Historical and Contemporary Management of Infantile Hepatic Hemangioma: A 30-year Single-center Experience.

OBJECTIVE: To describe outcome of infants with hemangioma(s) of the liver. SUMMARY OF BACKGROUND DATA: Infantile hepatic hemangiomas exhibit a diverse phenotype. We report our 30-year experience and describe optimal management based on precise radiological classification. METHODS: Retrospective review of 124 infants (66 female) 1986-2016. Categorical analysis with Chi2 and nonparametric comparison. Data expressed as median (range) and P < 0.05 considered significant. RESULTS: Lesions classified as focal (n = 70, 56%); multifocal (n = 47, 38%) or diffuse (n = 7, 6%) and of these 80(65%) were symptomatic (eg, cardiac failure n = 39, 31%; thrombocytopenia n = 12, 10%).Increased hepatic artery velocity was seen in 63 (56%). Median hepatic artery velocity was greatest in diffuse lesions [245 (175-376) cm/s vs focal 120 (34-242) cm/s vs multifocal 93 (36-313) cm/s; P = 0.0001]. Expectant management alone was followed in 55 (44%). Medical therapy was utilised in 57(46%) and sufficient for symptom control in 29/57 (51%). Propranolol therapy (from 2008) was sufficient for symptom control in 22/28 (79%). Surgery (hepatic artery ligation n = 26; resection n = 13; embolization n = 1) was required in 40 (32%). Median maximal lesion diameter was 3 (0.5-17.1) cm and greater in those requiring surgery (7 cm vs 4.9 cm; P = 0.04). The proportion requiring surgery decreased markedly in the propranolol era [pre-propranolol 25/48 (52%) vs post-propranolol 16/76 (21%) (P = 0.0003)]. Systematic follow-up with ultrasound to a median of 2.6 (0.02-16) years. CONCLUSIONS: A proportion of infantile hepatic hemangiomas remain asymptomatic permitting observation until resolution but the majority require complex multi-modal therapy. First-line pharmacotherapy with propranolol has reduced but not abolished the need for surgery.

Contrast-Enhanced Ultrasound Quantification Assessment of Focal Fatty Variations in Liver Parenchyma: Challenging the Traditional Qualitative Paradigm of Uniform Enhancement With Adjacent Parenchyma.

OBJECTIVES: The purpose of this study was to quantify contrast-enhanced ultrasound enhancement of focal fatty sparing (FFS) and focal fatty infiltration (FFI) and compare it with adjacent liver parenchyma. METHODS: This was a retrospective observational study yielding 42 cases in the last 4 years. Inclusion criteria were a focal liver lesion, adequate video availability, and an established diagnosis of FFS or FFI based on clinical or imaging follow-up or a second modality. Contrast-enhanced ultrasound examinations were performed with a standard low-mechanical index technique. Commercially available software calculated quantitative parameters for a focal liver lesion and a reference area of liver parenchyma, producing relative indices. RESULTS: In total, 42 patients were analyzed (19 male) with a median age of 18 (interquartile range, 42) years and a median lesion diameter of 30 (interquartile range, 16) mm. The cohort included 26 with FFS and 16 with FFI. Subjectively assessed, 27% of FFS and 25% of FFI were hypoenhancing in the arterial phase, and 73% of FFS and 75% of FFI were isoenhancing. In the venous and delayed phases, all lesions were isoenhancing. The peak enhancement (P = .001), wash-in area under the curve (P < .01), wash-in rate (P = .023), and wash-in perfusion index (P = .001) were significantly lower in FFS compared with adjacent parenchyma but not the mean transit time. In the FFI subgroup, no significant difference was detected. Comparing relative parameters, only the wash-in rate was significantly (P = .049) lower in FFS than FFI. The mean follow-up was 2.8 years. CONCLUSIONS: Focal fatty sparing shows significantly lower and slower enhancement than the liver parenchyma, whereas FFI enhances identically. Focal fatty sparing had a significantly slower enhancement than FFI.

Contrast-enhanced ultrasound: a comprehensive review of safety in children.

Contrast-enhanced ultrasound (CEUS) has been increasingly used in pediatric radiology practice worldwide. For nearly two decades, CEUS applications have been performed with the off-label use of gas-containing second-generation ultrasound contrast agents (UCAs). Since 2016, the United States Food and Drug Administration (FDA) has approved the UCA Lumason for three pediatric indications: the evaluation of focal liver lesions and echocardiography via intravenous administration and the assessment of vesicoureteral reflux via intravesical application (contrast-enhanced voiding urosonography, ceVUS). Prior to the FDA approval of Lumason, numerous studies with the use of second-generation UCAs had been conducted in adults and children. Comprehensive protocols for clinical safety evaluations have demonstrated the highly favorable safety profile of UCA for intravenous, intravesical and other intracavitary uses. The safety data on CEUS continue to accumulate as this imaging modality is increasingly utilized in clinical settings worldwide. As of August 2021, 57 pediatric-only original research studies encompassing a total of 4,518 children with 4,906 intravenous CEUS examinations had been published. As in adults, there were a few adverse events; the majority of these were non-serious, although very rarely serious anaphylactic reactions were reported. In the published pediatric-only intravenous CEUS studies included in our analysis, the overall incidence rate of serious adverse events was 0.22% (10/4,518) of children and 0.20% (10/4,906) of all CEUS examinations. Non-serious adverse events from the intravenous CEUS were observed in 1.20% (54/4,518) of children and 1.10% (54/4,906) of CEUS examinations. During the same time period, 31 studies with the intravesical use of UCA were conducted in 12,362 children. A few non-serious adverse events were encountered (0.31%; 38/12,362), but these were most likely attributable to the bladder catheterization rather than the UCA. Other developing clinical applications of UCA in children, including intracavitary and intralymphatic, are ongoing. To date, no serious adverse events have been reported with these applications. This article reviews the existing pediatric CEUS literature and provides an overview of safety-related information reported from UCA uses in children.

European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB): An Update on the Pediatric CEUS Registry on Behalf of the "EFSUMB Pediatric CEUS Registry Working Group".

The European Federation of Ultrasound in Medicine and Biology (EFSUMB) created the "EFSUMB Pediatric Registry" (EFSUMB EPR) with the purpose of collecting data regarding the intravenous application of pediatric contrast-enhanced ultrasound (CEUS). The primary aim was to document the current clinical practice and usefulness of the technique and secondarily to assess CEUS safety in children. We issue the preliminary results of this database and examine the overall practice of CEUS in children in Europe.

Autoimmune pancreatitis in children: A single centre experience in diagnosis, management and long term follow up.

OBJECTIVES: Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis and data is limited in the paediatric population. We aim to describe in detail a cohort of paediatric patients with AIP including their presentation, investigations that led to their diagnosis, management and long-term follow up. METHODS: We retrospectively reviewed the data of 6 patients diagnosed with AIP over an 10-year period. Data including demographics, clinical information, laboratory parameters, serological markers, radiological and histological findings as well as longitudinal follow up were collected. RESULTS: Out of the six patients, one was diagnosed with definitive Type 1 AIP, two with definitive Type 2 AIP, two with probable Type 2 AIP and one with suspected Type 2 AIP. Median time of follow up was 3.9 years (range 2.6-10.1). 4 patients had pancreatic biopsies with 2 of these patients showing granulocytic epithelial lesions (GELs). 4 patients received steroids and two of them developed ulcerative colitis. Azathioprine was commenced on the patient with Type 1 AIP to help her wean off steroids that caused significant side effects on her. Only two patients developed exocrine insufficiency. CONCLUSIONS: The long term follow up of our cohort of paediatric AIP shows good prognosis. More follow up data on patients with AIP is needed to help further characterize and define the disease.

Contrast-enhanced ultrasound in the diagnosis of pediatric focal nodular hyperplasia and hepatic adenoma: interobserver reliability.

BACKGROUND: Focal nodular hyperplasia and hepatic adenoma are rare liver tumors in which specific features on contrast-enhanced ultrasound (US) have been reported but are little known in children. OBJECTIVE: To assess the interobserver agreement in diagnosing and differentiating focal nodular hyperplasia and hepatic adenoma in children using established adult contrast-enhanced US characteristics. MATERIALS AND METHODS: Thirty children with a definite or probable diagnosis of focal nodular hyperplasia or hepatic adenoma on magnetic resonance imaging (MRI)/histology who underwent contrast-enhanced US studies were included. Typical and additional contrast-enhanced US features of focal nodular hyperplasia and hepatic adenoma were included. The lesions were classified as definite/probable focal nodular hyperplasia, definite/probably hepatic adenoma or unclassified. The interobserver kappa of contrast-enhanced US characteristics was calculated. RESULTS: Focal nodular hyperplasia and hepatic adenoma in children demonstrate contrast-enhanced US characteristics similar to those in adults. Among the nine lesions with confirmed histological diagnosis, correct diagnosis was made in 7 (77.8%) based on contrast-enhanced US criteria. Two lesions were unclassified by both observers due to a mixed arterial filling pattern. Interobserver kappa for contrast-enhanced US diagnosis was 0.64 (P<0.0001). CONCLUSION: There is a good interobserver kappa for separating focal nodular hyperplasia from hepatic adenoma in children using established adult contrast-enhanced US features.

Is Testicular Macrocalcification a Risk for Malignancy?: Tumor Development on Ultrasonographic Follow-up of Preexisting Intratesticular Macrocalcification.

Testicular macrocalcification is an uncommon finding when imaging the scrotum with ultrasonography (US). It is not normally a recognized risk factor for development of testicular malignancy, and patients are not usually offered follow-up US examinations or counseled for self-examination. This aspect is in distinction to patients with testicular microlithiasis (usually with an additional risk factor), who are offered follow-up on the assumption that microlithiasis is associated with malignancy. We report a series of 6 patients with predetermined testicular macrocalcification, with development of a malignancy on follow-up US. We encourage US follow-up examinations for patients with macrocalcification, potentially in a similar manner as for those with testicular microlithiasis.

Retrospective Analysis of the Safety and Cost Implications of Pediatric Contrast-Enhanced Ultrasound at a Single Center.

OBJECTIVE: Because of concern over medical ionizing radiation exposure of children, contrast-enhanced ultrasound (CEUS) has generated interest as an inexpensive, ionizing radiation-free alternative to CT and MRI. CEUS has received approval for pediatric hepatic use but remains off-label for a range of other applications. The purposes of this study were to retrospectively analyze adverse incidents encountered in pediatric CEUS and to assess the financial benefits of reducing the number of CT and MRI examinations performed. MATERIALS AND METHODS: All pediatric (patients 18 years and younger) CEUS examinations performed between January 2008 and December 2015 were reviewed. All immediate reactions deemed due to contrast examinations were documented in radiology reports. Electronic patient records were examined for adverse reactions within 24 hours not due to an underlying pathologic condition. With tariffs from the U.K. National Institute of Clinical Excellence analysis, CEUS utilization cost ($94) was compared with the CT ($168) and MRI ($274) costs of the conventional imaging pathway. RESULTS: The records of 305 pediatric patients (187 boys, 118 girls; age range, 1 month-18 years) undergoing CEUS were reviewed. Most of the studies were for characterizing liver lesions (147/305 [48.2%]) and trauma (113/305 [37.1%]); the others were for renal, vascular, and intracavitary assessment (45/305 [14.8%]). No immediate adverse reactions occurred. Delayed adverse reactions occurred in two patients (2/305 [0.7%]). These reactions were transient hypertension and transient tachycardia. Neither was symptomatic, and both were deemed not due to the underlying disorder. The potential cost savings of CEUS were $74 per examination over CT and $180 over MRI. CONCLUSION: Pediatric CEUS is a safe and potentially cost-effective imaging modality. Using it allows reduction in the ionizing radiation associated with CT and in the gadolinium contrast administration, sedation, and anesthesia sometimes required for MRI.

Contrast-enhanced US-guided Interventions: Improving Success Rate and Avoiding Complications Using US Contrast Agents.

Ultrasonography (US) is an established modality for intervention. The introduction of microbubble US contrast agents (UCAs) has the potential to further improve US imaging for intervention. According to licensing, UCAs are currently approved for clinical use in restricted situations, but many additional indications have become accepted as having clinical value. The use of UCAs has been shown to be safe, and there is no risk of renal toxic effects, unlike with iodinated or gadolinium contrast medium. Broadly speaking, UCAs can be injected into the bloodstream (intravascular use) or instilled into almost any accessible body cavity (endocavitary use), either in isolation or synchronously. In microvascular applications, contrast-enhanced US (CEUS) enhances delineation of necrotic areas and the vascularized target to improve real-time targeting. The ability of CEUS to allow true assessment of vascularity has also been used in follow-up of devascularizing intervention. In macrovascular applications, real-time angiographic images can be obtained with CEUS without nephrotoxic effects or radiation. In endocavitary applications, CEUS can achieve imaging similar to that of iodinated contrast medium-based fluoroscopy; follow-up to intervention (eg, tubography and nephrostography) can be performed at the bedside, which may be advantageous. The use of UCAs is a natural progression in US-guided intervention. The aim of this article is to describe the indications, contraindications, and techniques of using UCAs as an adjunctive tool for US-guided interventional procedures to facilitate effective treatment, improve complication management, and increase the overall success of interventional procedures. Online supplemental material is available for this article. ©RSNA, 2016.

Intravenous and Intracavitary Use of Contrast-Enhanced Ultrasound in the Evaluation and Management of Complicated Pediatric Pneumonia.

Pediatric pneumonia can be complicated by necrotizing pneumonia or a parapneumonic effusion either in the form of an empyema or a clear effusion. Ultrasonography (US) and computed tomography represent well-established modalities for evaluation of complicated pediatric pneumonia. Contrast-enhanced ultrasound (CEUS) was recently introduced and is gaining increasing acceptance in pediatric imaging. In this case series, we present our initial experience with both intravenous and intracavitary use of CEUS in children with complicated pneumonia. Intravenous CEUS accurately and confidently showed necrotizing pneumonia and delineated pleural effusions, whereas intracavitary CEUS accurately identified the chest catheter location and patency and showed the presence of loculations, suggesting the use of fibrinolytics.

Multiparametric Sonography of Hematologic Malignancies of the Testis: Grayscale, Color Doppler, and Contrast-Enhanced Ultrasound and Strain Elastographic Appearances With Histologic Correlation.

Primary testicular lymphoma is rare and appears with nonspecific findings on grayscale and color Doppler sonography. We present 8 patients further examined with contrast-enhanced sonography, strain elastography, and histologic analysis after orchiectomy. Seven of 8 patients had a diagnosis of large B-cell lymphoma, and 1 of 8 had a diagnosis of granulocytic sarcoma, with solitary lesions (2 of 8), multiple lesions (3 of 8), or entire testicular involvement (3 of 8). Lesions appeared hypoechoic (7 of 8) or isoechoic (1 of 8), all with increased vascularity on color Doppler sonography and a nonbranching linear pattern of intratumoral vessels (7 of 8). Contrast-enhanced ultrasound (CEUS) confirmed this pattern and showed increased enhancement in all lesions. On strain elastography, all lesions were hard, with an elasticity score of greater than 4. Multiparametric sonography of testicular lymphoma identifies increased vascularity on color Doppler and contrast-enhanced ultrasound and increased lesion stiffness on strain elastography.

Role of Contrast-Enhanced Ultrasound (CEUS) in Paediatric Practice: An EFSUMB Position Statement.

The use of contrast-enhanced ultrasound (CEUS) in adults is well established in many different areas, with a number of current applications deemed "off-label", but the use supported by clinical experience and evidence. Paediatric CEUS is also an "off-label" application until recently with approval specifically for assessment of focal liver lesions. Nevertheless there is mounting evidence of the usefulness of CEUS in children in many areas, primarily as an imaging technique that reduces exposure to radiation, iodinated contrast medium and the "patient-friendly" circumstances of ultrasonography. This position statement of the European Federation of Societies in Ultrasound and Medicine (EFSUMB) assesses the current status of CEUS applications in children and makes suggestions for further development of this technique.

Congenital Vascular Malformations of the Liver: An Association With Trisomy 21.

A link between congenital vascular malformation (CVM) of the liver and trisomy 21 has been suggested. We reviewed all children with trisomy 21 referred for investigation to a specialist pediatric hepatobiliary unit (1985-2015). Forty-five children with trisomy 21 were identified; 7 (15%) had a defined CVMs (4 girls). All such infants were also diagnosed with a range of cardiac defects. CVMs were divided according to the nature of the vascular connection. Group (i) (n = 3): Abnormal venovenous anomaly. This included portocaval shunt and patent ductus venosus (n = 2). Group (ii) (n = 4): Involvement of all 3 vascular systems. Two infants had arterioportal hypertension caused by hepatic arteries feeding into a left portal vein aneurysm within the umbilical fissure. Two infants had more complex hepatic artery to hepatic vein shunts developing early cardiorespiratory failure with progressive jaundice. Our series shows a clear association between cardiac anomalies and CVM in children with trisomy 21.

Multiparametric Sonography of Testicular Hematomas: Features on Grayscale, Color Doppler, and Contrast-Enhanced Sonography and Strain Elastography.

We investigated the appearances of intratesticular hematomas on multiparametric sonography combining grayscale, color Doppler, and contrast-enhanced sonography and strain elastography. Over a 3-year period, 17 hematomas in 8 patients were analyzed. Blunt trauma (n = 7) and surgery (n = 1) were preceding events. Grayscale sonography showed well-defined oval or round predominantly hypoechoic and heterogeneous, peripherally (n = 13) or centrally (n = 4) located lesions. Three showed contrast enhancement of septations, and 4 showed peripheral lesion rim enhancement. On strain elastography, the mean strain ratio was 1.19 (range, 0.41-2.36), and elasticity score were 3 or lower (n = 13) and 5 (n = 4). Multiparametric sonography shows nonenhancement with contrast and predominantly "soft" elastographic properties and aids in the interpretation of the benign nature of intratesticular hematomas.

Tracheomegaly in infants with severe congenital diaphragmatic hernia treated with fetal endoluminal tracheal occlusion.

OBJECTIVE: To measure and evaluate the effects of tracheal dimensions on survival and ventilation in a large series of infants with congenital diaphragmatic hernia (CDH) treated antenatally with fetal endoluminal tracheal occlusion (FETO). STUDY DESIGN: Tracheal dimensions on chest radiograph (CR) were measured by 2 blinded radiologists. Survival, day 1 best oxygenation index and duration of ventilation, continuous positive airway pressure, and hospital stay were recorded. Survivors with a minimum 12-month follow-up were longitudinally compared for incidence of gastroesophageal reflux, chest infections, chest deformities, and hernia recurrence. RESULTS: Seventy infants with CDH (41 who underwent FETO) were treated between 2004 and 2010. Hernia repair was performed in 26 infants without FETO (8 with patch repair) and 35 infants with FETO (26 with patch repair; P = .0015). Infants with FETO had a wider trachea than those without FETO at T1 (P < .0001) and between T1 and the carina (P < .0001). Tracheal diameter was similar in survivors and nonsurvivors in the FETO group. Tracheal size was not correlated with day 1 best oxygenation index in the FETO group (R2 = 0.17) or the non-FETO group (R2 = 0.07). There were no between-group differences in duration of mechanical ventilation (P = .30), continuous positive airway pressure (P = .20), or hospital stay (P = .30). In the longitudinal study, tracheal widths were larger on the last CR than on preoperative CR in patients without FETO (T1, P = .02; widest point, P = .001; carina, P = .0001), and for patients with FETO at the widest point (P < .0001) and at the carina (P < .0001), but not at T1 (P = .12). There were no differences in clinical variables between the FETO and non-FETO groups. CONCLUSION: FETO has a significant impact on tracheal size of infants with CDH; however, tracheal size does not affect survival or the requirement for early respiratory support.

Cortical necrosis secondary to trauma in a child: contrast-enhanced ultrasound comparable to magnetic resonance imaging.

Cortical necrosis is an uncommon cause of renal impairment and is rarely a consequence of blunt abdominal trauma. We present a case of unilateral traumatic acute cortical necrosis in a child demonstrated on contrast-enhanced US with confirmation on MRI. Contrast-enhanced US provides a rapid, accurate evaluation of renal parenchyma abnormalities in blunt abdominal trauma in children without exposure to ionising radiation or the risk of sedation.

Multiparametric Ultrasound for Focal Testicular Pathology: A Ten-Year Retrospective Review.

Conventional ultrasonography (US), including greyscale imaging and colour Doppler US (CDUS), is pivotal for diagnosing scrotal pathologies, but it has limited specificity. Historically, solid focal testicular abnormalities often led to radical orchidectomy. This retrospective study evaluated the utilisation of contrast-enhanced ultrasound (CEUS) and strain elastography (SE) in investigating intratesticular focal abnormalities. A total of 124 cases were analysed. This study underscored the superior diagnostic capabilities of CEUS in detecting vascular enhancement in all malignant cases, even those with undetectable vascularity by CDUS. It also highlighted the potential of CEUS in identifying distinctive vascular patterns in benign vascular tumours. Definitive confirmation of benignity could be obtained when the absence of enhancement was demonstrated on CEUS. While SE alone offered no distinctive advantage in differentiating between benign and malignant pathologies, we demonstrated that incorporating a combination of CEUS and SE into the evaluation of focal testicular abnormalities could improve diagnostic performance metrics over conventional CDUS. Our findings underscore the role of advanced ultrasound techniques in enhancing the evaluation of focal testicular abnormalities in clinical practice and could aid a shift towards testis-sparing management strategies.

Global testicular infarction in the presence of epididymitis: clinical features, appearances on grayscale, color Doppler, and contrast-enhanced sonography, and histologic correlation.

Epididymitis is common, presenting indolently with unilateral scrotal pain and swelling. Diagnosis is based on clinical assessment and resolves with antibiotic therapy. Recognized complications are abscess formation and segmental infarction. Global testicular infarction is rare. Diagnosis is important and requires surgical management. On grayscale sonography, global infarction may be difficult to establish. The addition of color Doppler imaging is useful but is observer experience dependent with limitations in the presence of low flow. Contrast-enhanced sonography is useful for unequivocally establishing the diagnosis. We report global testicular infarction in 2 patients with epididymitis clearly depicted on contrast-enhanced sonography, allowing immediate surgical management.

Contrast-enhanced ultrasound in the evaluation of focal testicular complications secondary to epididymitis.

OBJECTIVE: The purpose of this study is to determine the effectiveness of contrast-enhanced ultrasound in evaluating incidental focal testicular lesions in epididymitis. MATERIALS AND METHODS: Intratesticular lesions ipsilateral to epididymitis were subject to B-mode color Doppler ultrasound and contrast-enhanced ultrasound, with their appearances reviewed in consensus. Final interpretation was by histologic analysis or follow-up ultrasound. RESULTS: Over 28 months, 16 focal testicular lesions (median lesion size, 24 mm; range, 14-48 mm) in 14 patients (median age, 49 years; range, 18-81 years) were examined. Lesions were oval (n = 14), wedge shaped (n = 1), or involved the entire testis (n = 1). Lesions were isoechoic (n = 1), hypoechoic (n = 4), or of mixed echogenicity (n = 11). Color Doppler ultrasound flow was not clearly depicted in 13 lesions but was present in three lesions, with contrast-enhanced ultrasound concordant with color Doppler ultrasound, showing unequivocal absence of vascularity and increased flow, respectively. In the avascular lesions, rim enhancement (n = 6), vascular projections (n = 4), and irregular (n = 10) and smooth (n = 2) borders were documented. The observers identified infarction (n = 9), abscess (n = 4), orchitis (n = 1), and tumor (n = 2). Histologic examination (seven lesions in five patients) confirmed infarction, abscess formation, and seminoma; follow-up ultrasound confirmed resolution for eight patients. CONCLUSION: Contrast-enhanced ultrasound is a useful adjuvant to color Doppler ultrasound examination of a focal lesion in the testis ipsilateral to epididymitis to improve the characterization of nonvascularized tissue.