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Offiah A.C.,Academic Unit of Child Health
Pediatric Radiology | Year: 2014

Background: Paediatric non-accidental injury is a considerable health problem, and imaging plays a fundamental role in its assessment. Since the introduction of joint guidelines published by the Royal College of Radiologists (RCR) and Royal College of Paediatrics and Child Health (RCPCH) in 2008, there has been a concerted effort to adopt a consensus approach to imaging in this area in the United Kingdom.Objective: This study aims to establish current practice amongst European Society of Paediatric Radiology (ESPR) members within their institutions with regards to the use of imaging in suspected non-accidental injury.Materials and methods: A web-based survey was created and circulated to all active ESPR members. The responses were collated and analysed.Results: We received 134 responses, accounting for 37% of the current membership across 24 European countries. These responses highlighted significant variation amongst institutions, with no consensus regarding radiographic and cross-sectional imaging in the investigation of suspected non-accidental injury.Conclusion: There is a need for a European consensus protocol to imaging in suspected non-accidental injury. We recommend implementing the joint RCR/RCPCH guidance, a move supported by the ESPR. © 2014, Springer-Verlag Berlin Heidelberg.


Thayyil S.,University College London | Sebire N.J.,University College London | Sebire N.J.,Great Ormond Street Hospital for Children National Health Service NHS Foundation Trust | Chitty L.S.,University College London | And 16 more authors.
The Lancet | Year: 2013

Summary Background Post-mortem MRI is a potential diagnostic alternative to conventional autopsy, but few large prospective studies have compared its accuracy with that of conventional autopsy. We assessed the accuracy of whole-body, post-mortem MRI for detection of major pathological lesions associated with death in a prospective cohort of fetuses and children. Methods In this prospective validation study, we did pre-autopsy, post-mortem, whole-body MRI at 1·5 T in an unselected population of fetuses (≤24 weeks' or >24 weeks' gestation) and children (aged <16 years) at two UK centres in London between March 1, 2007 and Sept 30, 2011. With conventional autopsy as the diagnostic gold standard, we assessed MRI findings alone, or in conjunction with other minimally invasive post-mortem investigations (minimally invasive autopsy), for accuracy in detection of cause of death or major pathological abnormalities. A radiologist and pathologist who were masked to the autopsy findings indicated whether the minimally invasive autopsy would have been adequate. The primary outcome was concordance rate between minimally invasive and conventional autopsy. Findings We analysed 400 cases, of which 277 (69%) were fetuses and 123 (31%) were children. Cause of death or major pathological lesion detected by minimally invasive autopsy was concordant with conventional autopsy in 357 (89·3%, 95% CI 85·8-91·9) cases: 175 (94·6%, 90·3-97·0) of 185 fetuses at 24 weeks' gestation or less, 88 (95·7%, 89·3-98·3) of 92 fetuses at more than 24 weeks' gestation, 34 (81·0%, 67·7-90·0) of 42 newborns aged 1 month or younger, 45 (84·9%, 72·9-92·1) of 53 infants aged older than 1 month to 1 year or younger, and 15 (53·6%, 35·8-70·5) of 28 children aged older than 1 year to 16 years or younger. The dedicated radiologist or pathologist review of the minimally invasive autopsy showed that in 165 (41%) cases a full autopsy might not have been needed; in these cases, concordance between autopsy and minimally invasive autopsy was 99·4% (96·6-99·9). Interpretation Minimally invasive autopsy has accuracy similar to that of conventional autopsy for detection of cause of death or major pathological abnormality after death in fetuses, newborns, and infants, but was less accurate in older children. If undertaken jointly by pathologists and radiologists, minimally invasive autopsy could be an acceptable alternative to conventional autopsy in selected cases. Funding Policy research Programme, Department of Health, UK. © 2013 Elsevier Ltd.


Elder C.J.E.,Academic Unit of Child Health | Natarajan A.,Royal Infirmary
Journal of Pediatric Endocrinology and Metabolism | Year: 2010

We report two cases of adolescent females with poorly controlled diabetes mellitus who were found to have gross hepatomegaly on annual review. With the additional findings of short stature (in one case), delayed puberty and a Cushingoid habitus they were diagnosed with Mauriac syndrome. Within our diabetes service we have incorporated regular abdominal examinations for all children and young people with long standing, poorly controlled diabetes (HbA1c persistently >9.5%). A brief review of the literature is included. © Freund Publishing House Ltd.


Johnson R.,University of Sheffield | Offiah A.,Academic Unit of Child Health | Cohen M.C.,Sheffield Childrens Nhs Foundation Trust
Forensic Science, Medicine, and Pathology | Year: 2015

Aplasia cutis congenita (ACC) is a rare condition that occurs in around 0.01 % of births. Characterized by a localized absence of skin, it affects the midline of the scalp in over 80 % of cases. We describe the case of an infant born via vaginal spontaneous delivery with ACC affecting the scalp. This extended to the subcutaneous tissue and bone leaving the dura mater exposed. The patient was managed conservatively. At 4 weeks of age, she had a fatal superior sagittal sinus hemorrhage. In a review of the literature, we found ten previous cases of hemorrhage of the superior sagittal sinus complicating an ACC. Including our case, ACC complicated by hemorrhage of the superior sagittal sinus shows a 36 % mortality (4/11). An analysis of these cases suggests that this tends to occur between 1 and 3 months of age, though it may occur in younger neonates. © 2015, Springer Science+Business Media New York.


Klusmann M.,University College London | Van'T Hoff W.,Great Ormond Street Hospital for Children | Monsell F.,Royal Hospital for Children | Offiah A.C.,Academic Unit of Child Health
Skeletal Radiology | Year: 2014

Nephropathic cystinosis is an autosomal recessive lysosomal storage disorder in which intracellular cystine accumulates. It is caused by mutations in the CTNS gene. Clinical manifestations include renal tubular Fanconi syndrome in the first year of life, rickets, hypokalaemia, polyuria, dehydration and acidosis, growth retardation, hypothyroidism, photophobia and renal glomerular deterioration. Late complications include myopathy, pancreatic insufficiency and retinal blindness. Skeletal manifestations described in these patients include failure to thrive, osteomalacia, rickets and short stature. This paper describes progressive bony abnormalities in three unrelated patients with nephropathic cystinosis that have not been reported previously. © 2013 ISS.


Calder A.D.,Great Ormond Street Hospital for Children NHS Foundation Trust | Offiah A.C.,Academic Unit of Child Health
Pediatric Radiology | Year: 2014

Despite advances in antenatal imaging and genetic techniques, post-delivery post-mortem foetal radiography remains the key investigation in accurate diagnosis of skeletal dysplasia manifesting in the foetus. Foetal radiography is best performed using pathology-specimen radiography equipment and is often carried out in the pathology department without involvement of the radiology unit. However, paediatric radiologists may be asked to interpret post-mortem foetal radiographs when an abnormality is suspected. Many foetal radiographs are carried out before 20 weeks' gestation, and the interpreting radiologist needs to be familiar with the range of normal post-mortem foetal appearances at different gestational ages, as well as the appearances of some of the more commonly presenting skeletal dysplasias, and will benefit from a systematic approach when assessing more challenging cases. In this pictorial essay, we illustrate various normal post-mortem foetal radiographic appearances, give examples of commonly occurring skeletal dysplasias, and describe an approach to establishing more difficult diagnoses. © 2014 Springer-Verlag Berlin Heidelberg.


Arundel P.,University of Sheffield | Arundel P.,Academic Unit of Child Health | Offiah A.,University of Sheffield | Bishop N.J.,University of Sheffield
Journal of Bone and Mineral Research | Year: 2011

We present the first report of the development of characteristic radiologic appearances of long bones during the first year of life in an infant with type V osteogenesis imperfecta (OI). We show the evolution of metaphyseal abnormalities from a rickets-like appearance to the classically described dense metaphyseal bands. These abnormalities suggest that the underlying defect in type V OI may involve a molecule common to both bone and cartilage that is involved in the regulation of growth plate development and metadiaphyseal ossification. Our findings provide new insights into skeletal development in type V OI and potentially yield useful clues to the identity of the defect underpinning the condition. Copyright © 2011 American Society for Bone and Mineral Research.


Emerson N.J.,University of Sheffield | Offiah A.C.,Academic Unit of Child Health | Reilly G.C.,University of Sheffield | Carre M.J.,University of Sheffield
Strain | Year: 2013

The accuracy of biomechanical simulation has been improved by using high-resolution computed tomography (CT) to define the geometry and material parameters. This technique has been used to assess numerous systems, including the mechanical properties of bone, fixation techniques post-fracture and the performance of bone microarchitecture. In this study, a semi-automated process for converting CT data into finite element (FE) models was used to model the mid-shaft (diaphysis) of porcine femoral samples under sub-maximal torsional and compressive load. Physical validation was undertaken to investigate if the all-important geometry and material property mapping functioned correctly. Porcine femoral specimens were imaged using contiguous helical CT, which was converted to FE models using ScanIP from Simpleware, Exeter, UK. The heterogeneous material properties were estimated using density-elasticity relationships proposed in literature for human bone samples. Laboratory testing performed favourably, with a linear strain response validating the use of the array of linear material models used in simulation. The simulation procedure also performed well. Linear regression and mean error calculation demonstrated accurate correlation between predicted (from simulation) and observed (measured within the laboratory) results that offered improvement over the accuracy within comparative testing for human samples. Using FE modelling on a patient-specific basis offers potential in a number of scenarios, including the determination of injury risk and design of protective equipment. The increased accessibility of animal samples allows large-scale fracture testing of complex loading mechanisms and the potential to consider younger animal samples (to investigate the behaviour of developing bone). Spiral fractures of long bones have been demonstrated to be an indicator of non-accidental injury in children. Combining the increased accuracy in torsional simulation in this study with younger sample testing may be employed to attempt to determine the causes of fracture from post fracture scans, aiding in the diagnosis of non-accidental injury. © 2013 Wiley Publishing Ltd.


Davis W.R.,University College London | Davis W.R.,The Royal London Hospital | Halls J.E.,St Georges Hospital | Offiah A.C.,Great Ormond Street Hospital | And 5 more authors.
Rheumatology | Year: 2011

Objective: To assess the reproducibility of a novel scoring system that we have developed for the objective assessment of acute inflammatory change in JDM. This system defines markers of inflammatory change in four muscle groups and the surrounding soft tissues. Methods: Forty-eight children (33 girls) underwent retrospective assessment of their MRI studies by two musculoskeletal paediatric radiologists for the presence of disease activity. Each observer performed the readings on two separate occasions. The degree of concordance between the two observers and between the two readings was assessed using kappa analysis. The reproducibility of the total score was determined using Bland-Altman analysis. Results: There was fair to moderate agreement between the two observers for all the examined disease activity markers in all muscle groups. There was good intra-observer agreement between the two readings. There was no difference according to the side evaluated. The mean total score (out of 20) for Observer 1 was 7.9 and for Observer 2 was 7.5 (mean difference -0.4, 95% limits of agreement -6.8 to 6.0), while the mean total scores for Observer 1 were 9.0 for the first reading and 7.9 for the second reading (mean difference 1.0, 95% limits of agreement -2.6 to 4.6). Conclusion: Markers of inflammatory change in JDM can be observed on MRI in a reliable fashion and have been used to make a reliable and objective scoring system. The accuracy of the proposed scoring system is acceptable for the single reader, although there is more variability between two different individuals. © The Author 2011. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.


Roposch A.,University College London | Liu L.Q.,University College London | Offiah A.C.,Academic Unit of Child Health | Wedge J.H.,University of Toronto
Journal of Bone and Joint Surgery - Series A | Year: 2011

Background: Osteonecrosis of the femoral head is a major potential complication following the treatment of developmental dysplasia of the hip. It remains unclear if the radiographic changes associated with osteonecrosis are clinically relevant. Methods: In the present cross-sectional study, we determined the relationship between morphological changes on radiographs (classified with use of the Bucholz-Ogden system) and health-related quality of life (assessed with the Health Utilities Index Mark 3 [HUI3]; maximum score, 1), physical function (assessed with the Activities Scale for Kids [ASK]; maximum score, 100), and hip function (assessed with the Children's Hospital Oakland Hip Evaluation Scale [CHOHES]; maximum score, 100). The study group included seventy-two children (mean age, 14 ± 2.5 years) with a diagnosis of osteonecrosis of the hip secondary to the treatment of developmental dysplasia of the hip. Patient assessments were standardized (intraclass correlation coefficient, ≥0.93). Radiographs were graded by three experts according to consensus. Analyses were adjusted for the number of previous surgical procedures on the hip and for the severity of residual hip dysplasia. Results: The median ASK score was 97 (interquartile range, 93 to 100), the median CHOHES score was 86 (interquartile range, 77 to 96), and the median HUI3 score was 1 (interquartile range, 0.9 to 1). The ASK summary scores were nearly equal (median, >90) across all radiographic grades. Adjusted mean scores showed a downward shift with worse radiographic grades. The ASK scores (p = 0.004) and CHOHES scores (p = 0.006) differed across radiographic grades, with Bucholz-Ogden grade-I and II hips demonstrating significantly better scores than grade-III and IV hips. Discussion: Osteonecrosis secondary to the treatment of developmental dysplasia of the hip is a relatively benign condition in children and teenagers. While it was associated with limited hip function, it was not associated with physical disability. However, we speculate that this function will decline with increasing age. With regard to clinical outcome, Bucholz-Ogden grade-I hips are similar to grade-II hips and grade-III hips are similar to grade-IV hips. Level of Evidence: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence. Copyright © 2011 by the Journal of Bone and Joint Surgery, Incorporated.

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