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The omphalomesenteric duct is an embryologic structure that connects the yolk sac with the primitive midgut of the developing fetus. Omphalomesenteric duct anomalies include a group of entities that result from failed resorption of the omphalomesenteric duct. These anomalies include Meckel diverticulum, omphalomesenteric fistula, fibrous bands, cysts, and umbilical polyps. Meckel diverticulum is the most common congenital anomaly of the gastrointestinal tract and is usually asymptomatic. Symptoms develop when Meckel diverticulum involves complications such as hemorrhage, inflammation, and perforation, or when it causes intussusception or bowel obstruction. Hemorrhage is the most common complication of Meckel diverticulum, and technetium 99m-pertechnetate scintigraphy is the imaging modality of choice for detecting acute bleeding. US and CT are commonly used for the evaluation of patients with other complications such as obstruction and inflammation. Nevertheless, the diagnosis of these complications can be challenging, as their clinical manifestations are usually nonspecific and can masquerade as other acute intraabdominal entities such as appendicitis, inflammatory bowel disease, or other causes of bowel obstruction. There are other umbilical disorders, such as urachal remnants and umbilical granuloma, that may present with symptoms and imaging findings similar to those of omphalomesenteric duct anomalies. An accurate preoperative diagnosis of omphalomesenteric duct anomaly is crucial for appropriate management and a better outcome, particularly when these anomalies manifest as a life-threatening condition. The authors review the anatomy, clinical features, and complications of omphalomesenteric duct anomalies in children, describing the relevant differential diagnoses and associated imaging findings seen with different imaging modalities. ©RSNA, 2021.

Study Objective To evaluate the diagnostic performance of a Volume and Solid Vascular Tissue Score (VSVTS) for preoperative risk assessment of pediatric and adolescent adnexal masses. Design A retrospective cohort study comprised of all female individuals who presented with an adnexal mass that was managed surgically between April 2011 and March 2016. Setting The Hospital for Sick Children (Toronto, Ontario, Canada). Participants Female individuals 1–18 years of age who presented to a large tertiary pediatric hospital with an adnexal mass that was managed surgically. Main Outcome Measures Main outcome measures included diagnostic performance of the VSVTS for malignancy via sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (LR+), negative likelihood ratio (LR−), and receiver operating characteristic area-under-the-curve (AUC) analysis. Results A total of 179 masses in 169 subjects were included. The malignancy rate was 10.6%. The AUC for the VSTVS was 0.919. A VSTVS cut-off value of 4 achieved a sensitivity of 79% (95% CI 0.54-0.93), specificity of 88% (95% CI 0.82-0.93), PPV of 0.44 (95% CI 0.33-0.56), NPV of 0.97 (95% CI 0.94-0.99), LR+ of 6.77 (95% CI 4.18-10.97), and LR− of 0.24 (95% CI 0.10-0.57). Conclusions A sonographic scoring system based on the volume and presence of solid vascular tissue improves PPV for preoperative risk stratification of adnexal masses in the pediatric and adolescent population compared to existing ultrasound-only approaches. Further prospective research is needed to determine how best to incorporate components of such scoring systems into clinical management algorithms.

Methods to identify children with cystic fibrosis (CF) at risk for development of advanced liver disease are lacking. We aim to determine the association between liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) with research ultrasound (US) patterns and conventional hepatic markers as a potential means to follow liver disease progression in children with CF. ELASTIC (Longitudinal Assessment of Transient Elastography in CF) is a nested cohort of 141 patients, ages 7-21, enrolled in the Prediction by US of Risk of Hepatic Cirrhosis in CF (PUSH) Study. We studied the association between LSM with research-grade US patterns (normal [NL], heterogeneous [HTG], homogeneous [HMG], or nodular [NOD]) and conventional hepatic markers. In a subgroup (n = 79), the association between controlled attenuation parameter (CAP) and US pattern was explored. Among 133 subjects undergoing VCTE, NOD participants (n = 26) had a significantly higher median (interquartile range) LSM of 9.1 kPa (6.3, 15.8) versus NL (n = 72, 5.1 kPa [4.2, 7.0]; P

Hepatocellular carcinoma (HCC) is rare in children and there is limited data on its imaging features. To describe imaging features of pediatric HCC and correlate them with clinical and laboratory findings. We retrospectively reviewed imaging in all pediatric HCC cases seen between January 2000 and January 2019. Imaging features defined in LI-RADS (Liver Imaging Reporting and Data System) and tumor extent by PRETEXT (pretreatment extent of disease) criteria were noted by two radiologists. Patient charts were reviewed to collect clinical features, alpha-fetoprotein (AFP) level and pathology findings. Of the 15 children (7 boys, 8 girls; mean age: 11.8 years, age range: 6–17 years) included in the study, 12/15 had computed tomography, 9/15 had magnetic resonance imaging and 9/15 had ultrasound exams available for review. Pathological types of HCC included classic (11/15, 73%), fibrolamellar (3/15, 20%) and mixed cholangiocarcinoma-HCC (1/15, 7%). Eighty percent occurred de novo in normal liver and 67% showed elevated AFP levels. Arterial phase hyperenhancement was seen in 83% of cases, washout in 86%, capsule in 50% and tumor-in-vein in 33%. The mean tumor size was 9.8 cm and 40% were multifocal on imaging. Staging revealed PRETEXT II tumors in 47%, III in 20% and IV in 33%. There were no PRETEXT I tumors. The two most common PRETEXT annotation factors were portal vein and caudate lobe involvement in 71% and 43% of cases, respectively. Fibrolamellar HCC demonstrated central scar, normal AFP levels and normal background liver. Pediatric HCC are large heterogeneous tumors, as reflected by high PRETEXT staging, and commonly include portal vein and caudate involvement. This affects resectability of these tumors at presentation. Central scar, normal AFP level and normal liver background may help differentiate fibrolamellar HCC from other types of HCC.

Abdominal US is emerging as an alternative to the upper gastrointestinal (GI) series as the preferred diagnostic imaging test for midgut malrotation complicated by volvulus. Unlike the upper GI series, US is free from ionizing radiation, does not require oral contrast agent, and can be performed portably and at times remotely from the interpreting radiologist, expediting diagnosis. Although some institutions do not have a standardized US protocol for midgut volvulus, many routinely use US in the setting of an infant or child with acute abdominal signs or symptoms to evaluate for common conditions such as hypertrophic pyloric stenosis, intussusception, necrotizing enterocolitis and appendicitis. Because these common conditions can overlap in age and clinical presentation with midgut volvulus, the aim of this pictorial essay is to provide instruction on the technique and diagnostic findings of midgut volvulus on US to ensure confident, accurate interpretation, and prompt treatment.

The clinical and plain radiographic differentiation of congenital intrinsic duodenal anomalies (atresia, web, stenosis) from intestinal malrotation is not always clear. Although sonography has been documented as an important diagnostic tool in the differentiation of these two entities, its role is still not widely appreciated and it is still not universally utilized in this clinical setting. To assess the usefulness of sonographic features of the duodenal and gastric wall in the differentiation of congenital intrinsic duodenal anomalies from midgut malrotation in a large series of neonates and to compare them with other features on abdominal radiographs, ultrasound and upper gastrointestinal series. Using the surgical database at our tertiary pediatric hospital, we identified neonates who had surgically proven congenital intrinsic duodenal anomalies or malrotation over a period of 15 years (2000–2015). We reviewed imaging findings in both groups of neonates (blinded to the final diagnosis) with attention to the echogenicity and thickness of the wall of the duodenum and stomach, the relationship between the superior mesenteric artery and vein, the position of the third portion of the duodenum and the presence of the whirlpool sign. Findings were compared between the groups using the unpaired t-test and Fisher exact test. We included 107 neonates in the study, 40 with a congenital intrinsic duodenal anomaly, 49 with malrotation (36 with volvulus) and 18 with a combination of both. Duodenal and gastric wall thickening and hyperechogenicity were significantly more common in the group with a congenital intrinsic duodenal anomaly compared to those with malrotation (P

There is a broad spectrum of soft-tissue masses in children that can be challenging to diagnose clinically and on imaging. This article reviews the typical clinical and imaging findings of the most common and relevant benign, intermediate and malignant pediatric soft-tissue tumors in the following categories of the 2013 World Health Organization (WHO) classification: adipocytic tumors (lipoma, lipoblastoma, and liposarcoma), fibroblastic/myofibroblastic tumors (nodular fasciitis, myositis ossificans, fibrous hamartoma of infancy, fibromatosis colli, desmoid-type fibromatosis, lipofibromatosis, and infantile fibrosarcoma), pericytic tumors (myofibroma/myofibromatosis), skeletal muscle tumor (rhabdomyosarcoma), nerve sheath tumors (neurofibroma, malignant peripheral nerve sheath tumor), and uncertain differentiation (synovial sarcoma). In general, ultrasound and magnetic resonance imaging are used as first- and second-line imaging modalities, with limited roles for plain radiographs, computed tomography, and fluorodeoxyglucose-positron emission tomography. Many of these tumors have nonspecific imaging findings although there are some key imaging clues that in conjunction with the clinical information allow a specific diagnosis or a narrow differential diagnosis. However, in many instances, histology is required for final diagnosis.

This article discusses the role of imaging of the thyroid gland in children, highlighting pearls and pitfalls. In the pediatric age group, thyroid imaging is mainly performed for the evaluation of congenital hypothyroidism, diffuse thyroid enlargement, and thyroid nodules. Permanent congenital hypothyroidism is most often caused by thyroid dysgenesis. Diffuse thyroid enlargement is mainly seen in teenage girls, and most often caused by Hashimoto disease, nodular hyperplasia, Graves disease, or diffuse papillary carcinoma. Thyroid nodules are less common in children than they are in adults, but more likely to be malignant; TI-RADS criteria may underestimate the risk of malignancy in children, and lead to erroneous classification downgrading. Knowledge of unique features of thyroid imaging in the pediatric population is critical for accurate diagnosis and management recommendations.

Soft-tissue sarcomas in children comprise a heterogeneous group of entities with variable manifestation depending on the age of the patient and the location of the tumor. MRI is the modality of choice for evaluating musculoskeletal soft-tissue tumors and plays a paramount role in both initial diagnosis and assessment of tumor response during and after treatment. Conventional MRI sequences, such as T1- and T2-weighted imaging, offer morphologic information, which is important for localizing the lesion and describing anatomic relationships but not accurate for determining its malignant or benign nature and may be limited in differentiating tumor response from therapy-related changes. Advanced multiparametric MRI offers further functional information that can help with these tasks by using different imaging sequences and biomarkers. The authors present the role of MRI in rhabdomyosarcoma and other soft-tissue sarcomas in children, emphasizing a multiparametric approach with focus on the utility and potential added value of diffusion-weighted imaging (DWI) and dynamic contrast-enhanced MRI in characterization and staging, determination of pretreatment extent, and evaluation of tumor response and recurrence after treatment. ©RSNA, 2020.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign neoplasm of neural crest origin more commonly seen in the craniofacial region. We report a case of MNTI of the epididymis in a 6-month-old male child with emphasis on the sonographic appearance which has not been previously described. In this case, the mass was inseparable from the testicle and therefore the differential diagnosis considered both extratesticular and intratesticular masses. MNTI should be added to the differential diagnosis of scrotal masses, particularly if they present in a child younger than 12 months of age.

Purpose To retrospectively define the strength of association between testicular microlithiasis and testicular neoplasia in a large geographically diverse pediatric population. Materials and Methods Retrospective review of scrotal ultrasonographic (US) examination reports and pathology specimens obtained between January 2000 and May 2014 at six academic pediatric hospitals in North America was performed. Reported cases were reviewed to confirm microlithiasis. Radiology and pathology data bases were searched for pathology-proven testicular tumors (benign or malignant germ cell or stromal tumors). Association strength (risk) was expressed in terms of odds ratios (ORs) with and without adjustment for fixed study site effects based on logistic regression. Results A total of 37 863 individuals underwent scrotal US during the study period. Mean age was 11.1 years ± 4.7 [standard deviation] in boys with microlithiasis and 9.1 years ± 5.9 in boys without microlithiasis (P < .001). Microlithiasis was confirmed in 2.90% of patients (1097 of 37 863; range, 1.61%-5.25% across sites). It was unilateral in 21.97% (241 of 1097) of patients and bilateral in 78.0% (856 of 1097). Tumor was identified in 4.64% (51 of 1097) of boys with microlithiasis and 0.33% (122 of 36 766) of boys without (unadjusted OR, 14.65; 95% confidence interval [CI]: 10.29, 20.84; adjusted OR, 14.19). Malignant germ cell tumors were identified in 2.8% (31 of 1097) of boys with microlithiasis and 0.12% (45 of 36 766) of boys without microlithiasis (unadjusted OR, 17.26; 95% CI: 11.8, 25.25; adjusted OR, 22.37). Sex cord-stromal tumors were identified in 0.46% (five of 1097) of boys with microlithiasis and 0.079% (29 of 36 766) of boys without (unadjusted OR, 5.8; 95% CI: 2.1, 16; adjusted OR, 6.39). Conclusion There is a strong association between testicular microlithiasis and primary testicular neoplasia in this pediatric population. © RSNA, 2017.

Tumors of the foot are rare in children. In this review the authors illustrate radiographic, sonographic and MR imaging findings of foot soft-tissue tumors in children based on all cases presenting at a tertiary pediatric hospital during the 15-year period of 1999-2014. Among these cases there were 155 tumors of the foot - 72 of the bones and 83 of the soft tissues. Vascular malformations, fibromatosis and sarcomas were respectively the most frequent benign, intermediate and malignant soft-tissue tumors. Some tumors showed specific imaging findings. In imaging investigations, ultrasound can be used as the first imaging modality for diagnostic workup of most lesions because it is noninvasive, low-cost and readily available, and can confirm the presence of the mass and evaluate cystic components, especially in young children who would otherwise require sedation for MR imaging. MR imaging is the reference standard technique because of its high tissue contrast, which allows for detection and characterization of soft-tissue and bone abnormalities. MR imaging is useful as the first imaging modality in select cases, including those with high suspicion of malignancy, very large lesions or pre-treatment lesions. Recognition of some typical imaging findings in pediatric soft-tissue foot tumors is helpful to establish diagnosis and facilitate patient management.

Sonography can be used in the management of pediatric soft-tissue vascular anomalies for diagnosing, for assessing lesion extent and for evaluating complications and response to therapy. The sonographic technique includes a combination of gray-scale imaging with color and spectral Doppler techniques. However the interpretation of the sonographic findings requires correlation with the clinical findings, some of which can be easily obtained at the time of scanning. This has to be combined with the use of appropriate nomenclature and the most updated classification in order to categorize these children into the appropriate management pathway. In this article, which is part 1 of a two-part series, the authors review the current classification of vascular anomalies, provide a clinical and a sonographic approach to these lesions, and review the most relevant clinical and sonographic features of vascular tumors including infantile and congenital hemangiomas, tufted angioma, kaposiform hemangioendothelioma, pyogenic granuloma, intramuscular capillary-type hemangioma and angiosarcoma.

Vascular malformations are a heterogeneous group of entities, many of which present in the pediatric age group. Sonography plays a major role in the management of children with these vascular anomalies by providing information that helps in diagnosing them, in assessing lesion extent and complications, and in monitoring response to therapy. The interpretation of sonographic findings requires correlation with clinical findings, some of which can be easily obtained at the time of scanning. This has to be combined with the use of appropriate nomenclature and the most updated classification in order to categorize these patients into the appropriate management pathway. Some vascular malformations are part of combined vascular anomalies or are associated with syndromes that include other disorders, frequently limb overgrowth, and these are now being reclassified based on their underlying genetic mutation. Sonography has limitations in the evaluation of some vascular malformations and in these cases MR imaging might be considered the imaging modality of choice, particularly for lesions that are large, that involve multiple compartments or are associated with other soft-tissue and bone abnormalities. In this article, which is part 2 of a two-part series, the authors review the most relevant clinical and sonographic features of arteriovenous, capillary, venous and lymphatic malformations as well as vascular malformations that are part of more complex conditions or associated with syndromes, including Parkes-Weber syndrome, phosphatase and tensin homologue (PTEN) hamartoma tumor syndromes, Klippel-Trénaunay syndrome, CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi and skeletal anomalies) syndrome, fibro-adipose vascular anomaly and Proteus syndrome.

MR imaging is used to assess iron overload in patients with hemoglobinopathies and in those who have undergone multiple blood transfusions. Sometimes splenic nodules are found incidentally on these examinations and this may cause diagnostic uncertainty. To determine the prevalence, imaging characteristics and evolution of splenic nodules found on MR imaging for iron overload evaluation. Retrospective review of all MR imaging examinations performed for iron overload assessment from 2005 to 2015 in a tertiary pediatric hospital. The presence of focal splenic nodules including number, size, signal characteristics and changes on follow-up MR imaging were recorded. Relevant patient clinical information including underlying hematological disease was also documented. A total of 318 patients had MR imaging for iron overload assessment. Of these, 25 (8%) had at least one incidental splenic nodule. Sickle cell disease was present in 22 patients (88%) and thalassemia in 3 (12%). On intermediate-weighted spin-echo images, the nodules had high signal intensity compared to the remainder of the spleen in 23 patients (92%) and low signal intensity in the remaining 2 (8%). In all patients (100%) the nodules showed progressive loss of signal intensity with increasing echo time values. Follow-up MR imaging was performed in 20 (80%) patients, which showed an increase in the size of the splenic nodules in 7 patients (35%) stability in 11 (55%) and a decrease in size in 2 (10%). It is not uncommon to find splenic nodules during MR evaluation of iron overload. In patients with sickle cell disease, most of these nodules are thought to represent preserved splenic tissue and appear hyperintense compared to the remainder of the spleen. They frequently remain stable on follow-up imaging, although about a third of them may show growth. Awareness of these nodules is important to avoid concern for potential malignancy and unnecessary investigations.

To assess if a heterogeneous pattern on research liver ultrasound examination can identify children at risk for advanced cystic fibrosis (CF) liver disease.Planned 4-year interim analysis of a 9-year multicenter, case-controlled cohort study (Prospective Study of Ultrasound to Predict Hepatic Cirrhosis in CF). Children with pancreatic insufficient CF aged 3-12 years without known cirrhosis, Burkholderia species infection, or short bowel syndrome underwent a screening research ultrasound examination. Participants with a heterogeneous liver ultrasound pattern were matched (by age, Pseudomonas infection status, and center) 1:2 with participants with a normal pattern. Clinical status and laboratory data were obtained annually and research ultrasound examinations biannually. The primary end point was the development of a nodular research ultrasound pattern, a surrogate for advanced CF liver disease.There were 722 participants who underwent screening research ultrasound examination, of which 65 were heterogeneous liver ultrasound pattern and 592 normal liver ultrasound pattern. The final cohort included 55 participants with a heterogeneous liver ultrasound pattern and 116 participants with a normal liver ultrasound pattern. All participants with at least 1 follow-up research ultrasound were included. There were no differences in age or sex between groups at entry. Alanine aminotransferase (42 ± 22 U/L vs 32 ± 19 U/L; P = .0033), gamma glutamyl transpeptidase (36 ± 34 U/L vs 15 ± 8 U/L; P .001), and aspartate aminotransferase to platelet ratio index (0.7 ± 0.5 vs 0.4 ± 0.2; P .0001) were higher in participants with a heterogeneous liver ultrasound pattern compared with participants with a normal liver ultrasound pattern. Participants with a heterogeneous liver ultrasound pattern had a 9.1-fold increased incidence (95% CI, 2.7-30.8; P = .0004) of nodular pattern vs a normal liver ultrasound pattern (23% in heterogeneous liver ultrasound pattern vs 2.6% in normal liver ultrasound pattern).Research liver ultrasound examinations can identify children with CF at increased risk for developing advanced CF liver disease.

An earlier incorrect version of this article appeared online. This article was corrected on December 17, 2019.

Objectives Early identification of children with cystic fibrosis (CF) at risk for severe liver disease (CFLD) would enable targeted study of preventative therapies. There is no gold standard test for CFLD. Ultrasonography (US) is used to identify CFLD, but with concerns for its diagnostic accuracy. We aim to determine if differences in standard blood tests, imaging variables and noninvasive liver fibrosis indices correlate with liver US patterns, and thus provide supportive evidence that a heterogeneous US liver pattern reflects clinically relevant liver disease. Methods We studied baseline research abdominal US and bloodwork from 244 children with pancreatic insufficient CF, ages 3 to 12 years, enrolled in a prospective study of the ability of US to predict CF cirrhosis (PUSH study). Children with a heterogeneous (HTG) liver pattern on US (n = 62) were matched 1 : 2 in design with children with normal US (NL, n = 122). Analyses included children with nodular (NOD, n = 22) and homogeneous hyperechoic (HMG, n = 38) livers. Results Univariate analysis showed significant differences between US groups for standard blood tests, spleen size, and noninvasive liver fibrosis indices. Multivariable models discriminated NOD versus NL with excellent accuracy (AUROC 0.96). Models also distinguish HTG versus NL (AUROC 0.76), NOD versus HTG (0.78), and HMG versus NL (0.79). Conclusions Liver US patterns in children with CF correlate with platelet count, spleen size and indices of liver fibrosis. Multivariable models of these biomarkers have excellent discriminating ability for NL versus NOD, and good ability to distinguish other US patterns, suggesting that US patterns correlate with clinically relevant liver disease.

Autoinflammatory diseases constitute a family of disorders defined by aberrant stimulation of inflammatory pathways without involving antigen-directed autoimmunity. They may be divided into monogenic and polygenic types. Monogenic autoinflammatory syndromes are those with identified genetic mutations, such as familial Mediterranean fever, tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS), mevalonate kinase deficiency or hyperimmunoglobulin D syndrome, cryopyrin-associated periodic fever syndromes (CAPS), pyogenic arthritis pyoderma gangrenosum and acne (PAPA) syndrome, interleukin-10 and interleukin-10 receptor deficiencies, adenosine deaminase 2 deficiency and pediatric sarcoidosis. Those without an identified genetic mutation are known as polygenic and include systemic-onset juvenile idiopathic arthritis, idiopathic recurrent acute pericarditis, Behcet syndrome, chronic recurrent multifocal osteomyelitis and inflammatory bowel disease among others. Autoinflammatory disorders are defined by repeating episodes or persistent fever, rash, serositis, lymphadenopathy, arthritis and increased acute phase reactants, and thus may mimic infections clinically. Most monogenic autoinflammatory syndromes present in childhood. However, because of their infrequency, diverse and nonspecific presentation, and the relatively new genetic recognition, diagnosis is usually delayed. In this article, which is Part 1 of a two-part series, the authors update monogenic autoinflammatory diseases in children with special emphasis on imaging features that may help establish the correct diagnosis.

Study Objective To evaluate the diagnostic performance of the Decision Tree System (DTS) rules 2 and 3 for surgically managed adnexal masses in the North American population and to compare it to the risk stratification criteria used at The Hospital for Sick Children (≥8 cm and complex/solid). Design A retrospective cohort study of patients who presented with adnexal masses and were surgically treated between April 2011 and March 2016. Setting The Hospital for Sick Children (Toronto, Ontario, Canada). Participants Patients 1-18 years of age with adnexal masses who underwent surgical treatment. Main Outcome Measures Main outcome measures included diagnostic performance (preoperative sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) for malignancy) of the DTS and ≥8 cm and complex/solid criteria. Results The malignancy rate was 11.0%. The DTS had a sensitivity (95% CI) of 84% (79-90), specificity of 77% (71-83), PPV of 30% (17-42), and NPV of 98% (94-100). The ≥8 cm and complex/solid criteria had a sensitivity of 89% (85-94), specificity of 71% (64-77), PPV of 27% (16-38), and NPV of 98% (96-100). Conclusions Our study demonstrated that DTS rules 2 and 3 had similar diagnostic performance to the ≥8 cm and complex/solid criteria in the same population, with a very high NPV and a low PPV. Future prospective investigation should be conducted to further assess how DTS components can be incorporated into future algorithms for the management of adnexal masses in the pediatric population.

The diagnosis of soft-tissue masses in children can be difficult because of the frequently nonspecific clinical and imaging characteristics of these lesions. However key findings on imaging can aid in diagnosis. The identification of macroscopic fat within a soft-tissue mass narrows the differential diagnosis considerably and suggests a high likelihood of a benign etiology in children. Fat can be difficult to detect with sonography because of the variable appearance of fat using this modality. Fat is easier to recognize using MRI, particularly with the aid of fat-suppression techniques. Although a large portion of fat-containing masses in children are adipocytic tumors, a variety of other tumors and mass-like conditions that contain fat should be considered by the radiologist confronted with a fat-containing mass in a child. In this article we review the sonographic and MRI findings in the most relevant fat-containing soft-tissue masses in the pediatric age group, including adipocytic tumors (lipoma, angiolipoma, lipomatosis, lipoblastoma, lipomatosis of nerve, and liposarcoma); fibroblastic/myofibroblastic tumors (fibrous hamartoma of infancy and lipofibromatosis); vascular anomalies (involuting hemangioma, intramuscular capillary hemangioma, phosphate and tensin homologue (PTEN) hamartoma of soft tissue, fibro-adipose vascular anomaly), and other miscellaneous entities, such as fat necrosis and epigastric hernia.

Systemic connective tissue disorders are characterized by the presence of autoantibodies and multiorgan system involvement. Juvenile systemic lupus erythematosus with or without associated antiphospholipid syndrome; juvenile dermatomyositis; sclerodermiform syndromes, including systemic and localized sclerodermas and eosinophilic fasciitis; mixed connective tissue disease; and Sjogren syndrome are the disorders that affect children most frequently. Diagnosis is difficult, because the clinical presentation of patients is diverse, from mild to severe disease. In addition, all organs may be affected. However, a variety of imaging techniques are now available to investigate rheumatic disease in children. These imaging modalities offer the potential for earlier diagnosis and improved assessment of therapeutic response. This article reviews the main connective tissue disorders that affect children, highlighting their key imaging features on images acquired with different diagnostic imaging modalities and correlating these features with clinical and pathologic findings, when available. ©RSNA, 2019.

Angiomatoid fibrous histiocytoma is a rare soft-tissue tumor that more often affects children and young adults. There is little information available regarding the imaging appearance of angiomatoid fibrous histiocytoma in children.To describe the ultrasonographic (US) and magnetic resonance (MR) imaging findings of angiomatoid fibrous histiocytoma in children.A retrospective analysis was done of US and MR imaging findings in children with angiomatoid fibrous histiocytoma. Clinical findings and histopathology with molecular analysis results were also collected.There were 7 children with angiomatoid fibrous histiocytoma with a median age of 6 years (age range: 16 months-14 years). Patients presented clinically with a soft-tissue mass in the extremities or in the trunk. Four children had anemia, and three of them had additional systemic symptoms. Two patients had US and three had MR imaging while the remaining two had both. Lesion size ranged from 1.3 cm to 7.2 cm. In four patients, angiomatoid fibrous histiocytoma presented as a nonspecific predominantly solid mass. The other three patients had a combination of the following imaging findings: intralesional blood-filled cystic spaces with fluid-fluid levels, enhancing fibrous pseudocapsule and hemosiderin deposition. These findings correlated well with histopathology.The imaging detection of intralesional blood-filled cystic spaces with fluid-fluid levels, enhancing fibrous pseudocapsule and hemosiderin deposition in a soft-tissue tumor in a child may suggest the diagnosis of angiomatoid fibrous histiocytoma. A history of systemic symptoms and anemia in the presence of a soft-tissue mass may also be a clue for the diagnosis of angiomatoid fibrous histiocytoma.

No study reported in the literature comprehensively compares findings on neonatal abdominal radiographs with sonography. To compare the findings on abdominal radiographs and sonograms in infants in intensive care, to better understand how the various intestinal gas patterns on radiographs relate to the spectrum of appearances on sonography and, second, to evaluate the ability of sonography to differentiate necrotizing enterocolitis from other intestinal pathology. We prospectively evaluated sonograms and radiographs, blinded to the other modality and to clinical information. Patients’ charts were reviewed by a third blinded reader and used as a reference standard for diagnosis. We made associations between sonographic findings, radiographic intestinal gas patterns and clinical diagnoses. We included 75 infants with gestational ages between 23 weeks and 41 weeks. Sonographic abnormalities were present in infants with all radiographic intestinal gas patterns, including normal patterns. We only saw absent intestinal perfusion and fluid collections on sonography (suggesting intestinal necrosis and sealed perforation) in infants with intestinal dilatation with elongation on radiographs. Separation of intestinal loops on radiographs was most commonly caused by reasons other than intestinal wall thickening. Increased intestinal echogenicity or free fluid with echoes on sonography correlated with a diagnosis of necrotizing enterocolitis, whereas anechoic free fluid correlated with absence of necrotizing enterocolitis. Sonography is complementary to radiographs in infants with suspected intestinal pathology, with a spectrum of appearances seen on each modality. Some sonographic findings either strongly suggest necrotizing enterocolitis or supply evidence against this diagnosis.

The inguinal canal is often seen at the edge of the field of view on plain radiography, computed tomography, or magnetic resonance imaging and may often not be scanned when performing sonography of the scrotum or abdomen. As a result, pathology in this anatomical region may be easily overlooked. The peculiar embryology of the inguinal canal makes the identification of pathology in the inguinal region significant, as some of the processes that take place within the scrotum may originate in the abdomen, and vice versa. This article reviews the relevant embryology of the inguinal canal, discusses abdominal and scrotal conditions that involve the inguinal region, and illustrates associated pathology.

Pancreatic tumors are rare in children, resulting in limited information regarding their frequency and imaging appearances. To review all pancreatic tumors seen in children over a decade at a large tertiary pediatric institution and to describe multimodality imaging findings. We conducted a retrospective review of imaging studies performed in children with pancreatic tumors between January 2000 and December 2012, analyzing imaging features on available US, CT and MR examinations. We reviewed patient charts for clinical features, management and final diagnosis. We included 23 children in this study. Of these, 12 had solid and papillary epithelial neoplasms (SPEN), 3 had neuroendocrine tumors, 3 had lymphoma, and 1 each had hemangioendothelioma, benign congenital cysts with adipose tissue, dendritic cell sarcoma, metastatic rhabdomyosarcoma, and lipoma. All children with SPEN were teenage girls. SPEN showed characteristic imaging features including well-defined margins with surrounding capsule, solid-cystic components and hemorrhage. Pancreatic tumors are uncommon in children. SPEN is the most common tumor and is seen predominantly in teenage girls and shows characteristic imaging features.

In some series of malrotation small numbers of children are described in whom the position of the duodenojejunal flexure was considered to be normal on straight anteroposterior (AP) view of an upper gastrointestinal (UGI) series.The purpose of this study was to illustrate children with disorders of midgut rotation in whom the diagnosis was difficult because on the straight AP view of the UGI series the duodenojejunal flexure was either not clearly depicted or was projected to the left of the midline close to its expected normal position at or close to the level of the duodenal cap.We reviewed 111 children with malrotation to determine the frequency that duodenojejunal flexure was not clearly depicted or was close to normal position.Seven patients had close to normal position of duodenojejunal flexure on AP view. The correct diagnosis was made on initial UGI series in four patients based on other features on AP and lateral views. In two of the other three patients, a repeat UGI series facilitated the correct diagnosis. In the final patient, an abnormal position of a nasojejunal tube suggested the correct diagnosis.Accurate diagnosis of anomalies of midgut rotation requires careful assessment of the entire duodenal sweep on both AP and lateral views to avoid false-negative interpretations.

Zebra spleen is the normal pattern of splenic enhancement during the arterial phase of CT and MRI and is attributed to different flow rates. The purpose of this study was to describe the appearance and occurrence of bands of hypoechogenicity in the spleen on unenhanced sonograms of children with no splenic abnormalities.We reviewed 100 abdominal ultrasound studies to evaluate the ultrasound characteristics of the spleen. Demographic data were collected for all patients.Homogeneous echogenicity of the spleen was found in 92 children. Heterogeneous echogenicity was present in eight. Three of the eight had discrete macronodules due to known splenic disease. The other five had bands of hypoechogenicity. These five had no known splenic disease, but one had mild splenomegaly of unknown cause.The pattern of hypoechoic bands occurred in 5% of our series. This pattern cannot be explained simply by different flow rates and probably reflects different structural components of the parenchyma. At ultrasound this pattern should be considered a normal finding that may simulate a splenic mass.

Purpose To determine interobserver agreement (IOA) and accuracy of conventional MR enterography (MRE), qualitative diffusion, and apparent diffusion coefficient (ADC) values for detecting clinically active inflammation. Methods MREs in 57 consecutive children with suspected inflammatory bowel disease were retrospectively reviewed. Results Substantial IOA for conventional MRE (kappa=0.65) and qualitative diffusion (kappa=0.64), but fair to good IOA for ADC, (intra-class coefficient=0.63) were seen. Conventional MRE detected active clinical inflammation well (area under curve [AUC] 0.725), while qualitative diffusion and ADC did not perform well (AUC=0.572 and 0.461, respectively). Conclusion DWI can be helpful in diagnosing inflammatory bowel disease but does not perform well in identifying those with active inflammation.

The diagnosis of soft tissue masses in children can be challenging because there is a broad spectrum of entities that can present as such. Ultrasonography is the primary diagnostic imaging modality in the pediatric age and is especially useful for small and superficial lesions. MR imaging can be helpful, particularly in the evaluation of large and deep soft tissue lesions. Correlation of the imaging findings with the clinical information is crucial in the diagnostic work-up. This article reviews the most common causes of soft tissue masses that require imaging in children.

Background Infants with stage 4 and 4S neuroblastoma (NB) have a superior prognosis to older children. However, they often require intensive therapy including abdominal radiation. We aimed to investigate the long-term hepatic outcomes in infants with stage 4S and 4 NB. Methods We reviewed the charts of 38 infants diagnosed with stage 4S and 4 NB between 1984 and 2002. We included only those with available follow-up 5 years following diagnosis. We assessed hepatic imaging and function (transaminases, bilirubin, alkaline phosphatase) at all available time points from diagnosis. Abnormalities present at more than 5 years from diagnosis were considered persistent late changes. Results We identified 15 stage 4S and 12 stage 4 patients. Twelve of 15 stage 4S patients had hepatic involvement at diagnosis, 8 of whom required abdominal radiation. Five of eight demonstrated late imaging changes. Two of four with hepatic metastases but no radiation demonstrated late imaging changes. The late imaging changes resolved over time and without intervention in 3/7 survivors. The persistent lesions included liver fibrosis (1) and focal nodular hyperplasia (FNH) (3). Five of 12 stage 4 NB patients had hepatic involvement at diagnosis; none required radiation or had late hepatic imaging changes. Conclusions In stage 4S NB, adverse hepatic effects are infrequent, may resolve over time, and occur with or without radiation. FNH should be considered in those with persistent late imaging changes. Adverse hepatic outcomes after liver involvement or radiation in infants with stage 4 NB rarely occur. Pediatr Blood Cancer 2012; 58: 283–288. © 2011 Wiley Periodicals, Inc.

In neonates and children, sonographic examinations of the renal pyramids may depict a spectrum of unique changes in echogenicity due to the effects of physiologic processes or a wide variety of pathologic processes that may affect the collecting ducts or interstitium of the pyramids. Focused sonographic evaluation of the pyramids with high-frequency transducers produces the most detailed images of the pyramids, revealing some appearances not previously reported, to the authors' knowledge. The authors highlight the clinical settings in which they have documented detailed changes in the echogenicity of the pyramids. The patterns of altered echogenicity alone may reflect a specific cause but in many instances are nonspecific, with clinical and biochemical correlation required to establish a more precise diagnosis. However, there is a lack of histologic data to completely explain the mechanism of many of these changes in echogenicity in all of the processes. As the authors have expanded their use of the focused sonographic technique, they have been able to depict altered echogenicity in the pyramids in greater numbers of children in whom an explanation for the changes is not always immediately apparent; for now, the cause must be considered idiopathic. More work is required to expand the use of this focused technique together with clinical, biochemical, and histologic correlation in an attempt to offer more complete explanations for the changes in echogenicity of the pyramids.

The relationship between sonographic features of renal venous thrombosis (RVT) and outcome has not been described in a large series of patients. To analyze sonographic findings of RVT and their evolution in a large series of patients and to attempt to identify features that might predict outcome. Retrospective analysis of sonograms and medical records of neonates and infants diagnosed with RVT during the period 1998-2007. Of 22 children (mean age: 3 days; age range: 0–107 days), RVT was bilateral in 12. Of 34 affected kidneys, thrombus in the main renal vein was seen in 17 and typical RVT sonographic findings without main renal vein thrombus were seen in the remaining 17. All children had US follow-up (range: 0.6–97.2 months). Three children with bilateral RVT died. Nine kidneys atrophied. Imaging findings associated with subsequent kidney atrophy included markedly reduced perfusion at diagnosis, subcapsular collections, patchy cortical echotexture and profoundly hypoechoic and irregular renal pyramids. Six patients (eight kidneys) presented with renal calcifications on initial sonogram before 7 days of life, suggesting antenatal RVT. Sonography is useful in neonatal and early infant RVT and might help predict renal atrophy. Antenatal RVT appears to be relatively common.

We report an unusual sonographic presentation of late-onset hemorrhagic cystitis caused by BK virus infection in an 8-year-old who had undergone hematopoietic stem cell transplantation for treatment of relapsed acute lymphoblastic leukemia. Sonography showed the presence of multiple bladder nodules in a background of diffuse bladder wall thickening. The dominant bladder nodule showed increase in size on serial sonograms and was vascularized on Doppler interrogation. Biopsy confirmed BK virus-associated cystitis. Recognition of this sonographic appearance in the appropriate clinical setting might obviate the need for biopsy in these children.

Background Pancreatic metastases in childhood cancer have been rarely reported in the radiology literature although ample evidence exists in pathology reports for its occurrence in patients with alveolar rhabdomyosarcomas (RMS). Objective Assess the occurrence of pancreatic metastases in alveolar rhabdomyosarcomas, increase awareness of this association and reassess current staging protocols. Materials and methods Three major oncology centers reviewed their records and imaging examinations. Patients’ history and demographics, primary tumor site and histology, presence of tumor recurrence, and presence and location of other metastases were reviewed. Results Pancreatic metastases occurred in eight patients with alveolar RMS. Four of these presented at diagnosis and four with disease recurrence. In recurrent disease, the duration between the diagnosis of the primary tumor and pancreatic metastases varied from 8 months to 6 years (mean ± SD: 2.38 ± 2.49 years). In all patients who received PET scans, pancreatic metastases showed a marked FDG-uptake, but had variable detectability with CT. Pancreatic metastases were not associated with certain primary tumor locations or presence of other metastases, mandating an evaluation of the pancreas in all cases of alveolar rhabdomyosarcomas. Conclusion Radiologists should be sensitized and actively evaluate the pancreas in patients with alveolar RMS. Optimizing CT and PET-CT protocols may increase the diagnostic yield.

Objective Germ cell tumours are the most common ovarian neoplasms in childhood and, of these, teratomas, whether mature or immature, are the most frequently found. Mature teratoma is a benign tumour, whereas the immature type, although also benign, has a more aggressive course, with a propensity to recurrence. A review of the literature revealed that there are some imaging features that may help to differentiate between these 2 types of teratoma, although no systematic comparison has been made. The objective of this study was to review imaging features of ovarian teratomas in children and to assess differentiating imaging features between the mature and immature types of ovarian teratoma. Methods Retrospective analysis of all patients who presented to our institution during a 9-year period (September 1999 to August 2008) with ovarian teratoma as confirmed on histology. Results Forty-one patients with pathologically proven ovarian teratoma were found. The patient ages ranged from 4–18 years at presentation (mean [standard deviation] age, 12.4 ± 3.4 years; median age, 13 years). Thirty patients (73.2%) were found to have mature ovarian teratoma, and 11 (26.8%) had immature teratoma. A component of endodermal sinus tumour was found in one of the immature teratomas. On ultrasonography, the appearance of the immature teratomas was purely solid in 3 (27.3%), mixed solid and cystic in 6 (54.5%), and predominantly cystic in 2 (18%). The mature ovarian teratomas demonstrated a predominantly cystic appearance in 22 (73.3%) and a mixed solid and cystic appearance in 8 (26.6%); there were no cases with a pure solid appearance. The prevalence of the more cystic appearance of the mature type showed significant statistical difference when compared with its prevalence in the immature type ( P = .0008, χ2 test). Other imaging features, such as size, presence of fat, or calcifications, did not show a significant difference between the 2 types of teratoma. Conclusions The predominance of a cystic component and a pure solid component in ovarian teratoma are significant differentiating factors between the mature type and the more aggressive immature type of teratoma.

Objective: To report a case of calcineurin-induced pain syndrome (CIPS) in a child undergoing his second hematopoietic stem cell transplant (HSCT). Case Summary: A 6.1-year-old child received cyclosporine and methotrexate for acute graft-versus-host disease (aGVHD) prophylaxis after his first HSCT for acute myeloblastic leukemia. Amlodipine was given for the treatment of hypertension. Symptoms of CIPS were not observed. After the second HSCT at the age of 6.7 years, the child received cyclosporine (target trough whole blood cyclosporine concentration range 150–200 μg/L). starting on day −3, and mycophenolate mofetil for aGVHD prophylaxis. With the first cyclosporine dose, the patient complained of leg pain that was most severe during the cyclosporine infusion. Analgesic agents and a change from intravenous to oral administration of cyclosporine were Ineffective In controlling the pain. Magnetic resonance imaging findings on day 10 showed periosteal soft tissue changes and mild bone marrow edema of the femora and tibiae. Tacrolimus was substituted for cyclosporine on day 20; on day 21 amlodipine was initiated to manage hypertension. Trough whole blood tacrolimus concentrations ranged from 1.7 to 6.2 μg/L. Pain was reduced in seventy by day 29 and completely resolved once tacrolimus was discontinued on day 42. In this case, CIPS was considered to be probably associated with cyclosporine according to the Naranjo probability scale. Discussion: CIPS is hypothesized to result from calcineurin-induced vascular changes that disturb bone perfusion and permeability, leading to intraosseous vasoconstriction and bone manow edema. In our patient, symptoms were most acute during the infusion, when whole blood cyclosporine concentrations were likely to be the highest. Our patient's symptoms were resolved when tacrolimus was substituted for cyclosporine and amlodipine was initiated. Conclusions: interventions aimed at reducing pain associated with CIPS may include the initiation of calcium-channel blocker therapy and conversion to an alternative calcineurin inhibitor.

Decreased perfusion and trauma during laparoscopic harvesting are proposed causative factors for DGF and rejection in children following renal transplantation with laparoscopic donor nephrectomy (LDN) allograft. We performed a retrospective review of 11 children who underwent LDN transplant and 11 preceding patients who underwent ODN transplant. Intraoperative DUS findings, creatinine values and clearance, time to nadir creatinine and AR episodes were compared. There were no significant differences in the BMI, vascular anatomy, side of nephrectomy, or warm ischemia time in the two groups. Mean follow-up duration was 11.4 and 30.4 months in LDN and ODN groups. DUS showed initial turbulent flow in five of the LDN and four of the ODN group with an average RI of 0.59 and 0.66 in the ODN and LDN groups, respectively (NS). Three patients in the ODN group had an abnormal RI compared to none in the LDN group (p = 0.034). The creatinine values, creatinine clearances (at 24 h, one, four wk and last follow-up) and AR episodes were similar in both groups. Pneumoperitoneum during LDN does not appear to have an adverse impact on early graft reperfusion.

Peritoneal malignancies are a rare occurrence in children, often metastatic and rarely originating primarily in the peritoneum. The imaging findings of these entities in the pediatric age have not been recently reviewed or they have been previously described or depicted mostly as part of articles discussing each entity separately. This is a review of the most relevant peritoneal malignancies in childhood emphasizing imaging features. Knowledge of these appearances may facilitate diagnosis and staging of these neoplasms.

Bone marrow transplantation is frequently performed to restore hematologic and immunologic competence after chemotherapy and radiation therapy for a range of childhood malignancies, as well as to treat various congenital conditions in which hematologic and immunologic functions are depressed or absent. Potentially devastating complications may occur during the pre-engraftment period after bone marrow transplantation, when marrow aplasia may supervene for several weeks until engraftment occurs, as well as during the post-engraftment period (the 3 months after engraftment) and in subsequent months and years. Complications of bone marrow transplantation may be classified either according to the time interval between transplantation and the occurrence of the complication or according to the organ system affected. The range of complications that may affect the central nervous system and the rest of the body may be detected with ultrasonography, computed tomography, and magnetic resonance imaging. Neurologic, paranasal sinus, pulmonary, and abdominopelvic complications all may be seen after bone marrow transplantation. Graft-versus-host disease and lymphoproliferative disorders also may occur. The increasing use of bone marrow transplantation mandates that the radiologist be familiar with the full range of potential complications and their imaging appearances.

The objective of this study was to assess the diagnostic agreement between bowel sonography and MR enterography in children with suspected or confirmed inflammatory bowel disease (IBD).We prospectively studied 33 children undergoing clinically indicated MR enterography. Bowel sonography was performed within 2 hours before MR enterography. The reliability of each modality was assessed by computing the interreader kappa agreement on nine inflammatory markers in eight bowel segments and the interreader intraclass correlation coefficients (ICC) in each bowel segment. The agreement between both modalities in each bowel segment was also calculated using the Kendall coefficient of concordance (KCC) in addition to the sensitivity and specificity of sonography using those of MR enterography as the reference standard.Sonographic readings are substantially reliable in the colon and terminal and distal ileum (ICC = 0.79-0.88) but are highly unreliable in the mid and proximal small bowel. MR enterography has moderate to almost perfect reliability for all bowel segments (ICC = 0.43-0.96). Sonography was in substantial agreement with MR enterography in most of the large bowel (KCC = 0.67-0.83) and terminal and distal ileum (KCC = 0.75) and in moderate agreement in the remaining bowel segments (KCC = 0.45-0.48). Sonographic findings are specific for disease using MR enterography as the reference standard in all bowel segments (0.83-1.00), but sensitivity markedly varies according to disease location.Sonography is highly specific and shows substantial agreement with MR enterography in detecting inflammatory and damage changes in the colon and terminal and distal ileum, the segments most likely to be affected by IBD. Therefore, sonography can be a good complement to MR enterography in evaluations of patients with known or suspected IBD, particularly if MR enterography access is limited.

Magnetic resonance (MR) imaging can be used in the management of pediatric soft-tissue vascular anomalies for diagnosing and assessing extent of lesions and for evaluating response to therapy. MR imaging studies often involve a combination of T1- and T2-weighted images in addition to MR angiography and fat-suppressed post-contrast sequences. The MR imaging features of these vascular anomalies when combined with clinical findings can aid in diagnosis. In cases of complex vascular malformations and syndromes associated with vascular anomalies, MR imaging can be used to evaluate accompanying soft-tissue and bone anomalies. This article reviews the MR imaging protocols and appearances of the most common pediatric soft-tissue vascular anomalies.

Pediatric thyroid nodules, while uncommon, have high malignancy risk. The objectives of the study were (1) to identify sonographic features predictive of malignancy; (2) to create a prediction model; and (3) to assess inter-observer agreement among radiologists.All available cases of thyroid nodules, surgically removed between 2000 and 2009. Three radiologists reviewed the sonographic images; 2 pathologists reviewed the tissue specimens. Adult prediction models were applied. Interobserver variability was assessed.Twenty-seven subjects, mean age 13.1±3.4 years, were included. Nineteen nodules were differentiated thyroid carcinomas. On multivariate analysis, size was the only significant predictor of malignancy. On recursive partitioning analysis, size >35 mm with microcalcification and ill-defined margins yielded the best prediction model. Radiologist inter-observer agreement regarding malignancy was moderate (κ=0.50).Larger size, microcalcifications and ill-defined margins on ultrasound demonstrate the best predictive model for malignancy in the pediatric population. Experienced pediatric radiologists demonstrate moderate inter-observer agreement in prediction of malignancy.

With most conditions that result in hyperechoic renal pyramids, the involvement is usually relatively homogeneous and symmetric, involving all pyramids and both kidneys. An exception to this pattern has been shown in furosemide-induced medullary nephrocalcinosis (MNC), in which the renal involvement may be asymmetric but rarely unilateral. 1 Cases of markedly asymmetric MNC with hyperechoic pyramids predominantly involving 1 kidney have been previously described in children with specific contralateral unilateral renal disease, such as renal vein thrombosis and hydronephrosis. 2 The purpose of this report is to illustrate the case of a girl with substantial unilateral renal artery stenosis who was found to have unilateral hyperechoic renal pyramids in the contralateral kidney, possibly due to MNC. To our knowledge, this finding in this clinical setting has not been previously reported in the literature.

About one-half of patients with meconium ileus (MI) present with a complication such as volvulus, atresia, meconium peritonitis or giant cystic meconium peritonitis. The treatment of these complications requires surgery. However, the preoperative diagnosis of complicated MI is difficult. We describe two neonates with complicated small-bowel obstruction, one with MI related to cystic fibrosis and the other not related to cystic fibrosis. In both, contrast enema depicted a spiral appearance of the distal small bowel, which at surgery proved to be the result of volvulus associated with antenatal bowel perforation. This appearance of the small bowel on contrast enema in this clinical setting has not been previously described. The recognition of this spiral appearance of the distal small bowel suggests the need for surgery.

The nonoperative management of intussusception continues to evolve and is the subject of ongoing debate. Our purpose was to assess our current enema reduction rate and to focus on two specific issues that have received little attention in the literature: first, the value and safety of using delayed, repeated reduction attempts and, second, the management of intussusceptions due to lead points.We performed a retrospective analysis of all intussusception cases seen at the Hospital for Sick Children, Toronto, Canada, a tertiary pediatric hospital, from May 1999 to December 2002.There were 163 children with a total of 219 intussusceptions. Enema reduction was attempted in 211 (96%). Reduction rate with air enema was 90.2%. Delayed reduction attempts were used in 25 patients (15.3%) in 26 intussusceptions (12.3%) and were successful in 50% of the cases. Lead points were documented in 13 children (8%); sonography depicted the lead points in seven (53.8%) of the 13. The reduction rate of intussusceptions due to lead points was 63.6% (14/22).Air enema associated with the use of delayed, repeated reduction attempts is a safe and effective approach for intussusception reduction with a high success rate. Delayed, repeated reduction attempts should be considered when the initial attempt manages to move the intussusceptum and the patient remains clinically stable. The management of intussusceptions due to lead points remains a challenge. Sonography does not depict all lead points, and the indication for other imaging studies should be tailored according to each particular patient. We recommend attempted enema reduction in all patients with lead points.

In the previous two parts of this review on intussusception, the diagnosis and management of symptomatic, "idiopathic" ileocolic and ileoileocolic intussusceptions, which are considered to result from hyperplasia of lymphoid tissue in the distal ileum, were discussed. In this third part, those intussusceptions with an identifiable cause including pathologic lead point, those due to gastrojejunostomy or other feeding tubes, and those that are seen in the postoperative period as well as those that may be asymptomatic or may reduce spontaneously (usually limited to the small bowel) are discussed.

Children with symptomatic ileocolic or ileo-ileocolic intussusceptions can be successfully managed in one of a number of different ways. The nonoperative enema reduction technique has major advantages over surgical reduction and high success rates can be achieved using pneumatic or hydrostatic reduction techniques under fluoroscopic or sonographic guidance. This article highlights current concepts and some controversial issues related to management of intussusception, including patient selection for attempted enema reduction, the advantages and disadvantages of each technique, complications, the value of delayed, repeated reduction attempts, the role of imaging after attempted enema reduction, and recurrence of intussusception.