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Background: Interventional radiology (IR) is becoming more relevant in patient care and is associated with increased patient radiation exposure and radiation-induced adverse effects. Diagnostic reference levels (DRLs) are crucial for radiation control. There is a paucity of published DRLs for IR in South Africa and sub-Saharan Africa. Objectives: This study aimed to determine local DRLs for fluoroscopically-guided IR procedures and compare the achieved DRLs with published local and international DRLs. Method: Retrospective, descriptive, single-centre study. Kerma air product (KAP), reference point air kerma (Ka,r) and fluoroscopy time (FT) were collected for patients (12 years and older) who underwent IR procedures at a university hospital from 01 January 2019 to 31 December 2019. The 75th percentile of the distribution of each dose parameter (KAP, Ka,r and FT) per procedure was calculated and taken as the local diagnostic reference levels (LDRL). The established LDRLs were compared to published DRLs. Results: A total of 564 cases were evaluated. The 13 most frequent procedures (with 15 or more cases) represented 86.1% (487/564). Percutaneous transhepatic biliary drainage was the most common procedure (n = 146, 25.9%). Diagnostic cerebral angiogram DRLs exceeded the published DRL data ranges for all parameters (DRL 209.3), and interventional cerebral angiogram exceeded published ranges (DRL 275). Uterine artery embolisation (UAE) exceeded these ranges for KAP and Ka,r. (KAP-954.9 Gy/cm2, Ka,r-2640.8 mGy). Conclusion: The LDRLs for diagnostic cerebral angiogram, interventional cerebral angiogram and UAE exceeded published international DRL ranges. These procedures require radiation optimisation as recommended by the International Commission on Radiological Protection (ICRP). Contribution: In addition to informing radiation protection practices at the level of the institution, the established LDRLs contribute towards Regional and National DRLs.

Background: Diagnostic reference levels (DRLs) are a crucial element of auditing radiation doses in paediatric computed tomography (CT). Currently, there are no national paediatric CT DRLs in South Africa. Objectives: The aim of this article was to establish local paediatric DRLs for CT examinations at two academic hospitals and to compare paediatric CT radiation output levels with established DRLs in the developed and developing world. Method: Computed Tomography Dose Indexvolume (CTDIvol) and dose length product (DLP) values were collected from CT examinations performed at two university hospitals for patients aged 0–15 years, during 01 November 2016–30 April 2017. The 75th percentile of the data distribution was calculated for each CT examination type and age group, further categorised into routine working hours and after-hours for both hospitals and statistically compared. Results: Of the 1031 CT examinations performed, CT brain examination was the most common (755/1031; 72.23%). DLP values were increased in the after-hours categories compared to regular working hours at both hospitals. The largest increase was in the 0–1 year age group (150.56%). With the exception of CT Chest and CT abdomen in the 0–1 year age group, the CTDIvol and DLP values compared favourably to international standards. Conclusion: Most of the calculated DRLs are acceptable and internationally comparable. This likely indicates effective reduction techniques and protocols. Computed tomography body examination protocols for 0–1 year patients should be reviewed. Strategies should be implemented to limit higher doses in after-hours examinations.

Background: The role of the voiding cystourethrogram (VCUG) in the follow-up of children with posterior urethral valves (PUVs) post-ablation has been considered a standard practice. The urethral ratio and gradient of change have proven to be useful. Objectives: We aimed to review the role of the ‘ideal’ ratio on predicting residual PUV post-ablation. Methods: A systematic review of the PubMed, SCOPUS and Web of Science databases was performed (April 2019). The search terms included ‘Urethral Ratio and Posterior urethral valve ablation’. All cited reference lists were further evaluated for additional inclusive studies. Results: Eleven studies were identified, of which nine were relevant to the topic. Case reports, comments and adult and animal studies were excluded, leaving four studies for critical review. In total, 338 patients were assessed. The control group consisted of 167 age-matched, male children. Study regions included India and Australia. The ages ranged from 15 days to 3.4 years. Ablation methods included the use of a resectoscope with cutting diathermy, cold knife or Bugbee electrode. The mean urethral ratios in the control group ranged from 1.04 to 1.73. The suggested predictive urethral cut-off ratios recommended include 2.2 (p = 0.001), 2.5–3 and 3.5. Conclusion: Although the precise cut-off ratio could not be clearly defined in this review, a urethral ratio less than a range of 2.2–3.5 has proven to be a beneficial predictor of ablation success and should thus be incorporated into standard VCUG reporting templates in the follow-up of PUVs in male children in resource-limited settings.

Primary pancreatic tumours are a rare and unusual entity in children. In this article, we present the case of an 8-month-old girl who presented with obstructive jaundice. The differential diagnosis based on imaging studies was that of a pancreatic vascular neoplasm; however, with the laboratory evidence of Kasabach–Merritt phenomenon (KMP), this prompted the diagnosis of pancreatic kaposiform hemangioendothelioma. A core biopsy of the pancreatic mass was taken at laparotomy and confirmed this diagnosis. The pancreas is an exceedingly rare site of occurrence for this tumour, with only nine cases being published to date. The clinical, biochemical, imaging and pathological findings are discussed to highlight a rare and potentially life-threatening vascular tumour.

Chest wall or pleural-based tumours represent a heterogeneous group of lesions that are infrequent in children and infants; however, a large proportion of these lesions are malignant in nature. Categorising them on the basis of primary versus secondary, site of origin (osseous and cartilage, or soft tissue) and tissue composition may assist in narrowing the differential diagnosis. We present a case of a 7-year-old boy with a progressive history of dyspnoea. The initial chest radiograph (CXR) demonstrated complete opacification of the left hemithorax with no air bronchograms. This was associated with the cut-off of the left main bronchus and mediastinal shift to the right. The post-contrast computed tomography (CT) of the chest showed multiple left-sided enhancing pleural-based masses with collapse of the left lung. These lesions were locally invasive as demonstrated by the intra and extra-thoracic extension. There were no associated erosions of the adjacent ribs or intra-tumoural calcifications. Based on the imaging findings, the diagnosis of extra-skeletal Ewing sarcoma (ES-EWS) of the chest wall was made with a differential diagnosis of rhabdomyosarcoma. A core biopsy was performed of the pleural-based mass, and histology with immunohistochemistry confirmed the diagnosis of a malignant small round blue cell tumour; subtype Ewing sarcoma family tumour (ESFT). The child was subsequently commenced on chemotherapy. The diagnosis of ES-EWS should be considered when a child or adolescent presents with an ill-defined, eccentric, chest wall mass in the absence of a lesion with a primary osseous origin. Imaging plays a key role in tumour staging, therapeutic planning and follow-up of patients.

Childhood ataxia and central nervous system hypomyelination (CACH), also known as ‘vanishing white matter disease’ (VWM), is a leukoencephalopathy with autosomal recessive inheritance. It is characterised by normal psychomotor development initially, with an onset of neurological deterioration that follows a chronic and progressive course. Stress conditions such as febrile infections, minor head trauma or even acute fright provoke major episodes of neurological deterioration. We present a case of a 2-year-old child who presented with spasticity and cerebellar ataxia. After magnetic resonance imaging (MRI) of the brain, CACH/VWM was diagnosed on the basis of the typical clinical and MRI findings. As there is no known cure for CACH/VWM, our patient was followed up over 3 years with MRIs of the brain to assess the progressive involvement of the cerebral white matter. In those patients with suggestive or inconclusive MRI findings for CACH/VWM, particularly in the presymptomatic stage and adult onset variants, involvement of the inner rim of the corpus callosum should prompt the inclusion of CACH/VWM in the differential diagnosis. Biochemical markers such as the asialotransferrin:transferrin ratio in the cerebrospinal fluid can also potentially be used as a screening tool in this subset of patients prior to gene mutation analysis.

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in the paediatric age group, ranking fourth in frequency after central nervous system tumours, neuroblastomas and nephroblastomas. Embryonal RMS of the biliary tree is considered a rare entity, with the most common clinical presentation being that of obstructive jaundice. We present the case of a 4-year-old boy who presented with hepatomegaly and obstructive jaundice. Biochemically, there was evidence of elevated ductal enzymes with conjugated hyperbilirubinaemia. The magnetic resonance imaging (MRI) features were consistent with a biliary RMS with the differential diagnosis of a choledochal cyst initially included based on the computed tomography images. The diagnosis of embryonal biliary RMS was later confirmed on histology. This case illustrates the importance of considering malignant aetiologies in paediatric cases of obstructive jaundice, as this entity is infrequently described in the literature and may mimic the appearance of a choledochal cyst. The demonstration of enhancement of intraductal material within the biliary tree on MRI and the presence of arterial waveforms within the intraductal mass on ultrasound assists in the differentiation between biliary RMS and a choledochal cyst.

Immune Reconstitution Inflammatory Syndrome (IRIS) refers to a collection of inflammatorydisorders, predominantly related to infectious processes that manifest after the initiation ofantiretroviral therapy (ART) and can be classified as unmasking or paradoxical. The prevalenceof IRIS in children in sub-Saharan Africa is low. Approximately half of all cases are associatedwith Mycobacterium tuberculosis. It may be difficult to distinguish IRIS from tuberculosis andother opportunistic infections radiologically; therefore, radiological findings must be interpretedwith clinical and laboratory findings. In this review article, we describe the clinical andradiological manifestations of IRIS in children and provide illustrative radiological examples.

Approximately 20% of paediatric patients with epilepsy are refractory to medical therapies. In this subgroup of patients, neuroimaging plays an important role in identifying an epileptogenic focus. Successful identification of a structural lesion results in a better outcome following epilepsy surgery. Advances in imaging technologies, methods of epileptogenic region localisation and refinement of clinical evaluation of this group of patients in epilepsy centres have helped to widen the spectrum of children who could potentially benefit from surgical treatment. In this review, we discuss ways to optimise imaging techniques, list typical imaging features of common pathologies that can cause epilepsy, and potential pitfalls to be aware of whilst reviewing imaging studies in this challenging group of patients. The importance of multidisciplinary meetings to analyse and synthesise all the non-invasive data is emphasised. Our objectives are: to describe the four phases of evaluation of children with drug-resistant localisation-related epilepsy; to describe optimal imaging techniques that can help maximise detection of epileptogenic foci; to describe a systematic approach to reviewing magnetic resonance imaging of children with intractable epilepsy; to describe the features of common epileptogenic substrates; to list potential pitfalls whilst reviewing imaging studies in these patients; and to highlight the value of multimodality and interdisciplinary approaches to the management of this group of children.

Vascular abnormalities associated with neurofibromatosis type 1 (NF1) are well described. Spinal arterio-venous fistula (AVF), however, is a rare finding in NF1 and may present with neurological symptoms that require treatment. Management of spinal AVFs can be endovascular or surgical. We present a patient with known NF1 and neurological symptoms owing to a spinal AVF demonstrated by imaging, who required a combination of endovascular and surgical management.

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The ivy sign refers to diffuse bilateral leptomeningeal enhancement on post- contrastT1-weighted magnetic resonance imaging (MRI) and increased signal intensity in bilateralsubarachnoid spaces and perivascular spaces on T2-weighted fluid attenuation inversionrecovery (FLAIR) MRI sequences in patients with moyamoya disease.

The dural tail sign was first described by Wilms in 1989, as a post-contrast linear thickening of the meninges that resembles a tail extending from a mass on T1-weighted MR imaging. The sign consists of a tapering rim ranging from 0.5 to 3.0 cm in length with an inner surface that is either smooth or slightly nodular.

The molar tooth sign was initially identified in Joubert syndrome, named after Marie Joubert who first described it in 1968 as a rare autosomal recessive disorder characterised by the neuroradiological hallmark of the molar tooth sign caused by cerebellar vermian hypoplasia. Subsequently, it emerged that the molar tooth sign encompasses many syndromes that are now grouped together and termed Joubert syndrome and related disorders (JSRDs). Knowledge of the newer classification system and the subtypes is important and helps to direct and interpret imaging studies based on clinical signs so as to avoid delay in diagnosis of the hepatic oculorenal and renal subtypes of JSRDs in patients in whom the molar tooth sign is identified on brain MRI.

Rigler’s sign was first described in 1941 by L G Rigler as a new radiological sign for recognising free air in the peritoneal cavity on supine radiograph. The presence of pneumoperitoneum allows free intraperitoneal air to be contrasted with intraluminal gas, accentuating the wall of gas-containing viscera. It is observed in infants and very ill patients where only limited radiographs of the abdomen are possible. The football sign was first described by R E Miller in the 1960s. Seen on supine abdominal radiographs, this describes an oval radiolucency resembling an American football. It is important for the radiologist to recognise the supporting signs of pneumoperitoneum, such as Rigler’s sign and the football sign, on supine abdominal radiographs, especially in neonates and infants, where erect chest/abdominal radiographs are not always possible.

The sandwich sign refers to the sandwiching of mesenteric vessels and fat by enlarged mesenteric nodes on cross-sectional imaging, commonly occurring in lymphoma, but not specific to lymphoma. The sign is radiologically indistinguishable from post-transplant lymphoproliferative disorders. The radiological significance of the sandwich sign is in suggesting the diagnosis of lymphoma so that appropriate treatment may be initiated early as the tumour has a rapid growth pattern.

Variations and abnormalities of skull appearance and shape are generally related to a primary maldevelopment of the brain. The copper-beaten skull appearance is typically associated with craniosynostosis, where premature fusion of the cranial bone sutures results in the growing brain exerting pressure on the malleable cranium, producing a pattern known as the copper-beaten skull appearance.

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