Nonspecific Gastrointestinal Symptoms as the First Sign of Ganglioneuroblastoma Intermixed-Case Report and Literature Review.

Nonspecific gastrointestinal symptoms remain a problem for pediatricians because, out of a thousand trivial cases, there are rare diseases that require in-depth diagnostics and extensive knowledge to identify them. These complaints may be caused by a neoplastic process. We present the case of a 5-year-old boy whose diagnostic pathway lasted about 3 months. He was admitted to hospital due to severe abdominal pain. Physical examination revealed a bloated, hard, and painful abdomen. In the standing X-ray, the features of intestinal obstruction were visualized. An ultrasound examination showed a possible malignant lesion in the location of the left adrenal gland. After the surgical removal of the pathological mass and histopathological examination, the diagnosis of ganglioneuroblastoma intermixed was made. This tumor, along with neuroblastoma, ganglioneuroma, and ganglioneuroblastoma nodular, belongs to neuroblastic tumors (NTs), which originate from primitive cells of the sympathetic nervous system. NTs are quite rare, but they are still the majority of extracranial solid tumors in children, and their symptoms often appear relatively late when the neoplastic process is already advanced. The purpose of this review is to present current information about ganglioneuroblastoma, with a special emphasis on nonspecific gastrointestinal symptoms as first sign of this tumor and its diagnostics.

Surveillance of Fontan-associated liver disease: current standards and a proposal from the European Society of Paediatric Radiology Abdominal Task Force.

Since Francis Fontan first introduced the eponymous technique, the Fontan procedure, this type of surgical palliation has allowed thousands of children affected by specific heart malformations to reach adulthood. Nevertheless, abdominal, thoracic, lymphatic and neurologic complications are the price that is paid by these patients. Our review focuses on Fontan-associated liver disease; the purpose is to summarize the current understanding of its physiopathology, the aim of follow-up and the specific radiologic follow-up performed in Europe. Finally, we as members of the Abdominal Task Force of the European Society of Paediatric Radiology propose a consensus-based imaging follow-up algorithm.

Radiologic follow-up in Fontan-associated liver disease in Europe: European Society of Paediatric Radiology survey demonstrates the need for a consensus protocol.

Fontan surgery is a life-saving procedure for newborns with complex cardiac malformations, but it originates complications in different organs. The liver is also affected, with development of fibrosis and sometimes cirrhosis and hepatocellular carcinoma. There is no general agreement on how to follow-up these children for the development of liver disease. To understand the current practice on liver follow-up, we invited members of the European Society of Paediatric Radiology (ESPR) to fill out an online questionnaire. The survey comprised seven questions about when and how liver follow-up is performed on Fontan patients. While we found some agreement on the use of US as screening tool, and of MRI for nodule characterization, the discrepancies on timing and the lack of a shared protocol make it currently impossible to compare data among centers.

Practical approach for the diagnosis of biliary atresia on imaging, part 2: magnetic resonance cholecystopancreatography, hepatobiliary scintigraphy, percutaneous cholecysto-cholangiography, endoscopic retrograde cholangiopancreatography, percutaneous liver biopsy, risk scores and decisional flowchart.

We aim to present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses to biliary cirrhosis, end-stage liver failure and death within the first years of life. Differentiating biliary atresia from other nonsurgical causes of neonatal cholestasis is difficult as there is no single method for diagnosing biliary atresia and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. In this second part, we discuss the roles of magnetic resonance (MR) cholecystopancreatography, hepatobiliary scintigraphy, percutaneous biopsy and percutaneous cholecysto-cholangiography. Among imaging techniques, ultrasound (US) signs have a high specificity, although a normal US examination does not rule out biliary atresia. Other imaging techniques with direct opacification of the biliary tree combined with percutaneous liver biopsy have roles in equivocal cases. MR cholecystopancreatography and hepatobiliary scintigraphy are not useful for the diagnosis of biliary atresia. We propose a decisional flowchart for biliary atresia diagnosis based on US signs, including elastography, percutaneous cholecysto-cholangiography or endoscopic retrograde cholangiopancreatography and liver biopsy.

Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound.

We present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses towards biliary cirrhosis, end-stage liver failure and death by age 3. Differentiation of biliary atresia from other nonsurgical causes of neonatal cholestasis is challenging because there is no single method for diagnosing biliary atresia, and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. Concerning imaging, our systematic literature review shows that ultrasonography is the main tool for pre- and neonatal diagnosis. Key prenatal features, when present, are non-visualisation of the gallbladder, cyst in the liver hilum, heterotaxy syndrome and irregular gallbladder walls. Postnatal imaging features have a very high specificity when present, but a variable sensitivity. Triangular cord sign and abnormal gallbladder have the highest sensitivity and specificity. The presence of macro- or microcyst or polysplenia syndrome is highly specific but less sensitive. The diameter of the hepatic artery and hepatic subcapsular flow are less reliable. When present in the context of acholic stools, dilated intrahepatic bile ducts rule out biliary atresia. Importantly, a normal US exam does not rule out biliary atresia. Signs of chronic hepatopathy and portal hypertension (portosystemic derivations such as patent ductus venosus, recanalised umbilical vein, splenomegaly and ascites) should be actively identified for - but are not specific for - biliary atresia.

Two-dimensional (2D), three-dimensional static (3D) and real-time (4D) contrast enhanced voiding urosonography (ceVUS) versus voiding cystourethrography (VCUG) in children with vesicoureteral reflux.

BACKGROUND: Two-dimensional (2DUS) contrast enhanced voiding urosonography has been used in the diagnosis and treatment monitoring of the vesicoureteral reflux in children for over 15 years. The opportunity of performing this examination with the use of three-dimensional static (3DUS) and real-time (4DUS) techniques opens up new diagnostic horizons. OBJECTIVE: To analyze if 3DUS/4DUS bring additional information leading to an increased detection rate or change in the grading of reflux compared to 2DUS and voiding cystouretrography. MATERIAL AND METHODS: We evaluated 69 patients (mean 4.1 years) who underwent 2DUS/3DUS/4DUS contrast enhanced voiding urosonography (ceVUS) and voiding cystourethrography (VCUG) for the diagnosis and grading of vesicoureteral reflux. RESULTS: 2DUS and 3DUS/4DUS urosonography diagnosed 10 more refluxes (7.25%) than cystourethrography and in 3 refluxes (2.17%) detected a higher grade. In 9 refluxes (6.52%) 3DUS/4DUS urosonography and cystourethrography diagnosed a higher grade than 2DUS. There was a statistically significant difference between cystourethrography and 3DUS/4DUS urosonography when the number of detected refluxes and differences in grading were compared. 4DUS enabled a better visualization of reflux than 3DUS. CONCLUSIONS: 3DUS/4DUS techniques bring additional information leading to a change in reflux grading compared to 2DUS and a detect higher number of refluxes compared to cystourethrography.

Testicular adrenal rest tumors in boys with congenital adrenal hyperplasia: 3D US and elastography--do we get more information for diagnosis and monitoring?

BACKGROUND: Testicular adrenal rest tumors (TART) are the nodular testicular lesions deriving from the adrenal remnant tissue reported in boys and men with congenital adrenal hyperplasia. Until now, the diagnostics of TART have been based on a combination of clinical features, imaging methods (primarily two dimensional ultrasound--2D US), response of the foci to glycocorticosteroid (GCS) therapy and exclusion of the neoplastic process. Application of 2D US supplies however a limited range of information about the volume, demarcation, structure and vascularization of the lesions. OBJECTIVE: To define whether the use of 3D US, power Doppler and elastography changes the algorithm of the diagnostics and monitoring or treatment of TART. MATERIAL AND METHODS: In this study, modern ultrasound techniques such as 3D US and elastography were introduced in two boys with TART. RESULTS: The 3D power Doppler option gives the opportunity for accurate assessment of the volume of testes and adrenal tissue foci and their vascularization. Sonographic elastography allows the assessment of stiffness of adrenal tissue areas compared to normal testis parenchyma. CONCLUSION: The use of these modern techniques enables more adequate and advanced diagnostics, and more precise monitoring of the effects of treatment in patients with TART.

Ultrasound presentation of abdominal non-Hodgkin lymphomas in pediatric patients.

INTRODUCTION: Burkitt's lymphoma accounts for approximately 25% of lymphomas diagnosed in children of developmental age. The tumor is localized mainly in the intestine (usually in the ileocecal region), mesenteric lymph nodes and extraperitoneal space. The clinical symptoms are non-specific and include: abdominal pain, vomiting, gastrointestinal bleeding, and acute abdomen suggesting appendicitis or intestinal intussusception. On ultrasound examination, Burkitt's lymphoma may manifest itself in various ways, depending on the origin of the lesion. AIM: The aim of this paper was to review the ultrasound manifestation of abdominal Burkitt's lymphoma in children. MATERIAL AND METHODS: The analysis included 15 pediatric patients with Burkitt's non-Hodgkin lymphoma in the abdominal cavity. The mean age of the patients was 9.5. Abdominal and gastrointestinal ultrasound examinations were conducted using a Siemens scanner with a convex transducer of 3.5-5 MHz and linear array transducer of L4 - 7.5 MHz. RESULTS: Ultrasound examinations conducted in the group of 15 patients revealed pathological masses localized in the gastric wall in 3 patients (20%), in the ileocecal region in 10 patients (67%) and a disseminated process in 2 patients (13%). In 12 patients with a diagnosed Burkitt's non-Hodgkin lymphoma in an extragastric localization, differences in the morphology of the lesions were observed. CONCLUSIONS: The clinical and ultrasound picture of abdominal Burkitt's lymphoma in children is variable. A careful ultrasound assessment of all abdominal organs conducted with the use of convex and linear probes increases the chances of establishing an adequate diagnosis.

Errors in the ultrasound diagnosis of the kidneys, ureters and urinary bladder.

The article presents the most frequent errors made in the ultrasound diagnosis of the urinary system. They usually result from improper technique of ultrasound examination or its erroneous interpretation. Such errors are frequent effects of insufficient experience of the ultrasonographer, inadequate class of the scanner, insufficient knowledge of its operation as well as of wrong preparation of patients, their constitution, severe condition and the lack of cooperation during the examination. The reasons for misinterpretations of ultrasound images of the urinary system may lie in a large polymorphism of the kidney (defects and developmental variants) and may result from improper access to the organ as well as from the presence of artefacts. Errors may also result from the lack of knowledge concerning clinical and laboratory data. Moreover, mistakes in ultrasound diagnosis of the urinary system are frequently related to the lack of knowledge of the management algorithms and diagnostic possibilities of other imaging modalities. The paper lists errors in ultrasound diagnosis of the urinary system divided into: errors resulting from improper technique of examination, artefacts caused by incorrect preparation of patients for the examination or their constitution and errors resulting from misinterpretation of ultrasound images of the kidneys (such as their number, size, fluid spaces, pathological lesions and others), ureters and urinary bladder. Each physician performing kidney or bladder ultrasound examination should possess the knowledge of the most frequent errors and their causes which might help to avoid them.

2D/3D/4D contrast-enhanced voiding urosonography in the diagnosis and monitoring of treatment of vesicoureteral reflux in children - can it replace voiding cystourethrography?

INTRODUCTION: Vesicoureteral reflux appears in 20-50% of pediatric patients with recurrent urinary tract infections. The most common method of diagnosing this disease is voiding cystourethrography. However, contemporary pediatric radiology does not favor this method due to exposure to X-radiation. AIM: The aim of this study was to assess the usefulness of 2D/3D/4D contrast-enhanced voiding urosonography in the diagnosis and treatment monitoring of vesicoureteral reflux in children and the possibility of using contrast-enhanced voiding urosonography to replace voiding cystourethrography. MATERIAL AND METHODS: Voiding cystourethrography and contrast-enhanced voiding urosonography were conducted in 80 pediatric patients in order to assess sensitivity, specificity, positive and negative predictive values as well as the number of vesicoureteral refluxes detected by each of the two methods. The second stage of the study involved performing voiding urosonography in an extended protocol in 58 children in order to determine the usefulness of three-dimensional (3D/4D) examinations in the assessment of vesicoureteral reflux and the ability to assess the urethra. RESULTS: The concordance between the two methods was 86.95%. The sensitivity of voiding urosonography was 84.51%, specificity - 90.99%, positive predictive value - 85.71% and negative predictive value - 90.17%. A 3D/4D assessment of the urinary bladder and transperineal 2D morphological assessment of the urethra were possible in all patients (100%). Assessment of the urethra during micturition with the use of 2D/3D/4D techniques was possible in all patients in whom voiding was elicited (95.83%), and 3D/4D assessment of vesicoureteral reflux was possible in all patients with reflux (100%). Although the application of 3D/4D techniques allowed accurate specification of the grade of reflux in all cases (100%), it appeared particularly useful in differentiating between grades II and III (70.97%). CONCLUSIONS: Contrast-enhanced voiding urosonography allows the diagnosis and monitoring of treatment of vesicoureteral reflux in pediatric patients as well as assessment of the urethra in both girls and boys. The method is characterized by high sensitivity and specificity. Moreover, it is safe, relatively inexpensive and can replace voiding cystourethrography.