Surgical approach to forearm pronation deformity in patients with cerebral palsy: a systematic review.

Background: Pronation deformity in patients with cerebral palsy can have a major impact on upper limb functionality. There is lack of consensus in the literature about the preferred surgical technique to address this deformity. Study aim: To evaluate and synthesize the outcome of different surgical techniques for pronation deformity in patients with cerebral palsy. Methodology: The databases MEDLINE and Embase were searched for publications up to December 2021. Articles were considered eligible for inclusion when the included patients had a pronation deformity caused by cerebral palsy and results of surgical intervention for pronation deformity were examined. Evaluation of the quality of the retrieved study was conducted using the MINORS tool. Meta-analysis was not possible due to the heterogeneity of interventions and reported outcomes. Results: Nineteen studies, involving 475 patients and eight different techniques were included. All studies reported gain of active supination in most patients. The effect of surgery on functional gain was less clear and there was a large heterogeneity of reported functional outcome measures. There were 46 reported complications. Overall quality of study design was poor, illustrated by the average MINOR score of 6.9/16. Overall, there is a high risk of bias due to poor internal and external validity of the studies. Conclusion: Despite positive reports on gain in supination and functionality after most procedures addressing pronation deformity in CP patients, no conclusions can be drawn concerning the preferred technique due to the low quality of the evidence.

Monitoring the initial recovery after fusion surgery using activity trackers in adolescent idiopathic scoliosis: going in the lumbar spine decreases the daily step count.

PURPOSE: Although the functional outcome (e.g. the return to daily activities) plays an important role in the evaluation of treatment success for the paediatric patient, clinicians currently cannot make accurate and objective predictions regarding the very early (≤ 6 weeks) functional outcome and its recovery over time. The purpose of the present study is to objectively measure initial postoperative physical activity levels and examine the relationship with patient characteristics, fusion levels and pain. METHODS: Step count (SC) was obtained pre- (Pre-Op) and postoperatively (Post-3W: 3 weeks after surgery; Post-6W: 6 weeks after surgery) using an accelerometer. Patients were grouped based on LIV (thoracic (T-group) and lumbar (L-group)) and fusion length (FL ≤ 10 levels = SF-group and FL ≥ 11 levels = LF-group). Differences in the daily SC between groups (LIV and FL) and the three timepoints was investigated using a two-way ANOVA. RESULTS: The SC was significantly lower at both Post-3W (p < 0.001) and Post-6W (p < 0.001) compared to the preoperative SC, and significantly (p < 0.001) increased from Post-3W to Post-6W (Pre-Op = 13,049 ± 3214 steps/day; Post-3W = 6486 ± 2925 steps/day; Post-6W = 8723 ± 3020 steps/day). At both post-op timepoints the T-group had a higher SC compared to the L-group. CONCLUSION: A fusion surgery with the LIV at L2 or below has a negative impact on the very early postoperative activity levels. The initial functional outcome level of AIS patients was not related to the presently collected patient characteristics. This suggests that objective activity trackers provide novel information and could have an added value in very early rehabilitation programs.

Risk and Resilience Predictors of Recovery After Spinal Fusion Surgery in Adolescents.

OBJECTIVE: This prospective study examined risk and resilience predictors of pain and functional recovery in the first 6 months after spinal fusion surgery in adolescents. METHODS: Adolescents with adolescent idiopathic scoliosis undergoing spinal fusion surgery (n=100, aged 12 to 18 y, 77% girls) completed assessments before surgery and at 3 weeks, 6 weeks, and 6 months after surgery. Recovery trajectories in pain, health-related quality of life (HRQOL), and objectively registered physical activity were identified. Presurgical pain catastrophizing and pain intensity (risk), and psychological flexibility, and postsurgical pain acceptance (resilience) were examined as predictors of recovery. RESULTS: Latent growth class analyses revealed 4 distinct pain recovery trajectories (ie, Severe-Moderate [11%, n=9], Mild-No [58%, n=49], Moderate-Mild [24%, n=20], and Moderate-Severe [7%, n=6] pain trajectory), 2 HRQOL recovery trajectories; 2 trajectories characterizing recovery in average daily physical activity at moderate-to-vigorous intensity (MVPA); and 3 trajectories characterizing recovery in total physical activity volume characterized by the average daily number of steps. Subsequent multivariate analyses of variance revealed that presurgical pain intensity (partial η2=0.21, P<0.001) and pain catastrophizing (partial η2=0.13, P<0.01) were both predictive of poorer recovery in HRQOL, and pain catastrophizing additionally predicted poorer pain recovery (partial η2=0.15, P<0.05). Psychological flexibility (partial η2=0.25, P<0.001) and postsurgical pain acceptance (partial η2=0.07, P<0.05) were predictive of more favorable recovery trajectories in HRQOL, and psychological flexibility additionally predicted more favorable recovery trajectories in postsurgical pain (partial η2=0.15, P<0.05). Daily MVPA trajectories were not significantly predicted by any of the hypothesized factors, while presurgical pain catastrophizing levels were predictive of a delayed recovery trajectory in the daily amount of steps (partial η2=0.17 P<0.01). CONCLUSIONS: Presurgical screening could include assessment of pain intensity, pain catastrophizing, psychological flexibility, and pain acceptance to identify adolescents who are at risk for poorer recovery. These are potentially modifiable factors that can be targeted in presurgical interventions to prevent poor and foster adaptive outcomes after major surgery in adolescents.

The management of chronic paediatric Monteggia fracture-dislocation.

BACKGROUND: Monteggia fracture-dislocation is a rare and often missed injury in the paediatric population. The neglected radial head dislocation might go unnoticed for several months, but usually becomes symptomatic at a certain point in time, posing a complex clinical problem. Management strategies for chronic Monteggia lesions have been proposed by many authors. A consensus therapy is nonetheless lacking. In recent years an increasing number of case series reporting the outcomes of various treatment options have been published to gain insight into this challenging pathology. The purpose of this review is to provide a general background on chronic, paediatric Monteggia fracture-dislocation, followed by a systematic analysis and discussion of various management strategies and their outcomes, described in recent outcome studies. METHODS: A literature search was conducted within the online databases PubMed, Cochrane Central, EMBASE and Google Scholar, to identify outcome studies on the management of chronic Monteggia lesions published between January 2015 and April 2020. A total of 23 outcome studies were identified and included in this study. RESULTS: Obtaining stable radial head reduction can be regarded as the main objective of any management strategy for chronic, paediatric Monteggia fracture-dislocation. In recent literature, many surgical techniques have been put forward to obtain this goal, with the mainstay of most treatment strategies being ulnar osteotomy and open reduction with or without reconstruction of the annular ligament. Watchful neglect is a strategy that got more or less abandoned and is challenged in recent literature. CONCLUSIONS: Due to the complexity of long-standing radial head dislocation and the unpredictability of outcomes in the treatment of chronic Monteggia lesions, early diagnosis and achieving a stable reduction, preferably in the acute setting, are paramount. Because of the tendency to obtain more satisfactory radiological and clinical results in younger patients, with a short injury-to-surgery interval, it is advisable to promptly proceed to surgical treatment when chronic Monteggia fracture-dislocation is diagnosed.

A study on the efficacy of AFO stiffness prescriptions.

PURPOSE: Ankle foot orthosis (AFO) stiffness is a key characteristic that determines how much support or restraint an AFO can provide. Thus, the goal of the current study is twofold: (1) to quantify AFO prescriptions for a group of patients; (2) to evaluate what impact these AFO have on the push-off phase. METHOD: Six patients were included in the study. Three patients were prescribed an AFO for ankle support and three patients were prescribed an AFO for ankle and knee support. Two types of AFO - a traditional polypropylene AFO (AFOPP) and a novel carbon-selective laser sintered polyamide AFO (AFOPA), were produced for each patient. AFO ankle stiffness was measured in a dedicated test rig. Gait analysis was performed under shod and orthotic conditions. RESULTS: Patient mass normalized AFOPP stiffness for ankle support ranged from 0.042 to 0.069 N·m·deg-1·kg-1, while for ankle and knee support it ranged from 0.081 to 0.127 N·m·deg-1·kg-1. On the group level, the ankle range of motion and mean ankle velocity in the push-off phase significantly decreased in both orthotic conditions, while peak ankle push-off power decreased non-significantly. Accordingly, on the group level, no significant improvements in walking speed were observed. However, after patient differentiation into good and bad responders it was found that in good responders peak ankle push-off power tended to be preserved and walking speed tended to increase. CONCLUSIONS: Quantification of AFO stiffness may help to understand why certain orthotic interventions are successful (unsuccessful) and ultimately lead to better AFO prescriptions. Implications for rehabilitation AFO ankle stiffness is key characteristic that determines how much support or restraint an AFO can provide. In a typical clinical setting, AFO ankle stiffness is not quantified. AFO has to meet individual patient's biomechanical needs. More objective AFO prescription and more controlled AFO production methods are needed to increase AFO success rate.

Three-dimensional correction of fibular hemimelia using a computer-assisted planning : technical report and literature review.

The purpose of this survey in Belgium and the Netherlands was to assess treatment variation in glenohumeral osteoarthritis between experienced and less experienced orthopedic surgeons, and to investigate perioperative treatment after shoulder arthroplasty in a large group of orthopedic surgeons. Orthopedic surgeons specialized in shoulder surgery were invited to complete a survey between November 2013 and February 2015. Seventy-one percent of the approached surgeons com-pleted the survey. Less experienced surgeons (< 6 years) and surgeons from the Netherlands find patient characteristics (e.g. smoking p=0.01) more relevant than more experienced surgeons (≥ 6 years) and surgeons from Belgium. Less experienced surgeons will less likely (p=0.001) perform resurfacing arthroplasty compare to experienced surgeons. The less and the experienced surgeons use similar indications for a reverse shoulder arthroplasty regarding age limit and cuff arthropathy without osteoarthritis. Less experienced surgeon will more likely (p=0.003) prescribe a low molecular weight heparin during the hospital stay after a shoulder arthroplasty. In this survey, we found a decrease in the use of resurfacing arthroplasty and a strong increase in the use of reverse shoulder arthroplasty. Besides, there is little consensus concerning pre-operative planning, patient characteristics, surgical technique, and patient reported outcome measures. Level of evidence: IV.

Myhre syndrome: A first familial recurrence and broadening of the phenotypic spectrum.

Myhre syndrome is a rare multisystem connective tissue disorder, characterized by short stature, facial dysmorphology, variable intellectual disability, skeletal abnormalities, arthropathy, cardiopathy, laryngotracheal anomalies, and stiff skin. So far, all molecularly confirmed cases harbored a de novo heterozygous gain-of-function mutation in SMAD4, encoding the SMAD4 transducer protein required for both transforming growth factor-beta and bone morphogenic proteins signaling. We report on four novel patients (one female proband and her two affected children, and one male proband) with Myhre syndrome harboring the recurrent c.1486C>T (p.Arg496Cys) mutation in SMAD4. The female proband presented with a congenital heart defect, vertebral anomalies, and facial dysmorphic features. She developed severe tracheal stenosis requiring a total laryngectomy. With assisted reproductive treatment, she gave birth to two affected children. The second proband presented with visual impairment following lensectomy in childhood, short stature, brachydactyly, stiff skin, and decreased peripheral sensitivity. Transmission electron microscopy (TEM) of the dermis shows irregular elastin cores with globular deposits and almost absent surrounding microfibrils and suggests age-related increased collagen deposition. We report on the first familial case of Myhre syndrome and illustrate the variable clinical spectrum of the disorder. Despite the primarily fibrotic nature of the disease, TEM analysis mainly indicates elastic fiber anomalies.

Defining the Clinical, Molecular and Ultrastructural Characteristics in Occipital Horn Syndrome: Two New Cases and Review of the Literature.

Occipital horn syndrome (OHS) is a rare connective tissue disorder caused by pathogenic variants in ATP7A, encoding a copper transporter. The main clinical features, including cutis laxa, bony exostoses, and bladder diverticula are attributed to a decreased activity of lysyl oxidase (LOX), a cupro-enzyme involved in collagen crosslinking. The absence of large case series and natural history studies precludes efficient diagnosis and management of OHS patients. This study describes the clinical and molecular characteristics of two new patients and 32 patients previously reported in the literature. We report on the need for long-term specialized care and follow-up, in which MR angiography, echocardiography and spirometry should be incorporated into standard follow-up guidelines for OHS patients, next to neurodevelopmental, orthopedic and urological follow-up. Furthermore, we report on ultrastructural abnormalities including increased collagen diameter, mild elastic fiber abnormalities and multiple autophagolysosomes reflecting the role of lysyl oxidase and defective ATP7A trafficking as pathomechanisms of OHS.

Mesomelic dysplasia with acral synostoses Verloes-David-Pfeiffer type: follow-up study documents progressive clinical course.

Verloes-David-Pfeiffer mesomelia-synostoses syndrome is an autosomal-dominant form of mesomelic dysplasia comprising typical acral synostoses combined with ptosis, hypertelorism, palatal abnormality, CHD, and ureteral anomalies. Since the original reports in 1995, two other patients have been described with this syndrome, one of them the patient reported in 1998 by Day-Salvatore. In this article, we report on the follow-up of some of the original cases and review the literature. We confirm that the Verloes-David-Pfeiffer syndrome (VDPS) is a progressive skeletal disorder that despite repeated corrective surgical intervention leads to severe limb deformities. No mutations were detected in the FLNB gene. To date, the cause and the pathogenesis of VDPS remain unknown. The latter is characterized in this study as a syndromic type of skeletal dysplasia because besides congenital malformations and multiple acromelic synostoses arising prenatally, VDPS manifests in postnatal life as a severe osteochondrodysplasia.

Endothelial outgrowth cells are not derived from CD133+ cells or CD45+ hematopoietic precursors.

OBJECTIVE: Two types of endothelial progenitor cells (EPCs), early EPCs and late EPCs (also called endothelial outgrowth cells [EOCs]), were described in vitro previously. In this report, we dissect the phenotype of the precursor(s) that generate these cell types with focus on the markers CD34, CD133, and vascular endothelial growth factor receptor-2 (VEGFR2) that have been used to identify putative circulating endothelial precursors. We also included CD45 in the analysis to assess the relation between CD34+ hematopoietic progenitors (HPC), CD34+ endothelial precursors, and both in vitro generated EPC types. Addressing this issue might lead to a better understanding of the lineage and phenotype of the precursor(s) that give rise to both cell types in vitro and may contribute to a consensus on their flowcytometric enumeration. METHODS AND RESULTS: Using cell sorting of human cord blood (UCB) and bone marrow (BM) cells, we demonstrate that EOC generating precursors are confined to a small CD34+ CD45- cell fraction, but not to the CD34+ CD45+ HPC fraction, nor any other CD45+ subpopulation. CD34+ CD45+ HPC generated monocytic cells that displayed characteristics typical for early EPCs. Phenotypic analysis showed that EOC generating CD34+ CD45- cells express VEGFR2 but not CD133, whereas CD34+ CD45+ HPC express CD133 as expected, but not VEGFR2. CONCLUSION: EOCs are not derived from CD133+ cells or CD45+ hematopoietic precursors.

Functional recovery after a reverse prosthesis for reconstruction of the proximal humerus in tumor surgery.

UNLABELLED: An alternative treatment for primary bone tumors of the proximal humerus was assessed. Four patients, who made full functional recovery after complete resection of the proximal humerus inclusive of the rotator cuff and subsequent reconstruction with a reverse shoulder prosthesis, were examined clinically and radiographically. Distinct medialization of the center of rotation of the glenohumeral joint (28 mm) and elongation of the remaining deltoid muscle (116%) were measured. Increased scapular rotation (118%) was observed. The radiologic results and thoracoscapular rhythm analyses were implemented in a three-dimensional computerized model of the glenohumeral joint. This allowed us to calculate a doubling of the moment of the deltoid abductor muscle in the true scapular plane. After tumor surgery, in which the proximal humerus is resected without reinserting the rotator cuff, full functional recovery of the shoulder can be obtained with a total shoulder prosthesis, medializing the glenohumeral center of rotation and elongating the remaining deltoid muscle. LEVEL OF EVIDENCE: Therapeutic study, Level IV (case series-no, or historical control group).

Quantified measurement of subacromial impingement.

We modified the Hawkins impingement maneuver in order to develop a quantifiable and reproducible impingement test. The involved anatomic structures were examined with magnetic resonance imaging of 3 cadaveric shoulders. The reproducibility of the clinical sign was assessed with an interobserver and intraobserver reliability test, with calculation of the intraclass correlation coefficient (ICC). The quantified Hawkins maneuver appears to be a reproducible clinical test (median interobserver ICC, 0.9331; median intraobserver ICC, 0.9748) and creates bony contact between the greater tuberosity and the anterolateral acromion. This test enables the clinician to quantify the subacromial space.