Management of Pediatric Volar Plate Avulsion Fractures of the Proximal Interphalangeal Joint: A Systematic Review.

BACKGROUND: Sudden, forced hyperextension injuries to the proximal interphalangeal joint leading to volar plate avulsion fractures are common hand injuries in children. Suboptimal management of these fractures can lead to the development of long-term complications such as stiffness and flexion contracture. METHODS: MEDLINE (PubMed), Scopus, Embase, Google Scholar, and Cochrane CENTRAL databases were systematically searched, and additional studies were found through reference of articles up to June 15, 2023. Identified articles were assessed using predetermined inclusion/exclusion criteria. RESULTS: Twenty-five articles were included, involving 268 patients with ages from 3 to 17 years. Fractures with less than 30% joint involvement, classified as Eaton type I or II, or designated as "Stable" in the Keifhaber-Stern classification, were treated through nonsurgical means. Surgical interventions, encompassing open reduction and internal fixation, were reserved for fractures with more than 30% joint involvement and/or meeting criteria such as Eaton type IIIa or IIIb and Keifhaber-Stern "Tenuous" or "Unstable." Positive outcomes were seen in 99.5% of patients receiving nonsurgical treatment, compared with 85.7% in the surgical cohort. CONCLUSIONS: The literature demonstrated positive outcomes for fractures presenting with less than 30% joint involvement that were managed nonsurgically. In fractures with more than 30% joint involvement, surgical interventions yielded positive results. To further substantiate these findings, larger prospective studies with uniform measures are needed to validate the results of this study.

Outcomes of Composite Grafts for Pediatric Fingertip Amputations: A Systematic Review.

Introduction The aim of this study was to explore the outcomes of composite grafts in fingertip amputations in children as well as the contributing factors that may affect outcomes. Methods Literature search was conducted across six databases in March 2022 to select studies on the use of composite grafts on fingertip amputations in the pediatric population. Results Twelve articles with 735 composite grafts were identified for review. Most fingertip injuries occurred in the less than 5-year age group and were due to crush type injuries. In studies that reported "complete" graft take as a separate outcome measure, 17.3% of fingertips with this result were observed. In the studies that reported "complete" and "partial" graft take together as an outcome measure, 81.6% of fingertips achieved this outcome. A lower proportion of failed graft take was observed in more distal fingertip amputations. Infection (3.8%) and nail abnormalities (3.4%) were the most common complications following composite grafting. Conclusion Composite grafting can be considered as a useful method of treatment in this population. Clinicians should be aware of the potential complications following this method of treatment such as infection and nail abnormalities. More proximal fingertip amputations may warrant other surgical interventions (beyond Level II on the modified Ishikawa/Ishikawa classification). Significant heterogeneity was observed within the studies, mainly due to lack of standardization in assessment and reporting of outcomes.

Development and validation of a WHO ICF compliant hand and upper limb assessment tool for children: The Reach Out questionnaire.

BACKGROUND: Current tools for evaluating hand and upper limb function in children do not represent all domains of the World Health Organization International Classification of Disability, Functioning and Health (ICF) framework and may not capture an accurate progression or regression of function over time. PURPOSE: Based on this framework, we have developed an assessment tool (Reach Out) to evaluate function in children aged from 2 to 16 years following consultation with an advisory panel of specialists. STUDY DESIGN: Primary clinical study. METHODS: Construct validity along with test-retest reliability, inter-rater reliability and sensitivity to change have all been analyzed to validate the Reach Out assessment tool. RESULTS: The assessment tool has been validated in a total of 231 patients. Significant construct validity of 0.64 (P < .00001, 95% confidence interval = 0.56-0.71, n = 231) for both age groups and diagnostic groups was observed. The Reach Out questionnaire was internally consistent with a Cronbach's Alpha of > 0.8 for most domains in most age groups. Test re-retest scores showed that the questionnaire was reliable with most domains of the questionnaire achieving high scores of reliability (P ≤ .03). We also received positive feedback from participants and parents. CONCLUSIONS: The use of this new tool will help identify both progression and regression of function, allowing a more tailored and holistic approach to treatment in children with conditions affecting the hand and upper limb through the incorporation of International Classification of Disability, Functioning and Health domains. This tool is quicker to complete and can be applied to a wide range of ages and diagnostic groups compared to previous assessment tools.

Resect, rewire, and restore: Nerve transfer salvage of neurological deficits associated with soft tissue tumors in a retrospective cohort series at a tertiary reconstructive center.

AIMS: We aimed to explore the effectiveness of nerve transfer as an intervention to restore neurological deficits caused by extremity tumors through direct nerve involvement, neural compression, or as a consequence of oncological surgery. METHODS: A retrospective cohort study of consecutive cases was conducted, including all patients who underwent nerve transfers to restore functional deficits in limbs following soft tissue tumor resection. The threshold for a successful nerve transfer was a BMRC motor grade of 4/5 and sensory grade of 3-3+/4 with protective sensation. RESULTS: In total, 29 nerve transfers (25 motor and 4 sensory) were completed in 11 patients, aged 12-70 years at referral, over a 6-year period to 2020. This included 22 upper limb and 3 lower limb motor nerve transfers. The timing of delayed nerve transfer reconstructions was 1-15 months following primary oncological resection, with immediate simultaneous reconstructions performed in 4 cases. The threshold for success was achieved in 82% of upper limb and 33% of lower limb motor nerve transfers, while all sensory transfers were successful in restoring protective sensation. CONCLUSION: Nerve transfer surgery, a well-established technique in restoring deficits following traumatic nerve injury, is further demonstrably relevant in extremity oncological reconstruction, especially as it can be performed remotely to the tumor location or resection site and introduces a healthy nerve or fascicle to rapidly reinnervate distal muscles without sacrificing major function. This study further illustrates the importance of early recognition and referral to specialist services where multi-disciplinary surgical resection and reconstructive planning can be conducted. LEVEL OF EVIDENCE: IV Clinical Case Series.

Utility of a Pediatric Adaptive Sports Clinic: A Case Series Review.

BACKGROUND: Limited funding is available for athletes with disabilities in the United Kingdom. This compounds the barriers to participation and development that already exist. METHOD: To combat this growing problem, a Multi-Disciplinary Pediatric Adaptive Sports Clinic was formed. RESULT: Fifteen athletes with disabilities attended the Clinic from November 2017 to November 2019. In our cohort, there were 10 males and 5 females (age range: 13-18 years). Most athletes participated at a grassroots level (n = 9). The range of diagnoses included cerebral palsy, Ehlers Danlos syndrome and congenital hand differences. Forty-four appointments were made after the initial meeting with a 95% attendance rate. Improvements beyond the minimal clinically important differences (MCID) for the Patient Specific Functional Scale, Numerical Pain Rating Scale, and Medical Research Council Manual Muscle Testing Scale were noted in over half of cases. CONCLUSION: With a focus on injury prevention and strength and conditioning techniques, this clinic supported athletes to successfully compete from a recreational to an elite level across all types of sports and adolescent ages by providing patient-specific regimens. Our case series provides preliminary evidence to suggest the formation of similar clinics that can support athletes with disabilities across a range of sports.

Early MRI for Pediatric Wrist Injuries-Prospective Case Series of 150 Cases.

Background Pediatric carpal injuries are a clinical challenge due to their non-specific clinical features and occult nature on plain radiography. We hypothesized that early magnetic resonance imaging (MRI) will allow prompt diagnosis and treatment stratification, and that distal pole fracture of the scaphoid requires a shorter duration of immobilization. This study aims to assess the injury pattern and clinical outcomes of under-16-year-olds treated with acute post-traumatic wrist injuries in accordance with the unit's protocol. Methods All patients under the age of 16 years treated for suspected pediatric wrist injuries in our tertiary pediatric hand and upper limb service were included. Prospectively collected data included patient demographics, radiological findings, treatment and adherence to the unit's protocol. Results There were 151 patients with a mean age of 12 years. The majority (72%) had occult bony injury with radiological evidence of fracture on MRI. The sensitivity and specificity of plain film radiography were 42.7% and 71.4%, respectively. Almost one in four patients benefitted from early MRI demonstrating no injuries, permitting early mobilization and discharge. The scaphoid was the most commonly injured carpal bone. Non-displaced fractures of the distal pole of the scaphoid in patients over 10 years old were treated with 4 weeks' immobilization with no adverse outcome. Conclusion Standardized care in our unit has yielded good results with low complication rate and fewer hospital appointments. Our results support the routine early use of MRI and a shorter duration of immobilization in fracture of the distal pole of the scaphoid at 4 weeks. Level of Evidence This is a level IV, case series study.

Role of Wrist Arthroscopy in Juvenile Inflammatory Arthritis.

Background and Purposes The wrist is the fourth most common joint to be involved in juvenile inflammatory arthritis (JIA), which is a common rheumatological condition affecting children. Wrist arthroscopy is well established in rheumatoid arthritis, but remains unexplored in JIA. The aim of this study is to investigate the role of wrist arthroscopy in JIA, with focus on those who are refractory to medical management. Methods This is a prospective observational study, including consecutive patients with JIA undergoing arthroscopy between January 2016 and December 2020. Those over the age of 18 years and those with other rheumatological diagnoses were excluded. Data including pre-, intra-, and postoperative variables, demographics, and patient-reported outcomes were collated and are reported using standard measures. Results A total of 15 patients underwent arthroscopy ( n = 20 wrists). Synovitis was noted in all wrists on arthroscopy and synovectomy was performed in all cases. Other procedures were performed as indicated during the procedure. The median follow-up duration was 11.3 (interquartile range [IQR] 8.1-24.2) months. Median reduction of 4 (IQR 2.25-6) points on the Visual Analogue Score for pain on loading was noted postoperatively. Grip strength was improved in n = 11/20 wrists and functional improvement was noted in n = 18/20 wrists. Restriction of range of motion was achieved with a shrinkage procedure in patients with hypermobile joints. There were no postoperative complications, and no patients were lost to follow-up. Conclusion In experienced hands, wrist arthroscopy is feasible, safe, and efficacious in the management of JIA, among patients who are refractory to medical management. Level of Evidence This is a Level II study.

Microsurgery in pediatric upper limb reconstructions: An overview.

The use of microsurgery has spread during the last decades, making resolvable many complex defects considered hitherto inapproachable. Although the small vessel diameter in children was initially considered a technical limitation, the increase in microsurgical expertise over the past three decades allowed us to manage many pediatric conditions by means of free tissue transfers. Pediatric microsurgery has been shown to be feasible, gaining a prominent place in the treatment of children affected by limb malformations, tumors, nerve injuries, and post-traumatic defects. The aim of this current concepts review is to describe the more frequent pediatric upper limb conditions in which the use of microsurgical reconstructions should be considered in the range of treatment options.

Efficacy of mTOR inhibitors (sirolimus) in isolated limb overgrowth: a systematic review.

We systematically assessed the literature on the use of sirolimus for the treatment of isolated limb overgrowth conditions and its various modalities of administration in PubMed, Scopus, Ovid MEDLINE, Web of Science, Google Scholar, Cochrane Database of Systematic Reviews, references of journals and grey literature using pre-trialled Medical Subject Headings terms and articles. Eleven articles were included, and 39 patients were identified for review. Sirolimus was given orally in 38 patients and topically in one patient. Sirolimus was found to be highly effective in treatment of isolated limb overgrowth conditions with improvement of symptoms (physical, emotional, social) reported in all but one patient. Dosage and adverse effects seemed to be closely correlated. The result of our study suggests that sirolimus should be considered as an adjuvant or first-line management in isolated limb overgrowth in prospective trials.

Wrist Ganglion Cysts in Children: An Update and Review of the Literature.

Ganglion cysts are the most common soft tissue tumor of the hand and wrist, affecting pediatric and adult populations. Despite their frequency, there is no consensus within the literature regarding the best management of pediatric wrist ganglia, and there are few recent publications examining this topic. We provide an up-to-date literature review examining the current issues and controversies in the management of pediatric wrist ganglia.

Successful Delayed Neurolysis of the Median Nerve in a Teenager 7 years After Supracondylar Fracture: A Case Report and Review of the Literature.

ABSTRACT: Supracondylar humeral fractures are the commonest elbow fractures in childhood, associated with a high rate of neurological complications. We present the case of a 14-year-old adolescent girl with persisting daily pain, lack of sensation, and strength to her left hand after a displaced supracondylar fracture 7 years earlier. At the time of the fracture, she underwent closed reduction and percutaneous Kirschner wire fixation. During revision surgery, half of the median nerve was found embedded in bone at the level consistent with the healed fracture site. Neurolysis of the fascicles adherent to the bone was performed. Postoperatively, the patient had complete recovery of sensation, reduced pain, and a 2.5-fold improvement of grip strength. This case shows that there is a role for delayed neurolysis in children.

Current concepts in diagnosis and management of common upper limb nerve injuries in children.

Peripheral nerve injuries (PNI) of the upper limb are a common event in the paediatric population, following both fractures and soft tissues injuries. Open injuries should in theory be easier to identify and the repair of injured structures performed as soon as possible in order to obtain a satisfying outcome. Conversely, due to the reduced compliance of younger children during clinical assessment, the diagnosis of a closed nerve injury may sometimes be delayed. As the compliance of patients is influenced by pain, anxiety and stress, the execution of the clinical manoeuvres intended to identify a loss of motor function or sensibility, can be impaired. Although the majority of PNI are neuroapraxias resulting in spontaneous recovery, there are open questions regarding certain aspects of closed PNI, e.g. when to ask for electrophysiological exams, when and how long to wait for a spontaneous recovery and when a surgical approach becomes mandatory. The aim of the article is therefore to analyse the main aspects of the different closed PNI of the upper limb in order to provide recommendations for timely and correct management, and to determine differences in the PNI treatment between children and adults.

Scaphoid Fractures below the Age of 10: Case Series and Review of the Literature.

Background Scaphoid fractures are relatively uncommon in children, especially below the age of 10 due to the ossification of the scaphoid bone, which starts around 4 to 6 years of age and continues until 13 to 15 years of age, where pediatric scaphoid fractures peak. This makes the diagnoses challenging in this age group. Methods The primary aim of this study was to analyze prospectively collected data in managing scaphoid fractures. All cases in children up to the age of 10 years, treated in a tertiary pediatric hand and upper limb from January 2014 to June 2018 were included. Parameters studied were patient demographics, clinical presentation, mechanism of injury, investigations, type of fracture, associated injuries, treatment offered, outcomes and complications. The secondary aim was to review the literature due to the limited knowledge about these fractures in these low age groups. Results A total of 23 patients with documented scaphoid fractures in children up to the age of 10 years were found. Final diagnosis in all these patients was done with magnetic resonance imaging (MRI). The mean age was 9.8 years, with female preponderance. Scaphoid waist was the most common location. Five patients had associated fractures of the capitate and one patient had associated second metacarpal base fracture. All patients were managed nonoperatively. The average time of immobilization was 6.6 weeks (range: 4-10 weeks). A majority of patients had minor symptoms after the fracture, most likely due to the immobilization time. Conclusion Scaphoid fractures are rare in the pediatric population up to the age of 10. MRI is most often needed to confirm diagnosis. Nonoperative management of most scaphoid fractures in this age group is safe and feasible with no significant long-term morbidity. Surgical management of scaphoid fractures might be required in very selected cases. Level of Evidence This is a Level IV study.

Carpal tunnel syndrome in paediatric patients: A novel association with Kosaki overgrowth syndrome.

Carpal tunnel syndrome in a paediatric population is vanishingly rare and usually associated with lysosomal storage disorders such as mucopolysaccharidosis (MPS). Overgrowth syndromes similarly are rare and are characterised by increased skeletal growth alongside typical dysmorphic features and intellectual delay and as such the acronym OGID (overgrowth intellectual delay) is now widely used. Kosaki overgrowth syndrome (KOGS) is a newly recognised OGID with only 6 cases to date reported in the literature. Here we report a 7th case of KOGS with a new finding of carpal tunnel syndrome not previously described. We discuss similarities between the intraoperative findings during carpal tunnel decompression with findings seen in patients with MPS.

Kosaki overgrowth syndrome: A novel pathogenic variant in PDGFRB and expansion of the phenotype including cerebrovascular complications.

Heterozygous activating variants in platelet-derived growth factor, beta (PDGFRB) are associated with phenotypes including Kosaki overgrowth syndrome (KOGS), Penttinen syndrome and infantile myofibromatosis (IM). Here, we present three new cases of KOGS, including a patient with a novel de novo variant c.1477A > T p.(Ser493Cys), and the oldest known individual age 53 years. The KOGS phenotype includes characteristic facial features, tall stature, scoliosis, hyperelastic thin skin, lipodystrophy, variable intellectual and neurological deterioration, and abnormalities on brain imaging. Long-term outcome is unknown. Our cases confirm the phenotypic spectrum includes progressive flexion contractures, camptodactyly, widely spaced teeth, and constriction rings. We also propose novel occasional features including craniosynostosis, ocular pterygia, anterior chamber cleavage syndrome, early osteoporosis, increased pigmentation, recurrent haematomas, predisposition to cellulitis, nail dystrophy, carpal tunnel syndrome, recurrent hypoglycaemia in infancy, joint dislocation, and splenomegaly. Importantly, we report fusiform aneurysm of the basilar artery in two patients. Complications include thrombosis and stroke in the oldest reported patient and fatal rupture at the age of 21 in the patient with the novel variant. We conclude that cerebrovascular complications are part of the phenotypic spectrum of KOGS and KOGS-like disorders and suggest vascular imaging is indicated in these patients.

Surgical Transgastric Necrosectomy for Necrotizing Pancreatitis: A Single-stage Procedure for Walled-off Pancreatic Necrosis.

OBJECTIVE: The aim of this study was to evaluate the role of surgical transgastric necrosectomy (TGN) for walled-off pancreatic necrosis (WON) in selected patients. BACKGROUND: WON is a common consequence of severe pancreatitis and typically occurs 3 to 5 weeks after the onset of acute pancreatitis. When symptomatic, it can require intervention. METHODS: A retrospective review of patients with WON undergoing surgical management at 3 high-volume pancreatic institutions was performed. Surgical indications, intervention timing, technical methodology, and patient outcomes were evaluated. Patients undergoing intervention <30 days were excluded. Differences across centers were evaluated using a P value of <0.05 as significant. RESULTS: One hundred seventy-eight total patients were analyzed (mean WON diameter = 14 cm, 64% male, mean age = 51 years) across 3 centers. The majority required inpatient admission with a median preoperative length of hospital stay of 29 days (25% required preoperative critical care support). Most (96%) patients underwent a TGN. The median duration of time between the onset of pancreatitis symptoms and operative intervention was 60 days. Thirty-nine percent of the necrosum was infected. Postoperative morbidity and mortality were 38% and 2%, respectively. The median postoperative length of hospital length of stay was 8 days, with the majority of patients discharged home. The median length of follow-up was 21 months, with 91% of patients having complete clinical resolution of symptoms at a median of 6 weeks. Readmission to hospital and/or a repeat intervention was also not infrequent (20%). CONCLUSION: Surgical TGN is an excellent 1-stage surgical option for symptomatic WON in a highly selected group of patients. Precise surgical technique and long-term outpatient follow-up are mandatory for optimal patient outcomes.

Treatment and outcomes of arthrogryposis in the upper extremity.

Upper extremity involvement in patients with arthrogryposis multiplex congentia is quite frequent. Treatment initially consists of stretching and splinting as significant gains can be seen in the first years of life. The goal of any surgical procedure is to improve upper extremity function and performance of daily living activities, yet it is important to treat each patient individually and understand that areas do not always need to be addressed surgically. Despite overall lower functioning scores in this patient population, quality of life scores are comparable to the general aged adjusted population. This article will discuss the clinical presentation, treatment procedures and outcomes when addressing the upper extremities of patients presenting with arthrogryposis.

Recommendations for the management of MPS IVA: systematic evidence- and consensus-based guidance.

INTRODUCTION: Mucopolysaccharidosis (MPS) IVA or Morquio A syndrome is an autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the N-acetylgalactosamine-6-sulfatase (GALNS) enzyme, which impairs lysosomal degradation of keratan sulphate and chondroitin-6-sulphate. The multiple clinical manifestations of MPS IVA present numerous challenges for management and necessitate the need for individualised treatment. Although treatment guidelines are available, the methodology used to develop this guidance has come under increased scrutiny. This programme was conducted to provide evidence-based, expert-agreed recommendations to optimise management of MPS IVA. METHODS: Twenty six international healthcare professionals across multiple disciplines, with expertise in managing MPS IVA, and three patient advocates formed the Steering Committee (SC) and contributed to the development of this guidance. Representatives from six Patient Advocacy Groups (PAGs) were interviewed to gain insights on patient perspectives. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with experience managing patients with MPS IVA and the manuscript was evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers. RESULTS: A total of 87 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) disease-modifying interventions (enzyme replacement therapy [ERT] and haematopoietic stem cell transplantation [HSCT]); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions (including spinal, limb, ophthalmic, cardio-thoracic and ear-nose-throat [ENT] surgeries). Consensus was reached on all statements after two rounds of voting. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance). CONCLUSION: This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS IVA and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps.