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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.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.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.Cross-union of the tibia and fibula is a promising complementary procedure for treating refractory CPT patients.Level IV-case series.

Purpose Langenskiöld described a reconstructive soft-tissue procedure for irreducible lateral congenital patellar dislocations. Paley further detailed the technique in the surgical management of congenital femoral deficiency. The aim of this study was to evaluate the outcomes of patients with congenital, chronic and recurrent patellar dislocations treated with the modified Langenskiöld procedure. Methods This is a retrospective case series. Between 2011 and 2018, 18 knees in 13 patients (mean age 15.8 years (sd 4.4; 12 to 29.9), nine female) with diagnoses of recurrent (six patients, eight knees), chronic (four patients, six knees) and congenital (three patients, four knees) patellar dislocations were treated with the modified Langenskiöld procedure. Results There were no recurrent lateral dislocations in the congenital or recurrent groups. One of the patients in the congenital group had an overcorrection with some medial patellar maltracking but until this time has not required any further surgery. In the chronic group two of the six knees developed further dislocations; these were both on the same patient, who had no dislocations until one year after surgery. Mean Kujala score was 83.7 (sd 17; 47 to 100) for all groups. In spite of preoperative knee flexion contractures of up to 30° in three patients (six knees), all patients had full extension postoperatively. Eight patients reported being satisfied with their outcome, one was somewhat satisfied, two were very dissatisfied, and two did not respond. Conclusion The modified Langenskiöld reconstruction provides a powerful correction for challenging cases of congenital and recurrent patellar dislocations. Re-dislocation as well as overcorrection can occasionally occur. Level of Evidence Level IV

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.

BACKGROUND Multiple hereditary exostoses (MHE) is a rare bone disease that results in growth of benign cartilage-capped tumors and a number of skeletal deformities. Forearm deformities are present in up to 60% of patients with MHE, and radial head subluxation or dislocation occurs in 20% to 30%. Radial head subluxation/dislocation results in a shortened forearm and loss of motion. The purpose of this study was to identify radiographic variables that are most predictive of radial head subluxation/dislocation in an effort to determine the need for prophylactic treatment. METHODS We retrospectively reviewed the cases of consecutive patients with MHE treated in our center between April 2007 and December 2019. Radiographic measurements included the presence or absence of distal ulnar osteochondromas, total ulnar bow, total radial bow, and percent ulnar length. Participants were separated into 3 groups based on the status of the radial head: located, subluxated, and dislocated. Radiographic measurements were compared using a Kruskal-Wallis H test with Dunn post-hoc analysis. A prediction model was run using a binomial logistic regression, and a prediction matrix was created. RESULTS A total of 88 patients were included in the study. There were significant differences in the located group compared with the dislocated group in terms of pronation, supination, and extension. The percent ulnar length, total ulnar bow, and total radial bow differed significantly between the located and dislocated groups (p < 0.0001); however, in the binomial regression analysis, only the percent ulnar length and total ulnar bow could be used to distinguish between the located group and the subluxated/dislocated group. Both of these measurements were significant predictors of subluxation/dislocation. There was no radial head subluxation/dislocation in patients with an ulnar bow of

(1) Background: Patients treated with the two previous generations of ulnarization developed a bump related to the ulnar head becoming prominent on the radial side of the hand. To finally remedy this problem, a third generation of ulnarization was developed to keep the ulnar head contained. While still ulnar to the wrist center, the center of the wrist remains ulnar to the ulnar head, with the ulnar head articulating directly with the trapezoid and when present the trapezium. (2) Methods: Between 2019 and 2021, 22 radial club hands in 17 patients were surgically corrected with this modified version of ulnarization. (3) Results: In all 17 patients, the mean HFA (hand–forearm-angle) correction was 68.5° (range 12.2°–88.7°). The mean ulna growth was 1.3 cm per year (range 0.2–2 cm). There were no recurrent radial deviation deformities more than 15° of the HFA. (4) Conclusions: This new version of ulnarization may solve the problem of the ulna growing past the carpus creating a prominent ulnar bump. The results presented are preliminary but promising. Longer-term follow-up is needed to fully evaluate this procedure.

Rotationplasty is a reconstructive option for severe congenital femoral deficiency (CFD). The senior author (D.P.) developed five new rotationplasty techniques for use in CFD based on the Paley classification, including the Paley–Brown (fusion femur to pelvis), Paley (fusion femur to femoral head), Paley–Winkelman (insertion tibial condyle to acetabulum), PaleySUPERhip–Van Nes (hip osteotomy with knee fusion) and PaleySling–Van Nes (hip reconstruction with knee fusion revision) rotationplasty techniques. The purpose of this study is to retrospectively evaluate the complications, radiographic outcomes and need for secondary surgery in 19 rotationplasty cases performed by the senior author (D.P.) for severe CFD from 2009 to 2019. Rotationplasty comprised only 2% of the authors treated CFD cases during this period. Average age at surgery was 8.6 years old. Average follow-up was 3.3 years. Sixteen concomitant procedures were performed including temporary arthrodesis, tibial osteotomy and SUPERhip procedure. The most common complication was wound necrosis/dehiscence, which occurred in 52% of the cases related to the circumferential incision and required a total of 31 additional debridements. Additional complications were successfully treated and included sciatic nerve palsy decompressed by abducting the femur, a tibial delayed union that underwent bone grafting, two distal femur failed epiphysiodesis treated by revision with one osteotomy and a thigh compartment syndrome requiring debridement. Indication specific rotationplasty successfully addresses the severe degree of femoral deficiency, deformity, and discrepancy in patients with CFD, despite high rates of wound complications.

Limb deformity corrective surgery has exceedingly benefited by the introduction of hexapod systems. Although the principles of deformity correction planning still remain the same as with the Ilizarov system, the corrective phase is made easier thanks to the computer-assisted simultaneous correction of deformity in all space planes. In this chapter, a new CORA-centric hexapod system named “Orthex” is described. With due respect to other existing hexapod systems, the “Orthex” fixator is credited by the authors to be more friendly, intuitive, and sophisticated in the software-based analysis of deformity, spatial location of the frame relative to the bone, and deformity correction planning. Indeed, the “Orthex” also allows the user to plan the deformity correction in consecutive phases (in “series” correction) rather than a simultaneous correction of the deformity and overcomes some of the limitations of other hexapod systems such as: frame mounting rules, need to import external data and measurements, and brand-specific nomenclature. Practical applications with clinical case descriptions are reported in the present chapter.

BACKGROUND Congenital femoral deficiency (CFD) is a rare condition that affects the morphology of the hip and surrounding soft tissues. Bony deformity and distorted muscular anatomy are well known, but no studies have described the relationship of the femoral neurovascular (NV) bundle to surgically relevant anatomic landmarks. The authors compared the location of the femoral NV bundle on the affected side in patients with CFD with the unaffected side. The authors hypothesized that the bundle on the pathologic side would be in an abnormal position relative to the unaffected side. METHODS Thirty-three patients diagnosed with unilateral CFD who had undergone preoperative magnetic resonance imaging of the pelvis were included in our study. The authors identified the femoral NV bundle on the axial cuts and measured its distance from the anterior superior iliac spine (ASIS), anterior inferior iliac spine (AIIS), and lesser trochanter (LT). Anatomic percent change and absolute measurements were then compared and correlated with associated boney deformities and the Paley classification. RESULTS The distance from the femoral NV bundle to the ASIS, AIIS, and LT was significantly different compared with the unaffected side. The AIIS absolute distance and AIIS percent change significantly correlated with the neck-shaft angle of the proximal femur. CONCLUSIONS In patients with CFD, the femoral NV bundle seems to be further from the LT and closer to the AIIS on the affected side when compared with the unaffected side. magnetic resonance imaging may be helpful to understand the course of the femoral NV bundle before reconstruction in patients with CFD; however, the authors recommend identification of the femoral NV bundle before transection of the proximal rectus femoris tendon to provide safe surgical care. LEVEL OF EVIDENCE Level IV-case-control study of diagnostic studies.

Unloading of the hip was originally considered important in the treatment of Perthes disease. However, various methods of unloading were unsuccessful in altering the natural history of the disease. This may have been due to the misconception that non-weight bearing and unloading were equivalent. To truly remove the compressive forces from the hip, muscular forces must be neutralized. This can be accomplished by hip joint distraction with an external fixator. It was postulated that if the femoral head could be distracted back into the acetabulum, the epiphyseal cartilage would proliferate to fill the gap between the collapsed femoral head and the acetabulum, and improved range of motion could be expected. Surgical techniques and postoperative management of hip joint distraction for treatment of Perthes disease are described in detail. Clinically, patients who had hip joint distraction were active and had little gait abnormality, pain, or weakness after treatment.

Restoration of sphericity to the femoral head has the best prognosis after Perthes disease, typically accomplished via an osteochondroplasty or femoral head reduction osteotomy (FHRO). Three types of FHRO—Ganz, Paley, and Burian—have been developed due to the different patterns of femoral head deformity necessitating a change in the orientation of the osteotomy. The Ganz-type FHRO is made parallel to the femoral neck, while the Paley type is made perpendicular to the elongation axis of the femoral head, increasing the amount that can safely be reduced and more ideally reshaping the femoral head in cases with elongation not perpendicular to the femoral neck. The Burian FHRO, with its wedge resection, allows the advancement of the medial segment of the femoral head laterally, reducing the risk of a vascular necrosis while being easier to perform when elongation of the femoral head occurs more medially.

Fibular hemimelia (FH) presents with foot and ankle deformity and leg length discrepancy. Many historic reconstructions have resulted in poor outcomes. This report reviews modern classification and reconstruction methods. The Paley SHORDT procedure (SHortening Osteotomy Realignment Distal Tibia) is designed to correct dynamic valgus deformity. The Paley SUPERankle procedure (Systematic Utilitarian Procedure for Extremity Reconstruction) is designed to correct fixed equino-valgus foot deformity. The leg length discrepancy in FH is successfully treated with serial lengthening and epiphysiodesis. Implantable intramedullary lengthening devices have led to all internal lengthenings. Recent advancements in techniques and implants in extramedullary implantable limb lengthening (EMILL) have allowed internal lengthenings in younger and smaller patients, who would traditionally require external fixation. These new internal techniques with lengthenings of up to 5 cm can be repeated more easily and frequently than external fixation, reducing the need to achieve larger single-stage lengthenings (e.g., 8 cm). Modern reconstruction methods with lengthening are able to achieve limb length equalization with a plantigrade-stable foot, resulting in excellent functional result comparable or better than a Syme’s amputation with prosthetic fitting.

Success in treatment of congenital pseudarthrosis of the tibia (CPT) is defined as obtaining union without refracture. The success rate of most techniques (rodding, Ilizarov, rodding with Ilizarov, free vascularized fibula, etc.) is on average 50%. Recently a new technique has emerged that improves this success rate to as high as 100% in some reports. This new method involves creating a cross-union between the tibia and fibula. The Paley cross-union protocol starts with preoperative zoledronic acid infusion to protect the tibia, fibula and autogenous bone graft from resorption by the hyperosteoclastic cells in CPT. This is combined by radical circumferential hamartoma resection from tibia and fibula. The bones are fixed internally with telescopic rod and locking plate for the tibia and rodding with a wire for the fibula. Decancellousization, a new method of autogenous bone graft procurement from the ilium makes it possible to harvest enough graft even from the ilium of a 12-month-old child, to fill the interosseous space between the tibia and fibula. Insertion of BMP2 between the graft and the anterior and posterior muscles helps induce osteogenesis by cell recruitment from the muscles and from the bone graft. The cross-union is already bridged by 6 weeks in most cases and the CPT is healed by 12 weeks. The results are independent of association with NF1 or fibrous dysplasia, failed prior surgery or age under 3 years. This procedure has many steps and requires meticulous preparation and fixation of the tibia and fibula as well as great skill to harvest and adequate amount of bone graft in a small child. The author's technique has been reproduced by others with equal success.

Tibial hemimelia is a rare congenital deficiency with a wide spectrum of pathology and deformity. This paper aims to give a comprehensive review of tibial hemimelia, with a concise summary of the history, pathology, and clinical findings of tibial hemimelia, while providing treatment recommendations and a review of the current literature. Classifications and surgical treatments are discussed, including amputation, limb reconstruction, and lengthening. Type-specific treatments are also discussed, including staged distraction correction of joint contractures of knee and ankle, Weber patelloplasty, fibular centralization, knee and ankle arthrodesis, implantable articulated distractors, and the role of femoral shortening. Amputation is a simpler and easier solution for many patients; however, reconstruction options continue to evolve, improve, and provide better functional outcomes in many cases. Factors favoring surgical reconstruction include the presence of a knee joint/proximal tibia, and the presence of a patella and quadriceps mechanism.

Introduction This study compared bone transport to acute shortening/lengthening in a series of infected tibial segmental defects from 3 to 10 cm in length. Methods In a retrospective comparative study 42 patients treated for infected tibial non-union with segmental bone loss measuring between 3 and 10 cm were included. Group A was treated with bone transport and Group B with acute shortening/lengthening. All patients were treated by Ilizarov methods for gradual correction as bi-focal or tri-focal treatment; the treating surgeon selected either transport or acute shortening based on clinical considerations. The principle outcome measure was the external fixation index (EFI); secondary outcome measures included functional and bone results, and complication rates. Results The mean size of the bone defect was 7 cm in Group A, and 5.8 cm in Group B. The mean time in external fixation in Group A was 12.5 months, and in Group B was 10.1 months. The external fixation index (EFI) measured 1.8 months/cm in Group A and 1.7 months/cm in Group B (P = 0.09). Minor complications were 1.2 per patient in the transport group and 0.5 per patient in the acute shortening group (P = 0.00002). Major complications were 1.0 per patient in the transport group versus 0.4 per patient in the acute shortening group (P = 0.0003). Complications with permanent residual effects (sequelae) were 0.5 per patient in the transport group versus 0.3 per patient in the acute shortening group (P = 0.28). Conclusions While both techniques demonstrated excellent results, acute shortening/lengthening demonstrated a lower rate of complications and a slightly better radiographic outcome. Bone grafting of the docking site was often required with both procedures. Level of evidence: Level III; Retrospective comparative study

Fibular hemimelia presents with foot deformity and leg length discrepancy. Previous classifications have focused on the degree of fibular deficiency rather than the type of foot deformity. Published methods of surgical reconstruction have often failed due to residual or recurrent foot deformity. The purpose of this report is to introduce new classification and reconstruction methods. The Paley SHORDT procedure is used to stabilize the ankle when there is a hypoplastic distal fibula with a dynamic valgus deformity. It involves shortening and realignment of the distal tibia relative to the fibula. In contrast, the Paley SUPERankle procedure is used when there is a fixed equinovalgus foot deformity. The SUPERankle uses a supramalleolar shortening-realignment osteotomy and/or subtalar osteotomies with anlage resection. Due to the bony instead of soft tissue correction of deformity, residual or recurrent deformity is prevented. Weakening of gastro-soleus and peroneal muscles is avoided by shortening of the tibia instead of tendon lengthening. The limitation of ankle motion is related to ankle dysplasia rather than surgery or lengthening. A plantigrade-stable foot and ankle leads to an excellent functional result comparable or better than a Syme's amputation with prosthetic fitting. Serial lengthening procedures combined with the SHORDT or SUPERankle reconstruction lead to limb length equalization with a plantigrade, painless, functional foot.

Triple pelvic osteotomy (TPO) involves periacetabular osteotomies of the ilium, ischium, and pubis to reorient the acetabulum. This operation is indicated in certain situations for the treatment of developmental dysplasia of the hip, dysplastic neuromuscular hips, and for containment of the femoral head in cases of Legg-Calvé-Perthes disease.This retrospective cohort study compares radiographic outcomes of patients who underwent TPO using two different techniques and describes a novel single-incision direct lateral approach. TPO was performed on 22 patients by the senior author. The first 10 patients underwent TPO through a single-incision anterolateral approach. The last 12 patients underwent TPO using the direct lateral approach. Preoperative and postoperative pelvic radiographs were reviewed for each patient, and the migration index and center-edge angle were recorded.The migration index and center-edge angle were evaluated and were not found to be significantly different between the anterolateral and direct lateral groups. The direct lateral approach is described.The direct lateral approach for TPO is equivalent to the anterolateral approach on radiographic evaluation. Advantages of the direct lateral approach include direct visualization of the ischial osteotomy, effective mobilization of the acetabulum, and safety of the sciatic nerve.

ObjectivesThe purpose of this study was to compare the results and complications of tibial lengthening over an intramedullary nail with treatment using the traditional Ilizarov method.MethodsIn this matched case study, 16 adult patients underwent 19 tibial lengthening over nails (LON) procedures. For the matched case group, 17 patients who underwent 19 Ilizarov tibial lengthenings were retrospectively matched to the LON group.ResultsThe mean external fixation time for the LON group was 2.6 months and for the matched case group was 7.6 months. The mean lengthening amounts for the LON and the matched case groups were 5.2 cm and 4.9 cm, respectively. The radiographic consolidation time in the LON group was 6.6 months and in the matched case group 7.6 months. Using a clinical and radiographic outcome score that was designed for this study, the outcome was determined to be excellent in 17 and good in two patients for the LON group. The outcome was excellent in 14 and good in five patients in the matched case group. The LON group had increased blood loss and increased cost. The LON group had four deep infections; the matched case group did not have any deep infections.ConclusionsThe outcomes in the LON group were comparable with the outcomes in the matched case group. The LON group had a shorter external fixation time but experienced increased blood loss, increased cost, and four cases of deep infection. The advantage of reducing external fixation treatment time may outweigh these disadvantages in patients who have a healthy soft-tissue envelope. Cite this article: J. E. Herzenberg. Tibial lengthening over intramedullary nails: A matched case comparison with Ilizarov tibial lengthening. Bone Joint Res 2016;5:1–10. doi: 10.1302/2046-3758.51.2000577

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.

Stump overgrowth is the main problem of limb amputation in children. Many surgical procedures have been developed to overcome the problem, but all have shown inconsistent results. The only surgical procedure that has been successful in preventing overgrowth is capping of the amputated limb with a cartilaginous cap taken from the amputated limb, usually from the head of fibula. A donor site is not available in revision cases. Iliac crest apophysis transfer was suggested to treat the condition, but has never been previously reported. The purpose of this study is to review the results of iliac crest apophysis transfer to prevent stump overgrowth. Five children with amputation stump overgrowth underwent iliac apophyseal transfer to cap the resection site of the overgrowth. Retrospective review showed that three of the five suffered recurrent stump overgrowth two to four years after the index surgery. All three were revised again. One patient was lost to follow-up. Although many studies showed capping of the stump with cartilaginous cap to be successful in preventing stump overgrowth, iliac crest apophysis transfer was only successful in one of four cases available to follow-up (25%). This is not a reliable enough method to be used routinely.

Femoral head reduction osteotomy (FHRO) is the surgical treatment of choice for a misshapen (nonspherical) and enlarged (coxa magna) femoral head in a spherical acetabulum, most often occurring as a sequela of Legg–Calves–Perthes disease, but also arising secondary to skeletal dysplasia and avascular necrosis. Left untreated, the nonspherical femoral head will cause pain due to femoro-acetabular impingement and subsequent femoro-acetabular cartilage damage and labral tear.

Congenital femoral deficiency (CFD) presents a spectrum of deficiency, deformity and dysplasia of the upper femur, hip joint and acetabulum. The Paley classification divides this spectrum into separate pathoanatomical groups that can be treated using discreetly different operative procedures specific to the pathoanatomy of each type of CFD. The SUPERhip1 procedure is used to reconstruct Paley type 1 CFD cases, while the SUPERhip2 procedure is used to reconstruct Paley type 2 CFD cases.

Fusion of the knee is a singularly difficult operation to accomplish. The indications are usually chronic infection after multiple previous total knee procedures, complicated traumatic injury to the knee leaving no significant surfaces for a replacement procedure, or a tumorous condition. On occasion, there may be an unusual case that is considered for conversion to total knee arthroplasty after a successful fusion. This presents one of the most difficult of all reconstructive procedures for the knee. Infection must be ruled out, the condition of the extensor mechanism must be evaluated, and the status of the skin must be clarified. There are a few papers in the literature that support this approach and report reasonable results.