BACKGROUND:Dynamic valgus of the ankle and subtalar joint occurs in patients with fibular hemimelia and a hypoplastic distal fibula. The purpose of this study is to relate clinical dynamic valgus to radiographic measurements and to radiographically evaluate the shortening osteotomy for realignment of the distal tibia (ShORDT) for treatment of this condition. METHODS:A retrospective study was conducted on patients with fibular hemimelia who underwent the ShORDT procedure. Radiographic measurements included: lateral distal tibial angle (LDTA), talocrural angle (TCA), anterior distal tibial angle (ADTA), and distal fibular station (DFS). Logistic regression was performed to determine if any measurements could predict dynamic valgus. Measurements were compared at different time intervals. Linear regression was performed to determine if age, follow-up duration, or a synostosis/tether were related to changes in DFS following surgery. RESULTS:Twenty patients met the inclusion criteria (9 females/11 males). The mean age at surgery was 6.9 years (±3.5 y) with a mean follow-up of 4.0 years (±1.3 y). Logistic regression found DFS ( P <0.001) to be predictive of dynamic valgus. DFS demonstrated a significant improvement from preoperative to postoperative measurements ( P <0.001), recurrence from postoperative to final ( P <0.001), and no difference between the preoperative and final values ( P =1.00). Linear regression found that a synostosis/tether ( P <0.001) and follow-up duration ( P =0.02) were predictive of change in DFS postoperatively. CONCLUSIONS:DFS is a reproducible radiographic measurement in patients with fibular hemimelia to radiographically confirm dynamic valgus. In general, the deformity correction achieved recurs in the growing patient. Use of a solid tether or synostosis between the tibia and fibula may mitigate the recurrence. LEVEL OF EVIDENCE:Level III-Retrospective cohort study.

This is a study of the relationship between the segmental bone lengths of foot height, tibia length, femur length, and upper body length to determine whether they follow a pattern that is consistent with the Lucas sequence.

A pre-meeting workshop on Long Bone Pathology in Children with Achondroplasia was held in Salzburg, Austria at the 11th International Conference on Children's Bone Health (ICCBH) 22-25 June 2024. There remains poor understanding and awareness amongst physicians managing achondroplasia of the underlying pathophysiology, radiology, natural history and orthopaedic procedures available for long bone deformities and restrictions. The structure of the workshop consisted of presentation of the results of a multinational patient survey on views of leg lengthening in achondroplasia, lectures, a debate and an interactive round table discussion. In total 150 attendees from 71 different cities and 31 countries were in attendance.

This is a retrospective chart and radiographic review of 145 patients who underwent full-body EOS imaging; 109 males and 36 females. The mean ages of the female and male subsets are 28.8 (SD = 11.6) years and 29.5 (SD = 11.8) years, respectively. The sum of the foot height (Ft) and the tibial length (T) for each subject was compared to their femur length (Fe). Subsequently, the sum of the tibial (T) and femoral lengths (Fe) were compared to their respective upper body lengths (UB), as measured from the tops of the femoral heads. A linear regression test was performed to determine whether a Lucas sequence-based relationship exists between Ft + T and Fe, and between T + Fe and UB. The regression for the relationship between Ft + T and Fe for the entire cohort (R = 0.82, R2 = 0.70), the female subset (R = 0.94, R2 = 0.88) and the male subset (R = 0.75, R2 = 0.57), all demonstrated a strong positive correlation between Ft + T and Fe and showed that Ft + T is a likely predictor of Fe. The regression test for the entire cohort demonstrated a moderately positive correlation between T + Fe and UB (R = 0.41, R2 = 0.17, F(1, 145) = 29.42, p = 2.4E-07). A stronger correlation was found for the relationship between T + Fe and UB (R = 0.57, R2 = 0.32, F(1, 35) = 16.64, p = 2.5E-05) for the female subset relative to the male subset (R = 0.20, R2 = 0.038, F(1, 35) = 4.37, p = 0.04). There appears to be a Lucas sequence relationship between the lengths of the foot height, tibial length, femoral length and upper body length, which together make up standing height. This mathematical proportion relationship is stronger in females than males.

Lower extremity rotational alignment must be quantified in cases of recurrent patellofemoral instability. Femoral and tibial rotation can be measured with both physical examination and radiographic studies. Derotational osteotomies of both the femur and the tibia can be used to correct patients with excessive femoral anteversion and tibial external rotation (miserable malalignment syndrome). In this article, we attempt to provide guidelines for assessing lower extremity rotation and an understanding of normal femoral and tibial rotational values. We then provide examples of one type of corrective femoral osteotomy and one type of tibial rotational osteotomy. (C) 2019 Elsevier Inc. All rights reserved.

Torsional malalignment of the legs is common in children, and those that do not remodel may benefit from surgical correction. Traditionally, this is corrected with an open osteotomy. Guided growth is the gold standard for minimally invasive angular correction and has been investigated for use in torsional deformities. This study presents our preliminary results of rotationally guided growth in the femur and tibia using a novel technique of peripheral flexible tethers. A total of 8 bones in 5 patients were treated with flexible tethers consisting of separated halves of a hinge plate (Orthopediatrics Pega Medical, Montreal, QC, Canada), which were fixed to the epiphysis and metaphysis at 45° angles to the physis and connected with Fibertape (Arthrex, Naples, FL, USA). The implants are placed medially and laterally in the opposite 45° inclination, determined by the desired direction of rotation. Additionally, the average treatment time was 12 months. All patients corrected the rotational malalignment by clinical evaluation. The average rotational change was 30° in the femurs and 9.5° in the tibias. Further, the average follow-up was 18 months, with no recurrence of the rotational deformity. There was no change in longitudinal growth in the patients who underwent bilateral treatment. Rotational guided growth with flexible tether devices is a novel technique that successfully corrects torsional malalignment without invasive osteotomy surgery.

Background: Congenital synostosis of the knee is a rare condition with limited data on treatment options and outcomes. This study reports clinical findings, treatment approach, and surgical/clinical outcomes for congenital synostosis of the knee. Methods: An institutional review board-approved retrospective review of patients with congenital synostosis of the knee presenting to 2 institutions between 1997 and 2021 was performed. Results: Eight patients (13 knees) with a median follow-up of 11.3 years (3.3 to 17 y) were included. Seven patients had associated syndromes. Patients presented with an average knee flexion deformity of 100° (range 60 to 130°) and delayed walking ability. Seven patients had associated upper extremity hypoplasia/phocomelia. The average age at the index surgery was 4.3 years (range 1.2 to 9.2 y). Synostosis resection with gradual deformity correction was performed in most patients. An attempt was made at a mobile knee in some patients, but all went on to knee fusion. Mean flexion deformity at final follow-up was 11.6° (range: 0 to 40°) and 5 limbs were fused in full extension. Mean limb length discrepancy at final follow-up was 6.8 cm (range: 0 to 8 cm). All patients maintained their improved ambulation status at final follow-up. Twenty-two complications were identified. Conclusions: Reliable correction of the deformity associated with congenital knee synostosis was achieved at a median follow-up of 11 years. Importantly, all patients maintained their improved ambulation at final follow-up. This is the largest study on patients with congenital knee synostosis and outlines a reconstructive approach to improve ambulatory status. Level of Evidence: Level IV

Valgus lower extremity alignment with excessive femoral anteversion and tibial external torsion (miserable malalignment syndrome) results in patellar maltracking. Sagittal plane deformities (rotational alignment) should be quantified along with typical frontal plane alignment. Femoral and tibial rotation can be assessed with both physical examination and radiographic studies. Derotational osteotomies of both the femur and the tibia can then be used for correction. In this chapter, we attempt to provide guidelines for assessing lower extremity rotation and an understanding of normal femoral and tibial rotational values. We then provide examples of corrective femoral osteotomies and tibial rotational osteotomies. Oper Tech Sports Med 31:151038 (c) 2023 Published by Elsevier Inc.

This study aims to develop multipliers for the spine and sitting height to predict sitting height at maturity. With the aid of longitudinal and cross-sectional clinical databases, we divided the total sitting height, cervical, thoracic, and lumbar lengths at skeletal maturity by these same four factors at each age for each percentile given. A series of comparisons were then carried out between the multipliers as well as the percentiles and the varied racial and ethnic groups within them. Regarding sitting height, there was little variability and correlated with the multipliers calculated for the thoracic and lumbar spine. The multiplier method has demonstrated accuracy that is not influenced by generation, percentile, race, and ethnicity. This multiplier can be used to anticipate mature sitting height, the heights of the thoracic, cervical, and lumbar spine, as well as the lack of spinal growth after spinal fusion surgery in skeletally immature individuals.

Abstract Background Great difficulty and more failures were the descriptions of the treatment of congenital patella dislocation in pediatric patients. This study aims to evaluate the outcomes of patients with congenital patellar dislocations treated with the modified Langenskiöld procedure. Methods The medical records of 16 knees in 11 patients with a diagnosis of congenital patella dislocation were collected from September 2016 to March 2019. They were treated with the modified Langenskiöld procedure. The mean follow-up period was 37.8 months. The outcome measures were the Lysholm score, Kujala score, patellar stability, and knee range of motion. Results Eleven patients, namely, eight girls and three boys, with 16 knees were enrolled. The mean age at the time of operation was 3.1 years. The post-operative mean Lysholm score was 94.8 (SD 5.1; 87–100), whereas the Kujala score was 95 (SD 5.9; 86–100). There were no recurrent dislocations, and all patients had full extension postoperatively. Conclusion The modified Langenskiöld procedure is a promising solution for the treatment of congenital patella dislocations. Level of evidence Level IV; Case Series; Treatment Study.

We reviewed 18 limbs in 17 patients who underwent ankle fusion with simultaneous tibial lengthening with a magnetic internal lengthening nail. All patients had preoperative limb length discrepancy (LLD) (mean 4.9 cm (2.6-7.6 cm)) with ankle deformity. The ankle was fused from medial or lateral approaches using screws/plate constructs placed adjacent to the retrograde Precise nail. Lengthening was carried out by a distal 1/3 tibial osteotomy. Clinical and radiographic measures were performed after a mean follow-up of 20 months (12-37 months). The mean amount of lengthening performed was 4 cm (1.8-7.2 cm). The final mean LLD was 1 cm (0.7-1.1 cm), which was statistically significant (p<0.01) as compared to preoperative. The foot was plantigrade in all cases. The mean foot rotation was 10° (5-15°) external, relative to the knee. At final follow-up all patients reported minimal to no pain, and all claimed to be walking more functionally than before surgery. Ankle fusion and limb lengthening was achieved in all cases. Combining both treatments by using an internal lengthening nail was very effective and avoided leaving patients with a dysfunctional LLD or of having a separate limb lengthening procedure. This is the first report of such a combined treatment of ankle fusion with internal tibial lengthening nail.

BACKGROUND:The treatment of congenital pseudarthrosis of the tibia (CPT) remains a challenge because of the difficulties of achieving and maintaining bone union, as well as complications of joint deformity and limb-length discrepancy. The purpose of this study was to evaluate the efficacy of cross-union of the tibia and fibula in achieving union and preventing refracture for patients with refractory CPT as a complementary approach to improve upon conventional surgical treatments.METHODS:A retrospective study including patients with refractory CPT who attended our department between June 2014 and August 2020. Eighteen CPT patients, who had sustained refracture that required cast immobilization or secondary surgery, and were managed by pseudarthrosis resection, cross-union of the tibia and fibula, bone morphogenetic protein-2 and autogenous iliac bone grafting, were included. Clinical outcomes of the bone union rate and the frequency of refracture after performing cross-union of the tibia and fibula were assessed during the follow-up period.RESULTS:The mean follow-up period was 4.3 years (range: 1.5 to 6.25 y). The mean age of the patients at surgery was 5.4 years (range: 2.6 to 10 y), and all 18 (100%) of the 18 patients had final healing at the site of pseudarthrosis. The average time spent to achieve radiologic bone union of the pseudarthrosis after operation was 2.96 months (range: 2.2 to 4.1 mo). Two (11.1%) patients had an average 2.5 cm limb-length discrepancy, none (0%) sustained refracture which needed cast immobilization or secondary surgery. Patients were all pain-free and move actively.CONCLUSIONS:Cross-union of the tibia and fibula is a promising complementary procedure for treating refractory CPT patients.LEVEL OF EVIDENCE:Level IV-case series.

Congenital femoral deficiency (CFD) Paley type 1b is characterized by severe bony deformity of the upper femur, extra-articular contractures of the hip, and, delayed ossification of the femoral neck and/or subtrochanteric region. The Systematic Utilitarian Procedure for Extremity Reconstruction of the hip (SUPERhip) procedure for the correction of CFD deformities was developed in 1997. Initially, a non-fixed angle device (rush rod) was used for fixation. Late complications of persistent delayed ossification and recurrent varus deformity occurred. In order to reduce and treat such complications, fixation with a fixed angle device and the off-label use of BMP2 to induce ossification of the un-ossified femoral neck were employed. The purpose of this study is to determine if the use of a fixed angle device, and, BMP2 inserted into a drill hole in the cartilage of the femoral neck, decreases the incidence of these late complications. We retrospectively reviewed 72 SUPERhip procedures performed for Paley type 1b CFD between 1997 and 2012. Due to recurrent varus or persistent delayed ossification of the femoral neck, 34 revision SUPERhip procedures were performed. In total, 106 SUPERhip procedures were studied. Sixty-eight SUPERhips were performed using internal fixation without BMP2, while 38 SUPERhips were performed with both internal fixation and the addition of BMP2. Forty-one were performed using non-fixed angle internal fixation while 65 had fixed angle internal fixation. Fixed angle devices significantly reduced the incidence of recurrent varus compared with non-fixed angle devices. Inserting BMP2 in the femoral neck significantly reduced the incidence of persistent delayed ossification. Using only a fixed angle device but no BMP2 did not reduce the incidence of delayed ossification. The combination of both a fixed angle device and BMP2 reduced the incidence of recurrent coxa vara and persistent delayed ossification of the femoral neck. The SUPERhip procedure corrects the pathoanatomy of the proximal femur in CFD Paley type 1b but is associated with a very high risk of recurrence of coxa vara and persistence of femoral neck delayed ossification, unless, a fixed angle internal fixation device is used to prevent recurrent coxa vara and BMP2 is used to induce ossification of the femoral neck.

American Journal of Medical Genetics Part AVolume 188, Issue 3 p. 1000-1004 RESEARCH LETTER Tibia hemimelia in a patient with CHARGE syndrome: A rare but recurrent phenomenon Sietse M. Aukema, Corresponding Author Sietse M. Aukema sietse.aukema@mumc.nl smaukema@gmail.com Department of Clinical Genetics, Maastricht University Medical Center, Maastricht, The Netherlands Correspondence Sietse M. Aukema, Department of Clinical Genetics, Maastricht University Medical Center, Maastricht, the Netherlands. Email: sietse.aukema@mumc.nl; smaukema@gmail.com Constance T. R. M. Stumpel, Department of Clinical Genetics and GROW-School for Oncology & Developmental Biology, Maastricht University Medical Center+, Maastricht, The Netherlands. Email: c.stumpel@outlook.comSearch for more papers by this authorChrista M. de Geus, Christa M. de Geus Department of Clinical Genetics, University of Groningen, University Medical Center Groningen, Groningen, The NetherlandsSearch for more papers by this authorSimon G. F. Robben, Simon G. F. Robben Department of Radiology, Maastricht University Medical Center, Maastricht, The NetherlandsSearch for more papers by this authorKim J. A. F. van Kaam, Kim J. A. F. van Kaam Department of Clinical Genetics, Maastricht University Medical Center, Maastricht, The NetherlandsSearch for more papers by this authorHeleen M. Staal, Heleen M. Staal Department of Orthopaedics, Maastricht University Medical Centre, Maastricht, The NetherlandsSearch for more papers by this authorAdhiambo M. Witlox, Adhiambo M. Witlox Department of Orthopaedics, Maastricht University Medical Centre, Maastricht, The NetherlandsSearch for more papers by this authorNicole A. J. de la Haye, Nicole A. J. de la Haye Department of Pediatrics, Maastricht University Medical Centre (MUMC+), Maastricht, The NetherlandsSearch for more papers by this authorMerel Klaassens, Merel Klaassens Department of Pediatrics, Maastricht University Medical Centre (MUMC+), Maastricht, The NetherlandsSearch for more papers by this authorAudrey Coumans, Audrey Coumans Department of Obstetrics and Gynaecology, Maastricht University Medical Center, Maastricht, The NetherlandsSearch for more papers by this authorAlexander P. A. Stegmann, Alexander P. A. Stegmann orcid.org/0000-0002-9736-7137 Department of Clinical Genetics and GROW-School for Oncology & Developmental Biology, Maastricht University Medical Center+, Maastricht, The NetherlandsSearch for more papers by this authorDror Paley, Dror Paley Paley Orthopedic and Spine Institute, West Palm Beach, Florida, USASearch for more papers by this authorConstance T. R. M. Stumpel, Corresponding Author Constance T. R. M. Stumpel c.stumpel@outlook.com Department of Clinical Genetics and GROW-School for Oncology & Developmental Biology, Maastricht University Medical Center+, Maastricht, The Netherlands Correspondence Sietse M. Aukema, Department of Clinical Genetics, Maastricht University Medical Center, Maastricht, the Netherlands. Email: sietse.aukema@mumc.nl; smaukema@gmail.com Constance T. R. M. Stumpel, Department of Clinical Genetics and GROW-School for Oncology & Developmental Biology, Maastricht University Medical Center+, Maastricht, The Netherlands. Email: c.stumpel@outlook.comSearch for more papers by this author Sietse M. Aukema, Corresponding Author Sietse M. Aukema sietse.aukema@mumc.nl smaukema@gmail.com Department of Clinical Genetics, Maastricht University Medical Center, Maastricht, The Netherlands Correspondence Sietse M. Aukema, Department of Clinical Genetics, Maastricht University Medical Center, Maastricht, the Netherlands. Email: sietse.aukema@mumc.nl; smaukema@gmail.com Constance T. R. M. Stumpel, Department of Clinical Genetics and GROW-School for Oncology & Developmental Biology, Maastricht University Medical Center+, Maastricht, The Netherlands. Email: c.stumpel@outlook.comSearch for more papers by this authorChrista M. de Geus, Christa M. de Geus Department of Clinical Genetics, University of Groningen, University Medical Center Groningen, Groningen, The NetherlandsSearch for more papers by this authorSimon G. F. Robben, Simon G. F. Robben Department of Radiology, Maastricht University Medical Center, Maastricht, The NetherlandsSearch for more papers by this authorKim J. A. F. van Kaam, Kim J. A. F. van Kaam Department of Clinical Genetics, Maastricht University Medical Center, Maastricht, The NetherlandsSearch for more papers by this authorHeleen M. Staal, Heleen M. Staal Department of Orthopaedics, Maastricht University Medical Centre, Maastricht, The NetherlandsSearch for more papers by this authorAdhiambo M. Witlox, Adhiambo M. Witlox Department of Orthopaedics, Maastricht University Medical Centre, Maastricht, The NetherlandsSearch for more papers by this authorNicole A. J. de la Haye, Nicole A. J. de la Haye Department of Pediatrics, Maastricht University Medical Centre (MUMC+), Maastricht, The NetherlandsSearch for more papers by this authorMerel Klaassens, Merel Klaassens Department of Pediatrics, Maastricht University Medical Centre (MUMC+), Maastricht, The NetherlandsSearch for more papers by this authorAudrey Coumans, Audrey Coumans Department of Obstetrics and Gynaecology, Maastricht University Medical Center, Maastricht, The NetherlandsSearch for more papers by this authorAlexander P. A. Stegmann, Alexander P. A. Stegmann orcid.org/0000-0002-9736-7137 Department of Clinical Genetics and GROW-School for Oncology & Developmental Biology, Maastricht University Medical Center+, Maastricht, The NetherlandsSearch for more papers by this authorDror Paley, Dror Paley Paley Orthopedic and Spine Institute, West Palm Beach, Florida, USASearch for more papers by this authorConstance T. R. M. Stumpel, Corresponding Author Constance T. R. M. Stumpel c.stumpel@outlook.com Department of Clinical Genetics and GROW-School for Oncology & Developmental Biology, Maastricht University Medical Center+, Maastricht, The Netherlands Correspondence Sietse M. Aukema, Department of Clinical Genetics, Maastricht University Medical Center, Maastricht, the Netherlands. Email: sietse.aukema@mumc.nl; smaukema@gmail.com Constance T. R. M. Stumpel, Department of Clinical Genetics and GROW-School for Oncology & Developmental Biology, Maastricht University Medical Center+, Maastricht, The Netherlands. Email: c.stumpel@outlook.comSearch for more papers by this author First published: 11 December 2021 https://doi.org/10.1002/ajmg.a.62600Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Volume188, Issue3March 2022Pages 1000-1004 RelatedInformation