Guided growth surgery for angular deformity of the knee: one centres experience.

BACKGROUND: Angular deformity in the lower extremity can result in pain, gait disturbance, cosmetic deformity and joint degeneration. Up until the introduction of guided growth, which has since become the widely accepted treatment for frontal plane angular angular deformity around the knee in skeletally immature patients, treatment consisted of staples, corrective osteotomy or an angular epiphysiodesis. Guided growth modulation uses the tension band principle with the goal of treatment being to normalise the lower limb mechanical axis resulting in lower morbidity than previous treatments. In order to assess the success of this procedure we reviewed our results in an attempt to identify patients who may not benefit from this elegant procedure. METHODS: We performed a retrospective review of prospectively collected surgical records and diagnostic imaging in our paediatric tertiary national referral centre to identify all patients who had guided growth surgery for coronal plane angular deformity of the knee from 2007 to 2023. We noted the patient demographics, diagnosis, peri-operative experience and outcome. All patients were followed until skeletal maturity, until their hardware was removed or at least 2 years. RESULTS: Two hundred thirty-six patients were assessed for eligibility. Of the 282 treated knees which met the criteria for final assessment 55 (19.5%) were unsuccessful. Complications were few but included infection and metal-work prominence. Procedures that were less likely to be successfully included growth disturbances following trauma (18.8% failure) or infection (40%), tumour (66.6%), mucopolysaccharidoses type I (15.7%), spondyloepiphyseal dysplasia (25%) or Blount's disease (60%). Idiopathic angular deformity showed an 89.5% success rate with guided growth. CONCLUSION: In our hands, guided growth had an 80.5% success rate when all diagnoses were considered. We continue to advocate the use of guided growth as a successful treatment option for skeletally immature patients with limb deformity however caution should be employed when considering its use in certain patient groups. LEVEL OF EVIDENCE: Level III, retrospective cohort study.

Children's Hip Predictive (CHiP) Score: A Triage Tool for Hip Dislocation in Children Referred With Suspected Hip Dysplasia.

BACKGROUND: A fundamental tenent of treating developmental dysplasia of the hip is to identify patients with dislocated hips early so as to avoid the long-term sequelae of late diagnosis. The aim of this study was to develop a readily useable triage tool for patients with suspected hip dislocation, based on the clinical history and examination findings of the referring practitioner. METHODS: All primary care referrals (n=934) over a 3-year period for suspected developmental dysplasia of the hip to a tertiary pediatric center were evaluated. Defined parameters with respect to history and clinical examination were evaluated. Multivariable logistic regression was used to establish predictors of hip dislocation, and from this a predictive model was derived which incorporated significant predictors of dislocation. An illustrative nomogram translated this predictive model into a usable numerical scoring system called the Children's Hip Prediction score, which estimates probability of hip dislocation. RESULTS: There were 97 dislocated hips in 85 patients. The final predictive model included age, sex, family history, breech, gait concerns, decreased abduction, leg length discrepancy, and medical/neurological syndrome. The area under receiver operating curve for the model is 0.761. A Children's Hip Prediction score of≥5 corresponds to a sensitivity of 76.3% and a score of≥15 has a specificity of 97.8%, corresponding to an odds ratio of 27.3 for increased risk of dislocation. CONCLUSION: We found that a novel clinical prediction score, based on readily available history and examination parameters strongly predicted risk of dislocations in hip dysplasia referral. It is hoped that this tool could be utilized to optimize resource allocation and may be of particular benefit in less well-resourced health care systems. LEVEL OF EVIDENCE: Level II.

Correction to: Day-case pelvic osteotomy for developmental dysplasia of the hip.

[This corrects the article DOI: 10.1302/1863-2548.14.200164.].

Establishing a Virtual Clinic for Developmental Dysplasia of the Hip: A Prospective Study.

BACKGROUND: The aim was to describe the introduction and operation of a virtual developmental dysplasia of the hip (DDH) clinic. Our secondary objectives were to provide an overview of DDH referral reasons, treatment outcomes, and adverse events associated with it. METHODS: A prospective observational study involving all patients referred to the virtual DDH clinic was conducted. The clinic consultant delivered with 2 DDH clinical nurse specialists (CNS). The outcomes following virtual review include further virtual review, CNS review, consultant review or discharge. Treatment options include surveillance, brace therapy, or surgery. Efficiency and cost analysis were assessed. RESULTS: Over the 3.5-year study period, 1002 patients were reviewed, of which 743 (74.2%) were female. The median age at time of referral was 7 months, (interquartile range of 5 to 11) with a median time to treatment decision of 9 days. Median waiting times from referral to treatment decision was reduced by over 70%. There were 639 virtual reviews, 186 CNS reviews, and 144 consultant reviews. The direct discharge rate was 24%. One hundred one patients (10%) had dislocated or subluxed hips at initial visit while 26.3% had radiographically normal hips. Over the study period 704 face to face (F2F) visits were avoided. Cost reductions of €170 were achieved per patient, with €588,804 achieved in total. Eighteen parents (1.8%) opted for F2F instead of virtual review. There were no unscheduled rereferrals or recorded adverse events. CONCLUSION: We report the outcomes of the first prospective virtual DDH clinic. This clinic has demonstrated efficiency and cost-effectiveness, without reported adverse outcomes to date. It is an option to provide consultant delivered DDH care, while reducing F2F consults. LEVEL OF EVIDENCE: Level III.

Day-case pelvic osteotomy for developmental dysplasia of the hip.

PURPOSE: In this article we report the results of a pilot study analysing the implications of performing pelvic osteotomies for developmental dysplasia of the hip (DDH) as a day case. We assess the advantages of performing paediatric pelvic osteotomies as day-case procedures from a financial perspective and from an in-patient bed resource point of view. METHODS: This was a prospective cohort study analysing Salter and Pemberton pelvic osteotomies performed for DDH over a three-year period from 1st January 2017 to 30th September 2019. All patients residing within 50 km of the hospital were eligible for day-case procedures. All other cases were performed as in-patients. A detailed financial costing analysis was performed and the in-patient resources utilized were documented and compared between the two models of care. RESULTS: In total, 84 Salter and Pemberton osteotomies were performed between 1st January 2017 to 30th September 2019. Of these cases, 35 were performed as day-case procedures. A total reduction in 70 in-patient bed days was reported. Total costs for a single in-patient requiring two nights of admission amounted to €5,752, whereas the discharge cost of a day case was reported at €2,670. The savings made by our institution amounted to €3,082 per day case. A total saving of €102,696 was made over three years. In all, seven day-case patients re-attended due to inadequate pain control. They required overnight admission and were discharged uneventfully the following day. CONCLUSION: Day-case pelvic osteotomies significantly reduce the number of in-patient bed days used in an elective paediatric orthopaedic setting. Significant financial savings in excess of €3,000 per case are possible. The introduction of day-case pelvic osteotomy procedures can significantly improve the cost-effectiveness of managing DDH provided there are clear protocols in place with close clinical follow-up. LEVEL OF EVIDENCE: IV.

A radiographic clinic for developmental dysplasia of the hip (DDH).

BACKGROUND: We describe the first radiographic clinic in the literature for DDH and how this novel clinic can significantly improve the efficiency and cost-effectiveness of service in a tertiary referral centre. AIMS: A radiographic clinic for the management of developmental dysplasia of the hip was introduced in 2017 in our institution. We performed a detailed cost analysis to assess the economic savings made with the introduction of this new clinic. We assessed the efficiency of the service by identifying how many unnecessary outpatient visits were prevented. We also assessed the difference in times from referral to review between the two clinics. METHODS: Analysis of the clinic activity in 2017 was possible as all data was collected prospectively by the DDH CNS and stored in our database. Cost analysis was performed, and the savings made per patient along with the financial benefit to our institution was recorded. RESULTS: The new radiographic clinic reduced the cost of reviewing one patient by €162.51 per patient. There was a 73% discharge rate from the clinic which prevented 251 unnecessary patient visits to the outpatient department over the course of the year. There was a significant 11-day reduction in waiting times between referral and review when comparing the radiographic to the conventional clinic (p < 0.05). CONCLUSIONS: A radiographic clinic for the management of developmental dysplasia of the hip has a significant effect on the efficiency and overall cost-effectiveness of service provision in a tertiary referral centre.

Non-vascularised Fibular Autograft for Reconstruction of Paediatric Bone Defects: An Analysis of 10 Cases.

AIM AND OBJECTIVE: Fibular autograft is a known technique for the reconstruction of traumatic and non-traumatic bone defects in both adult and paediatric populations. We aim to describe our outcomes using various stabilisation methods for non-vascularised fibular autograft to reconstruct both benign and malignant tumours in a paediatric population in a National Paediatric Centre over the past 14 years. MATERIALS AND METHODS: This was a retrospective review of 10 paediatric cases with non-traumatic primary bone defects in a National Paediatric Centre. Criteria for inclusion were all non-traumatic primary bone defects requiring reconstruction with a non-vascularised fibular autograft in the diaphyseal or metaphyseal regions of the bone. The primary outcome measures were union and time to union (weeks). Time to union was illustrated using Kaplan-Meier curves. Secondary outcome measures included postoperative fracture, infection (deep and superficial), time to full weight-bearing and all-cause revision surgery. RESULTS: The mean length of follow-up was 63 months for the entire cohort (9-168, SD = 48.6). There was no loss to follow-up. Six lesions were located in the tibia, two in the femur and the remaining two were located in the ulna and third metacarpal. Union was ultimately achieved in 8 of the 10 patients using this donor autograft. The mean time to union was 28 weeks (10-99, SD = 29.8). There were four complications of autograft fracture. The mean time to fracture was 17 weeks (9-32, SD = 10.71). In all four of these cases, the patient achieved union at final follow-up. There were two superficial and two deep infections recorded. Three resolved with the use of antimicrobial therapy and one deep infection ultimately required insertion of an intercalary prosthesis to treat the infected non-union of the fibular graft site. CONCLUSION: The use of non-vascularised fibular autograft for the reconstruction of tumours is an effective surgical technique in a paediatric cohort. We report the largest known series of malignant paediatric tumours treated with this technique to date. CLINICAL SIGNIFICANCE: Non-vascularised fibular autograft is successful in the reconstruction of large bone defects secondary to malignant paediatric bone tumours. HOW TO CITE THIS ARTICLE: Sheridan GA, Cassidy JT, Donnelly A, et al. Non-vascularised Fibular Autograft for Reconstruction of Paediatric Bone Defects: An Analysis of 10 Cases. Strategies Trauma Limb Reconstr 2020;15(2):84-90.

Progression of Hip Dysplasia in Mucopolysaccharidosis Type I Hurler After Successful Hematopoietic Stem Cell Transplantation.

BACKGROUND: Dysostosis multiplex contributes substantially to morbidity in patients with Hurler syndrome (mucopolysaccharidosis type I Hurler phenotype [MPS I-H]), even after successful hematopoietic stem cell transplantation (HSCT). One of the hallmarks of dysostosis multiplex in MPS I-H is hip dysplasia, which often requires surgical intervention. We sought to describe in detail the course of hip dysplasia in this group of patients, as assessed by radiographic analysis, and to identify potential outcome predictors. METHODS: Longitudinal data were obtained from digitally scored pelvic radiographs of patients with MPS I-H using OrthoGon software for parameters including, but not limited to, the acetabular index, migration percentage, Smith ratio, and neck-shaft angle. Scoring was performed independently by two blinded observers. Additional information on genotype, enzyme replacement therapy pre-HSCT, donor chimerism, and enzyme activity post-HSCT were obtained. General trends and potential correlations were calculated with mixed-model statistics. RESULTS: Fifty-two patients (192 radiographs) were included in this analysis. Intraobserver and interobserver variation analysis showed an intraclass correlation coefficient ranging from 0.78 to 1.00. Among the twenty-one patients with follow-up beyond the age of five years, the acetabular index was in the range of severe hip dysplasia in up to 86% of the patients. Severe coxa valga was seen in 91% of the patients. Lateral and superior femoral displacement were highly prevalent, with the migration percentage outside the reference range in up to 96% of the patients. Finally, anterior pelvic tilt increased with age (p = 0.001). No correlations were identified between clinical parameters and radiographic findings. CONCLUSIONS: Our study shows that progressive acetabular dysplasia as well as coxa valga and hip displacement are highly prevalent and progressive over time in patients with MPS I-H, despite successful HSCT. These data may provide essential natural history determinations for the assessment of efficacy of new therapeutic strategies aimed at improving skeletal outcomes in patients with MPS I-H.

The prevalence of abnormal preoperative coagulation tests in pediatric patients undergoing spinal surgery for scoliosis.

BACKGROUND CONTEXT: Multilevel spinal fusion surgery for deformity correcting spinal surgery in pediatric patients with scoliosis has typically been associated with significant blood loss. The mechanism of bleeding in such patients is not fully understood. Coagulation abnormalities, which may be associated with scoliosis, are thought to play a role. PURPOSE: To document and compare the prevalence of preoperative coagulation abnormalities among patients with scoliosis attending a pediatric orthopedic department for spinal fusion surgery with patients attending for minor surgery. STUDY DESIGN: An observational study. All patients were recruited from a pediatric tertiary referral center in Dublin, Ireland. PATIENT SAMPLE: Coagulation profile results were prospectively collected over a 2-year period from 165 spinal surgery patients. In total, 175 patients were included in the non-scoliosis group. These patients attended the day ward for minor procedures and were recruited over a 4-month period. OUTCOME MEASURES: The primary outcome measure was the coagulation profiles, which included prothrombin time, activated partial thromboplastin time (APTT), and thrombin time (TT). Levels of Coagulation Factors II, V, VII, and X were also recorded. METHODS: All blood samples were sent to the haematology laboratory to establish the coagulation profile. The primary outcome was the presence of an abnormal coagulation screening test (if any of PT, APTT, or TT were abnormal). Prothrombin time, APTT, and TT were also analyzed as individual continuous variables, as well as Coagulation Factors II, V, VII, and X. Regression analysis was used to compare the coagulation profile of scoliosis patients with that of non-scoliosis patients. There were no outside funding sources or any potential conflict of interest associated with this study. RESULTS: The scoliosis patients were more likely to have an abnormal preoperative screening test compared with non-scoliosis patients, with an odds ratio of 2.6. Further analysis showed statistically significant longer clotting times for patients with scoliosis compared with those without; PT (t=3.37, p=.001), APTT (t=4.26, p<.001), TT (t=4.52, p<.001). Of the coagulation factors analyzed, only factor X was significantly different in scoliosis patients compared with non-scoliosis controls (t=-4.41, p<.001). CONCLUSIONS: Children with scoliosis have a higher prevalence of preoperative coagulation abnormalities compared with normal healthy patients.

Fibroma of the peroneus longus tendon sheath in a child: a case report.

Tendon sheath fibromas are rare, benign soft-tissue tumours and usually involve tendons of the upper extremities, particularly the fingers. The most common presentation is a painless, slow-growing swelling. Tendon sheath fibromas are composed of dense fibrocollagenous stromas with scattered, spindle-shaped fibroblasts and narrow, slit-like vascular spaces. Ultrasonography and magnetic resonance imaging are useful diagnostic tools. We report on an 11-year-old boy with a fibroma involving the right peroneus longus tendon sheath.

Treatment of hip dysplasia in patients with mucopolysaccharidosis type I after hematopoietic stem cell transplantation: results of an international consensus procedure.

BACKGROUND: Mucopolysaccharidosis type I (MPS-I) is a lysosomal storage disorder characterized by progressive multi-organ disease. The standard of care for patients with the severe phenotype (Hurler syndrome, MPS I-H) is early hematopoietic stem cell transplantation (HSCT). However, skeletal disease, including hip dysplasia, is almost invariably present in MPS I-H, and appears to be particularly unresponsive to HSCT. Hip dysplasia may lead to pain and loss of ambulation, at least in a subset of patients, if left untreated. However, there is a lack of evidence to guide the development of clinical guidelines for the follow-up and treatment of hip dysplasia in patients with MPS I-H. Therefore, an international Delphi consensus procedure was initiated to construct consensus-based clinical practice guidelines in the absence of available evidence. METHODS: A literature review was conducted, and publications were graded according to their level of evidence. For the development of consensus guidelines, eight metabolic pediatricians and nine orthopedic surgeons with experience in the care of MPS I patients were invited to participate. Eleven case histories were assessed in two written rounds. For each case, the experts were asked if they would perform surgery, and they were asked to provide information on the aspects deemed essential or complicating in the decision-making process. In a subsequent face-to-face meeting, the results were presented and discussed. Draft consensus statements were discussed and adjusted until consensus was reached. RESULTS: Consensus was reached on seven statements. The panel concluded that early corrective surgery for MPS I-H patients with hip dysplasia should be considered. However, there was no full consensus as to whether such a procedure should be offered to all patients with hip dysplasia to prevent complications or whether a more conservative approach with surgical intervention only in those patients who develop clinically relevant symptoms due to the hip dysplasia is warranted. CONCLUSIONS: This international consensus procedure led to the construction of clinical practice guidelines for hip dysplasia in transplanted MPS I-H patients. Early corrective surgery should be considered, but further research is needed to establish its efficacy and role in the treatment of hip dysplasia as seen in MPS I.

A single-center prospective evaluation of the Ponseti method in nonidiopathic congenital talipes equinovarus.

BACKGROUND: The Ponseti method has revolutionized the management of idiopathic congenital talipes equinovarus (CTEV). However, nonidiopathic CTEV is still often primarily treated by extensive surgical soft tissue release. We believe that nonoperative treatment of these patients using the Ponseti method may give very satisfactory results. METHODS: We examined the demographics of nonidiopathic CTEV and the success of the Ponseti method in this population over a 5-year period. We treated 29 patients with 43 nonidiopathic and 97 patients with 138 idiopathic CTEV feet. Patients with nonidiopathic CTEV made up 23% of all cases. The commonest etiologies were arthrogryposis (5 cases), trisomy 21 (4 cases), and spina bifida (3 cases). Average follow-up was 39 (nonidiopathic group) and 35 months (idiopathic group). RESULTS: The Ponseti method was initially successful in 91% of nonidiopathic and 98% of idiopathic feet. Recurrence of deformity occurred in 44% of nonidiopathic and 8% of idiopathic feet. Thirty-seven percent of nonidiopathic feet required extensive surgical release compared with 2% in the idiopathic group. CONCLUSIONS: Although the success rate of the Ponseti method in nonidiopathic CTEV is inferior to that in idiopathic CTEV, 63% of our nonidiopathic patients did not require extensive surgery. We believe that the Ponseti method should be used in all cases of nonidiopathic CTEV. LEVEL OF EVIDENCE: Level III--prospective cohort study.