Pediatric Hepatic Cystic Lesions: Differential Diagnosis and Multimodality Imaging Approach.

When a pediatric hepatic cystic lesion is identified at imaging, the differential diagnosis may be broad, including developmental, infectious, neoplastic, and posttraumatic or iatrogenic causes. The location of a cystic lesion and its number, size, composition, and relationship to the biliary system are features that help in narrowing the differential diagnosis. An incidentally detected simple hepatic cyst is the most commonly encountered. Ciliated foregut cysts are typically located in hepatic segment IVa. The presence of multiple cysts should raise suspicion for fibropolycystic liver disease, a group of related lesions-including biliary hamartoma and choledochal cyst-caused by abnormal embryologic development of the ductal plate. Communication of the cystic lesion with the biliary tree can confirm the diagnosis of choledochal cyst. In a neonate with jaundice, a cystic lesion at the porta hepatis should raise suspicion for choledochal cyst versus cystic biliary atresia. Hepatic abscess can appear cystlike, though typically with internal contents. In an immunocompromised child, multiple cystlike lesions should raise concern for fungal microabscesses. A complex cystic mass in a young child should raise suspicion for mesenchymal hamartoma, which can evolve into undifferentiated embryonal sarcoma if untreated. Hepatic hematoma and biloma can appear cystlike in children with a history of trauma or recent intervention. In neonates with an umbilical vein catheter (UVC), an intrahepatic cyst along the course of the UVC should raise concern for infusate extravasation. Familiarity with imaging findings and clinical features is essential for achieving accurate diagnosis of pediatric hepatic cystic lesions, which in turn can guide appropriate clinical management. Online supplemental material is available for this article. ©RSNA, 2022.

Pretransplantation and Post-Transplantation Liver Disease Assessment in Adolescents Undergoing Isolated Heart Transplantation for Fontan Failure.

OBJECTIVE: To describe the assessment of Fontan-associated liver disease and determine the clinical and imaging measures that may identify hepatic morbidity risk in isolated heart transplantation candidates and trend those measures post-isolated heart transplantation. STUDY DESIGN: Retrospective analysis of pre-isolated heart transplantation and post-isolated heart transplantation Fontan-associated liver disease (FALD) status using blood tests, magnetic resonance imaging (MRI), and liver biopsy analysis within 6 months before isolated heart transplantation and 12 months after isolated heart transplantation in 9 consecutive patients with Fontan. Pre- and post-isolated heart transplantation standard laboratory values; varices, ascites, splenomegaly, thrombocytopenia (VAST) score; Fontan liver MRI score; liver biopsy scores; Model for End-stage Liver Disease (MELD); MELD excluding the International Normalized Ratio (MELD-XI); AST to platelet ratio index, and cardiac catheterization data were compared. RESULTS: Pretransplantation maximum MELD and MELD-XI was 15 and 16, respectively. Central venous pressures and VAST scores decreased significantly post-transplantation. In 5 paired studies, Fontan liver MRI score maximum was 10 pretransplantation and decreased significantly post-transplantation. Arterially enhancing nodules on MRI persisted in 2 patients post-transplantation. Pretransplantation and post-transplantation liver biopsy scores did not differ in 4 paired biopsy specimens. CONCLUSIONS: Patients with FALD and MELD <15, MELD-XI <16, Fontan liver MRI score <10, and VAST score ≤2 can have successful short-term isolated heart transplantation outcomes. Liver MRI and VAST scores improved post-transplantation. Post-transplantation liver biopsy scores did not change significantly. Pretransplantation liver biopsy demonstrating fibrosis alone should not exclude consideration of isolated heart transplantation. The persistence of hepatic vascular remodeling and fibrosis post-isolated heart transplantation suggests that continued surveillance for hepatic complications post-transplantation for patients with Fontan is reasonable.

Radiographic findings predictive of irreducibility and surgical resection in ileocolic intussusception.

BACKGROUND: Ileocolic intussusception is a common cause of intestinal obstruction in young children. Radiographs may be of limited value in the diagnosis of intussusception and are sometimes utilized primarily to exclude pneumoperitoneum before therapeutic enema reduction. OBJECTIVE: The goal of this study was to determine if radiographic findings in ileocolic intussusception can offer prognostic information regarding the outcome of therapeutic air enema and, for those requiring surgical intervention, surgical outcomes and/or complications. MATERIALS AND METHODS: A single institution retrospective study was performed including all enemas for intussusception performed during a 5-year period from September 2012 to August 2017. Radiographs obtained before therapeutic enema, including our institution radiographs, outside facility radiographs, or scout images obtained during fluoroscopy or computed tomography (CT), were independently scored by two pediatric radiologists for normal bowel gas pattern, soft-tissue mass, paucity of bowel gas, meniscus sign and bowel obstruction. The reviewers were blinded to enema and surgical outcomes at the time of review. Differences were resolved by consensus. Cases were excluded in which there was no adequate pre-procedure radiograph. In total, 182 cases were reviewed. The medical records were reviewed for enema and surgical outcomes. RESULTS: Radiographic findings included normal bowel gas pattern in 13%, soft-tissue mass in 26%, paucity of bowel gas in 65%, meniscus sign in 12% and obstruction in 10% of the cases, with 17.5% of patients having more than one finding. In patients with bowel obstruction on radiographs, there was a statistically significant decrease in success of therapeutic enema (83% vs. 21%, P=0.0001), increase in complicated surgical reductions (47% vs. 4%, P=0.0012), and increase in bowel resection (42% vs. 4%, P=0.003) compared to patients with normal bowel gas pattern. CONCLUSION: Radiographs can offer prognostic information regarding the potential for therapeutic enema success, as well as potential surgical outcomes in patients failing enema reduction. Particularly, bowel obstruction significantly decreases the success of therapeutic enema and increases the need for bowel resection.

Incidence and importance of portal venous gas in children with hypertrophic pyloric stenosis.

BACKGROUND: Hypertrophic pyloric stenosis (HPS) is a common cause of gastric outlet obstruction in young infants. Infants with HPS present with projectile vomiting, sometimes have electrolyte abnormalities and typically undergo pyloromyotomy to alleviate the obstruction. Abdominal US is the gold standard imaging study for diagnosis. Case reports of incidental hepatic portal venous gas have been reported in infants with HPS; however, no large studies have been conducted to determine the incidence or possible clinical implications of this finding. OBJECTIVE: To assess the incidence of portal venous gas in infants with HPS and to determine whether the presence of this gas in infants with HPS indicates a more unstable patient, increased length of stay or worse outcome. MATERIALS AND METHODS: We conducted a retrospective review of sonographic reports containing "pyloric stenosis," excluding negative descriptor, at a tertiary-care children's hospital from November 2010 to September 2017. Data collected included pyloric thickness/length, liver evaluation, portal venous gas, any additional imaging, demographics, symptomatology days, electrolyte abnormality, and length of hospital stay. RESULTS: In a 7-year period, 545 US exams were positive for HPS. Of these, 334 exams included enough hepatic parenchyma to evaluate for portal venous gas. Infants in 6 of the 334 exams demonstrated portal venous gas (1.8%). Clinical presentation (length of symptoms and electrolyte abnormalities), demographics (male predominance and age at presentation) and imaging characteristics (pyloric thickness and length) were similar for the HPS groups with and without portal venous gas. There was no significant difference in outcome or length of hospital stay. CONCLUSION: Visualization of portal venous gas in infants with HPS is not rare and appears benign, without need for further imaging. Portal venous gas in infants with HPS does not portend a more severe patient presentation or outcome.

Congenital Limb Overgrowth Syndromes Associated with Vascular Anomalies.

Congenital limb length discrepancy disorders are frequently associated with a variety of vascular anomalies and have unique genetic and phenotypic features. Many of these syndromes have been linked to sporadic somatic mosaicism involving mutations of the phosphoinositide 3-kinase (PI3K)/protein kinase B (AKT)/mammalian target of rapamycin (mTOR) pathway, which has an important role in tissue growth and angiogenesis. Radiologists who are aware of congenital limb length discrepancies can make specific diagnoses based on imaging findings. Although genetic confirmation is necessary for a definitive diagnosis, the radiologist serves as a central figure in the identification and treatment of these disorders. The clinical presentations, diagnostic and imaging workups, and treatment options available for patients with Klippel-Trenaunay syndrome, CLOVES (congenital lipomatous overgrowth, vascular anomalies, epidermal nevi, and scoliosis/spinal deformities) syndrome, fibroadipose vascular anomaly, phosphatase and tensin homolog mutation spectrum, Parkes-Weber syndrome, and Proteus syndrome are reviewed. ©RSNA, 2019.

Magnetic Resonance Enterography Healing and Magnetic Resonance Enterography Remission Predicts Improved Outcome in Pediatric Crohn Disease.

BACKGROUND: Mucosal healing predicts clinical remission and improved outcomes in patients with Crohn disease (CD). Magnetic resonance enterography (MRE) is a noninvasive imaging modality that can assess small and large bowel wall inflammation. Evidence suggests that MRE may be an acceptable alternative to evaluate mucosal healing over endoscopy. Our objective is to determine whether MRE remission predicts clinical remission at follow-up in children with CD. METHODS: We performed an institutional review board-approved retrospecitve chart review using our prospectively maintained MRE CD database. Inclusion criteria were all children who underwent an MRE more than 6 months after diagnosis with CD who had follow-up of at least 1 year from imaging. RESULTS: A total of 101 children with CD underwent MRE, a median of 1.3 years from diagnosis with a median follow-up of 2.8 years after MRE. Active inflammation was detected in 65 MRE studies, whereas 36 MRE studies demonstrated MRE remission. A total of 88.9% of children demonstrating MRE remission were in clinical remission at follow-up, whereas only 44.6% of those demonstrating MRE active inflammation achieved clinical remission. Children demonstrating MRE-active inflammation were more likely to have a change in medication (44.6% vs 8.3%) and more likely to undergo surgery (18.5% vs 2.8%). CONCLUSIONS: MRE remission is associated with clinical remission at follow-up at least 1 year after MRE. MRE remission was associated with fewer medication changes and fewer surgeries suggesting that, similar to endoscopic remission, MRE remission demonstrates improved outcome. Additional research is needed to confirm that MRE can be used as a surrogate for mucosal healing.

Malignant degeneration of pulmonary juvenile-onset recurrent respiratory papillomatosis.

Juvenile-onset recurrent respiratory papillomatosis (JORRP) is a rare disease associated with the human papilloma virus (HPV) in which papillomas form along the aerodigestive tract in children. Pulmonary involvement is uncommon, but associated with worse clinical outcomes, including the rare complication of malignant transformation. We present a patient with JORRP in which lung disease underwent malignant transformation during adolescence. Our goal is to raise awareness of the potential for malignant transformation in children, as well as to familiarize pediatric radiologists with imaging features of malignant lung disease in JORRP. We advocate for the identification of the subgroup of JORPP patients with pulmonary disease who, due to increased risk for malignant transformation, may benefit from closer clinical and imaging surveillance by a multidisciplinary team.

Use of the star sign to diagnose internal fistulas in pediatric patients with penetrating Crohn disease by MR enterography.

Development of internal fistula due to extramural spread of inflammatory bowel disease is a characteristic feature of penetrating disease in patients with Crohn disease. The "star sign" is a radiological finding of internal fistula that has previously been described in the gastroenterology literature in adult Crohn disease patients undergoing MR enteroclysis. The goal of this paper is to review the clinical and imaging features of penetrating disease in pediatric Crohn disease patients, highlighting the star sign as a useful diagnostic tool for diagnosing internal fistula in children by MR enterography. The recognition of penetrating complications by MR imaging can have important therapeutic and prognostic implications.

Magnetic resonance imaging identifies unsuspected liver abnormalities in patients after the Fontan procedure.

OBJECTIVE: To determine whether abdominal magnetic resonance imaging (MRI) detects hepatic abnormalities before clinical or biochemical perturbations in patients after the Fontan procedure. STUDY DESIGN: Thirty-nine children and adolescents who underwent the Fontan procedure and were referred to a pediatric hepatologist by cardiology services between 2011 and 2012 were reviewed retrospectively. Physical examination findings, routine laboratory tests of liver function, evaluation for chronic liver disease, and abdominal MRI findings were recorded. MRI findings were evaluated relative to time elapsed since surgery by 2 radiologists (blinded). RESULTS: Assessment for coexisting chronic liver disease was negative in all patients. All patients had a normal serum albumin level and International Normalized Ratio. Twenty-six of the 39 patients (67%) underwent abdominal MRI, 4 had MRI-incompatible hardware, and 9 did not undergo MRI because of insurance denial. All MRI scans demonstrated morphologic liver changes with varying degrees of reticular contrast enhancement compatible with fibrosis and congestion. Reticular contrast enhancement was often nonuniform, and 9 patients (35%) had multifocal arterially enhancing lesions. CONCLUSION: MRI can identify hepatic abnormalities in patients after Fontan surgery that go undetected by standard clinical and laboratory assessments. These abnormalities are not uniformly distributed throughout the liver, and thus assessment by liver biopsy analysis is subject to sampling error.

Imaging findings in megacystis-microcolon-intestinal hypoperistalsis syndrome.

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare and frequently lethal form of severe functional intestinal obstruction more commonly found in girls. Imaging features characteristic of this disease include a large dilated bladder, microcolon and intestinal dysmotility. Additional imaging findings may include intestinal malrotation, hydronephrosis and vesicoureteral reflux. It is usually fatal in the first year of life. Because presenting clinical and imaging features can mimic other causes of neonatal bowel obstruction, we compiled examples of this disorder to help the pediatric radiologist recognize imaging findings associated with MMIHS and aid in the development of a long-term management plan and in counseling the family regarding implications of this disorder. We reviewed recent and historical literature relevant to MMIHS and present the imaging and clinical features of four patients with MMIHS treated at our institution as examples of this uncommon disorder.

An Abbreviated Non-Contrast MRI Protocol for Osteomyelitis May Reduce the Need for Sedation in Young Children.

Lengthy MRI examinations in young children often requires sedation. When sedation is unavailable, critical imaging may be delayed. Abbreviating the imaging protocol to a few essential sequences may reduce the need for sedation and prevent delays in patient care. We retrospectively evaluated an abbreviated noncontrast MRI protocol to diagnose lower extremity osteomyelitis in the pediatric population. The IRB approved this study. The radiology information system was searched for lower extremity MRI examinations for osteomyelitis in patients <20 years old from August 2020 to August 2021. Three noncontrast sequences (long axis T1 without fat saturation (FS), long axis STIR, and axial T2 with FS) were independently reviewed by 2 pediatric radiologists. The accuracy of the reviewers was compared to the clinical radiology report based on the unabridged contrast-enhanced standard department protocol. The search yielded 80 exams, mean age was 7 years old, 59% (47/80) were male, and 41% (33/80) were female. The accuracies of reviewer A and reviewer B were 95% and 89%, respectively. The reviewer inter-observer agreement for the diagnosis of osteomyelitis was strong (k = 0.79). The accuracy of an abbreviated noncontrast MRI protocol to evaluate lower extremity osteomyelitis in children approaches that of the unabridged protocol and has the potential to decrease the need for sedation in young children.

NUT midline carcinoma: an imaging case series and review of literature.

BACKGROUND: NUT midline carcinoma is a rare and aggressive tumor that has primarily been reported in children and young adults. The tumor is characterized by a rearrangement on the nuclear protein in testis (NUT) gene, located on chromosome 15q14, resulting in the BRD4-NUT fusion oncogene. This carcinoma most commonly presents in the midline and displays an invariably lethal clinical course. OBJECTIVE: To highlight the imaging features of NUT midline carcinoma. MATERIALS AND METHODS: IRB approval was obtained for chart review. We retrospectively reviewed the chart and imaging studies of three children. All three cases were diagnosed by karyotyping and confirmed by fluorescence in situ hybridization (FISH). Cross-sectional imaging including CT, MRI and, in one patient, PET was available for review. RESULTS: Two out of three children presented with midline and multifocal disease. The third case had a medial left thigh mass and no metastatic disease at initial presentation. The common imaging features include heterogeneous low density on CT and T1 hypointensity and low-level T2 hyperintensity on MRI with heterogeneous enhancement. All cases were confirmed pathologically and by molecular studies. CONCLUSION: NUT midline carcinoma usually presents in the midline, either in the head, neck or chest. We present three cases with the bulk of the tumor below the diaphragm, which is seen in the minority of patients with NUT midline carcinoma, according to the available literature. Metastatic disease is common at initial presentation and can be quite extensive. The most striking feature of this disease is its aggressive nature with exponential interval growth of tumor.

Improving ultrasound for appendicitis through standardized reporting of secondary signs.

OBJECTIVE: Our aim was to implement a standardized US report that included secondary signs of appendicitis (SS) to facilitate accurate diagnosis of appendicitis and decrease the use of computed tomography (CT) and admissions for observation. METHODS: A multidisciplinary team implemented a quality improvement (QI) intervention in the form of a standardized US report and provided stakeholders with monthly feedback. Outcomes including report compliance, CT use, and observation admissions were compared pretemplate and posttemplate. RESULTS: We identified 387 patients in the pretemplate period and 483 patients in the posttemplate period. In the posttemplate period, the reporting of SS increased from 5.4% to 79.5% (p<0.001). Despite lower rates of appendix visualization (43.9% to 32.7%, p<0.001) with US, overall CT use (8.5% vs 7.0%, p=0.41) and the negative appendectomy rate remained stable (1.0% vs 1.0%, p=1.0). CT utilization for patients with an equivocal ultrasound and SS present decreased (36.4% vs 8.9%, p=0.002) and admissions for observations decreased (21.5% vs 15.3%, p=0.02). Test characteristics of RLQ US for appendicitis also improved in the posttemplate period. CONCLUSION: A focused QI initiative led to high compliance rates of utilizing the standardized US report and resulted in lower CT use and fewer admissions for observation. Study of a Diagnostic Test Level of Evidence: 1.

The Skeletally Immature and Newly Mature Throwing Athlete.

Injuries to the shoulder and elbow in the pediatric and adolescent throwing athlete are common. Both knowledge of throwing mechanics and understanding of normal bone development in the immature skeleton are key to the diagnosis, treatment, and potential prevention of these common injuries. Pathologic changes from chronic repetitive trauma to the developing shoulder and elbow manifest as distinctly different injuries that can be predicted by the skeletal maturation of the patient. Sites of vulnerability and resulting patterns of injury change as the child evolves from the skeletally immature little league player to the skeletally mature high school/college athlete.

Complete sternal cleft associated with right clavicular, manubrial, and thyroid hypoplasia, pectus deformity, and spinal anomalies.

Sternal cleft anomalies are rare. Associated anomalies include pentalogy of Cantrell and posterior fossa abnormalities, hemangiomas, arteriopathy, cardiac anomalies, eye abnormalities, and sternal defects syndrome. There is only a single report of complete sternal cleft, pectus excavatum, and right clavicular hypoplasia in an adult. Thyroid hemiagenesis is also very rare. To our knowledge, this is the first case of complete sternal cleft, pectus deformity, and right clavicular hypoplasia in a child and the first case with right thyroid hemiagenesis.

Secondary signs may improve the diagnostic accuracy of equivocal ultrasounds for suspected appendicitis in children.

INTRODUCTION: Ultrasound (US) is the preferred imaging modality for evaluating appendicitis. Our purpose was to determine if including secondary signs (SS) improve diagnostic accuracy in equivocal US studies. METHODS: Retrospective review identified 825 children presenting with concern for appendicitis and with a right lower quadrant (RLQ) US. Regression models identified which SS were associated with appendicitis. Test characteristics were demonstrated. RESULTS: 530 patients (64%) had equivocal US reports. Of 114 (22%) patients with equivocal US undergoing CT, those with SS were more likely to have appendicitis (48.6% vs 14.6%, p<0.001). Of 172 (32%) patients with equivocal US admitted for observation, those with SS were more likely to have appendicitis (61.0% vs 33.6%, p<0.001). SS associated with appendicitis included fluid collection (adjusted odds ratio (OR) 13.3, 95% confidence interval (CI) 2.1-82.8), hyperemia (OR=2.0, 95%CI 1.5-95.5), free fluid (OR=9.8, 95%CI 3.8-25.4), and appendicolith (OR=7.9, 95%CI 1.7-37.2). Wall thickness, bowel peristalsis, and echogenic fat were not associated with appendicitis. Equivocal US that included hyperemia, a fluid collection, or an appendicolith had 96% specificity and 88% accuracy. CONCLUSION: Use of SS in RLQ US assists in the diagnostic accuracy of appendicitis. SS may guide clinicians and reduce unnecessary CT and admissions.

Gender-based differences in pediatric nuclear medicine.

Gender-based differences commonly encountered in pediatric nuclear medicine reflect both basic embryologic differences of the sexes, which are evident from infancy, and evolving physiological changes due to gender, which occur as the pediatric patient grows, undergoes puberty, and matures to adulthood. It is important for a nuclear medicine physician or radiologist to know both the gender and the age of a patient when interpreting her or his studies. It is also important that the reading physician be familiar with the normally evolving physiological changes that are specific for that patient's stage of development. It is particularly important that the reading physician consider such changes when comparing serial studies of the patient that are acquired during the patient's transitions through her or his different significant stages of development. Many pediatric nuclear medicine imaging protocols are modifications or adaptations of the protocols for adult imaging. Physicians reading pediatric studies must routinely incorporate knowledge on age and gender that is relevant to the patient for any given study. The age-defined gender-based subtleties of potential findings in pediatric nuclear medicine studies are often underrecognized. However, they are often of interest and at times important in the workup of both benign entities and pathologic processes of the pediatric patient.

Treatment of severe thrombocytopenia with IL-11 in children with Wiskott-Aldrich syndrome.

This report examines the safety and efficacy of IL-11 in treating severe thrombocytopenia secondary to Wiskott-Aldrich syndrome in two pediatric patients before allogeneic stem cell transplantation (SCT). Both patients had a substantial increase in their platelet counts and a decrease in bleeding episodes and platelet transfusions. The median platelet count increased from 32,000/mm3 to 64,000/mm3. Each subsequently received allogeneic SCT; 1 year after transplantation, both are reconstituted with 100% donor hematopoietic stem cells with sustained normal platelet counts (>200 K/mm3). Larger studies are required to confirm this observation of the safety and efficacy of IL-11 in this setting.