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To determine the frequency and nature of MRI lesions in children with neurofibromatosis type I (NF1), 50 patients aged 8 to 16 years were evaluated prospectively with cranial MRI. Forty-one children were asymptomatic with respect to central nervous system pathology, and 50 % were macrocephalic. Sixteen patients (32 %) had normal MRI examinations. Thirty-two patients (64 %) had high intensity lesions on T2-weighted images and 16 patients (32 %) had hyperintense lesions on T1-weighted images. Seven patients (14 %) had ventricular dilatation (associated with increased intracranial pressure in 2) and 11 patients (22 %) had optic pathway lesions (optic glioma in 7). MRI was superior to CT in differentiating optic sheath thickening and optic nerve tortuosity from optic glioma in four patients. An intracranial tumour (ependymoma) and sphenoid wing dysplasia were evident in individual patients. Findings previously unreported in NF1 included an aqueductal web resulting in hydrocephalus, intraocular neurofibroma resulting in retinal detachment, and asymptomatic enlargement of the septum pellucidum. T1 and T2 signal abnormalities in isolation were not associated with neurological deficits or the occurrence of macrocephaly, and all lesions that required intervention were suspected clinically. Macrocephaly in the absence of increased intracranial pressure or accelerated head growth is not an indication for neuroimaging in children with NF1. However, the majority of children (68 %) had disease-specific abnormalities and thus MRI may provide a useful adjunct to clinical evaluation in the diagnosis of equivocal cases. [ABSTRACT FROM AUTHOR]

Objective: To investigate the feasibility of diffusion-weighted magnetic resonance imaging (DW-MRI) as a predictive imaging marker after neoadjuvant chemotherapy in patients with rhabdomyosarcoma., Material and Methods: We performed a multicenter retrospective study including pediatric, adolescent and young adult patients with rhabdomyosarcoma, Intergroup Rhabdomyosarcoma Study group III/IV, treated according to the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS2005 or MTS2008 studies. DW-MRI was performed according to institutional protocols. We performed two-dimensional single-slice tumor delineation. Areas of necrosis or hemorrhage were delineated to be excluded in the primary analysis. Mean, median and 5th and 95th apparent diffusion coefficient (ADC) were extracted., Results: Of 134 included patients, 82 had measurable tumor at diagnosis and response and DW-MRI scans of adequate quality and were included in the analysis. Technical heterogeneity in scan acquisition protocols and scanners was observed. Mean ADC at diagnosis was 1.1 (95% confidence interval [CI]: 1.1-1.2) (all ADC expressed in * 10 -3 mm 2 /s), versus 1.6 (1.5-1.6) at response assessment. The 5th percentile ADC was 0.8 (0.7-0.9) at diagnosis and 1.1 (1.0-1.2) at response. Absolute change in mean ADC after neoadjuvant chemotherapy was 0.4 (0.3-0.5). Exploratory analyses for association between ADC and clinical parameters showed a significant difference in mean ADC at diagnosis for alveolar versus embryonal histology. Landmark analysis at nine weeks after the date of diagnosis showed no significant association (hazard ratio 1.3 [0.6-3.2]) between the mean ADC change and event-free survival., Conclusion: A significant change in the 5th percentile and the mean ADC after chemotherapy was observed. Strong heterogeneity was identified in DW-MRI acquisition protocols between centers and in individual patients., (© 2023. The Author(s).)

Rhabdomyosarcoma is the most common soft-tissue sarcoma in children. The most common sites are head and neck, genitourinary tract and extremities. In this review we outline the clinical and radiologic features of paediatric rhabdomyosarcoma, as well as imaging considerations and imaging of relapse.

Over recent years significant advances in histopathology and functional imaging techniques for the diagnosis and restaging of children with neuroblastoma have led to better quantification of disease and assessment of disease response, allowing for better treatment stratification. In this review we summarise recent changes to the International Neuroblastoma Response Criteria including the use of RECIST (Response Evaluation Criteria in Solid Tumours) guidance for measurable soft-tissue disease, replacement of technetium-99 m-methylene diphosphonate (MDP) bone scans with metaiodobenzylguanidine (MIBG) scan or [F-18]2-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET)/CT scanning, and a new category of minimal residual bone marrow disease.

Background: Intramural duodenal haematomas (IDHs) are a rare complication of endoscopic biopsy but can cause significant morbidity and mortality, including duodenal obstruction, hospitalization and needing intravenous feeding. They are extremely uncommon in those with normal haematology., Objective: To describe the occurrence of IDHs following endoscopic biopsy in our institution., Materials and Methods: We identified three patients who developed a post biopsy IDH during an 18-month period (2010-2012) in a retrospective search of our hospital pathology and imaging databases., Results: All three children had complex medical problems and presented with gastrointestinal symptoms including severe abdominal pain, reflux, poor feeding and abnormal gut transit time. All underwent normal upper GI endoscopy with duodenal biopsy. Following endoscopy, they presented with intermittent GI obstruction with severe abdominal pain, distension and bilious vomiting or symptoms of pancreatitis, had imaging features of IDH and were managed conservatively making a full recovery. Initial haematology including platelet counts were normal, but two children were subsequently found to have platelet dysfunction and the third to have an unclassified coagulopathy., Conclusion: IDHs may be the presenting factor in children with unsuspected bleeding problems. We present these findings to raise awareness of the imaging features and clinical impact of these cases because better understanding of these risk factors may help to avoid these complications in children in the future.

Palliative care is an expanding specialty within paediatrics, which has attracted little attention in the paediatric radiological literature. Paediatric patients under a palliative care team will have numerous radiological tests which we traditionally categorise under organ systems rather than under the umbrella of palliative medicine. The prevalence of children with life-limiting illness is significant. It has been estimated to be one per thousand, and this may be an underestimate. In this review, we will focus on our experience at one institution, where radiology has proven to be an invaluable partner to palliative care. We will discuss examples of conditions commonly referred to our palliative care team and delineate the crucial role of diagnostic radiology in determining treatment options.

Background: Primary yolk sac tumour of the liver is extremely rare, and when it occurs in a young child it can be confused with hepatoblastoma., Objective: To present a retrospective case series of primary hepatic yolk sac tumour from two institutions to highlight the imaging features., Materials and Methods: We conducted a retrospective review of pathological and imaging findings in biopsy-proven pure primary yolk sac tumour of the liver., Results: A total of three children (15 months to 6 years) presented between 2006 and 2012 with primary hepatic yolk sac tumour. Two children presented with acute abdomen after minor trauma caused by tumour rupture and haemoperitoneum. We identified five additional paediatric cases of pure hepatic yolk sac tumour with descriptions of imaging findings in the literature. In seven of these eight children, central necrosis was seen on cross-sectional imaging., Conclusion: Primary yolk sac tumour should be considered as an alternative diagnosis to hepatoblastoma in a young child with a large liver mass and significantly raised serum alpha-foetoprotein, but in view of the nonspecific imaging features histological examination is essential for diagnosis. Although there are no specific imaging findings of primary yolk sac tumour, presence of cystic or necrotic areas and a tendency for tumour rupture are suggestive features.

Background: Melanotic neuroectodermal tumour of infancy (MNTI) is a rare neoplasm of neural crest origin., Objective: To describe three further cases of MNTI, with emphasis on CT and MRI findings., Materials and Methods: Data for children with histologically confirmed MNTI following biopsy or surgery were retrieved. Three children with available imaging at the time of diagnosis were included in the study., Results: All three children had primary tumour in the head and neck region: one in the maxilla, one in the occipital bone (extra-axial but with intracranial extension) and one with an unusual tumour growing exophytically from the subcutaneous tissues adjacent to the occipital bone. All tumours were iso/hypointense both on T1- and T2-weighted MRI, and showed marked contrast enhancement in their non-ossified components. CT allowed identification of bone destruction and remodelling., Conclusion: Our findings are consistent with previously reported cases of MNTI regarding age at presentation and location in the head and neck region. Our MR findings did not demonstrate the typical pattern of T1-shortening expected from melanin deposition.

Background: Multifocal lymphangiomatosis is a rare systemic disorder affecting children. Due to its rarity and wide spectrum of clinical, histological and imaging features, establishing the diagnosis of multifocal lymphangiomatosis can be challenging., Objectives: The purpose of this study was to describe a new imaging sign in this disorder: paraspinal soft tissue and signal abnormality at MRI., Materials and Methods: We retrospectively reviewed the imaging, clinical and histopathological findings in a cohort of eight children with thoracic involvement from this condition., Results: Evidence of paraspinal chest disease was identified at MRI and CT in all eight of these children. The changes comprise heterogeneous intermediate-to-high signal parallel to the thoracic vertebrae on T2-weighted sequences at MRI, with abnormal paraspinal soft tissue at CT and plain radiography., Conclusion: Multifocal lymphangiomatosis is a rare disorder with a broad range of clinicopathological and imaging features. MRI allows complete evaluation of disease extent without the use of ionising radiation and has allowed us to describe a previously unreported imaging sign in this disorder, namely, heterogeneous hyperintense signal in abnormal paraspinal tissue on T2-weighted images.

Background: Although CT can be greatly beneficial, its relatively high radiation doses have caused public health concerns., Objective: To assess patterns in CT usage among patients aged less than 22 years in Northern England during the period 1993-2002., Materials and Methods: Electronic data were obtained from radiology information systems of all nine National Health Service trusts in the region., Results: A total of 38,681 scans had been performed in 20,483 patients aged less than 22 years. The number of CT examinations rose, with the steepest increase between 1997 and 2000. The number of patients scanned per year increased less dramatically, with 2.24/1,000 population aged less than 22 years having one scan or more in 1993 compared to 3.54/1,000 in 2002. This reflects an increase in the median number of scans per patient, which rose from 1 in 1993 to 2 by 1999. More than 70% of CT examinations were of the head, with the number of head examinations varying with time and patient age., Conclusion: The frequency of CT scans in this population more than doubled during the study period. This is partly, but not wholly, explained by an increase in the number of scans per patient.

Paediatric radiology requires dedicated equipment, specific precautions related to ionising radiation, and specialist knowledge. Developing countries face difficulties in providing adequate imaging services for children. In many African countries, children represent an increasing proportion of the population, and additional challenges follow from extreme living conditions, poverty, lack of parental care, and exposure to tuberculosis, HIV, pneumonia, diarrhoea and violent trauma. Imaging plays a critical role in the treatment of these children, but is expensive and difficult to provide. The World Health Organisation initiatives, of which the World Health Imaging System for Radiography (WHIS-RAD) unit is one result, needs to expand into other areas such as the provision of maintenance servicing. New initiatives by groups such as Rotary and the World Health Imaging Alliance to install WHIS-RAD units in developing countries and provide digital solutions, need support. Paediatric radiologists are needed to offer their services for reporting, consultation and quality assurance for free by way of teleradiology. Societies for paediatric radiology are needed to focus on providing a volunteer teleradiology reporting group, information on child safety for basic imaging, guidelines for investigations specific to the disease spectrum, and solutions for optimising imaging in children.

We report a 35-week preterm neonate with bladder agenesis and bilateral dysplastic kidneys. A suprapubic catheter was inadvertently inserted into one of the larger inferior cysts of the left dysplastic kidney. A postmortem MRI scan was performed with the findings being confirmed on autopsy. We are unaware of another postmortem MRI study demonstrating bladder agenesis.

Background: Fetal endoscopic tracheal occlusion (FETO) is a promising treatment for severe congenital diaphragmatic hernia, a condition that carries significant morbidity and mortality. It is hypothesised that balloon occlusion of the fetal trachea leads to an improvement in lung growth and development. The major documented complications of FETO to date are related to preterm delivery., Objective: To report a series of five infants who developed tracheomegaly following FETO., Materials and Methods: Review of all children referred with tracheomegaly to the paediatric intensive care and tracheal service at two referral centres., Results: Five neonates presented with features of respiratory distress shortly after birth and were subsequently found to have marked tracheomegaly. Two neonates had tracheomalacia in addition., Conclusion: There are no previous reports in the literature describing tracheomalacia, or more specifically, tracheomegaly, as a consequence of FETO. We propose that the particularly compliant fetal airway is at risk of mechanical damage from in utero balloon occlusion. This observation of a new problem in this cohort suggests a thorough evaluation of the trachea should be performed in children who have had FETO in utero. It may be that balloon occlusion of the trachea earlier in utero (before 26 weeks' gestation) predisposes to this condition.

Background: Malignant rhabdoid tumours (RT) are increasingly recognized in young children, probably as a consequence of advances in accurate histological diagnosis rather than a true increase in frequency. Although typically presenting as renal tumours in infancy, extrarenal tumours outside the central nervous system (CNS) in children less than 12 months of age are now well recognized, but previous literature on their imaging features is very limited., Objective: To demonstrate the imaging features of extrarenal RTs outside the CNS., Materials and Methods: A retrospective database review was made from 1989 to 2007 of patients diagnosed with extrarenal RT in infancy, i.e. below 12 months of age., Results: There were nine patients (six boys and three girls). The age at presentation varied from 1 to 11 months (average 6 months). Tumours were located in the thorax/mediastinum (n=3), liver (n=3), neck (n=1), shoulder (n=1) and axilla (n=1). The imaging modalities used included US (n=8), CT (n=7) and MRI (n=6). Bone scan was positive in one patient, while metastases at the time of diagnosis occurred in four patients. On MRI the tumours tended to show nonspecific hypointensity on T1-W images and heterogeneous hyperintensity on T2-W images, with heterogeneous enhancement., Conclusion: This is the largest radiological series of extrarenal RTs outside the CNS in infancy. In our series no imaging features were found specific to the diagnosis. A tendency towards large size and mediastinal/paravertebral location were noted. A hypodense solid component on CT and a heterogeneous hyperintensity on T2-W MR images suggest that this tumour should be considered in the routine differential diagnosis of soft-tissue tumours in infancy, in addition to rhabdomyosarcoma.

Background: Primary upper abdominal teratomas are extremely rare tumours, most commonly arising in infants. The radiological literature relating to them is sparse. Surgical resection is difficult due to distortion of the vascular anatomy., Objective: To reassess the value of preoperative imaging with specific reference to the presence/absence of typical features of teratoma, anatomical location and adjacent vascular anatomy., Materials and Methods: The histopathology database was used to identify infants with upper abdominal teratoma. Pathological, surgical and radiological data were reviewed., Results: The search of the database identified 12 infants (10 girls, 2 boys) with an abdominal/retroperitoneal teratoma during the period 1993 to 2006. All teratomas were benign. In the majority of infants, typical radiological features of teratoma were demonstrated (fat, calcium). Identification of the major abdominal vessels on CT scan (most commonly the inferior vena cava) was not possible in all infants. Distortion (and commonly encasement) of the adjacent major abdominal vessels was usually evident., Conclusion: Upper abdominal teratomas in infants have typical radiological features. Preoperative delineation of the major vascular anatomy is often imprecise. Significant distortion of vascular anatomy was present in all infants and awareness of this feature impacts on surgical planning.

Background: The radiological findings after laparoscopic heminephrectomy in children are not widely reported., Objective: To document the range of imaging appearances following laparoscopic heminephrectomy in children., Materials and Methods: We reviewed all imaging and all radiology reports performed on children in our institution who had undergone laparoscopic heminephrectomy over a 6-year period., Results: Between 2001 and 2007, 35 patients (24 girls, 11 boys) had laparoscopic heminephrectomy. The mean age at surgery was 3.5 years. The sites of surgery comprised 12 right upper, 5 right lower, 18 left upper and 3 left lower heminephrectomies. These numbers included three patients who had bilateral procedures. The most consistent postoperative finding was an avascular cyst related to the site of surgery, seen after 23 of 38 procedures. Of these 23 cysts, 13 were simple and 10 were septated and/or contained echogenic debris., Conclusion: A cyst related to the site of laparoscopic heminephrectomy is a frequent postoperative occurrence but is not widely recognized for being simply this. Simple, anechoic, and septated appearances are possible. This cyst may be confusing to the radiologist and the clinician if they are not aware of the history of laparoscopic heminephrectomy and the technique that has been used. Whilst the exact aetiology of this cystic lesion is uncertain, all the patients in our series had a heminephrectomy performed with the use of an Endoloop (Ethicon, Somerville, NJ). No patients have required intervention to date.

Background: The natural history of focal splenic lesions in paediatric Gaucher disease (GD) is unknown and these lesions are thought to persist despite enzyme replacement therapy (ERT)., Objective: To assess the prevalence, evolution and resolution of splenic nodules in a cohort of paediatric Gaucher patients treated with ERT., Materials and Methods: The US findings in 37 children with GD were retrospectively reviewed. A total of 28 children underwent serial abdominal US examinations as part of their initial assessment and during routine follow-up. All patients received ERT., Results: Six children (21%) had splenic nodules on US examination, either at presentation or on follow-up examination. In all six patients, the nodules had resolved on follow-up imaging, with resolution taking 17 months to 4 years 8 months. Two sets of siblings developed nodules that resolved over a similar time period., Conclusion: Disappearance of focal splenic lesions in children with GD during follow-up has not been previously reported. The development of new focal splenic lesions in children with GD whilst on ERT has not been previously documented.

We report an infant with known unilateral multicystic dysplastic kidney (MCDK) who underwent renal ultrasonography and Doppler spectral waveform analysis for investigation of hypertension. A tardus-parvus waveform was demonstrated in the renal artery on the normal side suggesting either renal artery or more proximal stenosis. Coarctation of the aorta was subsequently demonstrated.

Over the last 15 years, various oncology groups throughout the world have used the PRETEXT system for staging malignant primary liver tumours of childhood. This paper, written by members of the radiology and surgery committees of the International Childhood Liver Tumor Strategy Group (SIOPEL), presents various clarifications and revisions to the original PRETEXT system.

We describe the CT findings in an initially asymptomatic boy aged 2 years 9 months with a gastrocolic fistula following percutaneous endoscopic gastrostomy (PEG) placement. The findings consisted of an unusual configuration of the gastrostomy tubing on an abdominal radiograph and upper gastrointestinal study indicating the possibility of transcolic PEG placement, which was confirmed with limited section CT. This well-known and major complication following a common procedure may be recognized on plain abdominal radiography, but it has not to our knowledge been documented previously on CT in a child.

The incidence of conjoined twins is estimated to be around 1 in 250,000 live births. There is a distinct female predominance. In this paper the imaging of conjoined twins both antenatally and postnatally is reviewed, in particular taking into consideration recent advances with multidetector CT. Accurate counselling of parents regarding the likely outcome of the pregnancy and the likelihood of successful separation is dependent on good prenatal imaging with ultrasound and MRI. Planning of postnatal surgical separation is aided by accurate preoperative imaging which, depending on the conjoined area, will encompass many imaging modalities, but often relies heavily on CT scanning.

A variety of portal vein anomalies that occur in children can be identified by US, CT, MRI and portal venography. Although these abnormalities can also occur in adults, there are certain pathological processes and aberrations within the portal system that are specific to children. Knowledge of the embryology and anatomy of the portal vein is of benefit in the understanding of these anomalies. Identifying deviations from normal portal architecture is important in the work-up for surgery such as liver transplantation, and prior to interventional procedures such as stent placement or embolization. The aim of this paper is to summarize the various types of congenital and acquired portal vein abnormalities that occur in children, describe their radiological features and provide images to demonstrate the differences from normal portal venous anatomy.

Non-Hodgkin's lymphoma (NHL) is the fourth most common childhood malignancy. Uterine involvement with NHL is well described in adults, rare in children and has not been described in the first 2 years of life. While renal involvement in NHL is well recognised, diffuse renal enlargement is an uncommon finding. We report a unique case of B-cell lymphoma of primitive phenotype in a 15-month-old girl with uterine and renal involvement at presentation. We describe the US and MRI features at presentation that helped in the prospective diagnosis of this condition.

Background: Routine magnetic resonance imaging (MRI) surveillance of bone marrow change in patients with Gaucher disease (GD) is recommended, but interpretation of imaging findings in the developing skeleton may be difficult., Objectives: To assess the agreement between routine spinal MRI findings and clinical course in paediatric GD patients receiving enzyme replacement therapy (ERT)., Materials and Methods: A retrospective institutional review of all GD patients on ERT who had had repeated routine spinal MRI in accordance with national recommendations ( n=14) was carried out. Vertebral body bone marrow MRI T1 signal was assessed relative to intervertebral discs. MRI findings were then compared with recorded date(s) of ERT initiation and possible therapy changes., Results: Nine patients had spinal MRI both before and after the start of ERT. MRI T1 marrow signal was normal in two and abnormal in three of the nine patients both before and after the start of ERT. Two of the nine patients had normalization and one had conversion from normal to abnormal T1 signal. Interpretation was uncertain in one. Seven episodes of treatment intensification occurred. MRI T1 marrow signal was normal before five and uncertain before two of the seven episodes., Conclusion: Routine spinal MRI had low accuracy for predicting clinically indicated therapy intensification.

We report the first case of a child with Gaucher's disease and a large mesenteric mass, confirmed histologically to be Gaucher's cell infiltrates. We describe the radiological findings and discuss further management. The advent of enzyme replacement therapy has prolonged survival and the emergence of previously undocumented manifestations of the disease is being observed. The radiologist and clinician should be alert to the possible development of these new problems and the fact that in Gaucher's disease a palpable right upper-quadrant mass need not necessarily represent hepatomegaly.

Background: Childhood constipation is common and assessment is often difficult. Plain abdominal radiography is simple and commonly used to assess constipation. The role of radiography with the use of a simple scoring system has not been fully evaluated., Objective: To assess the reliability of scoring faecal loading on plain abdominal radiographs in children with intractable constipation., Materials and Methods: Plain abdominal radiographs from 33 constipated and 67 control children were independently assessed by three observers on two separate occasions. A scoring system was devised with scores from 0 (no stool) to 5 (gross faecal loading with bowel dilatation) in three areas of the colon, giving a total score of 0-15., Results: There were significant differences between the scores of the constipated and control radiographs for each observer (P = 0.05). There was no intra-observer variation (P = 0.12-0.69), but significant inter-observer variation was demonstrated (P = 0.00)., Conclusions: We have found this scoring system to be a clinically useful and a reproducible tool in assessing childhood constipation. Assessment of faecal loading is subjective and varies between observers, although one observer will consistently score faecal loading on the same radiograph on successive occasions. To limit exposure to ionising radiation, we recommend that radiography be reserved for the investigation of intractable constipation, and its accuracy is improved if all radiographs are scored by the same observer.

Background: The postnatal imaging of infants with antenatally detected mild hydronephrosis remains controversial., Objective: Our aim was to establish the role and timing of micturating cystourethrography (MCUG) in mild hydronephrosis., Materials and Methods: We performed a retrospective study of 61 infants (122 kidneys) referred with an antenatal diagnosis of hydronephrosis who showed persistent postnatal dilatation. All had follow-up postnatal ultrasound (US) and MCUG performed. The degree of dilatation at each follow-up scan was recorded., Results: Of the 122 kidneys, 65 showed mild hydronephrosis. A substantial proportion of these (21.5%) demonstrated reflux. Serial US of these infants showed that the 6-week scan was the most informative and that any changes that warranted further investigation had occurred by this time., Conclusion: We recommend that all infants with mild hydronephrosis should undergo MCUG. MCUG need not be delayed until 3 months but could be performed following a 6-week US scan.