Transition of Caregiver Perceptions after Pediatric Neuromuscular Scoliosis Surgery.

Introduction: Spinal fusion for children with neuromuscular scoliosis has been known to improve sitting balance and quality of life as well as for high caregiver satisfaction. However, most studies performed were single surveys, and it remains unclear whether high satisfaction levels are maintained. Thus, in this article, we report the short- and medium-term improvements in caregiver standing assessment after neuromuscular scoliosis surgery in children with Gross Motor Function Classification System (GMFCS) level IV or V. Methods: In total, 18 patients with GMFCS levels IV and V were included in this study. The underlying diseases were typical cerebral palsy in 12 cases, chromosomal abnormalities in 5 cases, and congenital myopathy in 1 case. The median age at the time of surgery was 14.5 years. The medians for the first and second follow-up surveys were after 1.4 and 5.9 years, respectively. All the patients had undergone posterior spinal fusion, whereas 12 had undergone pelvic fixation. These patients were assessed using a caregiver questionnaire, in addition to patient demographic data and radiographic assessments. Results: The median BMI was 15.4 kg/m2 preoperatively, 16.6 kg/m2 at the first survey, and 17.1 kg/m2 at the second survey. The main Cobb angles were 97.5°, 36.5°, and 37.0° and the spino-pelvic obliquity angles were 22.5°, 6.0°, and 6.5° preoperatively, at the first survey and at the second survey, respectively. In the questionnaire, most domains were rated similarly in the first and second surveys, but the ratings for the "children's QOL" and "digestion and defecation" domains were noted to increase, while that for the "transfer" and "satisfaction with treatment" domains have decreased. Conclusions: Neuromuscular scoliosis surgery in children has been associated with extremely high treatment satisfaction in the early postoperative period. However, some caregivers showed a decline in the "transfer" and "treatment satisfaction" domains over time.

A functional learning health system in Japan: Experience with processes and information infrastructure toward continuous health improvement.

Introduction and definition of the term Learning Health System (LHS) appears to have occurred initially around 2007. Prior to this and the introduction of electronic health records (EHR), a predecessor could be found in the Clinical Pathways concept as a standard medical care plan and a tool to improve medical quality. Since 1997, Japan's Saiseikai Kumamoto Hospital (SKH) has been studying and implementing Clinical Pathways. In 2010, they implemented EHR, which facilitated the collection of structured data in common templates that aligned with outcome measurements defined through Japan's Society of Clinical Pathways. For each patient at this hospital, variances from the desired outcomes have been recorded, producing volumes of structured data in formats that could readily be aggregated and analyzed. A visualization tool was introduced to display graphs on the home page of the EHR such that each patient can be compared to similar patients. Knowledge learned from patient care is shared regularly through Clinical Pathways meetings that are supported by all staff within the hospital. The SKH experience over the past two decades is worth exploring further in the context of the development of a fully functional LHS and the attributes/characteristics thereof. In this report, the SKH experience and processes are compared with previously published attributes of a fully functional LHS (ie, characteristics of an LHS that can indicate maturity). Specific examples of the SKH system are detailed with respect to leveraging knowledge gained to change performance that improves patient care as prescribed by learning health cycles. The SKH experience and its information infrastructure and culture exemplify a functional LHS, which is now being expanded to additional hospitals with the hope that it can be scaled and serve as a solid platform for measures aimed at improving medical care, thus establishing broader and more global learning health systems.

Impact of a learning health system on acute care and medical complications after intracerebral hemorrhage.

INTRODUCTION: Patients with stroke often experience pneumonia during the acute stage after stroke onset. Oral care may be effective in reducing the risk of stroke-associated pneumonia (SAP). We aimed to determine the changes in oral care, as well as the incidence of SAP, in patients with intracerebral hemorrhage, following implementation of a learning health system in our hospital. METHODS: We retrospectively analyzed the data of 1716 patients with intracerebral hemorrhage who were hospitalized at a single stroke center in Japan between January 2012 and December 2018. Data were stratified on the basis of three periods of evolving oral care: period A, during which conventional, empirically driven oral care was provided (n = 725); period B, during which standardized oral care was introduced, with SAP prophylaxis based on known risk factors (n = 469); and period C, during which oral care was risk-appropriate based on learning health system data (n = 522). Logistic regression analysis was performed to evaluate associations between each of the three treatment approaches and the risk of SAP. RESULTS: Among the included patients, the mean age was 71.3 ± 13.6 years; 52.6% of patients were men. During the course of each period, the frequency of oral care within 24 hours of admission increased (P < .001), as did the adherence rate to oral care ≥3 times per day (P < .001). After adjustment for confounding factors, a change in the risk of SAP was not observed in period B; however, the risk significantly decreased in period C (odds ratio 0.61; 95% confidence interval 0.43-0.87) compared with period A. These associations were maintained for SAP diagnosed using strict clinical criteria or after exclusion of 174 patients who underwent neurosurgical treatment. CONCLUSIONS: Risk-appropriate care informed by the use of learning health system data could improve care and potentially reduce the risk of SAP in patients with intracerebral hemorrhage in the acute stage.

Characteristic Reconstitution of the Spinal Langerhans Cell Histiocytosis in Young Children.

BACKGROUND: Pediatric Langerhans cell histiocytosis (LCH) often results in vertebral compression fracture. However, few reports have reported vertebral remodeling during the course of LCH. We aimed to investigate the longitudinal reconstitution and transformation of the affected vertebrae and the adjacent structures in young children with spinal LCH. METHODS: We recruited 13 patients, including 16 affected vertebrae, diagnosed with LCH via biopsy. The average age at first visit was 3.6 years. The average follow-up period was 10.2 years. Vertebral lesions involved L2 in 3 cases; T12, L1, or L5 in 2 cases; and C4, C5, C7, T5, T8, T9, or L3 in 1 case. We measured the ratios of the height of the affected vertebra and 1 vertebra above the affected one to that of the second vertebra above the affected one, local kyphotic angles, and the ratio of the height of the center of the adjacent disk to that of one disk above it. RESULTS: The collapse of the affected vertebra was most severe after 1 year of disease onset. The rate of reconstitution accelerated at 2 years or later of disease onset. The recovery speed of the anterior wall was faster than that of the center height. While the height of the affected vertebrae was restored, the thickness of the adjacent disk also increased. Further, the height of the adjacent vertebrae increased in a similar manner. The average local kyphosis angle shifted to lordosis within the first 3 years. CONCLUSIONS: The heights of not only the disk but also the adjacent vertebra increased during the vertebral collapse phase in pediatric spinal LCH patients. These transformations may affect the realignment of the sagittal spinal balance at the earlier stage of the disease. During the collapse phase, the heights of the adjacent vertebrae and disks increase but after the affected vertebrae reconstituted, the augmentation of adjacent vertebrae and disks diminished. LEVEL OF EVIDENCE: Level IV.

Bone metabolism and inflammatory characteristics in 14 cases of chronic nonbacterial osteomyelitis.

BACKGROUND: Chronic nonbacterial osteomyelitis (CNO) is a multifocal autoinflammatory disease that often impairs daily life in children. This study aimed to investigate the bone metabolic and inflammatory characteristics of patients with CNO, and to assess the differences between responders and nonresponders to conservative treatment. METHODS: We investigated the clinical symptoms; laboratory data including inflammatory and bone metabolic biomarkers; and imaging findings from plain radiography, magnetic resonance imaging (MRI), fluorodeoxyglucose-positron emission tomography (FDG-PET), and dual-energy x-ray absorption (DEXA) in 14 patients with CNO. All patients underwent first-line treatment comprising systemic nonsteroidal anti-inflammatory drugs with or without bisphosphonate. According to the response to the first-line treatment, the patients were divided into the clinical remission/partial response group and the no response group. The differences in bone metabolic and inflammatory characteristics between the two groups were assessed. RESULTS: All patients had low bone mineral density assessed with DEXA. The bone metabolic biomarkers (bone-specific alkaline phosphatase and tartrate-resistant acid phosphatase 5b) were increased in boys of all ages and in young girls. Multiple inflammatory regions were detected in all patients by using FDG-PET including asymptomatic regions. The no response group had higher immunoglobulin G (IgG) and a greater number of bone inflammatory lesions detected on MRI than the clinical remission/partial response group. CONCLUSION: Our data indicate the involvement of abnormal bone turnover, necessity of whole-body scanning, and association of higher serum IgG levels and greater numbers of inflammatory lesions with prolonged disease activity in patients with CNO.

Management of foot deformity in children.

This study describes the management of foot deformity in children. Severe congenital clubfeet treated using posteromedial release without talocalcaneal joint release were flexible and functional. Talectomy may be necessary for congenital clubfeet with arthrogryposis multiplex congenita. The diagnosis and severity of vertical talus were defined based on stress radiographs. For the deformity with spina bifida, a combination of talocalcaneal joint fusion and precise correction by soft tissue release and tendon transfer was performed. This combined surgery is effective, particularly in patients with equino-varus feet.

Scoliosis surgery for handicapped children.

INTRODUCTION: This study aimed to assess treatment outcomes and caregivers' satisfaction regarding scoliosis surgery for handicapped children. METHODS: Handicapped children are, by definition, noncommunicatory and/or nonambulatory. We recruited 26 handicapped children who were followed-up for >1 year after a scoliosis surgery. We recruited 40 patients with adolescent idiopathic scoliosis (AIS) who underwent a surgery during the same period as controls. We used a posterior approach in all the children. We determined preoperative body mass index (BMI), main Cobb angle, Cincinnati correction index (CCI), and fusion level; intraoperative time and blood loss per level; and postoperative complications. We also assessed caregivers' satisfaction with surgical treatments for these patients using the modified Bridwell's questionnaire. RESULTS: We have described the results as handicapped children/AIS. Median preoperative BMI was 16.1/18.6 kg/m2. Preoperative and final Cobb angles were 94.2°/59.7° and 39.7°/17.0°, respectively and CCI was 2.0/1.7. The number of fusion levels was 14.6/9.0. The operative time and blood loss per level were 40.1/44.1 minutes and 264/138 ml, respectively. Postoperative complications in handicapped children were adynamic ileus in 8 cases, dysphagia in 5, pneumonia in 3, urinary tract infection in 2, and superior mesenteric artery syndrome (SMA), surgical site deep infection, infectious enteritis, agitation, and liver dysfunction in 1 each. However, in the AIS group, there was only 1 case of SMA. Median caregivers' satisfaction score on the 0-10 visual analog scale was 9. Caregivers for 19 of the 26 handicapped cases (73%) recommended surgical treatment to caregivers of other children with the same disease. CONCLUSIONS: Surgical treatment for neuromuscular and syndromic scoliosis was associated with a high rate of postoperative complications. However, the caregivers' satisfaction score after surgery was high.

Giant cell tumour of the femur in a 9-year-old girl, resulting in severe leg length discrepancy.

Giant cell tumour of bone (GCTB) is a rare benign tumour noted before physeal closure. GCTB cases in patients younger than 10 years are especially uncommon. The authors report the case of a 9-year-old girl with a GCTB of the distal femur. After initial intralesional excision and curettage of the tumour, local recurrence occurred, which needed two more additional surgeries to treat. The primary tumour and the local relapse invaded the growth plate of the right distal femur, leading to growth disturbance of the femur and requiring leg lengthening using Ilizarov apparatus. Surgeons should note the possibility of this rare condition while treating radiographic lytic lesions even in skeletally immature patients. The occurrence of late limb length discrepancy would be more likely in younger patients.

Use of glenoid inclination angle for the assessment of unilateral congenital high scapula.

Little has been reported on the motion of the scapula in Sprengel's deformity. Fourteen patients with unilateral congenital high scapula underwent the Woodward procedure from 1986 to 2004. The median age of the patients at the time of surgery was 4.4 years and the median follow-up duration was 8.8 years. The range of motion of the shoulder and the glenoid inclination angle (GIA) were measured preoperatively and at the final visit. The range of motion of the shoulder and GIA improved at the final visit. There was a positive correlation between the maximum abduction angle of the upper limb and GIA at the final visit.

Polyostotic osteolysis and hypophosphatemic rickets with elevated serum fibroblast growth factor 23: A case report.

We report on a boy who presented with hypophosphatemic rickets with elevated serum fibroblast growth factor 23 (FGF23) and polyostotic osteolytic lesions at age 2 years. Tumor-induced hypophosphatemic rickets was suspected; however, bone biopsy for osteolytic changes revealed no tumorous change, except for irregularly dilated vessels associated with osteoclasts and fibrous proliferation. Venous sampling failed to point to FGF23-producing foci. After alfacalcidol and phosphate supplementation, the rachitic skeletal changes improved, but FGF23 increased and new osteolytic lesions developed. Serum levels of neopterin and a few cytokines, including plasma transforming growth factor-ß and soluble tumor necrosis factor receptor type II, were elevated. At age 4 years, high doses of phosphate resulted in increased serum phosphate levels, decreased neopterin and cytokines, decreased FGF23, and stabilization of osteolysis. We excluded germline mutations in PHEX, FGF23, DMP1, and ENPP1 (genes for hereditary hypophosphatemic rickets) and somatic mutations in the GNAS and HRAS/KRAS (the disease-causing genes for McCune-Albright syndrome and linear nevus sebaceous syndrome, respectively). We could not perform octreotide scintigraphy or fluorodeoxyglucose-positron emission tomography, and thus could not completely exclude occult FGF23-producing tumors. However, considering the course of the disease, it is intriguing to assume that dysregulation of osteoclast-macrophage lineage may have induced increased neopterin levels, increased cytokine levels, osteolytic process, and possibly FGF23 overproduction.

Rotational open-wedge osteotomy improves treatment outcomes for patients older than eight years with Legg-Calve-Perthes disease in the modified lateral pillar B/C border or C group.

PURPOSE: Since 2003, we have been performing rotational open-wedge osteotomy (ROWO) for older children with severe Legg-Calve-Perthes disease (LCPD). We compared the treatment outcomes before and after the introduction of ROWO for patients aged ≥ eight years, classified in the modified lateral pillar B/C border or C group. METHODS: Before the introduction of ROWO, conservative treatment (non-weight bearing [NWB]) was performed for all cases between 1986 and 2002, whereby there were 34 hips (NWB group). The median patient age, at the first visit, was 9.1 years. According to the lateral pillar classification, 21 hips were categorized into the B/C border group and the others into the C group. Meanwhile, 14 consecutive hips underwent ROWO combined with NWB between 2003 and 2008 (ROWO+NWB group). The median patient age, at the first visit, was 9.1 years. Six hips were in the lateral pillar B/C border group and the others were classified into the C group. There were no significant differences in pre-treatment patients' demographic data. RESULTS: At the latest follow-up, according to the Stulberg classification, there were 19 class II hips, 13 class III hips, and two class IV hips in the NWB group. In the ROWO+NWB group, there were 12 class II hips, two class III hips, and no class IV hips. CONCLUSIONS: The application of ROWO combined with NWB in the treatment of older children with severe LCPD improved their outcomes, compared to those obtained by NWB treatment alone.

Novel 2 radiographical measurements for atlantoaxial instability in children with Down syndrome.

STUDY DESIGN: A retrospective case-control study. OBJECTIVE: To assess the usefulness and reproducibility of 2 novel safe and simple radiographical measurements for atlantoaxial instability in children with Down syndrome. SUMMARY OF BACKGROUND DATA: In pediatric atlantoaxial instability, early diagnosis is important for improved outcomes because of poor postoperative recovery in progressed neurological symptoms. Conventional dynamic radiography of the cervical spine is associated with a potential risk of worsening neurological symptoms. METHODS: We retrospectively reviewed the medical records of 50 patients (24 boys and 26 girls) with atlantoaxial instability associated with Down syndrome. Of the 50 children, 11 had undergone and 4 had been scheduled for surgery (surgical group). In this investigation, in addition to the atlas-dens interval (ADI) and space available for spinal cord (SAC), we measured C1 inclination angle and C1/4 SAC ratio on lateral radiographs of the cervical spine in the neutral position. To assess the diagnostic abilities of these indices to determine indication for surgery, receiver operating characteristic analysis of each index was performed, and their diagnostic abilities were compared using the area under the receiver operating characteristic curve. Moreover, we assessed reproducibility of our 2 proposed indices. RESULTS: The discriminatory abilities of C1/4 SAC ratio (area under the receiver operating characteristic curve, 1.00) and C1 inclination angle (0.91) were comparable with those of ADI (0.98) and SAC (0.95). For the interobserver and intraobserver reliability of the novel indices, the correlation coefficients were in the range from 0.88 to 0.99. Correlation was observed between the ADI and C1/4 SAC ratio (r = 0.507, P < 0.01) and between the ADI and C1 inclination angle (r = 0.407, P < 0.01). CONCLUSION: The diagnostic abilities of the 2 novel radiographical measurements were comparable with those of ADI and SAC. Moreover, these novel measurements can be obtained safely on lateral radiographs of the cervical spine in the neutral position. LEVEL OF EVIDENCE: 4.

Flexibility of idiopathic congenital clubfeet treated by posteromedial release without talocalcaneal joint release.

We retrospectively reviewed 57 patients with 84 idiopathic clubfeet and followed them up for at least 15 years. All feet were rated as good or excellent according to the International Clubfoot Study Group score. Stress radiographs showed that the arc of forefoot motion of conservatively treated feet was 81%, in operated feet of patients aged more than 1 year it was 74%, and in feet of patients aged less than 1 year during surgery it was 65%, compared with those in contralateral normal feet. Despite the severity of the deformity, feet treated by our posteromedial release without talocalcaneal joint release were flexible and functional.

Use of dynamic spinal brace in the management of neuromuscular scoliosis: a preliminary report.

Neuromuscular scoliosis is difficult to treat with braces because the collapsing trunk with the spinal deformity cannot tolerate the hard materials used for most orthoses. The dynamic spinal brace (DSB) is a novel three-point support brace used in Japan. We present our preliminary findings of 52 pediatric patients with neuromuscular scoliosis treated using DSBs. A positive correlation was found between the Cobb angle at the initiation of bracing and the degree of scoliosis progression. We concluded that DSBs may be effective for early-stage scoliosis. We also found that DSB improved sitting stability and thereby caregiver satisfaction.

Use of real-time polymerase chain reaction for the diagnosis of infection and differentiation between gram-positive and gram-negative septic arthritis in children.

BACKGROUND: Diagnosis and identification of the etiological agent of septic arthritis (SA) in children is an important issue, as early treatment based on accurate diagnosis of joint infections can prevent potentially disabling complications. The purpose of this study was to evaluate the efficacy of real-time polymerase chain reaction (PCR) for the diagnosis of SA in children. PATIENTS AND METHODS: Twenty children with suspected SA who had joint pain and underwent surgical treatment were enrolled in this study. Their preoperative clinical and laboratory findings were investigated. Tissues obtained during operation were subjected to microbiological culture and real-time PCR, including methicillin-resistant Staphylococcus (MRS)-specific PCR and broad range universal PCR. Infection was confirmed if the result of microbiological culture was positive. Furthermore, abnormal clinical and laboratory findings and improvement in the symptoms and posttreatment data were also defined as the final diagnosis of infection. RESULTS: Out of the 20 patients, 19 were diagnosed with the infection. The remaining patient was postoperatively diagnosed with juvenile idiopathic arthritis. Abnormal preoperative body temperatures, white blood cell counts, C-reactive protein levels, and erythrocyte sedimentation rates were observed in 6, 9, 15, and 12 cases, respectively. The results of microbiological culture, MRS-PCR, and universal PCR were positive in 9, 2, and 15 cases, respectively. Analysis of the melting peak in universal PCR revealed that of the 15 cases, 10 had gram-positive and 5 had gram-negative infections. The sensitivity and specificity for the diagnosis of SA were, respectively, 0.47 and 1.00 in microbiological culture and 0.79 and 1.00 in real-time PCR. CONCLUSIONS: Successful diagnosis of infection and differentiation between gram-positive and gram-negative bacteria were achieved using MRS-PCR and universal PCR. Hence, real-time PCR is useful and has greater sensitivity than microbial culture for diagnosing SA in children. LEVEL OF EVIDENCE: Level II diagnostic study investigating a diagnostic test.

Scurvy in a child with autism: magnetic resonance imaging and pathological findings.

We present a case of scurvy in a 6-year-old boy with autism and an unbalanced diet. The patient was admitted with difficulties in walking. Magnetic resonance imaging findings of the thigh showed diffuse signal abnormality in the bone marrow, periosteum, and the femoral muscle. A biopsy specimen of the femur showed hematoma, proliferative fibroblasts, and few collagen fibers, which suggested a deficiency of vitamin C. Although recurrent periosteal hematoma may be suggestive of scurvy, this finding was subtle in the current case. It is important to be aware of this rare disease because it is easily cured with vitamin C supplementation.

Surgical and oncologic outcomes of laparoscopic partial nephrectomy: a Japanese multi-institutional study of 1375 patients.

BACKGROUND AND PURPOSE: Despite clear trends toward minimally invasive surgery, information about laparoscopic partial nephrectomy (LPN) in Japan is sparse. We conducted a retrospective survey to clarify time trends for LPN and analyze surgical and oncologic outcomes. PATIENTS AND METHODS: A nationwide survey was performed. Between 1998 and 2008, 1375 patients underwent LPN at 54 institutions. Complications, patterns of tumor recurrence, and recurrence-free survival were analyzed. RESULTS: Renal pedicle clamping was used in 1031 (75%) cases, and renal cooling was performed in 64%. Median warm/cold ischemic time was 37/53 minutes. Median tumor size was 2.26 cm (interquartile range 1.6 to 2.7). Multivariate analysis identified total operative time, operative blood loss, and surgical margin status as independently associated with high grade (grade 3-5) urologic and nonurologic complications. Despite increases in central tumor, a trend was seen toward shorter warm/cold ischemic time in recent cases, and the overall complication rate did not change throughout the study period. With a median follow-up of 26 months for 1193 malignancies, recurrence occurred in 22 (1.7%) patients, including local recurrence in 7 (0.5%), lung in 8 (0.7%), lymph nodes in 2 (0.1%), and bone in 4 (0.3%). Of the 26 cases with positive surgical margins, local tumor recurrence occurred in only one. CONCLUSIONS: This is the first nationwide survey of LPN in Japan to be reported. LPN could be performed with acceptable positive margins and complication rates. Most tumor recurrences occur as metastases, and surgical margin status appears to have little impact on oncologic outcomes.

Surgery for foot deformity in patients with congenital myopathy (multicore disease, congenital fiber-type disproportion, and centronuclear myopathy).

Foot deformities associated with congenital myopathy are rarely treated surgically; therefore, few reports are available. We present cases of three patients who underwent surgery for foot deformities caused by congenital myopathy. Posteromedial release without talocalcaneal joint release is our surgical procedure. Surgical procedures in each case were determined on the basis of radiographic findings. Surgery resulted in plantigrade feet in the three patients, all of whom were able to walk without pain more than 3 years postoperatively.

Foot anomalies in Antley-Bixler syndrome: three case reports.

Multiple joint contractures, including radiohumeral synostosis, are the hallmark of Antley-Bixler syndrome (ABS). The detailed description of the skeletal aberration, however, focused in feet is scarce. We carried out the scrutiny for foot lesion in three ABS patients with POR (nicotinamide adenine dinucleotide phosphate-oxidase-cytochrome P450 oxidoreductase) gene mutations, one of whom had undergone surgical intervention for difficulty in walking. Radiographs in all three patients showed middle cuneiform-second metatarsal synostosis and the fourth brachymetapody, irrespective of the severity of their systemic manifestations. In addition, talocalcaneal synostosis, lateral cuneiform-cuboid synostosis, defects of middle phalanx, and distal phalanx-middle phalanx synostosis were found in at least two patients. In conclusion, we found distinctive constellations of foot abnormalities in the patients of ABS with POR gene mutation, which may be useful in planning the treatment strategy, as well as in the diagnostic process.