Special Issue "Limb Lengthening and Reconstruction in Children"

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Surgery".

Deadline for manuscript submissions: closed (15 May 2021).

Special Issue Editor

Prof. Dr. David S. Feldman
E-Mail Website
Guest Editor
Paley Advanced Limb Lengthening Institute, St. Mary's Hospital, West Palm Beach, FL 33407, USA
Interests: deformity correction; limb lengthening; scoliosis; hip and joint preservation; multiple hereditary exostoses; arthrogryposis

Special Issue Information

Dear Colleagues,

In 1741, Nicholas Andry published Orthopediae (Greek for Straightening Children), which became the name of the subspeciality. Deformity correction in children remains one of the most challenging aspects of orthopedics. This symposium will discuss the state of the art of limb lengthening and deformity correction as applied to congenital and developmental deformities, limb length discrepancies and dysplasias of the upper and lower limbs (congenital femoral deficiency (CFD), fibular hemimelia (FH), tibial hemimelia (TH), radial club hand, Blount’s disease, multiple hereditary exostoses (MHE) and olliers), congenital pseudarthosis of the tibia (CPT), and skeletal dysplasias (achondroplasia, hypochondroplasia, pseudoachondroplasia, etc). Many of these conditions, which were previously only treatable by amputation and prosthetic fitting, can now successfully be treated by limb lengthening and reconstruction, employing new surgical reconstructive procedures and advanced limb lengthening technologies (computer dependent external fixators and fully implantable lengthening plates and nails).

Prof. Dr. David S. Feldman
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All papers will be peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 1600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • limb lengthening
  • deformity
  • congenital femoral deficiency
  • fibular hemimelia
  • tibial hemimelia
  • radial club hand
  • multiple hereditary exostoses
  • ollier’s disease
  • blount’s disease
  • achondroplasia
  • pseudoachondroplasia
  • hypochondroplasia
  • dwarfism

Published Papers (11 papers)

Order results
Result details
Select all
Export citation of selected articles as:

Research

Jump to: Review

Article
Radial Club Hand Treated by Paley Ulnarization Generation 3: Is This the New Centralization?
Children 2021, 8(7), 562; https://doi.org/10.3390/children8070562 - 29 Jun 2021
Viewed by 239
Abstract
(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 [...] Read more.
(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. Full article
(This article belongs to the Special Issue Limb Lengthening and Reconstruction in Children)
Show Figures

Figure 1

Article
Cross-Union Surgery for Congenital Pseudarthrosis of the Tibia
Children 2021, 8(7), 547; https://doi.org/10.3390/children8070547 - 24 Jun 2021
Viewed by 363
Abstract
Congenital Pseudoarthrosis of the Tibia (CPT) is a rare condition with a reputation for recurrent fractures and failure to achieve union. A large variety of surgical procedures have been attempted for the treatment of fractured cases of CPT with an average rate of [...] Read more.
Congenital Pseudoarthrosis of the Tibia (CPT) is a rare condition with a reputation for recurrent fractures and failure to achieve union. A large variety of surgical procedures have been attempted for the treatment of fractured cases of CPT with an average rate of union without refracture of only 50%. Intentional cross-union between the tibia and fibula has been reported to improve these results to 100% union with no refractures. This is a retrospective study of 39 cases of CPT in 36 patients treated by the Paley cross-union protocol with internal fixation, bone grafting, zoledronic acid infusion and bone morphogenic protein 2 (BMP2) insertion. All 39 cases of CPT united at the tibia and developed a cross-union to the fibula. Two patients had a persistent fibular pseudarthrosis, one that was later treated at the time of planned rod exchange and one that has remained asymptomatic. There were few postoperative complications. There were no refractures during the up to 7-year follow-up period. The most common problem was the Fassier-Duval (FD) rod pulling through the proximal or distal physis into the metaphysis (66.7%). This did not negatively affect the results and was remedied at the time of the planned rod exchange. The Paley Cross-Union Protocol is very technically demanding, but the results have radically changed the prognosis of this once sinister disease. Full article
(This article belongs to the Special Issue Limb Lengthening and Reconstruction in Children)
Show Figures

Figure 1

Article
Novel Approach to Improving Knee Range of Motion in Arthrogryposis with a New Working Classification
Children 2021, 8(7), 546; https://doi.org/10.3390/children8070546 - 24 Jun 2021
Viewed by 251
Abstract
Arthrogryposis multiplex congenita (AMC) is a rare condition defined as contrac-tures in multiple joints. Surgical interventions for severe knee flexion contractures have included posterior release, distraction and extension with external fixation and distal femoral extension osteotomies. These operations have been able to achieve [...] Read more.
Arthrogryposis multiplex congenita (AMC) is a rare condition defined as contrac-tures in multiple joints. Surgical interventions for severe knee flexion contractures have included posterior release, distraction and extension with external fixation and distal femoral extension osteotomies. These operations have been able to achieve knee extension, but not increase the range of motion. The purpose of this study was to review our experience with peroneal nerve decompression, posterior knee release and proximal femoral shortening. We retrospectively reviewed the medical charts and radiographs of all patients with a diagnosis of arthrogryposis who underwent aforementioned procedure. There were 39 patients with 73 knees included in the analysis with a mean follow-up of 21 months. The mean preoperative arc of motion was 45° and last followup arc of motion was 79° (p < 0.0001). The mean last followup flexion contracture was 8° (p < 0.0001). Additional subanalyses were performed on those with followup greater than 24 months and those with flexion contractures >60°; there were no differences found in these groups. This study demonstrates that it is possible to achieve a functional range of motion of the knees in patients with AMC while improving ambulatory function. Full article
(This article belongs to the Special Issue Limb Lengthening and Reconstruction in Children)
Show Figures

Figure 1

Article
Extensive Limb Lengthening for Achondroplasia and Hypochondroplasia
Children 2021, 8(7), 540; https://doi.org/10.3390/children8070540 - 24 Jun 2021
Viewed by 353
Abstract
Extensive limb lengthening (ELL) was completed in 75 patients: 66 achondroplasia and 9 hypochondroplasia. The average lengthening was 27 cm for achondroplasia (12–40 cm) and 17 cm for hypochondroplasia (range 10–25 cm). There were 48 females and 27 males. Lengthening was done either [...] Read more.
Extensive limb lengthening (ELL) was completed in 75 patients: 66 achondroplasia and 9 hypochondroplasia. The average lengthening was 27 cm for achondroplasia (12–40 cm) and 17 cm for hypochondroplasia (range 10–25 cm). There were 48 females and 27 males. Lengthening was done either by 2-segment (14 patients; both tibias and/or both femurs) or by 4-segment lengthenings (64 patients; both femurs and tibias at the same time). Most patients also had bilateral humeral lengthening. Patients had 2 or 3 lower limb lengthenings and one humeral lengthening. Lengthenings were either juvenile-onset (31), adolescent-onset (38) or adult-onset (6). The average age at final follow-up was 26 years old (range 17–43 years). There were few permanent sequelae of complications. The most serious was one paraparesis. All patients returned to activities of normal living and only one was made worse by the surgery (paraparesis). This is the first study to show that ELL can lead to an increase of height into the normal height range. Previous studies showed mean increases of height of up to 20 cm, while this study consistently showed an average increase of 30 cm (range 15–40 cm) for juvenile-onset and mean increase of 26 cm (range 15–30 cm) for adolescent-onset. This results in low normal height at skeletal maturity for males and females. The adult-onset had a mean increase of 16.8 (range 12–22 cm). This long-term follow-up study shows that ELL can be done safely even with large lengthenings and that 4-segment lengthening may offer advantages over 2-segment lengthening. While all but the more recent cases were performed using external fixation, implantable limb lengthening promises to be an excellent alternative and perhaps an improvement. Full article
(This article belongs to the Special Issue Limb Lengthening and Reconstruction in Children)
Show Figures

Figure 1

Article
Lengthening the Lower Extremities of Children with Ollier’s and Maffucci’s Enchondromatosis Using Implantable Lengthening Nails
Children 2021, 8(6), 502; https://doi.org/10.3390/children8060502 - 14 Jun 2021
Viewed by 430
Abstract
There are multiple forms of enchondromatosis with Ollier’s and Maffucci’s being the most prevalent types. Limb length discrepancy is a common problem in patients with Ollier’s and Maffucci’s enchondromatosis. There are multiple reports about lengthening bones in patients with enchondromatosis using external fixators. [...] Read more.
There are multiple forms of enchondromatosis with Ollier’s and Maffucci’s being the most prevalent types. Limb length discrepancy is a common problem in patients with Ollier’s and Maffucci’s enchondromatosis. There are multiple reports about lengthening bones in patients with enchondromatosis using external fixators. However, there are no case series regarding the use of implantable lengthening technology. The purpose of this paper is to describe our experience with implantable nail lengthening in patients with enchondromatosis. A retrospective chart and radiographic review of patients with enchondromatosis who underwent implantable nail limb lengthening was performed. Seven patients with 14 bony segments were reviewed. A total of 11/14 lengthenings were completed without difficulty. There were no issues in terms of fixation location in patients with Ollier’s disease. One patient with Maffucci’s syndrome experienced migration of the nail during two lengthenings due to a combination of intralesional fixation and preconsolidation. One patient with Ollier’s disease developed a knee extension contracture requiring manipulation under anesthesia. No other complications were recorded. The use of implantable nail lengthening to resolve limb length discrepancies in patients with Ollier’s disease appears to be safe and effective. Full article
(This article belongs to the Special Issue Limb Lengthening and Reconstruction in Children)
Show Figures

Figure 1

Article
Can Adding BMP2 Improve Outcomes in Patients Undergoing the SUPERhip Procedure?
Children 2021, 8(6), 495; https://doi.org/10.3390/children8060495 - 10 Jun 2021
Viewed by 415
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Limb Lengthening and Reconstruction in Children)
Show Figures

Figure 1

Article
Reconstruction of the Hip in Multiple Hereditary Exostoses
Children 2021, 8(6), 490; https://doi.org/10.3390/children8060490 - 08 Jun 2021
Viewed by 373
Abstract
The hip joint involvement in multiple hereditary exostoses (MHE) occurs in 30–90%, causing pain and limitation of motion by femoroacetabular impingement, coxa valga, acetabular dysplasia, hip joint subluxation, and osteoarthritis. The purpose of this study was to investigate the clinical and radiographic outcomes [...] Read more.
The hip joint involvement in multiple hereditary exostoses (MHE) occurs in 30–90%, causing pain and limitation of motion by femoroacetabular impingement, coxa valga, acetabular dysplasia, hip joint subluxation, and osteoarthritis. The purpose of this study was to investigate the clinical and radiographic outcomes of ten hips in seven patients treated by surgical dislocation and corrective osteotomies between 2004 and 2009. Surgical dislocation and excision of the osteochondromas and varus intertrochanteric osteotomies were performed in all cases when the neck–shaft angle was > 150°. Common sites of osteochondromas were medial, posterior, and anterior neck of the femur. Neck–shaft angle of the femur was improved from a mean of 157° to 139°, postoperatively. On an average, the center-edge angle improved from 20° to 30° postoperatively. We believe that Ganz’s safe surgical dislocation technique is the preferred treatment of MHE. This safeguards the circulation of the femoral head and the osteochondromas can be resected under direct vision. It can be combined with additional corrective osteotomies because the hip affected by MHE is frequently associated with dysplastic changes which can result in premature osteoarthritis. Full article
(This article belongs to the Special Issue Limb Lengthening and Reconstruction in Children)
Show Figures

Figure 1

Article
Rotationplasty for Severe Congenital Femoral Deficiency
Children 2021, 8(6), 462; https://doi.org/10.3390/children8060462 - 01 Jun 2021
Viewed by 613
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Limb Lengthening and Reconstruction in Children)
Show Figures

Figure 1

Review

Jump to: Research

Review
Deformity Reconstruction Surgery for Blount’s Disease
Children 2021, 8(7), 566; https://doi.org/10.3390/children8070566 - 30 Jun 2021
Viewed by 468
Abstract
Blount’s disease is an idiopathic developmental abnormality affecting the medial proximal tibia physis resulting in a multi-planar deformity with pronounced tibia varus. A single cause is unknown, and it is currently thought to result from a multifactorial combination of hereditary, mechanical, and developmental [...] Read more.
Blount’s disease is an idiopathic developmental abnormality affecting the medial proximal tibia physis resulting in a multi-planar deformity with pronounced tibia varus. A single cause is unknown, and it is currently thought to result from a multifactorial combination of hereditary, mechanical, and developmental factors. Relationships with vitamin D deficiency, early walking, and obesity have been documented. Regardless of the etiology, the clinical and radiographic findings are consistent within the two main groups. Early-onset Blount’s disease is often bilateral and affects children in the first few years of life. Late-onset Blount’s disease is often unilateral and can be sub-categorized as juvenile tibia vara (ages 4–10), and adolescent tibia vara (ages 11 and older). Early-onset Blount’s disease progresses to more severe deformities, including depression of the medial tibial plateau. Additional deformities in both groups include proximal tibial procurvatum, internal tibial torsion, and limb length discrepancy. Compensatory deformities in the distal femur and distal tibia may occur. When non-operative treatment fails the deformities progress through skeletal maturity and can result in pain, gait abnormalities, premature medial compartment knee arthritis, and limb length discrepancy. Surgical options depend on the patient’s age, weight, extent of physeal involvement, severity, and number of deformities. They include growth modulation procedures such as guided growth for gradual correction with hemi-epiphysiodesis and physeal closure to prevent recurrence and equalize limb lengths, physeal bar resection, physeal distraction, osteotomies with acute correction and stabilization, gradual correction with multi-planar dynamic external fixation, and various combinations of all modalities. The goals of surgery are to restore normal joint and limb alignment, equalize limb lengths at skeletal maturity, and prevent recurrence. The purpose of this literature review is to delineate basic concepts and reconstructive surgical treatment strategies for patients with Blount’s disease. Full article
(This article belongs to the Special Issue Limb Lengthening and Reconstruction in Children)
Show Figures

Figure 1

Review
Lengthening Reconstruction Surgery for Fibular Hemimelia: A Review
Children 2021, 8(6), 467; https://doi.org/10.3390/children8060467 - 02 Jun 2021
Viewed by 443
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Limb Lengthening and Reconstruction in Children)
Show Figures

Graphical abstract

Review
Deformity Reconstruction Surgery for Tibial Hemimelia
Children 2021, 8(6), 461; https://doi.org/10.3390/children8060461 - 31 May 2021
Viewed by 582
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Limb Lengthening and Reconstruction in Children)
Show Figures

Figure 1

Back to TopTop