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1.
Int J Legal Med ; 134(4): 1457-1463, 2020 Jul.
Article in English | MEDLINE | ID: mdl-31853676

ABSTRACT

PURPOSE: The aim of this study was to investigate whether post-mortem computed tomography (PMCT) provides additional information regarding the cause of death and underlying diseases in a general practitioners' (GP), out-of-hospital population. METHODS AND MATERIALS: Bodies donated to our anatomy department between January 2014 and January 2018, who consecutively underwent a total body PMCT and had given permission for retrieval of their medical records during life, were included. PMCT scans were assessed by a radiologist and compared with the cause of death as stated in the medical records. Discrepancies were analyzed with an adjusted Goldman classification. RESULTS: Ninety-three out of the 274 scanned donors during the inclusion period had given consent for the retrieval of their medical records, of which 79 GP's responded to the request thereof (31 men, 48 women, average age 72.8 years, range 36-99). PMCT identified 49 (62%) cases of cancer, 10 (12.7%) cardiovascular diseases, 8 (10.1%) severe organ failures, 5 (6.3%) cases with signs of pneumonia, 2 (2.5%) other causes, and 7 (8.9%) cases without an (underlying) definitive cause of death. Eleven major discrepancies on the Goldman classification scale, with possible relevance to survival between PMCT and GP records, were identified. CONCLUSION: PMCT can have added value for the detection of additional findings regarding the cause of death in an out-of-hospital, GP's population, especially to identify or exclude major (previously non-diagnosed) underlying diseases.


Subject(s)
Autopsy , Cause of Death , Forensic Medicine , Tomography, X-Ray Computed , Electronic Health Records/statistics & numerical data , General Practitioners , Humans , Radiologists
2.
Eur Radiol ; 29(2): 866-876, 2019 Feb.
Article in English | MEDLINE | ID: mdl-30054793

ABSTRACT

OBJECTIVES: The incidence of children developing metachronous contralateral inguinal hernia (MCIH) is 7-15%. Contralateral groin exploration during unilateral hernia repair can prevent MCIH development and subsequent second surgery and anaesthesia. Preoperative ultrasonography is a less invasive strategy and potentially able to detect contralateral patent processus vaginalis (CPPV) prior to MCIH development. METHODS: We queried MEDLINE, Embase and Cochrane library to identify studies regarding children aged < 18 years diagnosed with unilateral inguinal hernia without clinical signs of contralateral hernia, who underwent preoperative ultrasonography of the contralateral groin. We assessed heterogeneity and used a random-effects model to obtain pooled estimates of sensitivity, specificity and area under the receiver operating characteristic curve (AUC). RESULTS: Fourteen studies (2120 patients) were included, seven (1013 patients) in the meta-analysis. In studies using surgical exploration as reference test (n = 4, 494 patients), pooled sensitivity and specificity were 93% and 88% respectively. In studies using contralateral exploration as reference test following positive and clinical follow-up after negative ultrasonographic test results (n = 3, 519 patients), pooled sensitivity was 86% and specificity 98%. The AUC (0.984) shows high diagnostic accuracy of preoperative ultrasonography for detecting CPPV, although diagnostic ultrasonographic criteria largely differ and large heterogeneity exists. Reported inguinal canal diameters in children with CPPV were 2.70 ± 1.17 mm, 6.8 ± 1.3 mm and 9.0 ± 1.9 mm. CONCLUSION: Diagnostic accuracy of preoperative ultrasonography to detect CPPV seems promising, though may result in an overestimation of MCIH prevalence, since CPPV does not invariably lead to MCIH. Unequivocal ultrasonographic criteria are mandatory for proper diagnosis of CPPV and subsequent prediction of MCIH. KEY POINTS: • Diagnostic accuracy of preoperative ultrasonography for detection of CPPV in children with unilateral inguinal hernia is high. • Preoperative ultrasonographic evaluation of the contralateral groin assumedly results in an overestimation of MCIH prevalence. • Unequivocal ultrasonographic criteria are mandatory for proper diagnosis of CPPV and risk factor identification is needed to predict whether CPPV develops into clinically apparent MCIH.


Subject(s)
Hernia, Inguinal/diagnostic imaging , Hernia, Inguinal/surgery , Herniorrhaphy , Preoperative Care/methods , Child , Female , Hernia, Inguinal/pathology , Herniorrhaphy/methods , Humans , Laparoscopy , Male , Prevalence , ROC Curve , Risk Factors , Sensitivity and Specificity , Testicular Hydrocele/diagnostic imaging , Ultrasonography
3.
Eur J Pediatr ; 177(6): 791-803, 2018 Jun.
Article in English | MEDLINE | ID: mdl-29675642

ABSTRACT

Clinical post-mortem radiology is a relatively new field of expertise and not common practice in most hospitals yet. With the declining numbers of autopsies and increasing demand for quality control of clinical care, post-mortem radiology can offer a solution, or at least be complementary. A working group consisting of radiologists, pathologists and other clinical medical specialists reviewed and evaluated the literature on the diagnostic value of post-mortem conventional radiography (CR), ultrasonography, computed tomography (PMCT), magnetic resonance imaging (PMMRI), and minimally invasive autopsy (MIA). Evidence tables were built and subsequently a Dutch national evidence-based guideline for post-mortem radiology was developed. We present this evaluation of the radiological modalities in a clinical post-mortem setting, including MIA, as well as the recently published Dutch guidelines for post-mortem radiology in foetuses, neonates, and children. In general, for post-mortem radiology modalities, PMMRI is the modality of choice in foetuses, neonates, and infants, whereas PMCT is advised in older children. There is a limited role for post-mortem CR and ultrasonography. In most cases, conventional autopsy will remain the diagnostic method of choice. CONCLUSION: Based on a literature review and clinical expertise, an evidence-based guideline was developed for post-mortem radiology of foetal, neonatal, and paediatric patients. What is Known: • Post-mortem investigations serve as a quality check for the provided health care and are important for reliable epidemiological registration. • Post-mortem radiology, sometimes combined with minimally invasive techniques, is considered as an adjunct or alternative to autopsy. What is New: • We present the Dutch guidelines for post-mortem radiology in foetuses, neonates and children. • Autopsy remains the reference standard, however minimal invasive autopsy with a skeletal survey, post-mortem computed tomography, or post-mortem magnetic resonance imaging can be complementary thereof.


Subject(s)
Autopsy/methods , Cause of Death , Magnetic Resonance Imaging , Tomography, X-Ray Computed , Ultrasonography , Adolescent , Child , Child, Preschool , Fetal Death/etiology , Humans , Infant , Infant, Newborn , Netherlands , Radiography
4.
Hum Reprod ; 32(12): 2366-2372, 2017 Dec 01.
Article in English | MEDLINE | ID: mdl-29040511

ABSTRACT

STUDY QUESTION: Is testicular growth affected by a testicular biopsy intended for fertility preservation in pre-pubertal boys with cancer? SUMMARY ANSWER: Testicular growth of the biopsied testis is not impeded in comparison to the non-biopsied contralateral testis up until 1 year after surgery. WHAT IS KNOWN ALREADY: Fertility preservation in pre-pubertal boys by means of testicular biopsy has been conducted for more than 15 years. Although immediate adverse effects of testicular biopsy are rare (1%), no data exist on the effect of biopsy on testicular growth. STUDY DESIGN, SIZE, DURATION: In this prospective cohort study, between March 2011 and February 2017, 93 parents of pre-pubertal boys were offered cryopreservation of testicular tissue of their son, of whom 78 consented. Sixty-four boys were included in this follow-up study. PARTICIPANTS/MATERIALS, SETTING, METHODS: All boys with cancer at the paediatric oncology department of the Academic Medical Center (AMC) who needed gonadotoxic therapy and were unable to ejaculate were offered cryopreservation of testicular tissue prior to treatment. By testicular ultrasound before and after biopsy (1, 6 and 12 months after biopsy), volume and parenchymal abnormalities were assessed. Data were analysed using mixed-effects modelling. MAIN RESULTS AND THE ROLE OF CHANCE: Of the 64 included boys all were followed up at 1 month, 58 at 6 months and 55 at 12 months. Mean testicular volumes after 1, 6 and 12 months after biopsy were 1.7 ± 2.1, 1.7 ± 2.2 and 1.9 ± 2.4 for the biopsied testis and 1.8 ± 2.2, 1.8 ± 2.3 and 2.0 ± 2.2 for the non-biopsied testis, respectively. Biopsy of the testis did not have a significant impact on testicular growth. Immediate adverse effects of the biopsy, i.e. wound infections, were seen in 3/78 boys (3.8%). LIMITATIONS, REASONS FOR CAUTION: Although it is the largest cohort available to date, the number of patients included in our follow-up is still relatively small. A larger cohort would be able to evaluate growth more precisely. Follow-up was discontinued in a significant portion of boys, 12/76 (15.8%), mainly because of death due to primary illness but also because they could not be reached or declined further follow-up. WIDER IMPLICATIONS OF THE FINDINGS: These reassuring data may be used in counselling future boys who are eligible for fertility preservation and their parents. STUDY FUNDING/COMPETING INTEREST(S): Study funded by KIKA Foundation (Kika 86), Grant from the Netherlands Organisation for Health Research and Development (ZonMW TAS-116003002). The authors declare no conflict of interest. TRIAL REGISTRATION NUMBER: CCMO-register: NL27690.000.09.


Subject(s)
Biopsy/adverse effects , Fertility Preservation/methods , Neoplasms/therapy , Testis/growth & development , Testis/pathology , Adolescent , Child , Child, Preschool , Cryopreservation , Humans , Infant , Male , Neoplasms/complications , Netherlands , Prospective Studies , Time Factors
5.
Arch Dis Child Educ Pract Ed ; 101(4): 187-93, 2016 Aug.
Article in English | MEDLINE | ID: mdl-27325615

ABSTRACT

Defecation-related functional gastrointestinal disorders (FGIDs), such as infant dyschezia, functional constipation and functional non-retentive faecal incontinence, as defined by the Rome IV criteria, are common problems in childhood. The symptomatology varies from relatively mild, such as crying before passage of soft stools or infrequent defecation to severe problems with faecal impaction and the daily involuntary loss of faeces in the underwear. Conventional radiography is widely available, relatively cheap and is non-invasive. The drawback however, is radiation exposure. This review describes and evaluates the value of different existing scoring methods to assess faecal loading on an abdominal radiograph with or without the use of radio-opaque markers, to measure colonic transit time, in the diagnosis of these defecation-related FGIDs. Insufficient evidence exists for a diagnostic association between clinical symptoms of functional constipation or functional nonretentive faecal incontinence and faecal loading on an abdominal radiograph. Furthermore, evidence does not support the routine use of colonic transit studies to diagnose functional constipation. Colonic transit time measurement may be considered in discriminating between functional constipation and functional non-retentive faecal incontinence and in patients in which the diagnosis is not clear such as having an unreliable medical history. In children with the suspicion of defecation-related FGIDs, the diagnosis should be made based on the Rome IV criteria.


Subject(s)
Constipation/diagnosis , Gastrointestinal Diseases/diagnostic imaging , Radiography, Abdominal , Adolescent , Child , Child, Preschool , Defecation , Female , Humans , Infant , Male
6.
Eur J Nucl Med Mol Imaging ; 42(5): 706-15, 2015 Apr.
Article in English | MEDLINE | ID: mdl-25512056

ABSTRACT

PURPOSE: Treatment with (131)I-MIBG is associated with significant thyroid damage. This study was undertaken to investigate the long-term efficacy of current thyroid prophylaxis, to explore the relationship between thyroid dysfunction and thyroid volume after exposure to (131)I-MIBG and to evaluate the possible negative effects of (131)I(-) on the parathyroid glands. METHODS: Of 81 long-term surviving patients with neuroblastoma treated with (131)I-MIBG during the period 1999-2012, 24 were finally evaluated. Patients received thyroxine (T4), methimazole and potassium iodide as thyroid protection. In all patients (para)thyroid function was evaluated and ultrasound investigation of the (para)thyroid gland(s) was performed. Thyroid dysfunction was defined as a plasma thyrotropin concentration >5.0 mU/L (thyrotropin elevation, TE) or as the use of T4 at the time of follow-up. Hyperparathyroidism was defined as a serum calcium concentration above the age-related reference range in combination with an inappropriately high parathyroid hormone level. RESULTS: At a median follow-up of 9.0 years after (131)I-MIBG treatment, thyroid disorders were seen in 12 patients (50 %; 9 with TE, 5 with a thyroid nodule and 1 patient was subsequently diagnosed with differentiated thyroid carcinoma). No significant risk factors for the occurrence of thyroid damage could be identified. In 14 of 21 patients (67 %) in whom thyroid volume could be determined, the volume was considered small (<-2SD) for age and gender. Patients treated with T4 at the time of follow-up had significantly smaller thyroid volumes for age than patients without T4 treatment (p = 0.014). None of the patients was diagnosed with hyperparathyroidism. CONCLUSION: Thyroid protection during treatment with (131)I-MIBG needs attention and must be further improved, as thyroid disorders are still frequently seen despite current thyroid prophylaxis. Reduced thyroid volume in neuroblastoma survivors may be related to previous (131)I-MIBG therapy or current T4 treatment. No deleterious effects of (131)I-MIBG on the parathyroid glands could be found.


Subject(s)
3-Iodobenzylguanidine/adverse effects , Hypothyroidism/prevention & control , Neoplasms, Radiation-Induced/prevention & control , Neuroblastoma/radiotherapy , Radiopharmaceuticals/adverse effects , Radiotherapy/adverse effects , Thyroid Neoplasms/prevention & control , 3-Iodobenzylguanidine/therapeutic use , Child , Child, Preschool , Female , Humans , Hypothyroidism/etiology , Infant , Male , Neoplasms, Radiation-Induced/etiology , Radiopharmaceuticals/therapeutic use , Thyroid Neoplasms/etiology
7.
J Med Genet ; 51(2): 90-7, 2014 Feb.
Article in English | MEDLINE | ID: mdl-24253444

ABSTRACT

BACKGROUND: The T gene (brachyury gene) is the founding member of the T-box family of transcription factors and is vital for the formation and differentiation of the mesoderm and the axial development of all vertebrates. RESULTS: We report here on four patients from three consanguineous families exhibiting sacral agenesis, a persistent notochordal canal and abnormal ossification of the vertebral bodies, and the identification and characterisation of their underlying genetic defect. Given the consanguineous nature and the similarity of the phenotypes between the three families, we performed homozygosity mapping and identified a common 4.1 Mb homozygous region on chromosome 6q27, containing T, brachyury homologue (mouse) or T. Sequencing of T in the affected individuals led to the identification of a homozygous missense mutation, p.H171R, in the highly conserved T-box. The homozygous mutation results in diminished DNA binding, increased cell growth, and interferes with the normal expression of genes involved in ossification, notochord maintenance and axial mesoderm development. CONCLUSIONS: We have identified a shared homozygous mutation in three families in T and linked it to a novel syndrome consisting of sacral agenesis, a persistent notochordal canal and abnormal ossification of the vertebral bodies. We suggest that screening for the ossification of the vertebrae is warranted in patients with sacral agenesis to evaluate the possible causal involvement of T.


Subject(s)
Abnormalities, Multiple/genetics , Fetal Proteins/genetics , Notochord/abnormalities , Ossification, Heterotopic/genetics , Sacrum/abnormalities , Spine/abnormalities , T-Box Domain Proteins/genetics , Abnormalities, Multiple/diagnostic imaging , Abnormalities, Multiple/mortality , Amino Acid Sequence , Base Sequence , Cell Line, Tumor , Cell Proliferation , Chromosomes, Human, Pair 6/genetics , Comparative Genomic Hybridization , Consanguinity , Female , Genetic Association Studies , Homozygote , Humans , Infant , Infant, Newborn , Male , Mutation, Missense , Notochord/diagnostic imaging , Ossification, Heterotopic/diagnostic imaging , Ossification, Heterotopic/mortality , Pedigree , Protein Binding , Protein Transport , Sacrum/diagnostic imaging , Spine/diagnostic imaging , Syndrome , Ultrasonography, Prenatal
8.
Emerg Radiol ; 21(4): 349-58, 2014 Aug.
Article in English | MEDLINE | ID: mdl-24553773

ABSTRACT

Abusive head trauma is a severe form of child abuse. One important diagnostic finding is the presence of a subdural hematoma. Age determination of subdural hematomas is important to relate radiological findings to the clinical history presented by the caregivers. In court this topic is relevant as dating subdural hematomas can lead to identification of a suspect. The aim of our study is to describe the current practice among radiologists in the Netherlands regarding the age determination of subdural hematomas in children. This is a cross-sectional study, describing the results of an online questionnaire regarding dating subdural hematomas among pediatric and neuro-radiologists in the Netherlands. The questionnaire consisted of sociodemographic questions, theoretical questions and eight pediatric cases in which the participants were asked to date subdural hematomas based on imaging findings. Fifty-one out of 172 radiologists (30 %) filled out the questionnaire. The percentage of participants that reported it was possible to date the subdural hematoma varied between 58 and 90 % for the eight different cases. In four of eight cases (50 %), the age of the subdural hematoma as known from clinical history fell within the range reported by the participants. None of the participants was "very certain" of their age determination. The results demonstrate that there is a considerable practice variation among Dutch radiologists regarding the age determination of subdural hematomas. This implicates that dating of subdural hematomas is not suitable to use in court, as no uniformity among experts exists.


Subject(s)
Child Abuse/diagnosis , Diagnostic Imaging , Hematoma, Subdural/diagnosis , Child , Child, Preschool , Clinical Competence , Cross-Sectional Studies , Diagnosis, Differential , Female , Hematoma, Subdural/pathology , Humans , Infant , Male , Netherlands , Practice Patterns, Physicians' , Surveys and Questionnaires
9.
Eur J Pediatr ; 172(8): 1105-10, 2013 Aug.
Article in English | MEDLINE | ID: mdl-23640022

ABSTRACT

UNLABELLED: High plasma C-reactive protein (CRP) levels are associated with favorable outcome in adults with acute lung injury (ALI). The association between CRP levels and outcome has not been studied in ALI in children. We performed a historical cohort study in 93 mechanically ventilated children (0-18 years) with ALI. The CRP level within 48 h of disease onset was tested for association with 28-day mortality and ventilator-free days (VFD). Clinical parameters and ventilator settings were evaluated for possible confounding. Fourteen patients died within 28 days. The median (interquartile range) CRP level in nonsurvivors was 126 mg/L (64; 187) compared with 56 mg/L (20; 105) in survivors (p = 0.01). For every 10-mg/L rise in CRP level, the unadjusted odds (95% confidence interval (95% CI)) for mortality increased 8.7% (2.1-15.8%). Cardiovascular organ failure at onset of ALI was the strongest predictor for mortality (odds ratio, 30.5 (6.2-152.5)). After adjustment for cardiovascular organ failure, for every 10-mg/L rise in CRP level, the OR (95% CI) for mortality increased 4.7% (-2.7-12.6%; p = 0.22). Increased CRP levels were associated with a decrease in VFD (ρ = -0.26, p = 0.01). CONCLUSION: increased plasma CRP levels are not associated with favorable outcome in ALI in children. This is in contrast with findings in adults with ALI.


Subject(s)
Acute Lung Injury/blood , C-Reactive Protein/metabolism , Respiration, Artificial , Acute Lung Injury/mortality , Acute Lung Injury/therapy , Adolescent , Biomarkers/blood , Child , Child, Preschool , Cohort Studies , Female , Humans , Infant , Infant, Newborn , Intensive Care Units, Pediatric , Logistic Models , Male , Odds Ratio , Prognosis , Severity of Illness Index
10.
Acta Radiol ; 54(9): 1024-9, 2013 Nov.
Article in English | MEDLINE | ID: mdl-24179234

ABSTRACT

Bone age determination from hand radiographs is one of the oldest radiographic procedures. The first atlas was published by Poland in 1898, and to date the Greulich Pyle atlas, although it dates from 1959, is still the most commonly used method. Bone age rating is time-consuming, suffers from an unsatisfactorily high rater variability, and therefore already 25 years ago it was proposed to replace the manual rating by an automated, computerized method, a field nowadays referred to as computer-aided diagnosis (CAD). The pursuit of this goal reached a first stage of accomplishment in 1992-1996 with the presentation of several systems. However, they had limited clinical value, and efforts in CAD research were increasingly focused on lesion detection for cancer screening. It was only in 2008 that a fully-automated bone age method was presented, which appears to be clinically acceptable. In this paper we consider the requirements that should be met by an automated bone age method and review the state of the art. Integration in PACS and saving time are important factors for radiologists. But it is the validation of the methods which poses the greatest challenge, because there is no gold standard for bone age rating, and the direct comparison to manual rating is therefore not sufficient for demonstrating that manual rating can be replaced by automated rating. One needs additional studies assessing the precision of a method and its accuracy when used for adult height prediction, which serves as an objective.


Subject(s)
Age Determination by Skeleton/methods , Bone and Bones/diagnostic imaging , Diagnosis, Computer-Assisted/methods , Hand/diagnostic imaging , Adult , Humans , Image Processing, Computer-Assisted/methods , Radiology Information Systems
11.
Pediatr Radiol ; 42(6): 653-9, 2012 Jun.
Article in English | MEDLINE | ID: mdl-22237480

ABSTRACT

BACKGROUND: Laterality defects are quite common in thoracoileopagus and parapagus dicephalus but rare in other types of conjoined twins. OBJECTIVE: To present the presumed laterality defects in cephalothoracoileopagus and prosopothoracoileopagus conjoined twins, based on the unilateral or bilateral absence or duplication of the spleen. MATERIALS AND METHODS: Three human anatomical specimens of craniothoracoileopagus (CTIP) twins and one of prosopothoracoileopagus (PTIP) twins were investigated. The specimens were part of the Museum Vrolik collection of the Department of Anatomy and Embryology of the Academic Medical Centre, University of Amsterdam, The Netherlands. The specimens were taken out of their jars and scanned with multidetector CT and volumetric T2-weighted MRI at 1.5 T. RESULTS: The internal anatomy of the specimens was largely in accordance with previous reports. However, there was no recognisable spleen in the right twin in one CTIP specimen, in the left twin in one other CTIP specimen, and in both twins in the third CTIP specimen and in the PTIP specimen. CONCLUSION: Asplenia and polysplenia are considered reliable indicators of right and left isomerism, respectively. However, three of our four specimens had laterality patterns that did not correspond with those previously reported. Since no other parameters of laterality defects could be verified in these specimens, we concluded that asplenia was unlikely to be caused by laterality defects.


Subject(s)
Abnormalities, Multiple/diagnosis , Spleen/abnormalities , Twins, Conjoined , Cadaver , Female , Humans , Male , Radiography , Spleen/diagnostic imaging , Spleen/pathology
12.
Pediatr Radiol ; 42(5): 574-83, 2012 May.
Article in English | MEDLINE | ID: mdl-22252145

ABSTRACT

BACKGROUND: Children with severe neurological impairment and intellectual disability (ID) are susceptible for developing low bone mineral density (BMD) and fractures. BMD is generally measured with dual-energy X-ray absorptiometry (DXA). OBJECTIVE: To describe the occurrence of factors that may influence the feasibility of DXA and the accuracy of DXA outcome in children with severe neurological impairment and ID. MATERIALS AND METHODS: Based on literature and expert opinion, a list of disrupting factors was developed. Occurrence of these factors was assessed in 27 children who underwent DXA measurement. RESULTS: Disrupting factors that occurred most frequently were movement during measurement (82%), aberrant body composition (67%), small length for age (56%) and scoliosis (37%). The number of disrupting factors per child was mean 5.3 (range 1-8). No correlation was found between DXA outcomes and the number of disrupting factors. CONCLUSION: Factors that may negatively influence the accuracy of DXA outcome are frequently present in children with severe neurological impairment and ID. No systematic deviation of DXA outcome in coherence with the amount of disrupting factors was found, but physicians should be aware of the possible influence of disrupting factors on the accuracy of DXA.


Subject(s)
Absorptiometry, Photon , Artifacts , Bone Density , Fractures, Bone/diagnostic imaging , Fractures, Bone/etiology , Intellectual Disability/complications , Lumbar Vertebrae/diagnostic imaging , Nervous System Diseases/complications , Osteoporosis/diagnostic imaging , Osteoporosis/etiology , Whole Body Imaging , Adolescent , Checklist , Child , Child, Preschool , Humans , Infant , Male , Pilot Projects , Risk Factors , Young Adult
13.
Eur J Surg Oncol ; 48(7): 1536-1542, 2022 07.
Article in English | MEDLINE | ID: mdl-35307252

ABSTRACT

In-transit metastases (ITM) are defined as metastatic lymph nodes or deposits occurring between the primary tumor and proximal draining lymph node basin. In extremity rhabdomyosarcoma (RMS), they have rarely been reported. This study evaluates the frequency, staging and survival of patients with ITM in distal extremity RMS. METHODS: Patients with extremity RMS distal to the elbow or knee, enrolled in the EpSSG RMS 2005 trial between 2005 and 2016 were eligible for this study. RESULTS: One hundred and nine distal extremity RMS patients, with a median age of 6.2 years (range 0-21 years) were included. Thirty seven of 109 (34%) had lymph node metastases at diagnosis, 19 of them (51%) had ITM, especially in lower extremity RMS. 18F-FDG-PET/CT detected involved lymph nodes in 47% of patients. In patients not undergoing 18F-FDG-PET/CT lymph node involvement was detected in 22%. The 5-yr EFS of patients with ITM vs proximal lymph nodes vs combined proximal and ITM was 88.9% vs 21.4% vs 20%, respectively (p = 0.01) and 5-yr OS was 100% vs 25.2% vs 15%, respectively (p = 0.003). CONCLUSION: Our study showed that in-transit metastases constituted more than 50% of all lymph node metastases in distal extremity RMS. 18F-FDG-PET/CT improved nodal staging by detecting more regional and in-transit metastases. Popliteal and epitrochlear nodes should be considered as true (distal) regional nodes, instead of in-transit metastases. Biopsy of these nodes is recommended especially in distal extremity RMS of the lower limb. Patients with proximal (axillary or inguinal) lymph node involvement have a worse prognosis.


Subject(s)
Fluorodeoxyglucose F18 , Rhabdomyosarcoma , Adolescent , Child , Child, Preschool , Clinical Trials as Topic , Humans , Infant , Infant, Newborn , Lower Extremity , Lymph Nodes/pathology , Lymphatic Metastasis/pathology , Neoplasm Staging , Positron Emission Tomography Computed Tomography , Rhabdomyosarcoma/diagnostic imaging , Rhabdomyosarcoma/pathology , Young Adult
14.
Forensic Sci Med Pathol ; 7(4): 355-63, 2011 Dec.
Article in English | MEDLINE | ID: mdl-21519862

ABSTRACT

Abusive head trauma (AHT) is one of the most severe forms of physical child abuse. If a child initially survives severe AHT the neurological outcome can be poor. In recent years several children were seen who developed multicystic encephalomalacia (MCE) after documented severe AHT. A search of the Netherlands Forensic Institute database in The Hague was performed. Inclusion criteria were cases of AHT between 1999 and 2010 where the child was under the age of 1 year old at the time of trauma. Trauma mechanism and radiological information were collected. Five children, three boys and two girls (mean age 57 days, range 8-142 days) who developed cystic encephalomalacia after inflicted traumatic brain injury were included. Survival ranged from 27 to 993 days. In all cases judicial autopsy was performed. All cases came before court and in each case child abuse was considered to be proven. In two cases the perpetrator confessed, during police interrogation, to shaking of the child only. Although a known serious outcome, this is one of the few reports on MCE as a result of AHT. In all cases the diagnosis was confirmed at autopsy.


Subject(s)
Brain/pathology , Child Abuse , Encephalomalacia/pathology , Head Injuries, Closed/pathology , Atrophy , Diagnostic Imaging , Encephalomalacia/etiology , Female , Forensic Pathology , Head Injuries, Closed/complications , Humans , Infant , Infant, Newborn , Male , Shaken Baby Syndrome/complications
15.
Osteoporos Int ; 21(8): 1391-400, 2010 Aug.
Article in English | MEDLINE | ID: mdl-19937229

ABSTRACT

SUMMARY: Hand radiographs are obtained routinely to determine bone age of children. This paper presents a method that determines a Paediatric Bone Index automatically from such radiographs. The Paediatric Bone Index is designed to have minimal relative standard deviation (7.5%), and the precision is determined to be 1.42%. INTRODUCTION: We present a computerised method to determine bone mass of children based on hand radiographs, including a reference database for normal Caucasian children. METHODS: Normal Danish subjects (1,867), of ages 7-17, and 531 normal Dutch subjects of ages 5-19 were included. Historically, three different indices of bone mass have been used in radiogrammetry all based on A = piTW(1 - T/W), where T is the cortical thickness and W the bone width. The indices are the metacarpal index A/W(2), DXR-BMD = A/W, and Exton-Smith's index A/(WL), where L is the length of the bone. These indices are compared with new indices of the form A/(W(a) L(b)), and it is argued that the preferred index has minimal SD relative to the mean value at each bone age and sex. Finally, longitudinal series of X-rays of 20 Japanese children are used to derive the precision of the measurements. RESULTS: The preferred index is A/(W(1.33) L(0.33)), which is named the Paediatric Bone Index, PBI. It has mean relative SD 7.5% and precision 1.42%. CONCLUSIONS: As part of the BoneXpert method for automated bone age determination, our method facilitates retrospective research studies involving validation of the proposed index against fracture incidence and adult bone mineral density.


Subject(s)
Bone Density/physiology , Hand Bones/physiology , Radiographic Image Interpretation, Computer-Assisted/methods , Adolescent , Age Determination by Skeleton/methods , Aging/physiology , Algorithms , Child , Child, Preschool , Databases, Factual , Female , Hand Bones/diagnostic imaging , Humans , Male , Reference Values , Young Adult
16.
Ned Tijdschr Geneeskd ; 1642020 09 10.
Article in Dutch | MEDLINE | ID: mdl-33030321

ABSTRACT

Point-of-care ultrasonography (POCUS) was initially limited to simple applications such as diagnosing ascites, sampling pleural fluid and guiding venous access. Use of POCUS is currently on the rise. As radiologists, we endorse the use of ultrasonography as the stethoscope of the future, but we make some critical comments. The interpretation of ultrasonographic findings can have far-reaching therapeutic implications. The patient is therefore entitled to maximum ultrasonographic expertise. Intensive hands-on training starting in early medical school is mandatory and central archiving of ultrasonographic images and reports is essential.


Subject(s)
Point-of-Care Systems/standards , Radiologists , Radiology Information Systems , Ultrasonography/standards , Humans , Radiologists/education , Radiologists/standards , Ultrasonography/methods
17.
Eur J Paediatr Neurol ; 12(1): 41-5, 2008 Jan.
Article in English | MEDLINE | ID: mdl-17537653

ABSTRACT

We report a case of neonatal dural sinus malformation already visible on antenatal ultrasound. This is a rare disease entity in infants and children. Clinical diagnosis was made by demonstrating a cranial murmur on auscultation; macrocrania and signs of progressive cardiac failure. Imaging studies as cerebral ultrasound, postnatal MRI scan and MR angiography demonstrated a large dural sinus malformation originating from the sagittal sinus with extensive arteriovenous fistulae. Due to the extent of the lesion, the existing ischemic brain damage and involvement of the torcular, no therapeutic options were available and the child died of irreversible cardiac failure. The diagnosis was confirmed with autopsy. We discuss the clinical presentation, imaging and neuropathological results and relate our findings to embryological data and the existing literature.


Subject(s)
Central Nervous System Vascular Malformations , Cranial Sinuses/pathology , Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/pathology , Central Nervous System Vascular Malformations/physiopathology , Humans , Infant, Newborn , Magnetic Resonance Angiography , Magnetic Resonance Imaging , Radiography
19.
Forensic Sci Int ; 285: e17-e20, 2018 Apr.
Article in English | MEDLINE | ID: mdl-29370956

ABSTRACT

We present two cases of infants who died under suspicious circumstances. After clinical and legal investigations, non-accidental constrictive asphyxia inflicted by one of the parents was established. The first case presents a to date not yet reported, unique mechanism of trauma. In order to stop his daughter from crying, the father admitted that he sometimes sat on his baby while she was lying on the bed. Occasionally increasing his force by pulling with his hands on the bottom of the bed. In the second case tight swaddling and encircling chest compression was the causative mechanism. In both cases the father was sentenced to imprisonment with mandate psychiatric care. Only two previous reports of this uncommon and relatively unknown cause of child abuse, called constrictive asphyxia, are known. In all reported cases static loading of the chest resulted in rib fractures and demise of the child. This rare abusive mechanism should be known to pediatric radiologists and pathologists.


Subject(s)
Asphyxia/diagnosis , Child Abuse/diagnosis , Constriction, Pathologic/complications , Rib Fractures/diagnostic imaging , Asphyxia/etiology , Female , Humans , Infant , Male , Rib Fractures/etiology , Tomography, X-Ray Computed
20.
Int J Paleopathol ; 23: 15-25, 2018 12.
Article in English | MEDLINE | ID: mdl-29655998

ABSTRACT

Rickets and residual rickets are often encountered in Dutch archeological skeletal samples. However, no archeological Dutch paleopathological case of adult osteomalacia has been described in literature to date. This paper describes the first four archeological Dutch paleopathological cases of osteomalacia and assesses the value of the various modalities (macroscopic assessment, radiology and histology) that may be used for diagnosis. The skeletal remains investigated originate from the Meerenberg psychiatric hospital cemetery in Bloemendaal, the Netherlands, and date from 1891 - 1936. The remains of 69 adult individuals were inspected for macroscopic lesions which may be associated with osteomalacia. In cases suspect for osteomalacia, complimentary radiological and histological investigations (BSE-SEM and light microscopy) were performed. Macroscopically, four individuals presented with lesions (highly) suggestive of osteomalacia. Histological examination (both BSE-SEM and light microscopy) provided valuable information to come to an eventual diagnosis of osteomalacia in all four cases. Light microscopy proved to be an feasible alternative for BSE-SEM. The added value of radiological analyses was limited. The individuals identified were most likely patients in the psychiatric hospital, and the reason for their institutionalization and/or the regime in the institution may have played a role in the development of the osteomalacia observed.


Subject(s)
Osteomalacia/history , Osteomalacia/pathology , Adult , Female , History, 19th Century , History, 20th Century , Humans , Middle Aged , Netherlands , Osteomalacia/diagnostic imaging
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