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No previous research papers have reported a comparative survey of local radiologic diagnoses and central review in children with hepatoblastoma.To evaluate the utility of central review of children with hepatoblastoma enrolled in a clinical trial.The study included 91 children enrolled in a clinical trial conducted by the Japanese Study Group for Pediatric Liver Tumor. We compared the results of the initial pre-treatment extent of tumor (PRETEXT) disease staging performed at local sites with the results obtained on central review to determine the concurrence rates for tumor staging and additional criteria.The concurrence rate for PRETEXT staging was 70%. As the stage increased, the concurrence rate decreased. Using additional criteria, central review identified 143 lesions (157.1%), about 1.8 times higher than the number identified for the local site diagnoses. The additional criterion found most often on central review was "multifocal lesion" (n=19). The concurrence rate for lung metastases was high. However, our central review found many false-positive assertions of hepatic vein lesions, portal vein invasion and extrahepatic lesions among the local site diagnoses.In a clinical trial of hepatoblastoma, central review provided a more precise diagnosis than local site diagnoses with respect to severe PRETEXT stages III and IV cases and other cases including hepatic and portal vein invasion. The central review process appears to be effective and essential for improving the quality of clinical trials.

Liquid biopsy, a method of detecting genomic alterations using blood specimens, has recently attracted attention as a noninvasive alternative to surgical tissue biopsy. We attempted quantitative analysis to detect amplification of MYCN (MYCNamp) and loss of heterozygosity at 11q (11qLOH), which are clinical requisites as prognostic factors of neuroblastoma (NB). In this study, cell-free DNA (cfDNA) was extracted from plasma samples from 24 NB patients at diagnosis. Copy numbers of MYCN and NAGK genes were quantitatively analyzed by droplet digital PCR (ddPCR). 11qLOH was also assessed by detecting allelic imbalances of heterozygous single nucleotide polymorphisms in the 11q region. The results obtained were compared to those of specimens from tumor tissues. The correlation coefficient of MYCN copy number of cfDNA and tumor DNA was 0.88 (p 0.00001). 11qLOH was also accurately detected from cfDNA, except for one case with localized NB. Given the high accuracy of liquid biopsy, to investigate components of cfDNA, the proportion of tumor-derived DNA was estimated by examining the variant allele frequency of tumor-specific mutations in cfDNA. The proportion of tumor-derived DNA in cfDNA was 42.5% (range, 16.9%-55.9%), suggesting sufficient sensitivity of liquid biopsy for NB. In conclusion, MYCN copy number and 11qLOH could be quantitatively analyzed in plasma cfDNA by ddPCR assay. These results suggest that plasma cfDNA can be substituted for tumor DNA and can also be applied for comprehensive genomic profiling analysis.

To propose reference values for air-kerma at the reference point (Ka,r), air-kerma area product (PKA), fluoroscopy time (FT) and number of cine images (CI) for four age groups in Japan, a nationwide questionnaire was posted to 132 pediatric catheterisation of certified facility in Japan, using the conventional post system, to which 43 facilities responded. For diagnostic cardiac angiography, reference values were as follows: Ka,r: 86, 102, 165 and 264 mGy; PKA: 9.3, 9.5, 16 and 34 Gy.cm2; FT: 33, 29, 26 and 30 min and CI: 1904, 1966, 2405 and 1871 images. For therapeutic cardiac angiography, reference values were as follows: Ka,r: 107, 163, 103 and 202 mGy; PKA: 7.5, 18, 7 and 24 Gy.cm2; FT: 56, 52, 42 and 30 min and CI: 3886, 3232, 2212 and 4316 images for less than 1, 1–5, 6–10 and 11–15 y, respectively. To optimal patient exposure from diagnostic and therapeutic cardiac catheterisation, it is therefore necessary to establish reference values for pediatric cardiac catheterisation examinations for four age groups.

Purpose The present study introduced the half-dose method (HDM), which halves the radiation dose for conventional head computed tomography (CT), for postoperative hydrocephalus and follow-up for craniosynostosis at a children’s hospital. This study aimed to evaluate the contribution of selective head CT scanning optimization towards the overall reduction of radiation exposure. Materials and methods We retrospectively assessed 1227 and 1352 head CT examinations acquired before and after the introduction of the HDM, respectively, in children aged 0–15 years. The radiation exposure was evaluated using the CT dose index volume (CTDI-vol), dose-length product (DLP), rate of HDM introduction, and effect of reducing in-hospital radiation dose before and after the introduction of the HDM. For an objective evaluation of the image quality, head CT scans acquired with HDM and full-dose method (FDM) were randomly selected, and the image noise standard deviation (SD) was measured for each scan. In addition, some HDM images were randomly selected and independently reviewed by two radiologists. Results The HDM was introduced in 27.9% of all head CTs. The mean CTDI-vol of all head CTs was 21.5 ± 6.9 mGy after the introduction, a 14.9% reduction. The mean DLP was 418.4 ± 152.9 mGy.cm after the introduction, a 17.2% reduction. Compared to the FDM images, the noise SD of the HDM ones worsened by almost 0.9; however, none of the images were difficult or impossible to evaluate. Conclusion The HDM yielded diagnostically acceptable images. In addition, a change in protocol for only two diseases successfully reduced the patients’ overall radiation exposure by approximately 15%. Introducing and optimizing the HDM for frequently performed target diseases will be useful in reducing the exposure dose for the hospital’s patient population.

Surgical management of tumor thrombus extending to the major vascular system for children with hepatoblastoma is challenging and insufficiently discussed.We conducted a retrospective review of hepatoblastoma with tumor thrombus extending to the major vascular system (inferior vena cava, 3 hepatic veins, and portal vein trunk) treated at our center between May 2010 and June 2021. We describe our preoperative assessment, surgical strategies, and outcomes.We identified 9 patients (median age at the diagnosis: 3.4 years). All patients received chemotherapy before liver surgery. At the time of the diagnosis, tumor thrombus extended to the portal vein trunk (n = 6), inferior vena cava (n = 3), and 3 hepatic veins (n = 2). Among the 9 patients, 4 underwent liver resection. Liver transplantation was performed in 5 patients. The inferior vena cava wall was circumferentially resected for tumor removal in 1 patient and partially resected in 2 patients. One patient underwent liver transplantation using veno-venous bypass. Patients with tumor thrombus extending to the portal vein trunk were more likely to be managed by liver transplantation in comparison to those with tumor thrombus spreading to the inferior vena cava. The median follow-up period was 5.5 years. One patient underwent transhepatic balloon dilatation for biliary stricture after liver resection. Tumor recurrence was seen in 3 patients (33.3%; lung, n = 2; lymph node and liver, n = 1). No patients died during the follow-up period.Surgical intervention for pediatric hepatoblastoma with tumor thrombus extending into the major vascular system is safe, feasible, and achieves excellent outcomes.

Silver-Russell syndrome (SRS) is a congenital disorder characterized by prenatal and postnatal growth failure and craniofacial features. Hypomethylation of the H19/IGF2:IG-differential methylated region (H19LOM) is observed in 50% of SRS patients, and 15% of SRS patients with H19LOM had multilocus imprinting disturbance (MLID). Schimke immuno-osseous dysplasia (SIOD), characterized by spondyloepiphyseal dysplasia and nephropathy, is an autosomal recessive disorder caused by mutations in SMARCAL1 on chromosome 2. We report a patient with typical SRS-related features, spondyloepiphyseal dysplasia, and severe nephropathy. Molecular analyses showed H19LOM, paternal uniparental isodisomy of chromosome 2 (iUPD(2)pat), and a paternally inherited homozygous frameshift variant in SMARCAL1. Genome-wide methylation analysis showed MLID in this patient, although it showed no MLID in another patient with SIOD without SRS phenotype. These results suggest that iUPD(2)pat unmasked the recessive mutation in SMARCAL1 and that the SMARCAL1 gene mutation may have no direct effect on the patient's methylation defects.

Tracheal atresia causes some secondary changes (dilated trachea, flattened/inverted diaphragm, hyperintense and hyperinflated lungs). They can be reduced if a high airway fistula is present. This study evaluated fetal MR images of tracheal atresia and the secondary changes, focusing on the presence of a fistula. We assessed fetal MR images of tracheal atresia without fistula (n=4, median 26 weeks), tracheal atresia with fistula (n=4, median 33 weeks) and controls (n=30, median 32 weeks). We evaluated airway obstruction using true-positive rate in tracheal atresia and false-positive rate in controls indicating they are likely normal variants. Tracheal diameter, craniocaudal-anteroposterior ratio of the right hemidiaphragm, lung-to-liver signal intensity ratio, and cardiothoracic ratio were compared among the three groups using the Kruskal-Wallis test followed by pairwise comparison using the Mann-Whitney U test. True-positive rate was 100% in tracheal atresia, while false-positive rate was 20% in controls. The Kruskal-Wallis test showed differences among groups in craniocaudal-anteroposterior ratio and cardiothoracic ratio (P

Background The precise time of appearance of bone marrow edema in acute hematogenous pelvic osteomyelitis (AHPO) is unknown. The purpose of present research is to clarify the time of appearance of bone marrow edema on MRI in AHPO. Our hypothesis was that onset is slower than in long-bone osteomyelitis. Methods We selected twelve patients (mean, 11.8 years) with MRI findings and clinical diagnosis of AHPO. The signal ratios of bone marrow (BM) and gluteus maximus muscle (M, BM/M ratio) in fat-suppressed T2- and T1-weighted images (T2WI, T1WI) were calculated to evaluate changes in bone marrow signals. The correlation between BM/M ratios and days from onset was statistically evaluated and compared to lower extremity osteomyelitis. Results BM/M ratio of T2WI increased over time after onset of the primary symptom in all patients and showed a statistically positive correlation (r = 0.36). In seven patients in whom an MRI scan was conducted twice, all showed higher values for the second MRI, and changes were more pronounced over time. The mean BM/M ratio of T2WI was 4.1 when seven days or less had elapsed from the primary symptom and 6.4 when more than seven days had elapsed . BM/M ratios in the sacroiliac joint group were lower than in the non-sacroiliac joint group. Conclusion Unlike long-bone osteomyelitis, it took one week before findings for AHPO became fully evident. A definitive diagnosis can be made in patients with suspected sacroiliitis by performing a further MRI scan at seven days or later.

To understand the latest pediatric computed tomography (CT) exposure required for the revision of national DRLs.A questionnaire was sent to 409 facilities where the members of the Japanese Society of Radiological Technology and the Japanese Society of Pediatric Radiology are enrolled. We investigated the imaging conditions, CTDIIn all, 43 facilities (11%) responded to our survey. multi detector-row CT (MDCT) systems were available in all surveyed facilities. More than 98% of the MDCT systems had more than 64 detector rows. The CTDIIt is necessary to review the imaging protocol with the attending physician and radiologist and consider further optimization of medical exposure.

Objective Patient-reported outcomes (PROs) are important measures of the quality of life (QOL) and symptoms in patients with hepatitis C virus (HCV). We evaluated the PROs at the beginning of direct-acting antiviral (DAA) treatment and three years later. A low QOL in patients with chronic liver disease suggested a low muscle mass. We compared the relationship between the QOL and muscle mass.Methods DAAs were administered to 100 patients with HCV infection. The PROs included the cirrhosis-related symptom score (CSS), presence of restless legs syndrome, Pittsburg sleep quality index (PSQI) to evaluate sleep disturbance, SF-36 to measure the QOL, and calculated body muscle mass (CBMM) measured at the beginning of treatment and three years later. Computed tomography (CT) was used to screen 82 patients for hepatocellular carcinoma at the beginning of treatment and three years later. Cross-sectional CT images of the third lumbar vertebrae were analyzed to evaluate the body composition.Results The general health perception (GHN) of SF-36 was better at three years after DAA administration than at the beginning. Changes in the GHN (dGHN) were related to an improved sleep quality on the PSQI and CSS and increased CBMM. The dGHN was positively related to changes in the skeletal muscle. The sleep quality, sleep latency, fatigue, and abdominal fullness were related to dGHN. Conclusion The QOL is related to sleep disturbance and several other symptoms. Furthermore, in patients with an increased muscle volume after DAA treatment, increased muscle mass is associated with an improvement in the QOL., Internal medicine, 59(21), pp.2653-2660; 2020

The delayed local treatment approach (DL) in high-risk neuroblastoma (HR-NB) refers to the process in which tumor resection is performed after the completion of all the courses of chemotherapy, including myeloablative high-dose chemotherapy (HDC). Alternatively, in the conventional local treatment approach (CL), tumor resection is performed during induction chemotherapy. In this study, we compared the surgical outcomes in HR-NB patients treated by CL and DL.Forty-seven patients with abdominal HR-NB underwent primary tumor resection from 2002 to 2018. The timing of surgery was generally determined by following the trials and guidelines available at the time. The outcomes and surgical complications between the two strategies were compared.Operation time, blood loss, and postoperative WBC counts were lower in the DL group (n = 25) when compared to the CL group (n = 22), statistical significance notwithstanding. Major vascular structures were less frequently encased in the DL group tumors, while immediate surgical complications were significantly more frequent in the CL group (P 0.05). Furthermore, the 3-year EFSs were 50.0% and 53.9% in the DL and CL groups, respectively.DL appears to be a feasible and effective treatment option for HR-NB. Nonetheless, further verifications using larger cohorts are warranted.Treatment study, Level III.

Anaplastic large cell lymphoma (ALCL) accounts for 10-15% of childhood non-Hodgkin lymphoma cases; it is generally chemo-sensitive and is one of the most curable pediatric cancers. We report here a case of pediatric ALCL complicated with acute liver failure due to the aggravation of pre-existing biliary hepatopathy by lymphoma-associated hemophagocytic lymphohistiocytosis (HLH). Although the initial treatment response against ALCL was very good, poor and irreversible liver function due to biliary cirrhosis worsening by lymphoma-associated HLH prevented the patient from receiving further consolidation chemotherapies. To make matters worse, his condition was accompanied with intrahepatic fungal pseudoaneurysm and invasive fungal infection. Thus, we decided to perform an urgent living-donor liver transplantation from his father to correct the patient's liver function and make it possible to proceed with further ALCL therapy. After the living-donor liver transplantation, the patient successfully received consolidation therapy with brentuximab vedotin. To our knowledge, this may be an early reported case of a pediatric patient undergoing liver transplantation during treatment for ALCL. In most patients with HLH-associated ALCL, liver function improves when ALCL is controlled. However, acute liver failure is occasionally observed in HLH cases with pre-existing liver dysfunction. In such cases, liver transplantation should be considered to correct liver dysfunctions if the disease control of HLH is satisfactory.

Aim Sarcopenia is a harmful condition in patients with chronic liver disease. However, the evaluation of body muscle mass requires expensive instrumentation. The sarcopenia index (SI): (creatinine / cystatin C × 100) has been reported to correlate with muscle volume. A calculated body muscle mass (CBMM) using creatinine, cystatin C, and bodyweight also correlates with muscle mass. We evaluated the applicability of using SIs and CBMMs as screening methods for sarcopenia. Methods Patients (n = 303) with liver damage were evaluated for creatinine, cystatin C, and grip strength (GS). All patients were evaluated using cross-sectional computed tomography images of the third lumbar vertebrae to determine their skeletal muscle (SM) mass. CBMMs and SIs were compared with SMs, GSs, and sarcopenia. Results Correlation coefficients (R) between SMI (SM / height2 [m2 ]) and CBMM, and between GS and CBMM were 0.643 and 0.723, respectively. Factors contributing to low GSs; low SM indices; and sarcopenia were age and SM; sex, age, GS, SI, and CBMM indices; and sex, bodyweight, and CBMM, respectively, in the multivariate logistic analyses. Receiver operating characteristic curve analysis between sarcopenia and CBMM showed an area under the receiver operating characteristic curve of 0.78504 in women and 0.86067 in men. Cut-off CBMM values for sarcopenia were 27.903 (sensitivity 0.73958) in women and 39.731 (sensitivity 0.7941) in men. Conclusions CBMMs and SIs are simple and minimally invasive screening methods in which low levels are indicative of sarcopenia in patients with liver disease.

Background The SIOPEL-4 study has demonstrated that dose-dense cisplatin-based chemotherapy dramatically improves outcome in children with high-risk hepatoblastoma in western countries. However, the feasibility and safety of this regimen have not been clarified in Japanese patients. Methods A pilot study, JPLT3-H, was designed to evaluate the safety profile of the SIOPEL-4 regimen in Japanese children with newly diagnosed hepatoblastoma with either metastatic disease or low alpha-fetoprotein. Results A total of 15 patients (three female) were enrolled. Median age was 2 years (range, 0-14). Three patients were PRETEXT II (where PRETEXT is PRETreatment EXTent of disease), six PRETEXT III, and six PRETEXT IV. All patients had lung metastasis, none had low alpha-fetoprotein. Eight patients completed the prescribed treatment, and seven patients discontinued therapy prematurely, four due to progressive disease and three due to causes other than severe toxicity. Grade 4 neutropenia was documented in most patients in preoperative cycles A1-3 (11/15 in A1, 9/11 in A2, and 7/11 in A3) and in all considering all cycles. Grade 3-4 thrombocytopenia and grade 3 anemia were also frequently observed. Patients experienced several episodes of grade 3 febrile neutropenia, but none had grade 4 febrile neutropenia or severe infections. One patient had grade 3 heart failure only in the first cycle. Other grade 3 or 4 toxicities were hypomagnesemia, anorexia, nausea, mucositis, liver enzyme elevation, fever, infection, and fatigue. There were no unexpected severe toxicities. Conclusion The toxicity profile of JPLT3-H was comparable to that of SIOPEL-4. Dose-dense cisplatin-based chemotherapy may be feasible among Japanese patients with high-risk hepatoblastoma.

To report the clinical and imaging characteristics of BCG-osteomyelitis, and compare them with those of pyogenic osteomyelitis.Clinical and imaging findings were retrospectively evaluated in 14 children with BCG osteomyelitis, including 3 with Mendelian susceptibility to mycobacterial diseases (MSMD), and in 40 children with pyogenic osteomyelitis, using Fisher exact and Mann-Whitney U tests.BCG-osteomyelitis was an indolent inflammatory disease of young children (mean age 15.5 months). Immunocompetent patients came to medical attention over months after vaccination, while patients with MSMD much earlier (the average time lapse: 13.7 vs. 5.0 months). The former manifested with a slowly progressive, painless mass with only mildly increased acute-phase reactants, while the latter started with lymphadenitis with significant inflammatory reactions and later developed osteomyelitis. These clinical scenarios contrasted with acute febrile illness in pyogenic osteomyelitis. The imaging findings were identical in both immunocompetent and MSMD groups; however, the former showed monoostotic involvement, while the latter polyostotic affliction. The typical imaging finding of BCG-osteomyelitis comprises a large intraosseous abscess with modest reactive edema commonly associated with transphyseal extension from the metaphysis to the epiphysis, contrasting with the manifestation of pyogenic osteomyelitis; size of abscess (p=0.028), pattern of abscess extension (p0.001), and extent of surrounding edema (p0.001).BCG-osteomyelitis should be suspected in children under 2 years of age with insidious osteomyelitis, accompanied with characteristic imaging findings. Polyostotic BCG osteomyelitis is highly suggestive of MSMD. Awareness of the distinctive features of BCG-osteomyelitis enables the early diagnosis and timely therapeutic intervention.

A variety of underlying diseases can predispose infants and children to bacterial meningitis (BM). For the diagnosis, treatment, and prevention of its recurrence, radiologists should be familiar with its predisposing conditions so that they can suggest the appropriate imaging approach. Predisposing conditions of BM can be broadly classified into two categories: infection spread from the adjacent tissue to the cerebrospinal fluid (CSF) space and immunodeficiency. Diseases in the former category are further divided according to regardless of whether there is a structural defect between the CSF space and the adjacent tissue. When a structural defect is suspected in a patient with BM, computed tomography (CT) of the head and magnetic resonance (MR) imaging are first-line imaging examinations. Radionuclide cisternography should be implemented as a second-line step to identify the CSF leak site. In patients with suspected parameningeal infection without any structural defect, such as sinusitis or otitis media/mastoiditis, CT or MR images can identify not only the disease itself but also the associated intracranial complications. The purpose of this article is to discuss the diagnostic approach and imaging findings associated with the variety of conditions predisposing patients to recurrent BM, focusing on the role of radiology in their management.

Detecting an aberrant artery is the key to diagnosing bronchopulmonary sequestration (BPS) on fetal MRI. Few reports describe the best sequence to use for this purpose. We compared visualization of an aberrant artery in BPS on fetal MRI using single-shot turbo spin echo (SSTSE) and balanced steady state free precession (b-SSFP) sequences. We retrospectively reviewed the fetal MRI of 27 fetuses in which BPS was diagnosed prenatally (2003–2016). From these we selected 15 fetuses with the pathological diagnosis of BPS made postnatally at operation. All fetuses were examined in 1.5 T MRI units using SSTSE and b-SSFP sequences (mean gestational age: 28.4 ± 3.4 weeks). Two pediatric radiologists compared visualization of the aberrant artery using the two sequences. The presence of an aberrant artery was confirmed by postnatal contrast-enhanced CT. The influence of arterial diameter and background heterogeneity of the lung on the diagnostic power was also evaluated. All 15 fetuses clearly showed the aberrant artery on the SSTSE sequence. Conversely, the aberrant arteries detected on the SSTSE sequence were absent in the b-SSFP sequences of eight fetuses, and the other seven fetuses showed ambiguous visualization compared with the SSTSE sequence. Arterial diameter and background heterogeneity did not affect the diagnostic power in either sequence. The SSTSE sequence was superior to the b-SSFP sequence for clear visualization of the aberrant artery in BPS, regardless of the arterial diameter and background heterogeneity.

Pelvic osteomyelitis may occur in a metaphyseal equivalent, defined as a portion of flat or irregular bone that is adjacent to cartilage. The pelvic bone is known to have several metaphyseal equivalents and of these, the sacroiliac joint is the most frequent site of involvement. However, a sacral neurocentral synchondrosis has not been recognized as a metaphyseal equivalent, and there have been no previous reports describing this as the site of origin of sacral osteomyelitis. We here report two cases of sacral osteomyelitis originating in a neurocentral synchondrosis, another metaphyseal equivalent. We, as pediatric radiologists, should recognize a sacral neurocentral synchondrosis as another metaphyseal equivalent, especially in infants and younger patients.

Background Systemic juvenile xanthogranuloma is a very rare disease typically presents as skin lesions with yellow papules or nodules and is sometimes fatal. We report a case of congenital neonatal systemic juvenile xanthogranuloma with atypical skin appearance that made the diagnosis difficult. Case presentation A preterm Japanese female neonate with prenatally diagnosed fetal hydrops in-utero was born with purpuric lesions involving the trunk and face. Since birth, she had hypoxemic respiratory failure, splenomegaly, anemia, thrombocytopenia, coagulopathy, and was transfusion dependent for red blood cells, fresh frozen plasma, and platelets. Multiple cystic lesions in her liver, part of them with vascular, were detected by ultrasound. A liver biopsy was inconclusive. A skin lesion on her face similar to purpura gradually changed to a firm and solid enlarged non-yellow nodule. Technically, the typical finding on skin biopsy would have been histiocytic infiltration (without Touton Giant cells) and immunohistochemistry results which then would be consistent with a diagnosis of systemic juvenile xanthogranuloma, and chemotherapy improved her general condition. Conclusions This case report shows that skin biopsies are necessary to detect neonatal systemic juvenile xanthogranuloma when there are organ symptoms and skin eruption, even if the skin lesion does not have a typical appearance of yellow papules or nodules.