Single versus double hamstring tendon graft in anterior cruciate ligament reconstruction in the paediatric patient: a single-blind randomised controlled trial study protocol.

INTRODUCTION: There is currently no clear indication in the literature regarding a single or double hamstring tendon (single bundle) autograft for anterior cruciate ligament (ACL) reconstruction in the paediatric patient. The primary aim of this single blind randomised controlled trial is to determine whether a single or double hamstring tendon graft ACLR leads to superior clinical outcomes postsurgery in paediatric patients with ACL injury. METHODS AND ANALYSIS: Single site, prospective, single blind, randomised controlled trial with two parallel treatment arms. 100 patients aged 10-18 years who present with an isolated ACL tear±meniscal injury, verified on MRI, will be randomly allocated to one of the two surgical groups. The primary outcomes will be side-to-side difference in anterior tibial translation and graft failure incidence 12 months postsurgery. Primary and secondary outcomes will also be assessed at 2-year and 5-year postsurgery. ETHICS AND DISSEMINATION: Results will be presented in peer-reviewed journals and at international conferences and disseminated to participants and healthcare professionals via newsletters and hospital presentations. This study is approved by the Children's Health Queensland Hospital and Health Service Human Research Ethics committee. TRIAL REGISTRATION NUMBER: ACTRN12620001170910p; Australian New Zealand Clinical Trials Registry.

The ten-year evolutionary trajectory of a highly recurrent paediatric high grade neuroepithelial tumour with MN1:BEND2 fusion.

Astroblastomas are rare brain tumours which predominate in children and young adults, and have a controversial claim as a distinct entity, with no established WHO grade. Reports suggest a better outcome than high grade gliomas, though they frequently recur. Recently, they have been described to overlap with a newly-discovered group of tumours described as'high grade neuroepithelial tumour with MN1 alteration' (CNS HGNET-MN1), defined by global methylation patterns and strongly associated with gene fusions targeting MN1. We have studied a unique case of astroblastoma arising in a 6 year-old girl, with multiple recurrences over a period of 10 years, with the pathognomonic MN1:BEND2 fusion. Exome sequencing allowed for a phylogenetic reconstruction of tumour evolution, which when integrated with clinical, pathological and radiological data provide for a detailed understanding of disease progression, with initial treatment driving tumour dissemination along four distinct trajectories. Infiltration of distant sites was associated with a later genome doubling, whilst there was evidence of convergent evolution of different lesions acquiring distinct alterations targeting NF-κB. These data represent an unusual opportunity to understand the evolutionary history of a highly recurrent childhood brain tumour, and provide novel therapeutic targets for astroblastoma/CNS HGNET-MN1.

A comparison of virtual bronchoscopy versus flexible bronchoscopy in the diagnosis of tracheobronchomalacia in children.

INTRODUCTION: Flexible bronchoscopy (FB) is the current gold standard for diagnosing tracheobronchomalacia. However, it is not always feasible and virtual bronchoscopy (VB), acquired from chest multi-detector CT (MDCT) scan is an alternative diagnostic tool. We determined the sensitivity, specificity, and positive and negative predictive values of VB compared to FB in diagnosing tracheobronchomalacia. METHODS: Children aged <18-years scheduled for FB and MDCT were recruited. FB and MDCT were undertaken within 30-min to 7-days of each other. Tracheobronchomalacia (mild, moderate, severe, very severe) diagnosed on FB were independently scored by two pediatric pulmonologists; VB was independently scored by two pairs (each pair = pediatric pulmonologist and radiologist), in a blinded manner. RESULTS: In 53 children (median age = 2.5 years, range 0.8-14.3) evaluated for airway abnormalities, tracheomalacia was detected in 37 (70%) children at FB. Of these, VB detected tracheomalacia in 20 children, with a sensitivity of 54.1% (95%CI 37.1-70.2), specificity = 87.5% (95%CI 60.4-97.8), and positive predictive value = 90.9% (95%CI 69.4-98.4). The agreement between pediatric pulmonologists for diagnosing tracheomalacia by FB was excellent, weighted κ = 0.8 (95%CI 0.64-0.97); but only fair between the pairs of pediatric pulmonologists/radiologists for VB, weighted κ = 0.47 (95%CI 0.23-0.71). There were 42 cases of bronchomalacia detected on FB. VB had a sensitivity = 45.2% (95%CI 30.2-61.2), specificity = 95.5% (95%CI 94.2-96.5), and positive predictive value = 23.2 (95%CI 14.9-34.0) compared to FB in detecting bronchomalacia. CONCLUSION: VB cannot replace FB as the gold standard for detecting tracheobronchomalacia in children. However, VB could be considered as an alternative diagnostic modality in children with symptoms suggestive of tracheobronchomalacia where FB is unavailable. Pediatr Pulmonol. 2017;52:480-486. © 2016 Wiley Periodicals, Inc.

The radiological diagnosis of pneumonia in children.

Despite the importance of paediatric pneumonia as a cause of short and long-term morbidity and mortality worldwide, a reliable gold standard for its diagnosis remains elusive. The utility of clinical, microbiological and radiological diagnostic approaches varies widely within and between populations and is heavily dependent on the expertise and resources available in various settings. Here we review the role of radiology in the diagnosis of paediatric pneumonia. Chest radiographs (CXRs) are the most widely employed test, however, they are not indicated in ambulatory settings, cannot distinguish between viral and bacterial infections and have a limited role in the ongoing management of disease. A standardised definition of alveolar pneumonia on a CXR exists for epidemiological studies targeting bacterial pneumonias but it should not be extrapolated to clinical settings. Radiography, computed tomography and to a lesser extent ultrasonography and magnetic resonance imaging play an important role in complicated pneumonias but there are limitations that preclude their use as routine diagnostic tools. Large population-based studies are needed in different populations to address many of the knowledge gaps in the radiological diagnosis of pneumonia in children, however, the feasibility of such studies is an important barrier.

'Benign' ovarian teratoma and N-methyl-D-aspartate receptor (NMDAR) encephalitis in a child.

N-methyl-D-aspartate receptor (NMDAR) encephalitis is a life-threatening paraneoplastic neuropsychiatric encephalitis that predominantly affects young women and has a strong association with ovarian teratomas. Removal of the ovarian teratomas improves the prognosis and decreases the risk of recurrence. We present an 11-year-old girl with NMDAR encephalitis with small bilateral teratomas not initially appreciated on abdominal CT or pelvic MRI. A 12-mm teratoma was identified in the right ovary and a 7-mm teratoma was identified in the left ovary on US follow-up at 5 months. Intraoperative sonography was used to localize the teratomas for excision. In NMDAR encephalitis, the ovarian teratomas can be very small, particularly in children, and easily missed on cross-sectional imaging. Awareness of the association of NMDAR encephalitis and ovarian teratomas will improve the diagnostic accuracy and imaging interpretation. Periodic sonography and MRI might be warranted in children if the initial study is negative.

CT evaluation of extensor tendon entrapment as a complication of a distal radial fracture in a child.

Extensor indicis proprius (EIP) entrapment is a rare complication of a distal radial fracture. We report an 11-year-old with limited flexion of her index finger 1 year after a distal radial fracture. The utility of cross-sectional imaging in the diagnosis and preoperative planning of this complication is presented.

Importance of hepatic fibrosis in cystic fibrosis and the predictive value of liver biopsy.

UNLABELLED: Cystic fibrosis liver disease (CFLD), which results from progressive hepatobiliary fibrosis, is an important cause of morbidity and mortality, but it is difficult to identify before portal hypertension (PHT) ensues. Clinical signs, serum alanine aminotransferase (ALT) levels, and ultrasound (US) are widely applied, but their value in predicting the presence of cirrhosis, the development of PHT, or adverse outcomes is undetermined. The potential gold standard, liver biopsy, is not standard practice and, notwithstanding sampling error considerations, has not been systematically evaluated. Forty patients with cystic fibrosis (median age = 10.6 years) with abnormal clinical, biochemical, and US findings were subjected to dual-pass percutaneous liver biopsy. Clinical outcomes were recorded over 12 years of follow-up (median = 9.5 years for survivors). Logistic regression and receiver operating characteristic analyses were applied to predict hepatic fibrosis (which was assessed by fibrosis staging and quantitative immunohistochemistry) and the occurrence of PHT. PHT occurred in 17 of 40 patients (42%), including 6 of 7 (17%) who died during follow-up. Clinical examination, serum ALT levels, and US findings failed to predict either the presence of liver fibrosis or the development of PHT. Fibrosis staging on liver biopsy, where the accuracy was improved by dual passes (P = 0.002, nonconcordance = 38%), predicted the development of PHT (P < 0.001), which occurred more frequently and at a younger age in those with severe fibrosis. CONCLUSION: Clinical modalities currently employed to evaluate suspected CFLD help to identify a cohort of children at risk for liver disease and adverse outcomes but do not predict an individual's risk of liver fibrosis or PHT development. Liver fibrosis on biopsy predicts the development of clinically significant liver disease. Dual passes help to address sampling concerns. Liver biopsy has a relevant role in the management of patients with suspected CFLD and deserves more widespread application.

The vertebral body growth plate in scoliosis: a primary disturbance of growth?

UNLABELLED: STUDY DESIGN AND AIMS: This was an observational pilot study of the vertebral body growth plates in scoliosis involving high-resolution coronal plane magnetic resonance (MR) imaging and histological examination. One aim of this study was to determine whether vertebral body growth plates in scoliosis demonstrated abnormalities on MR imaging. A second aim was to determine if a relationship existed between MR and histological abnormalities in these vertebral body growth plates. METHODS: MR imaging sequences of 18 patients demonstrated the vertebral body growth plates well enough to detect gross abnormalities/deficient areas/zones. Histological examination of ten vertebral body growth plates removed during routine scoliosis surgery was performed. Observational histological comparison with MR images was possible in four cases. RESULTS: Four of the 18 MR images demonstrated spines with normal curvature and normal vertebral body growth plates. In 13 scoliotic spines, convex and concave side growth plate deficiencies were observed most frequently at or near the apex of the curve. One MR image demonstrated a 55 degrees kyphosis and no convex or concave side deficiencies. The degree of vertebral body wedging was independent of the presence of vertebral body growth plate deficiency. Histological abnormalities of the vertebral body growth plates were demonstrated in four with MR imaging abnormalities. CONCLUSION: This study demonstrated MR image abnormalities of scoliotic vertebral body growth plates compared to controls. A qualitative relationship was demonstrated between MR imaging and histological abnormalities. The finding that vertebral body growth plate deficiencies occurred both on the convex and concave sides of the spine, closest to the apical vertebra of the scoliosis curve, implied that they are less likely to be the result of adaptive changes to the physical forces involved in the scoliotic deformity. One explanation is that they represent a primary disturbance of growth.

Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease.

BACKGROUND: The objective of our study was to determine if hepatic ultrasound findings in paediatric patients with cystic fibrosis and suspected liver disease are related to histopathological results derived from liver biopsies. METHODS: A retrospective analysis of ultrasound and liver biopsy findings using published criteria was performed in 30 children with cystic fibrosis suspected as having liver disease on clinical, biochemical and ultrasonographic criteria. The results were correlated and assessed for intra- and interobserver agreement. RESULTS: A significant association was found for the prediction of fibrosis or cirrhosis on the basis of ultrasound (p=0.03). There was no significant association between normal or indeterminate ultrasound and histology results. A high intra- and interobserver variability was found in sonographic assessment of the hepatic echostructure. CONCLUSIONS: The diagnosis of early liver disease in cystic fibrosis cannot reliably be made on the basis of ultrasound alone. A normal ultrasound does not preclude significant liver fibrosis in cystic fibrosis. An abnormal US that suggests cirrhosis predicts the presence of moderate to severe liver disease.

Craniopagus: second Brisbane case. Case report.

Craniopagus is a rare and intriguing condition with an incidence of one in 2.5 million births. The chance of a neurosurgeon seeing a case in a working lifetime is unlikely. The chances of two cases from the same community within 12 months are remote in the extreme. The authors present a second case of craniopagus born and separated in Brisbane, Australia, in 2001 and discuss the intricacies of surgical separation and the lessons learned.

Assessment of image quality of a standard and two dose-reducing protocols in paediatric pelvic CT.

BACKGROUND: Concerns exist regarding the effect of radiation dose from paediatric pelvic CT scans and the potential later risk of radiation-induced neoplasm and teratogenic outcomes in these patients. OBJECTIVE: To assess the diagnostic quality of CT images of the paediatric pelvis using either reduced mAs or increased pitch compared with standard settings. MATERIALS AND METHODS: A prospective study of pelvic CT scans of 105 paediatric patients was performed using one of three protocols: (1) 31 at a standard protocol of 200 mA with rotation time of 0.75 s at 120 kVp and a pitch factor approximating 1.4; (2) 31 at increased pitch factor approaching 2 and 200 mA; and (3) 43 at a reduced setting of 100 mA and a pitch factor of 1.4. All other settings remained the same in all three groups. Image quality was assessed by radiologists blinded to the protocol used in each scan. RESULTS: No significant difference was found between the quality of images acquired at standard settings and those acquired at half the standard mAs. The use of increased pitch factor resulted in a higher proportion of poor images. CONCLUSIONS: Images acquired at 120 kVp using 75 mAs are equivalent in diagnostic quality to those acquired at 150 mAs. Reduced settings can provide useful imaging of the paediatric pelvis and should be considered as a standard protocol in these situations.