Non-Surgical Strategies for Managing Skeletal Deformities in a Child with X-Linked Hereditary Hypophosphatemic Ricket: Insights and Perspectives.

This case report sheds light on the management of skeletal deformity in a young child with X-linked hypophosphatemia (XLH), emphasizing the significance of a timely orthotic intervention alongside pharmacological treatment, which is a strategy not frequently highlighted in the XLH literature. The patient, a 2-year-and-7-month-old female, presented with classic XLH symptoms, including short stature, pronounced genu varum, and hypophosphatemia, with deformities observed in both the coronal and sagittal planes of the femur and tibia. Despite initial reliance on pharmacotherapy, which proved insufficient for skeletal realignment, the integration of orthotic treatment at age 3 marked a pivotal turn in the management strategy. By the age of 5 years and 9 months, this combined approach yielded significant improvements: the deformities in the femur and tibia were notably corrected, tibial torsion was addressed, and enhanced limb alignment was achieved, as corroborated by radiographic evidence. This case underscores the effectiveness of orthotic intervention as a critical and underemphasized adjunct to pharmacological therapy in managing XLH in early childhood. It advocates for the early inclusion of orthotic measures to optimize treatment outcomes and expand the range of management strategies for limb deformities.

Demographics and medical burden of osteogenesis imperfecta: a nationwide database analysis.

The epidemiological data on osteogenesis imperfecta (OI) in Asia is limited. This study, representing the first comprehensive epidemiological investigation on OI in Taiwan, reveals high medical resource utilization and underscores the importance of early diagnosis to enhance care quality. INTRODUCTION: This study examines osteogenesis imperfecta, a hereditary connective tissue disorder causing pediatric fractures and limb deformities, using a nationwide database from Taiwan to analyze clinical features and medical burden. METHODS: The study identified validated OI patients from the Catastrophic Illness Registry in the National Health Insurance Research Database from 2008 to 2019. Demographic data and medical resource utilization were analyzed. A multivariate Cox model assessed the influence of sex, validation age, and comorbidities. RESULTS: 319 OI patients (M/F = 153/166) were identified, with 58% validated before age 20. Prevalence and incidence were 0.8-1.3/100,000 and 0.02-0.09/100,000, respectively, with higher rates in the pediatric demographic. In the study period, 69.6% of the patients had admission history, primarily to pediatric and orthopedic wards. The median admission number was 3, with a median length of stay of 12 days and a median inpatient cost of approximately 3,163 USD during the period. Lower limb fractures were the main reason for hospitalization. 57% of OI patients received bisphosphonate treatment. The leading causes of mortality were OI-related deaths, neurovascular disease, and cardiovascular disease. The median age of validation in the non-survival group was significantly higher compared to the survival group (33 vs. 14 years), and patients validated during childhood required more inpatient fracture surgeries than those validated during adulthood. CONCLUSION: This study provides comprehensive real-world evidence on the clinical characteristics and high medical resource utilization of OI patients in a low prevalence region like Taiwan. Early diagnosis is crucial for improving care quality and enhancing health outcomes.

The use of ultrasound for monitoring reduction and ulnar nerve subluxation in pediatric humeral supracondylar fractures.

BACKGROUND: Traditional treatment for displaced humeral supracondylar fractures (SCFs) in children involves closed reduction (CR) under fluoroscopic guidance, percutaneous pinning, and immobilization with a long-arm cast. This study aims to explore the viability of using radiation-free ultrasound (US) for guiding CR and tracking ulnar nerve dynamics during medial pinning, contrasting the US method with the conventional cross pinning technique. MATERIALS AND METHODS: We assessed 70 children with acute displaced SCFs. The US group (n = 30) underwent US-guided reduction, whereas the traditional group (n = 40) underwent fluoroscopy-guided reduction. Both groups received percutaneous cross pinning and subsequent cast immobilization. Postoperative outcomes were compared between the two methods after a 6-month follow-up. In the US group, ultrasonography assessed fracture displacement distances before and after CR. The angle at which the ulnar nerve relocated to the cubital tunnel during elbow extension was documented using real-time US monitoring during medial pinning. RESULTS: The US group demonstrated improved reduction accuracy, increased range of motion, superior restoration of both Baumann and Humeroulnar angles, and a decreased incidence of malunions compared to the traditional group (all p < 0.05). The ultrasonographic measurement of fracture displacement was comparable with that of fluoroscopy (intraclass correlation coefficient > 0.90). In the US group, no ulnar nerve injury was noted, compared to 2.5 % in the traditional group, and real-time US observations revealed ulnar nerve hypermobility, with 53.3 % of patients exhibiting anterior ulnar nerve subluxation at 120° elbow flexion, 40 % at 90°, 16.7 % at 60°, and none at 30° flexion. CONCLUSION: Ultrasound is as reliable as fluoroscopy for evaluating fracture reductions. The use of intra-operative ultrasound significantly improves reduction accuracy and radiographic outcomes while reducing the risk of ulnar nerve injury.

Determining the Optimal Treatment for Idiopathic Clubfoot: A Network Meta-Analysis of Randomized Controlled Trials.

BACKGROUND: Clubfoot, or congenital talipes equinovarus deformity, is a common anomaly affecting the foot in infants. However, clinical equipoise remains between different interventions, especially those based on the Ponseti method. The aim of this study was to examine the clinical outcomes of the various interventions for treating idiopathic clubfoot. METHODS: Searches of the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, Embase, Scopus, and CINAHL were conducted. Randomized controlled trials comparing different interventions, including the Ponseti method, accelerated Ponseti method, Ponseti method with botulinum toxin type A (Botox) injection, Ponseti method with early tibialis anterior tendon transfer (TATT), Kite method, and surgical treatment, were included. Network meta-analyses (NMAs) were conducted according to the PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses) reporting guidelines. The primary outcomes were the change in total Pirani score and maximal ankle dorsiflexion. Secondary outcomes were the number of casts, time in casts, and rates of tenotomy, total complications, relapse, adverse events, and additional required major surgery. RESULTS: Eleven randomized controlled trials involving 740 feet were included. According to the SUCRA (surface under the cumulative ranking curve)-based relative ranking, the Ponseti method was associated with the best outcomes in terms of Pirani score changes, maximal ankle dorsiflexion, number of casts, adverse events, and total complications, whereas the accelerated Ponseti method was associated with the best outcomes in terms of time in casts and tenotomy rate. Early TATT ranked best in terms of relapse rate. The Ponseti method with Botox injection was associated with the best outcomes in terms of the need for additional major surgery. CONCLUSIONS: The NMAs suggest that the Ponseti method is the optimal treatment overall, despite potential drawbacks such as longer time in casts and higher rates of tenotomy, relapse, and the need for additional surgery compared with other modified approaches. Therefore, clinicians should consider how treatments can be tailored individually. LEVEL OF EVIDENCE: Therapeutic Level I . See Instructions for Authors for a complete description of levels of evidence.

Impact of bilateral motion control shoes with outsole adjustment on gait asymmetry in individuals with mild leg length discrepancy.

BACKGROUND: Footwear adjustment is recommended in the management plan of leg length discrepancy. However, it is not clear how the outsole of motion control shoe adjustment influences trunk symmetry and walking performance. RESEARCH QUESTION: Does bilateral adjustment of the outsole affect trunk and pelvis symmetry and ground reaction force during walking in individuals with leg length discrepancy? METHODS: 20 mild leg length discrepancy participants were recruited into a cross-sectional study. All subjects performed a walking trial with their habitual shoes to determine the outsole adjustment. Four trials of walking were carried out in the order of unadjusted and bilateral adjusted motion control air-cushion shoes. Shoulder level differences and trunk and pelvic motion were assessed, while the ground reaction force at the heel strike was reported. A paired t-test was performed to compare the differences between the conditions with a significance level at p < 0.05. RESULTS: During walking trials, mild leg length discrepancy participants with the adjusted shoe displayed lower variations in the maximum shoulder height differences (p = 0.001) and trunk rotation angle (p = 0.002) than those with the unadjusted shoe. Also, there was a significant reduction in the vertical ground reaction force (p = 0.030), but not in the anteroposterior or mediolateral directions, during walking in the adjusted shoe condition compared to the unadjusted shoe condition. SIGNIFICANCE: The outsole adjustment of the bilateral motion control shoes can improve trunk symmetry while decreasing the ground impact at the heel strike. The study provides additional information to prescribe or recommend footwear adjustment to improve walking symmetry in leg length discrepancy participants.

Short-Term Effects of Genicular Artery Embolization on Symptoms and Bone Marrow Abnormalities in Patients with Refractory Knee Osteoarthritis.

PURPOSE: To evaluate the short-term outcomes of genicular artery embolization (GAE) for knee osteoarthritis (OA) with and without bone marrow lesion (BML) and/or subchondral insufficiency fracture of the knee (SIFK). MATERIALS AND METHODS: This single-institution prospective observational pilot study analyzed 24 knees in 22 patients with mild to moderate knee OA, including 8 knees without BML, 13 knees with BML, and 3 knees with both BML and SIFK. The area and volume of BMLs on magnetic resonance images were measured before and after GAE. Baseline and postoperative pain and physical function were assessed using the visual analog scale (VAS) and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC). RESULTS: GAE significantly reduced the BML area and volume 3 months after embolization in the knees with BML (both P < .0005). GAE significantly decreased the VAS scores at 3 and 6 months after embolization in patients without BML (both P = .04) and those with BML (both P = .01). GAE also lowered the WOMAC scores 3 months after embolization in patients without and with BML (P = .02 and P = .0002, respectively). However, GAE did not significantly alter the BML area and volume (both P = .25), VAS scores (P = 1.00), and WOMAC scores (P = .08) in patients with BML and SIFK at 3 months after GAE. CONCLUSIONS: This observational pilot study suggested that GAE effectively reduces the BML area and volume and improves pain and physical function in patients with knee OA accompanied by BML but is inefficacious in those with both BML and SIFK.

Jigless knotless internal brace technique for acute Achilles tendon rupture: a case series study.

PURPOSE: To mitigate the risk of poor wound healing and of infection associated with the open repair of Achilles tendon midsubstance ruptures, minimally invasive techniques have been developed. We report our preliminary results after reviewing our "jigless knotless internal brace technique." METHODS: Patients were placed in prone position and a transverse 3-cm incision was made proximal to the palpable ruptured end. The proximal ruptured end was pulled out, gently debrided, and sutured using Krackow locking loops. Percutaneous sutures were crisscrossed through the distal tendon stump and looped around the Krackow sutures over the proximal stump. The ipsilateral Krackow sutures and the contralateral crisscrossed sutures were subcutaneously passed through two mini-incisions over the posterior calcaneus tuberosity and seated at the tuberosity with two 4.5-mm knotless suture anchors. All patients underwent the same post-operative rehabilitation protocol and regular follow-ups for at least 1 year. RESULTS: We recruited 10 patients (mean age, 37.3 years) who scored 100 points on the American Orthopaedic Foot and Ankle Society (AOFAS) scale, and who returned to their preoperative exercise levels 1-year post-operatively with no complications. CONCLUSION: Our method is simple, effective, and requires no special tools. It might be a reliable option for Achilles tendon repair. LEVEL OF EVIDENCE: III.

The "Double Medial Malleolus": A New Physical Finding in Talocalcaneal Coalition.

PURPOSE: It has been the observation of the senior author that there is a bony fullness or "double medial malleolus" over the middle facet as a consistent finding with most talocalcaneal coalitions (TCC). To document this observation, we reviewed records and radiographs in 3 patient groups. METHODS: Part 1: retrospective chart review was completed for 111 feet to determine the clinical presence of a palpable "double medial malleolus." Part 2: computed tomography (CT) scans for evaluation of tarsal coalition or symptomatic flatfoot between January 2006 and December 2014 were retrospectively reviewed for the same cohort. Soft tissue thickness was measured as the shortest distance between bone and skin surface at both the medial malleolus and the middle facet/coalition. The volume of the middle facet or coalition was measured at their midpoint. These findings were compared among feet with TCC (n=53), calcaneonavicular coalition (CNC) (n=20), and flatfoot (n=38). RESULTS: Part 1-clinical: from medical records, 38 feet (34%) had documented record of a palpable medial prominence. Of the feet reviewed with a "double medial malleolus," all had TCC (no false positives or false negatives). Clinical and CT prominence demonstrated significant correlation (rs=0.519, P=0.001). Part 2-radiographic: CT observation of "double medial malleolus" is significantly associated with TCC (P<0.001). CT observation of double medial malleolus is 81% sensitive and 79% specific as a predictive test for TCC. The middle facet-to-skin distance was significantly closer in those with TCC versus controls (P<0.001). The ratio was larger in patients with TCC versus CNC (P=0.006) or flatfeet (P<0.001). Volume was nearly twice the size in patients with TCC versus the controls (P<0.001). CONCLUSIONS: TCCs have a bony prominence below the medial malleolus on clinical exam and CT scan not present in flatfeet or CNCs. This abnormal middle facet is almost twice the size of the normal middle facet. Obesity or severe valgus may mask this finding. If a palpable bony prominence is noted just below the medial malleolus during examination of a painful foot with a decrease in subtalar motion, the likely diagnosis is TCC. With this added clinical finding, appropriate images can be ordered to confirm the diagnosis of the latter. We advise CT scans with 3D images for surgical planning. The primary finding for tarsal coalitions in textbooks is decreased subtalar motion. This new finding of a palpable enlarged medial prominence just below the medial malleolus is highly associated with TCCs. LEVEL OF EVIDENCE: Level III.

Defining the lateral edge of the femoroacetabular articulation: correlation analysis between radiographs and computed tomography.

PURPOSE: The purpose of this study was to analyze the variation in measuring the lateral center edge angle of Wiberg (LCEA) using the lateral edge of the sourcil (LCEA-S) compared to the lateral edge of the acetabulum (LCEA-E), and to correlate these measurements with three-dimensional computed tomography (3D-CT)-based analysis of the femoroacetabular articulation. METHODS: A retrospective analysis was performed on 24 patients (45 hips) treated for hip dysplasia at a single institution. All patients were required to have an anteroposterior (AP) pelvis radiograph and pelvic CT. LCEA-S and LCEA-E measurements were calculated from radiographs. Axial CT images were processed to standardize pelvic orientation and calculate the LCEA at three points (posterior, central, anterior) along the acetabular edge. Correlation analysis was used to evaluate radiographic and CT measures. RESULTS: Eight males and 16 females with an average age of 14.6 years were included. The LCEA-S (16.5° ± 2.0°) was found to be significantly less than the LCEA-E (26.0° ± 2.0°) (p < 0.001). The LCEA-S had the greatest correlation with the central measurement on the 3D-CT (r s = 0.893; p < 0.001). The LCEA-E had the greatest correlation with the anterior measurement on the 3D-CT (r = 0.834; p < 0.001). CONCLUSIONS: The LCEA can change significantly depending on the bony landmark used to define the lateral edge of the femoroacetabular articulation. The edge of the sourcil most closely correlates with the central weight-bearing portion of the articular surface on the 3D-CT and should be used to define the LCEA when treating patients with hip dysplasia. LEVEL OF EVIDENCE: Level III, retrospective comparison study.

Fracture risk and correlating factors of a pediatric population with attention deficit hyperactivity disorder: a nationwide matched study.

The aim of this study was to investigate the risk of fracture and the difference between sexes from a nationwide database of fracture risk among children aged 4-17 years with or without attention deficit hyperactivity disorder (ADHD, ICD-9-CD codes 314). The Longitudinal Health Insurance Database (LHID 2000) was used to analyze fracture characteristics of children from the National Health Insurance that covered 96.1% of the Taiwanese population (N=21.4 million). A total of 7200 ADHD children aged between 4 and 17 years whose diagnosis had been confirmed in at least three outpatient clinics between 1 January 2000 and 31 December 2009 were included, and a cohort of 36 000 children without ADHD matched for age, sex, and urbanization was recruited for analysis. The incidence rate of fractures in ADHD children was 21.0 (95% confidence interval=19.4-22.7) per 1000 person-years, significantly (P<0.001) higher than 15.0 (95% confidence interval=14.4-15.6) in non-ADHDs. After adjusting by age, sex, urbanization level, and geographic region, the statistically significant (P<0.001) hazard ratios (HR) of fracture for ADHD children compared with non-ADHD children included 1.62 in girls and 1.38 in boys, 1.53 in the skull, neck, and trunk (ICD-9-CM 800-809), 1.28 in the upper extremity (ICD-9-CM 810-819), and 1.84 in the lower extremity (ICD-9-CM 820-829). The HR also (P<0.001) increased significantly in all age groups, including 1.35 in 4-6, 1.37 in 7-9, and 1.54 in 10-17 years. ADHD should be listed among risk factors of children's fractures in each sex, all age groups, and all body areas that the parents, teachers, caregivers of ADHD children, and pediatric orthopedists should be aware of. Besides, ADHD girls were more affected than ADHD boys, especially after 10 years of age, whereas the adjusted HR was the highest in the lower extremities. Nationwide analysis matched for age and sex showed that ADHD should be considered the risk factor of children's fracture, especially for girls older than 10 years of age.

Biomechanical analysis of knee and trunk in badminton players with and without knee pain during backhand diagonal lunges.

The contribution of core neuromuscular control to the dynamic stability of badminton players with and without knee pain during backhand lunges has not been investigated. Accordingly, this study compared the kinematics of the lower extremity, the trunk movement, the muscle activation and the balance performance of knee-injured and knee-uninjured badminton players when performing backhand stroke diagonal lunges. Seventeen participants with chronic knee pain (injured group) and 17 healthy participants (control group) randomly performed two diagonal backhand lunges in the forward and backward directions, respectively. This study showed that the injured group had lower frontal and horizontal motions of the knee joint, a smaller hip-shoulder separation angle and a reduced trunk tilt angle. In addition, the injured group exhibited a greater left paraspinal muscle activity, while the control group demonstrated a greater activation of the vastus lateralis, vastus medialis and medial gastrocnemius muscle groups. Finally, the injured group showed a smaller distance between centre of mass (COM) and centre of pressure, and a lower peak COM velocity when performing the backhand backward lunge tasks. In conclusion, the injured group used reduced knee and trunk motions to complete the backhand lunge tasks. Furthermore, the paraspinal muscles contributed to the lunge performance of the individuals with knee pain, whereas the knee extensors and ankle plantar flexor played a greater role for those without knee pain.

How does knee pain affect trunk and knee motion during badminton forehand lunges?

Badminton requires extensive lower extremity movement and a precise coordination of the upper extremity and trunk movements. Accordingly, this study investigated motions of the trunk and the knee, control of dynamic stability and muscle activation patterns of individuals with and without knee pain. Seventeen participants with chronic knee pain and 17 healthy participants participated in the study and performed forehand forward and backward diagonal lunges. This study showed that those with knee pain exhibited smaller knee motions in frontal and horizontal planes during forward lunge but greater knee motions in sagittal plane during backward lunge. By contrast, in both tasks, the injured group showed a smaller value on the activation level of the paraspinal muscles in pre-impact phase, hip-shoulder separation angle, trunk forward inclination range and peak centre of mass (COM) velocity. Badminton players with knee pain adopt a more conservative movement pattern of the knee to minimise recurrence of knee pain. The healthy group exhibit better weight-shifting ability due to a greater control of the trunk and knee muscles. Training programmes for badminton players with knee pain should be designed to improve both the neuromuscular control and muscle strength of the core muscles and the knee extensor with focus on the backward lunge motion.

Arthroscopically assisted percutaneous fixation of displaced midclavicular fracture using a Knowles pin.

Clavicle fractures are common traumatic injuries of the shoulder girdle, with surgery being the recommended treatment for some displaced midshaft fractures. Open surgery is widely used; however, the primary concerns of this procedure include cosmetic problems and incisional complications, such as wound dehiscence, infection, numbness, and sensitivity. A new arthroscopically assisted technique was developed, in which the Knowles pin was used for percutaneous fixation to treat the displaced midclavicular fracture. This technique is an attractive alternative treatment option for midclavicular fractures.

Mesh Achilles tendon lengthening--a new method to treat equinus deformity in patients with spastic cerebral palsy: surgical technique and early results.

Equinus of the ankle is a common deformity in spastic cerebral palsy. Many methods have been developed to lengthen the Achilles tendon to correct the deformity. A new mesh Achilles tendon lengthening (ATL) procedure that might decrease immobilization and promote recovery was performed in 36 tendons with equinus deformity (22 patients, average age=6.2). The results were compared with those of two other methods: the Vulpius group and the Z-lengthening group. The corrected dorsiflexion angle of the ankle at a subsequent 2-year follow-up of the mesh ATL and Vulpius groups matched (25.5±3.0 and 27.1±3.5°, respectively), whereas that of the Z-lengthening group was higher (33.9±3.8°). Nevertheless, statistics of the timing of each patient's readiness to begin rehabilitation and walking as well as gaining better stability for running and one-legged hopping indicated that the mesh ATL group recovered significantly quicker than the Vulpius and Z-lengthening groups. The mesh ATL procedure achieves a successful correction of the equinus deformity in spastic cerebral palsy comparable with that of the Vulpius procedure, with the advantage of preserving the gastrocnemius without a complete section. This confers greater antigravity stability and quicker recovery in patients.

Synovial osteochondromatosis of the second metatarsophalangeal joint: a case report.

Synovial osteochondromatosis is usually monoarticular, involving a large joint. Common locations include the knee, elbow, shoulder, and hip. It is not very common in the ankle, and it is very rare in the smaller joints of the foot. To our knowledge, and with the exception of 4 cases that occurred in the great toe, this condition has never been described in other metatarsophalangeal joints. In this report, we presented a case of synovial osteochondromatosis in the second metatarsophalangeal joint and reviewed the literature.

Snapping hip caused by a venous hemangioma of the gluteus maximus muscle: a case report.

INTRODUCTION: Snapping hip, or coxa saltans, is defined as a clinical condition where a usually painful, audible snap occurs during hip flexion and extension. Its causes can be divided into external, internal or intra-articular origin. Accurate diagnosis is a prerequisite to successful treatment. We report a rare cause of snapping hip which is different from any previously reported cases. CASE PRESENTATION: A 23-year-old man presented to us with right hip pain of more than 10 years duration. Atrophy of the right gluteus maximus with snapping and tenderness were also noted. The imaging study revealed a focal intramuscular lesion in the lateral portion of the right gluteus maximus muscle. Surgery was performed and pathological examination concluded this mass to be a venous hemangioma. CONCLUSION: Intramuscular hemangioma, though rare, should be considered in the differential diagnosis of a snapping hip even though muscle fibrosis is most frequently encountered.