Detection of Bronchiolitis Obliterans Syndrome Following Pediatric Hematopoietic Stem Cell Transplantation. An Official American Thoracic Society Clinical Practice Guideline.

BACKGROUND: Many children undergo allogeneic Hematopoietic Stem Cell Transplantation (HSCT) for the treatment of malignant and non-malignant conditions. Unfortunately, pulmonary complications occur frequently post-HSCT, with bronchiolitis obliterans syndrome (BOS) being the most common non-infectious pulmonary complication. Current international guidelines contain conflicting recommendations regarding post-HSCT surveillance for BOS, and a recent National Institutes of Health workshop highlighted the need for a standardized approach to post-HSCT monitoring. As such, this guideline provides an evidence-based approach to detection of post-HSCT BOS in children. METHODS: A multinational, multidisciplinary panel of experts identified six questions regarding surveillance for, and evaluation of post-HSCT BOS in children. Systematic review of the literature was undertaken to answer each question. The Grading of Recommendations, Assessment, Development, and Evaluation approach was used to rate the quality of evidence and the strength of recommendations. RESULTS: The panel members considered the strength of each recommendation and evaluated the benefits and risks of applying the intervention. In formulating the recommendations, the panel considered patient and caregiver values, the cost of care, and feasibility. Recommendations addressing the role of screening pulmonary function testing and diagnostic tests in children with suspected post-HSCT BOS were made. Following a Delphi process, new diagnostic criteria for pediatric post-HSCT BOS were also proposed. CONCLUSIONS: This document provides an evidence-based approach to detection of post-HSCT BOS in children, while also highlighting considerations for implementation of each recommendation. Further, the document describes important areas for future research.

The SPEC score-A quantifiable CT scoring system for primary ciliary dyskinesia.

BACKGROUND: Structural lung changes seen on computed tomography (CT) scans in persons with primary ciliary dyskinesia (pwPCD) are currently described using cystic fibrosis (CF) derived scoring systems. Recent work has shown structural changes and frequencies that are unique to PCD, indicating the need for a unique PCD-derived scoring system. METHODS: Chest CT scans from 30 pwPCD, were described for structural changes including bronchiectasis, bronchial wall thickening, mucous plugging, atelectasis, air trapping, and interlobar septal thickening and, additionally, changes previously described as being frequent in pwPCD including extensive tree-in-bud pattern of mucous plugging, bronchoceles or nodules, thickening of interlobar and interlobular septa and whole lobe atelectasis. Based on these findings a novel and unique scoring system, the Specific PCD Evaluation by CT (SPEC) score was constructed. Scans were then re-scored using the SPEC score and results compared to corresponding measurements of lung function to assess structure-function correlation. RESULTS: Total SPEC scores ranged from 0 to 60 (max possible score 90). There was a strong negative correlation between the SPEC score (SPEC) and forced vital capacity (FVC), forced expiratory volume over 1 s (FEV1 ) and FEV1 /FVC ratio (-r = .784, -.865, -.872 respectively). CONCLUSIONS: Using PCD-derived data we describe the construct of a PCD-specific score for assessing lung structural damage on CT scans, the SPEC score. A strong correlation between the SPEC score and PFT variables was identified. The SPEC score holds the potential for describing longitudinal changes in CT scans and assessing the efficacy of interventive therapies in patients with PCD.

Refining diagnostic criteria for paediatric bronchiectasis using low-dose CT scan.

BACKGROUND: There is a current lack of consensus amongst paediatric radiologists and respiratory paediatricians as to the correct CT definition of bronchiectasis in children. Using contemporary low-dose CT, our objectives were to determine the upper limit of normal for broncho-arterial ratio (BAR) in children and to evaluate the effect of age and general anaesthesia. METHODS: Measurements of 330 broncho-arterial ratios from 51 children (0-19 years) undergoing low-dose CT chest for non-respiratory indications were performed by 3 blinded observers (two radiologists, one respiratory physician) using four different methods. Inter-observer reliability, mean BAR and reference ranges (mean±2SD) were calculated. Correlation between age and BARs were examined. Mean BAR for CT under general anaesthesia and CT awake were compared. RESULTS: Inter-observer correlation was extremely high for all measurements (0.93-0.97). There was a weak positive correlation between age and BAR in the CT-awake group (r = 0.33, 95%CI: 0.03-0.57; p = 0.031) using the inner-bronchial wall to artery, short-axis measurement. CT under general anaesthesia showed significantly higher BAR compared to CT-awake [mean difference 0.13 (95%CI: 0.05-0.22; p = 0.004)]. For the CT-awake group, the mean BAR was 0.65 (range: 0.42 to 0.89), with no child having a BAR above 0.9. CONCLUSION: Using a standardised approach, we have shown that a broncho-arterial ratio above 0.9 in children undergoing awake CT is abnormal and suggests airway widening or radiological bronchiectasis. Children undergoing CT under anaesthesia have higher BARs than those undergoing awake CT. A weak positive correlation between broncho-arterial ratio and age was observed, hence, age-adjusted cut-offs for BAR warrant further study.

Young women's attitudes towards, and experiences of, long-acting reversible contraceptives.

OBJECTIVES: To identify factors involved in women's decisions to choose particular contraceptive methods and more specifically, incentives and disincentives to use three long-acting reversible contraceptive (LARC) methods: injectables, implants, and intrauterine devices/systems (IUDs/IUSs). METHODS: A total of 502 women aged 18 to 30 completed a cross-sectional online questionnaire. RESULTS: The three most important factors in choosing a contraceptive method were: high efficacy at preventing pregnancy, protection against sexually transmitted infections, and non-interference with sexual intercourse. The most common incentives for LARC use were the high efficacy and long duration of action. Disincentives included the possibility of irregular bleeding and concerns about effects on fertility; fear of needles and pain was a particular disincentive for IUD/IUS use. Only 93 (18%) of the participants reported ever having used a LARC. CONCLUSIONS: Reported disincentives to LARC use (e.g., concern about effects on future fertility) indicated that many young women hold inaccurate beliefs about these methods. The relatively high proportions of women who held neutral attitudes about LARCs (21-40%, depending on the method) highlight the importance of education and contraceptive counselling to improve knowledge about the advantages of these methods.

Ganglion cysts in the paediatric wrist: magnetic resonance imaging findings.

BACKGROUND: The majority of published literature on ganglion cysts in children has been from a surgical perspective, with no dedicated radiologic study yet performed. OBJECTIVE: Our aim was to assess the magnetic resonance (MR) imaging appearance of ganglion cysts in a series of paediatric MR wrist examinations. MATERIALS AND METHODS: Ninety-seven consecutive paediatric MR wrist examinations were retrospectively reviewed for the presence of ganglion cysts. Only those studies with wrist ganglia were included. Cysts were assessed for location, size, internal characteristics and secondary effect(s). RESULTS: Forty-one ganglion cysts (2-32 mm in size) were seen in 35/97 (36%) patients (24 female, 11 male), mean age: 13 years 11 months (range: 6 years 3 months-18 years). The majority were palmar (63.4%) with the remainder dorsal. Of the cysts, 43.9% were related to a wrist ligament(s), 36.6% to a joint and 17.1% to the triangular fibrocartilage complex. Of the patients, 91.4% had wrist symptoms: pain (n=29, 82.9%), swelling (n=7, 20%) and/or palpable mass (n=4, 11.4%); 71.4% patients had significant additional wrist abnormalities. CONCLUSION: Ganglion cysts were frequently found in children referred for wrist MRI.

Normal paediatric bone marrow: magnetic resonance imaging appearances from birth to 5 years.

INTRODUCTION: Bone marrow (BM) assessment is an important aspect of paediatric MRI, with the marrow cavity visible on almost every clinical MR examination. In practice, however, assessment for marrow infiltration in paediatric patients can be challenging. Our aim was to review the MRI appearance of normal BM from 0 to 5 years. METHODS: Consecutive body MR examinations over 7 years were retrospectively reviewed in patients aged 0-5 years. Patients with anticipated BM abnormality were excluded. All patients had imaging of the spine and/or pelvis with T1-weighted (T1) ± T2 with fat saturation, post-contrast T1-weighted, diffusion-weighted or out-of-phase sequences. RESULTS: Nineteen patients were included: nine (47%) infants, 11 (58%) boys, mean age 18.2 months (range 1 day to 3 years and 10 months). On T1 imaging, 69% infant marrow sites and 24% in children >1 year were isointense, the remainder were hyperintense. One hundred per cent BM was T2 fat saturation hyperintense. Enhancement following contrast was seen in 16% of BM sites. Restricted diffusion was seen in 100% infant BM and 50% BM in children >1 year. On out-of-phase imaging, no signal loss was seen in infants, and 21% BM in children >1 year showed signal loss. CONCLUSION: Due to normal age-related differences in BM histology, MRI for marrow assessment in infants and young children can be misleading and may mimic marrow infiltration.

Potential pitfalls in imaging of paediatric cerebral sinovenous thrombosis.

Paediatric cerebral sinovenous thrombosis (CSVT) is a rare but serious condition. The imaging signs may be subtle with a number of potential pitfalls. We present a pictorial essay of the pitfalls of diagnosis of CSVT on CT and MRI. We describe, using examples, potential pitfalls on both imaging modalities including anatomical variants of the cerebral venous system, extra-axial fluid collections and enhancement of chronic thrombus. Pitfalls particular to CT are discussed including beam-hardening artefact, image windowing and neonatal physiological intravascular hyperdensity. We review the potential variability in the appearance of thrombus on MRI, dependent largely on the stage of thrombus evolution and the pulse sequence. The signal intensity of thrombi, although described as evolving in a typical pattern on T1- and T2-weighted MRI, may be affected by variability in the degree of oxygenation of red cells in the forming thrombus, dilution and secondary propagation of thrombosis. Individual MRI sequences should not be interpreted in isolation, but as a set, and compared with CT images if available.

Developmental dysplasia of the hip: controversies and current concepts.

Developmental dysplasia of the hip is an important but poorly understood disorder as evidenced by the vast amount of literature published to date on this topic. The precise definition of hip dysplasia is controversial and it encompasses a spectrum of abnormalities, rather than a single condition, which at one end overlap with normal hip maturation. We review the risk factors, clinical examination and radiological imaging of the hip in the infant and young child, the controversies of ultrasound screening for hip dysplasia and the current management, both operative and non-operative, of this condition according to patient age.

Ultrasonography in developmental dysplasia of the hip: what have we learned?

Developmental dysplasia of the hip (DDH) is an important but poorly defined entity, the natural history of which is incompletely understood. The term encompasses a disease spectrum ranging from a stable hip with a mildly dysplastic acetabulum to complete hip dislocation. Much controversy surrounds the diagnosis, imaging and management of this condition. We present a review of the history of US imaging of DDH from the radiologist's perspective, summarising the most widely recognised US methods described to date. We discuss controversies in the approach to US examination, with particular emphasis on current opinions. The existing scientific evidence for and variations in the practice of US screening for DDH, including recommendations from the United States Preventive Services Task Force and the ESPR DDH Taskforce Group respectively, are discussed.

Chloral hydrate sedation in radiology: retrospective audit of reduced dose.

BACKGROUND: Chloral hydrate (CH) is safe and effective for sedation of suitable children. OBJECTIVE: The purpose of this study was to assess whether adequate sedation is achieved with reduced CH doses. MATERIALS AND METHODS: We retrospectively recorded outpatient CH sedations over 1 year. We defined standard doses of CH as 50 mg/kg (infants) and 75 mg/kg (children >1 year). A reduced dose was defined as at least 20% lower than the standard dose. RESULTS: In total, 653 children received CH sedation (age, 1 month-3 years 10 months), 42% were given a reduced initial dose. Augmentation dose was required in 10.9% of all children, and in a higher proportion of children >1 year (15.7%) compared to infants (5.7%; P < 0.001). Sedation was successful in 96.7%, and more frequently successful in infants (98.3%) than children >1 year (95.3%; P = 0.03). A reduced initial dose had no negative effect on outcome (P = 0.19) or time to sedation. No significant complications were seen. CONCLUSION: We advocate sedation with reduced CH doses (40 mg/kg for infants; 60 mg/kg for children >1 year of age) for outpatient imaging procedures when the child is judged to be quiet or sleepy on arrival.

PHACE syndrome: MRI of intracerebral vascular anomalies and clinical findings in a series of 12 patients.

BACKGROUND: PHACE (posterior fossa defects, haemangioma, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities) syndrome describes a constellation of abnormalities that can occur in association with segmental craniofacial infantile haemangioma. OBJECTIVE: To report the spectrum of clinical and imaging abnormalities seen in a cohort of children. MATERIALS AND METHODS: A retrospective review of the clinical and imaging records of all patients diagnosed with PHACE syndrome between 1998 and 2009 was performed. Information sought included patient demographics, craniofacial segments involved, imaging findings and other extracutaneous abnormalities. RESULTS: Twelve patients were diagnosed with PHACE syndrome over 11 years. All patients had a segmental craniofacial haemangioma. Involved facial segments, in order of frequency, were frontotemporal (12), maxillary (8), mandibular (5) and frontonasal (1). The most common extracutaneous abnormalities were neurovascular anomalies (10), with many patients having multiple anomalies. The spectrum of arterial anomalies ranged from hypoplasia (9) to ectasia (3), anomalous origin/course (2) and persistent fetal anastomosis (2). Other anomalies found included cardiac anomalies (3), coarctation of the aorta (2), posterior fossa malformations (1) and sternal region anomalies (1). CONCLUSION: Intracranial anomalies are the most common extracutaneous feature of PHACE syndrome. The contribution of the radiologist in the recognition of such anomalies is important for the diagnosis of PHACE syndrome.