study verified the accuracy of the auxological prediction algorithm of LLD based on Shapiro's findings. The surgical outcome is comparable between Shapiro patterns 1 and 3. Auxological examination, assessment of bone age, and sexual maturation are crucial tools for accurate timing of surgery.

Retrograde Insertion Approach for Anterior Distal Femoral Hemiepiphysiodesis Procedure: A Case Report.

CASE: A 13-year-old adolescent boy with spastic diplegic cerebral palsy (CP) presented with crouch gait and bilateral knee flexion contractures (KFCs). After failure of conservative treatments, the patient was referred to orthopaedics for surgical intervention. Anterior distal femoral hemiepiphysiodesis (ADFH), using a retrograde approach, and Strayer gastrocnemius recession were performed bilaterally. No complications were reported during surgery/follow-up. Full-active knee extension and improved gait were achieved. CONCLUSION: KFC is prevalent in ambulatory patients with CP, reducing gait function. Retrograde ADFH is an effective surgical intervention to reduce KFC and improve gait, and offers simpler insertion/removal than the traditional antegrade technique.

[Fractures around the knee in children : Epiphysiolysis and physeal fractures of the distal femur and proximal tibia]. / Frakturen rund um das Knie im Wachstumsalter : Epiphysiolysen und epiphysäre Frakturen des distalen Femurs und der proximalen Tibia.

Epiphysiolysis and epiphyseal fractures of the distal femur and proximal tibia are an extremely rare entity, but due to their far-reaching consequences with associated functional restrictions of the knee joint, they must be recognized and treated thoroughly. Complete and correct diagnosis is essential and, diagnostically speaking and in addition to standard x­rays in two planes, the threshold for cross-sectional imaging examination techniques should be low. A conservative treatment attempt is possible for undisplaced fractures, but surgical retention and stabilization using wires and screws is usually indicated. Growth disorders often and inevitably occur after such injuries. Clinical monitoring of complications only ends once growth is complete.

Operative treatment of isolated epiphyseal fracture of the distal fibula: 1 case report and literature review.

Pediatric ankle injuries are common; ankle epiphyseal fractures are also common in children. But isolated distal epiphyseal fibular fractures of the distal fibula are clinically rare. We describe one unusual case of an adolescent with a completely displaced Salter-Harris type II distal fibular epiphyseal fracture. The attempt of closed reduction failed, and the patient required open reduction and internal fixation. The localized periosteum and the superior peroneal retinaculum were avulsed from the distal fibular metaphysis, with the peroneal tendons underneath exposed but no obvious subluxation. To the best of our knowledge, this combination of injuries has not been previously reported.

Endoprosthetic replacement with preservation of the epiphysis for proximal tibial reconstruction after osteosarcoma resection in children: a case report.

BACKGROUND: Limb salvage surgery is an important method for treating malignant tumors of the bone involving the adjacent parts of the major joints in children. This technique allows for preservation of limb function, especially in the lower limb. However, the reconstruction of the proximal end of the tibia after removing the tumor mass with a rational scale to preserve the total knee joint and reduce limb length discrepancy presents a challenge. CASE PRESENTATION: We present a case of osteosarcoma of the proximal tibia. After being treated with an extended tumor resection, the proximal tibia of the child was restructured using endoprosthetic replacement with epiphyseal preservation. This procedure preserves the entire articular surface and growth plate of the knee joint of the affected limb and provides a feasible alternative protocol for retaining the function and growth potential of the affected limb. The patient remained disease-free and normal limb motor function was observed during the 3.5 year follow-up since the initial surgery. CONCLUSIONS: Preservation of the epiphysis enabled our patient to perform better limb function after limb-saving surgery as a result of his undamaged knee joint and minimized limb-length discrepancy. We believe that endoprosthetic replacement with preservation of the epiphysis can provide the best strategy for reconstruction after resection of focal malignant tumors in long bones without epiphytic involvement.

Staples, tension-band plates, and percutaneous epiphysiodesis screws used for leg-length discrepancy treatment: a systematic review and proportional meta-analysis.

BACKGROUND AND PURPOSE: The primary aim of this systematic review and meta-analysis was to evaluate the success rate of 3 different epiphysiodesis techniques with implant usage for the treatment of leg-length discrepancy (LLD) in the pediatric population. The secondary aim was to address effectiveness (final LLD) and the reported complications of staples, tension-band plates (TBP), and percutaneous epiphysiodesis screws (PETS). METHODS: In this systematic review we searched MEDLINE (PubMed), Embase, Cochrane Library, Web of Science and Scopus for studies on skeletally immature patients with LLD treated with epiphysiodesis with an implant. The extracted outcome categories were effectiveness of epiphysiodesis (LLD measurements pre-/postoperatively, successful/unsuccessful) and complications that were graded on severity. RESULTS: 44 studies (2,184 patients) were included. 455 underwent epiphysiodesis with PETS, 578 patients with TBP, and 1,048 with staples. Successful epiphysiodesis was reported in 76% (95% confidence interval [CI] 61-89) with PETS (9 studies), 67% (CI 54-79) with TBP (10 studies), and 51% (CI 28-65) with Blount staples (8 studies). From pooled analysis, the severe complications rate was 7% for PETS, 17% for TBP, and 16% for Blount staples. Angular deformity was reported in 4% after PETS, 10% after TBP, and 17% after Blount staples. CONCLUSION: Our results showed that epiphysiodesis with PETS implants was the most successful technique. PETS had a higher success rate, fewer severe complications, and a lower proportion with angular deformity.

Distal radius fracture osteosynthesis by volar locking plate: influence of epiphyseal screw positioning on risk of loss of reduction.

Ulnar variance is an important radiological parameter for good functional outcome after distal radius fracture osteosynthesis. Secondary loss of reduction due to radial shortening is a common complication after volar locking plate fixation. Some authors recommend beginning by placing the most ulnar epiphyseal screw, ensuring that it is positioned as close as possible to the distal radioulnar and radiocarpal joints. The hypothesis of our study was that the positioning of the ulnar epiphyseal screw relative to the distal radioulnar and radiocarpal joints influences the maintenance of reduction during follow-up. 190 distal radius fractures were treated with volar locking plate fixation and divided into two cohorts: cohort A with <2 mm and cohort B with ≥2 mm loss of ulnar variance. Minimum follow-up was 45 days. The positioning of the most ulnar epiphyseal screw was evaluated using a single variable, the ulno-distal index. Means were compared using t-tests and proportions using chi-squared tests. The alpha risk was set at 5%. The intra- and inter-observer reliability of the ulno-distal index measurement were assessed. Mean ulno-distal index was significantly lower in cohort A at 11.28 mm, compared to 13.33 mm in cohort B; p < 0.0001. Ulno-distal index <12 mm was a significant protective factor: p < 0.0001 and relative risk 0.558. No other intrinsic or extrinsic factors of secondary loss of reduction significantly influenced the risk of ulnar variance alteration. The study confirmed the hypothesis that, in distal radius fracture treated with volar locking plate fixation, the closer the ulnar epiphyseal screw to the distal radioulnar joint and radiocarpal joint, the lower the risk of ulnar variance alteration.

Anterior Distal Femoral Hemiepiphysiodesis Using Coronally Oriented 8-plates for the Correction of Fixed Knee Flexion Deformities in Children-Preliminary Results.

INTRODUCTION: The original technique for guided growth of the distal femur for correction of pediatric fixed knee flexion deformities (FKFDs) involves the utilization of two 8-plates inserted on either side of the trochlear groove, a technique that has been frequently linked to the development of persistent postoperative knee pain and crepitus. The present study aimed to assess the preliminary results of a novel technique where the two 8-plates are fixed in the coronal plane, one on each of the medial and lateral surfaces, so that they occupy the anterior part of the distal femur. METHODS: Our study was a prospective case series that included cases with FKFD of >10 degrees in children with at least 12 months of predicted growth remaining. The preoperative knee flexion contracture angle was documented. The surgical procedure entailed the insertion of 2 coronally oriented 8-plates on the medial and lateral surfaces of the distal femur as anterior as possible to the axis of the femur without encroachment on the trochlear groove. The duration of time required to attain full knee extension and any complications encountered were recorded. Wilcoxon signed-rank was used to compare the preoperative and final contracture angles. The level of statistical significance was set at P <0.05. RESULTS: Thirteen knees in 8 patients (6 boys and 2 girls) were included. The median age was 11 years (6 to 14). There was a significant improvement in the FKFD for the whole cohort from 25 degrees (14 to 42) to 0 degrees (-9 to 8), P <0.05. The median rate of correction was 2.0 degrees/month (0.9 to 5.8). The time till full correction was 14 months (4 to 25). Postoperative knee pain and metalware-related complications were not reported by any patient during follow-up. CONCLUSIONS: Guided growth of the distal femur using coronally oriented 8-plates is an effective procedure for the treatment of FKFDs in children. This modified technique may achieve faster correction while minimizing the risk of postoperative knee pain compared with the conventional technique. LEVEL OF EVIDENCE: Level IV-case series.

Operative Treatment of Distal Tibial Fractures in Children Managed With Epiphyseal Screws: A Comparison of Outcomes When Screws Are Retained Versus Removed Following Fracture Union.

BACKGROUND: Fractures of the distal tibial epiphysis in children are often accompanied by articular surface disruption. With increased displacement, internal fixation is frequently performed with an epiphyseal screw to close the fracture gap. Despite limited, high-level clinical evidence to support implant removal, epiphyseal screws are commonly removed after fracture healing due to potentially increased contact forces on the tibiotalar joint. The purpose of this study was to investigate and compare outcomes and complications in children that underwent surgical treatment of distal tibial epiphyseal fractures with placement of an epiphyseal screw(s) and had the implant(s) retained versus removed at a minimum of 2-year postoperative follow-up. METHODS: Children younger than 18 years from two urban tertiary care centers who underwent operative management of distal tibia Salter-Harris III and IV fractures using epiphyseal screws (2013-2020) were divided into two cohorts: retained epiphyseal screws and implant removed. Demographics, intraoperative, postoperative, and radiographic data were collected. Patient-reported outcomes (PROs) using the Foot and Ankle Ability Measure (FAAM) and Single Assessment Numeric Evaluation (SANE) questionnaires were collected at the final follow-up. Statistical analysis, including power analysis, was performed. RESULTS: Fifty-two children were included (30 males, 22 females) with a mean age of 13.3 years at the time of injury (range, 7.7-16.4 years). Thirty-five children retained the implants; seventeen had implants removed. All completed the FAAM questionnaires at a mean follow-up of 4.4 ± 1.9 years, while 29 completed the SANE questionnaire at a mean follow-up of 4.4 ± 1.7 years. No statistically significant difference in patient demographics, surgical variables, or PROs was observed. Six children experienced complications from the initial surgery, including infections and complex regional pain syndrome, with no difference in complication rates between the cohorts ( P =0.08). Furthermore, no complication was observed as a result of implant removal. CONCLUSIONS: Children with retained epiphyseal implants have similar functional outcomes as compared with those who had implants removed after distal tibial epiphyseal fracture fixation and union. LEVEL OF EVIDENCE: Level III-Retrospective comparative study.

Lateral Femoral Condyle Allograft in the Treatment of Elbow Capitellar Avascular Necrosis: A Case Report.

CASE: A 27-year-old woman developed capitellar osteonecrosis after long-term corticosteroid use to treat non-Hodgkin lymphoma. She underwent an osteochondral reconstruction using a lateral femoral condyle (LFC) allograft. This graft was selected because it has a similar radius of curvature to the capitellum. The patient had osseous integration, painless, near full range of motion of her elbow 6 months postoperatively and good shoulder function 1.0 year postoperatively. CONCLUSION: The LFC allograft should be considered a viable option in treating capitellar osteonecrosis.

Anterior Proximal Tibial Screw Hemiepiphysiodesis Decreases Posterior Tibial Slope in Knee Flexion Contracture: A Case Report.

CASE: A 13-year-old adolescent boy with hemiplegic cerebral palsy suffering from fixed knee flexion deformity of 10° despite extensive conservative treatment. Owing to a posterior tibial slope (PTS) of 16°, anterior hemiepiphysiodesis was applied to the proximal tibia. The 2 screws were removed after 9 months. Final follow-up at 16 months showed complete knee extension and a PTS of 4°. CONCLUSION: The presented technique is a good alternative in knee flexion deformity with an increased PTS and has surprisingly not been described in the literature. This might be worth considering for other pathologies such as pediatric anterior cruciate ligament injury with an increased PTS.

Microwave Ablation of the Pig Growth Plate: Proof of Concept for Minimally Invasive Epiphysiodesis.

BACKGROUND: Different surgical methods for epiphysiodesis of limb length discrepancy (LLD) have been described. Although these methods are variably effective, they are associated with morbidity (pain and limp) and potential complications. Microwave ablation is a less-invasive opportunity to halt growth by selectively destroying the growth plate via thermal energy to treat LLD in children. QUESTIONS/PURPOSES: In this proof-of-concept study using an in vivo pig model, we asked: (1) What is the durability of response 2 to 4 months after microwave ablation of the tibial growth plate as measured by length and angulation of the tibia via a CT scan? (2) Was articular cartilage maintained as measured by standard histologic staining for articular cartilage viability? METHODS: To develop an in vivo protocol for microwave ablation, we placed microwave antennas adjacent to the proximal tibia growth plate in the cadaveric hindlimbs of 18 3-month-old pigs. To determine the suitable time, we varied ablation from 90 to 270 seconds at 65-W power settings. After sectioning the tibia, we visually assessed for discoloration (implying growth plate destruction) that included the central growth plate but did not encroach into the epiphysis in a manner that could disrupt the articular surface. Using this information, we then performed microwave ablation on three live female pigs (3.5 to 4 months old) to evaluate physiologic changes and durability of response. A postprocedure MRI was performed to ensure the intervention led to spatial growth plate alterations similar to that seen in cadavers. This was followed by serial CT, which was used to assess the potential effect on local bone and growth until the animals were euthanized 2 to 4 months after the procedure. We analyzed LLD, angular deformity, and bony deformity using CT scans of both tibias. The visibility of articular cartilage was compared with that of the contralateral tibia via standard histologic staining, and growth rates of the proximal tibial growth plate were compared via fluorochrome labeling. RESULTS: Eighteen cadaveric specimens showed ablation zones across the growth plate without visual damage to the articular surface. The three live pigs did not exhibit changes in gait or require notable pain medication after the procedure. Each animal demonstrated growth plate destruction, expected limb shortening (0.8, 1.2, and 1.5 cm), and bony cavitation around the growth plate. Slight valgus bone angulation (4º, 5º, and 12º) compared with the control tibia was noted. No qualitatively observable articular cartilage damage was encountered from the histologic comparison with the contralateral tibia for articular cartilage thickness and cellular morphology. CONCLUSION: A microwave antenna placed into a pig's proximal tibia growth plate can slow the growth of the tibia without apparent pain and alteration of gait and function. CLINICAL RELEVANCE: Further investigation and refinement of our animal model is ongoing and includes shorter ablation times and comparison of dynamic ablation (moving the antennae during the ablation) as well as static ablation of the tibia from a medial and lateral portal. These refinements and planned comparison with standard mechanical growth arrest in our pig model may lead to a similar approach to ablate growth plates in children with LLD.

Correcting for asymmetry of the proximal tibial epiphysis is warranted to determine postoperative alignment deviations in kinematic alignment from planned alignment of the tibial component on the native tibia.

BACKGROUND: In total knee arthroplasty, unrestricted kinematic alignment aims to restore pre-arthritic lower limb alignment and joint lines. Joint line orientations of the contralateral healthy proximal tibia might be used to evaluate accuracy of tibial component alignment post-operatively if asymmetry is minimal. Our objective was to evaluate left-to-right asymmetry of the proximal tibial epiphysis in posterior tibial slope and varus-valgus orientation as related to unrestricted kinematic alignment principles. METHODS: High resolution CT images (0.5 mm slice thickness) were acquired from bilateral lower limbs of 11 skeletally mature subjects with no skeletal abnormalities. Images were segmented to generate 3D tibia models. Asymmetry was quantified by differences in orientations required to shape-match the proximal epiphysis of the mirror 3D tibia model to the proximal epiphysis of the contralateral 3D tibia model. FINDINGS: Systematic and random differences (i.e. mean ± standard deviation) in tibial slope and varus-valgus orientation were - 0.8° ± 1.2° and - 0.2° ± 0.8°, respectively. Ninety five percent confidence intervals on the means included 0° indicating that systematic differences were minimal. INTERPRETATION: Since random differences due to asymmetry are substantial in relation to random surgical deviations from pre-arthritic joint lines previously reported, post-operative computer tomograms of the contralateral healthy tibia should not be used to directly assess accuracy of tibial component alignment on a group level without correcting for differences in tibial slope and varus-valgus orientation due to asymmetry.

Does the intercondylar approach provide a better outcome for chondroblastoma of the distal femur in skeletally immature patients?

Aims: The epiphyseal approach to a chondroblastoma of the intercondylar notch of a child's distal femur does not provide adequate exposure, thereby necessitating the removal of a substantial amount of unaffected bone to expose the lesion. In this study, we compared the functional outcomes, local recurrence, and surgical complications of treating a chondroblastoma of the distal femoral epiphysis by either an intercondylar or an epiphyseal approach. Methods: A total of 30 children with a chondroblastoma of the distal femur who had been treated by intraregional curettage and bone grafting were retrospectively reviewed. An intercondylar approach was used in 16 patients (group A) and an epiphyseal approach in 14 (group B). Limb function was assessed using the Musculoskeletal Tumor Society (MSTS) scoring system and Sailhan's functional criteria. Results: At final follow-up, the mean MSTS score was 29.1 (SD 0.9) in group A and 26.7 (SD 1.5) in group B (p = 0.006). According to Sailhan's criteria, the knee function was good and fair in 14 (87.5%) and two (12.5%) patients of group A, and eight (57.1%) and six (42.9%) patients of group B, respectively (p = 0.062). The lesion had recurred in one patient (6.2%) in group A and four patients (28.6%) in group B. Limb shortening > 1 cm was recorded in one patient (6.2%) from group A and six patients (42.8%) from group B. Joint degeneration was noted in one patient from group A and three patients from group B. Conclusion: An intercondylar approach to a chondroblastoma of the middle two-quarters of the distal femoral epiphysis results in better outcomes than a medial or lateral epiphyseal approach: specifically, better limb function, a lower rate of recurrence, and a lower rate of physeal damage and joint degeneration.

All-epiphyseal anterior cruciate ligament reconstruction yields superior sports performances than the trans-epiphyseal technique in skeletally immature patients: a systematic review.

BACKGROUND: Anterior cruciate ligament (ACL) tears in skeletally immature patients are increasingly common. Evidence comparing the outcomes of all-epiphyseal versus trans-epiphyseal ACL reconstruction in skeletally immature patients is limited, and the current literature could benefit from a comprehensive systematic review. The present study compared all-epiphyseal versus trans-epiphyseal ACL reconstruction in skeletally immature patients. The outcomes of interest were to compare joint laxity, patient-reported outcome measures (PROMs), return to sport, and complications. METHODS: This study was conducted according to the 2020 Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. In November 2023, the following databases were accessed: PubMed, Web of Science, Google Scholar, and Embase. No additional filters were used in the database search. All the clinical studies investigating ACL reconstruction in skeletally immature patients were accessed. Only articles that clearly stated the surgical technique (all- or trans-epiphyseal) were eligible. Only articles with a minimum of 6 months of follow-up were included. Only articles that clearly stated that surgeries were conducted in children with open physis were eligible. RESULTS: Data from 1489 patients (1493 procedures) were collected, of which 32% (490 of 1489 patients) were female. The mean length of follow-up was 46.6 months. The mean age of the patients was 12.7 years. No difference was found in joint laxity (Table 3): positive pivot shift (P = 0.4), positive Lachman test (P = 0.3), and mean arthrometer laxity (P = 0.1). No difference was found in PROMs (Table 4): International Knee Documentation Committee (IKDC) (P = 0.3), Lysholm (P = 0.4), and Tegner (P = 0.7). The trans-epiphyseal technique was associated with a greater rate of patients unable to return to sports (1% versus 7%, P = 0.0001) and with a longer time to return to sports (7.7 versus 8.6 months, P = 0.01). Though the trans-epiphyseal technique was associated with a lower rate of return to sport, this difference was not statistically significant (P = 0.8). No difference was evidenced in the rate of patients who had reduced their league or level of sports activity (P = 0.6) or in the rate of patients who had returned to their previous league or level of sports activity (P = 0.7). No difference was found in the rate of complication: re-tear (P = 0.8), reoperation (P = 0.7), increased laxity (P = 0.9), and persistent instability sensation (P = 0.3). CONCLUSION: Trans-epiphyseal ACL reconstruction was associated with a greater rate of patients unable to return to sport and with a longer time to return to sport compared with the all-epiphyseal technique in skeletally immature patients. Level of evidence Level III, systematic review.

Factors influencing correction rate following hemiepiphysiodesis using tension band plates for deformities around the knee: a multivariate analysis study.

PURPOSE: The aim of this study was to appraise various factors influencing the correction rate in temporary hemiepiphysiodesis (THE) around the knee joint. Specifically, the study analysed the relationship of correction rate with age, gender, aetiology, type and location of deformity. METHODS: The retrospective study included children who underwent THE for a coronal plane deformity (genu valgus or varum) around the knee joint (distal femur or proximal tibia) over a ten year period (2010-2020). The primary outcome of interest was the correction rate of the deformity. RESULTS: Thirty-three children (27 females and 6 males) with a mean age of 8.1 years involving 86 plates were included in the study. The mean correction achieved was 12.2° over a treatment period of 13.3 months. Subgroup analysis showed significant differences between the type (varus (0.8° per month), valgus (1.1° per month)) and the location of deformity femur (1.2° per month) and tibia (0.7° per month)]. On multivariate analysis, the location and the duration of treatment showed significant associations with the correction rate. CONCLUSION: The correction of coronal deformities following temporary hemiepiphysiodesis is influenced by several factors. Valgus, femoral and deformities in younger children correct at a faster rate. Location of deformity and duration of treatment emerged as potential factors affecting the correction rate.

Natural history of Wolcott-Rallison syndrome: A systematic review and follow-up study.

BACKGROUND AND AIMS: To systematically review the literature for reports on Wolcott-Rallison syndrome, focusing on the spectrum and natural history, genotype-phenotype correlations, patient and native liver survival, and long-term outcomes. METHODS: PubMed, Livio, Google Scholar, Scopus and Web of Science databases were searched. Data on genotype, phenotype, therapy, cause of death and follow-up were extracted. Survival and correlation analyses were performed. RESULTS: Sixty-two studies with 159 patients met the inclusion criteria and additional 30 WRS individuals were collected by personal contact. The median age of presentation was 2.5 months (IQR 2) and of death was 36 months (IQR 50.75). The most frequent clinical feature was neonatal diabetes in all patients, followed by liver impairment in 73%, impaired growth in 72%, skeletal abnormalities in 59.8%, the nervous system in 37.6%, the kidney in 35.4%, insufficient haematopoiesis in 34.4%, hypothyroidism in 14.8% and exocrine pancreas insufficiency in 10.6%. Episodes of acute liver failure were frequently reported. Liver transplantation was performed in six, combined liver-pancreas in one and combined liver-pancreas-kidney transplantation in two individuals. Patient survival was significantly better in the transplant cohort (p = .0057). One-, five- and ten-year patient survival rates were 89.4%, 65.5% and 53.1%, respectively. Liver failure was reported as the leading cause of death in 17.9% of cases. Overall survival was better in individuals with missense mutations (p = .013). CONCLUSION: Wolcott-Rallison syndrome has variable clinical courses. Overall survival is better in individuals with missense mutations. Liver- or multi-organ transplantation is a feasible treatment option to improve survival.

Epiphyseal-entry cannulated screws for temporary guided growth of the knees: a retrospective review of 89 cases.

Guided growth affects the physis in children to produce a desired effect. Several devices achieve alteration of growth, including staples, plates, and screws. Complications can include device failures, failure to modulate growth as expected, and unintended physeal arrest. We present the results of a unique technique designed to minimize these complications. This was a retrospective review of guided growth at the knee at a single institution utilizing cannulated screws with epiphyseal-entry points. Each case was reviewed to determine the presence of complications related to guided growth, including implant breakage, implant pull-out or pull-through, iatrogenic physeal arrest, failure to modulate growth, and the incidence of revision surgeries. There were 89 patients who had 146 epiphyseal-entry guided growth procedures with a total of 221 4.5 mm cannulated screws. There were no iatrogenic physeal arrests. Five (2.26%) screws in 4 (4.49%) patients had either a broken screw or screw pull-out/pull-through requiring revision procedures. Three patients had osteotomies after skeletal maturity. Four had revision-guided growth for other reasons: 2 due to a lack of timely follow-up, 1 for iatrogenic genu varum without implant failure, and 1 due to recurrent deformity after implant removal. Revision procedures were unanticipated in 6 (6.74%) patients. This study describes a technique for placing cannulated screws at the knee with epiphyseal starting points. Our rate of complications and number of revision surgeries compare favorably with those noted for other techniques. Guided growth using epiphyseal-entry cannulated screws is a safe, effective option for most patients.

Physeal Migration During Knee-guided Growth With Tension Band Plates: Influence of Implant Position.

OBJECTIVE: Physeal migration during guided growth with tension band plates (TBPs) has been poorly described. The positioning factors associated with this phenomenon and its clinical implications are unknown. Our aim is to determine the influence of implant position on the risk of physeal migration during knee-guided growth with TBP. METHODS: Retrospective study of 491 patients who underwent temporary hemi or epiphysiodesis with TBP around the knee between 2007 and 2019. We identified 29 patients who presented physeal migration during follow-up. Demographic and clinical data were collected, and the following measures were obtained from the immediate postoperative radiographs: epiphyseal screw base-physis distance/epiphyseal screw tip-physis distance, interscrew angle, epiphyseal screw-physis angle(ES-PHa)/metaphyseal screw-physis angle, plate-physis angle, epiphyseal screw-plate angle/metaphyseal screw-plate angle, and epiphyseal screw-physis length ratio. Using follow-up radiographs, the type of physeal migration of the epiphyseal screw (touch, occupy, or traverse) and the status of the physis after implant removal (unaltered, physeal bar, and skeletal maturity) were also recorded. A descriptive analysis of the cases and a case-control comparison of imaging studies were performed. RESULTS: The median patient age at intervention was 12.2 years (interquartile range: 11.3 to 14.1), and 76% were males. A statistically significant difference between cases and controls was obtained for epiphyseal screw base-physis distance (3.7 vs 6.3; P = 0.029), epiphyseal screw tip-physis distance (3.6 vs 7.85; P = 0.002), ES-PHa (-0.1 vs 7.45; P = 0.007), and plate-physis angle (85.45 vs 88.60; P = 0.012). In a categorical analysis, a significant difference was found for the ES-PHa categories ( P = 0.002) and for the ES-PHa/metaphyseal screw-physis angle categorical pair ( P = 0.018). In 16, 17, and 12 cases the physis was touched, occupied, or traversed, respectively, although we found no physeal alterations after plate removal. CONCLUSIONS: In our study, physeal migration of TBP is not an uncommon phenomenon, although no physeal abnormalities were detected. Convergent placement of the epiphyseal screw with the base or tip close to the physis should be avoided as this position is associated with a higher risk of physeal migration. LEVEL OF EVIDENCE: Level III-case-control study.

The transphyseal osteotomy for the slipped proximal tibial epiphysis in tibia vara.

This study aimed to describe a novel transphyseal osteotomy (TPO) for acute deformity correction in children with bilateral tibia vara and the atraumatic 'slipped proximal tibial epiphysis' (SPTE) entity. We described the clinical and radiological findings in five children (10 limbs) with tibia vara that were treated with the TPO. The criteria for the SPTE were met in nine (9/10) cases. The surgical technique and short-term results of the TPO are reported. The median age was 9 years (range, 6-9), with obesity (BMI > 95th centile) present in all children. The medial tibial plateau was not significantly depressed (the median angle of depression of the medial plateau measured 30° (range, 20°-32°). The mean medial proximal tibial angle of 33° (range, 8°-71°) was corrected to 82° (range, 77°-86°), the mean anatomic posterior proximal tibial angle of 48° (range, 32°-70°) was corrected to 72° (range, 61°-86°), and the median internal tibial rotation of 45° (range, 20°-50° internal rotation) was corrected to neutral rotation (range, 10° internal-10° external rotation). There were two complications: one case of recurrent deformity and one case of intra-articular extension of the osteotomy. We describe a novel TPO that aims to simultaneously correct all aspects of the deformity, stabilise the physis, and prevent recurrence through epiphysiodesis. Further research is required to determine its efficacy and safety. The atraumatic SPTE appears to represent a specific morphological presentation in tibia vara. Level of evidence: 4.