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Introduction: Hepatopulmonary fusion (HPF) is a rare anomaly specifically associated with right-sided congenital diaphragmatic hernia (CDH). Fewer than 50 cases of HPF have been reported, most at the time of surgery or postmortem with an associated high morbidity and mortality rate. Prenatal diagnosis and optimal management of these rare cases have not been established. Case Presentation: We present a case of HPF diagnosed prenatally by fetal ultrasound and magnetic resonance imaging allowing postnatal and intraoperative planning leading to successful HPF separation and repair of the CDH. Discussion: The prenatal recognition allowed the use of preoperative computed tomography angiogram and right cardiac catheterization to plan the surgical approach. Intraoperative ultrasound confirmed abnormal vasculature associated with the HPF facilitating a successful complete HPF separation and CDH repair was performed. The patient survived and continues to do well on long-term follow-up. Prenatal recognition may help reduce the high morbidity and mortality associated with HPF.

To evaluate the agreement between observers using Greulich-Pyle (GP) and Gilsanz-Ratib (GR) methods, between four specialities (radiology, pediatrics, pediatric endocrinology and pediatric radiology) and between observers and automated tool in the bone age estimation. A total of 99 observers participated in this questionnaire-based study. BoneXpert was used for the automated tool. Experienced, senior, and junior observers were defined by their experience, and the bone age determined by experienced observers was regarded as the ground truth. Agreement between observers was evaluated using the coefficient of variance (CV) and intraclass correlation coefficient (ICC), and they were reevaluated after adding BoneXpert to the observers. Agreement of BoneXpert, the senior, and the junior observers was also evaluated using the root-mean-square-error (RMSE) values and Blant Altman method by comparing with the ground truth. The CV ranged from 4.98% to 22.08%. The ICC were 0.980 for GP, 0.980 for GP and BoneXpert, 0.973 for GR, and 0.976 for GR and BoneXpert, and the ICC between four specialities ranged form 0.963 to 0.990. BoneXpert tool had the lowest RMSE values (0.504 years for GP atlas). Automated bone age estimation showed comparable results with GP and GR methods and its utilization may decrease inter-observer variability.

A 12-year-old Hispanic girl presented with fatigue, lightheadedness, and intermittent headaches. She was depressed and appeared pale to her mother. Her examination was unremarkable except for palpebral conjunctival pallor and was otherwise noncontributory. She had a profound hypoproliferative microcytic anemia with low iron level, low transferrin saturation, and a normal ferritin level. The patient experienced improvement in clinical symptoms following transfusion of packed red blood cells and oral iron therapy. At follow-up 2 months later, she presented with similar symptoms and persistent microcytic anemia with low iron levels. Her ferritin level was increased along with markedly elevated C-reactive protein and erythrocyte sedimentation rate. An oral iron challenge demonstrated lack of absorption, and hepcidin level was also significantly elevated. Thorough gastrointestinal and rheumatologic evaluations were performed to search for a source of inflammation. Key components of the patient’s social history supplemented by serology, radiographic, and pathologic findings ultimately cinched an unexpected diagnosis.

Two-point modified Dixon (mDixon) turbo spin-echo (TSE) sequence provides an efficient, robust method of fat suppression. In one mDixon acquisition, four image types can be generated: water-only, fat-only, in-phase and opposed-phase images. To determine whether PD mDixon TSE water-only and, by proxy, PD in-phase images generated by one acquisition can replace two conventional PD TSE sequences with and without fat suppression in routine clinical MR examination of the knee. This is a retrospective study of 50 consecutive pediatric knee MR examinations. PD mDixon TSE water-only and PD fat-saturated TSE sequences (acquired in the sagittal plane with identical spatial resolution) were reviewed independently by two pediatric radiologists for homogeneity of fat suppression and detection of intra-articular pathology. Thirteen of the 50 patients underwent arthroscopy, and we used the arthroscopic results as a reference standard for the proton-density fat-saturated and proton-density mDixon results. We used the Kruskal-Wallis rank test to assess difference in fat suppression between the proton-density mDixon and proton-density fat-saturated techniques. We used kappa statistics to compare the agreement of detection of intra-articular pathology between readers and techniques. We also calculated sensitivity, specificity and accuracy between arthroscopy and MR interpretations. Proton-density mDixon water-only imaging showed significant improvement with the fat suppression compared with proton-density fat-saturated sequence (P=0.02). Each observer demonstrated near-perfect agreement between both techniques for detecting meniscal and ligamentous pathology and fair to substantial agreement for bone contusions, and chondral and osteochondral lesions. Two-point mDixon water-only imaging can replace conventional proton-density fat-saturated sequence. When same-plane proton-density fat-saturated and non-fat-saturated sequences are required, proton-density water-only and proton-density in-phase image types acquired in the same acquisition shorten the overall examination time while maintaining excellent intra-articular lesion conspicuity.

Palpable subcutaneous masses present in various shapes and sizes in the pediatric population and, accordingly, represent a variety of underlying causes. Lymphatic and venous malformations are among the most common pediatric subcutaneous lesions. However, there are congenital and acquired, as well as benign and malignant, soft-tissue masses that can mimic them clinically and at imaging.Here, we review the natural history, wide range of clinical presentations, and varied but characteristic imaging appearance of lymphatic malformations that can pose diagnostic difficulties in children. In addition, the clinical and imaging characteristics of some pediatric soft-tissue pathologies that can mimic lymphatic malformations and clues to reach a proper diagnosis are highlighted.

OBJECTIVE. The purpose of this article is to review the unusual clinical and radiographic features of venous malformations that can give rise to diagnostic confusion. Entities that can have overlapping clinical and imaging features with venous malformations are also reviewed. CONCLUSION. Venous malformations are congenital endothelial malformations secondary to errors in vascular morphogenesis and are usually diagnosed in the first 2 decades of life. The clinical and imaging features of venous malformations often overlap those of other pathologic entities, creating diagnostic confusion. Furthermore, the clinical presentation and imaging appearance of venous malformations can vary, making the diagnosis challenging. Thorough knowledge of the various clinical and imaging features not only of venous malformations but also of the major potential mimic lesions is crucial for clinicians caring for patients with these lesions.

We describe the largest reported case of pilomatrixoma in the literature. While pilomatrixomas typically present as small soft-tissue nodules of the head, neck and upper extremities, they can also present as much larger masses in atypical locations. When they present in their usual size, pilomatrixomas have typical imaging features and can be correctly diagnosed with imaging studies before histological confirmation. Their clinical and imaging diagnosis become challenging when they are very large, as in our case. A giant pilomatrixoma should also be considered for paediatric patients presenting with a large subcutaneous soft-tissue mass.

Pancreatic neuroendocrine tumors are not included in the diagnostic criteria for tuberous sclerosis complex, although an association has been described. To investigate the association of pancreatic neuroendocrine tumor in children and young adults with tuberous sclerosis complex and define MRI characteristics of the tumor. We retrospectively evaluated the abdominal MRI scans of 55 children and young adults with tuberous sclerosis complex for the presence of a pancreatic mass. The scans were performed over a period of 7 years to monitor renal pathology. We obtained each patient’s clinical history and treatment protocol from the hospital’s electronic medical records. A solid pancreatic mass was identified in 5/55 (9%, 95% confidence interval [CI] 3–20%) patients (4 male) with a mean age of 12.6 years. Four of the lesions were located in the pancreatic tail and one in the pancreatic body. All of the lesions were solid, ovoid and well demarcated, with a mean diameter of 3.1 cm. The masses uniformly demonstrated T1 and T2 prolongation, but their diffusion behavior and post-contrast enhancement varied. The two surgically resected lesions were synaptophysin (+) non-functional pancreatic neuroendocrine tumors on pathology. Two of the patients who did not have surgery were treated with everolimus; one of the lesions has shown interval decrease in size and the other has remained stable. Pancreatic tumor is relatively common in children and young adults with tuberous sclerosis complex.

Poland syndrome is characterized by hypoplastic unilateral chest wall structures. These chest wall deformities may be associated with upper extremity anomalies. The association of Poland syndrome with either intercostal liver herniation or a spinocerebral deformity has been described, but there is no report of both findings encountered simultaneously. This is the first report of a newborn child with Poland syndrome associated with an intercostal liver segment herniation and thoracic myelomeningocele with features of an Arnold-Chiari II cerebral malformation.

Hematopoiesis, the formation of blood cells, commences and is confined to the yolk sac of the fetus for the first 6 weeks. Between the sixth and twentieth weeks of age, the reticuloendothelial system, the liver and spleen, take over the process of blood cell formation [1]. From the sixteenth week, hematopoiesis commences in the bone marrow coinciding with the development of bone cavities [1, 2]. From then on, bone marrow remains the primary site for hematopoiesis. With advancing years, the number of sites and volume of hematopoietic bone marrow progressively decreases.

Pediatric blunt scrotal trauma is most often the consequence of sports injury and presents a diagnostic challenge because swelling and pain make a scrotal physical exam difficult. US with color flow and duplex Doppler is the first-line imaging modality with the goal of accurate and timely diagnosis of injury requiring surgery to preserve fertility and hormonal function. US imaging findings following blunt scrotal trauma include hydrocele, hematocele, testicular hematoma, testicular fracture, testicular rupture, compromised perfusion/testicular torsion and testicular dislocation. Importantly, several of these findings may coexist. Our goal is to present the pertinent intrascrotal anatomy, US imaging findings for each testicular injury, and contemporary management for each, with emphasis on what our pediatric urology colleagues need to know for optimal patient care.

A 14-year-old boy presented with nausea and left-sidedabdominal pain of 2 days’ duration and had elevated whiteblood cells. Abdominal CT with contrast agent shows a 4×4×3.7-cm well-defined, hypodense structure in the splenichilumwithsurroundingfatstrandingandfreefluid(Fig.1). Itwas not a simple cyst and did not show significantenhancement. Differential diagnoses included complicatedpancreatic pseudocyst, mesenteric cyst and torsion of asplenule. US displays the lesion as a solid structure withidentical echogenicity to the spleen without blood flow(Fig. 2). Diagnosis of torsion of a splenule was made; thesplenule was later removed.Ectopic splenic tissue is a common anomaly (10–30% ofautopsies).Failedfusionofmesenchymal splenicbudsresultsin splenules in the left side of the abdomen, usually in thesplenic hilum (75%) [1, 2]. Torsion of a splenule is rare andimaging is usually inconclusive but an early US exam canprove the diagnosis, showing identical echo to the spleen andabsence of blood flow before necrosis develops.References

Pulmonary sequestration is a focal area of nonfunctioning, dysplastic lung parenchyma that lacks communication with the normal tracheobronchial tree. It is supplied by the systemic arterial circulation and has two types, intralobar and extralobar, that can be differentiated from each other by the pleural covering and the venous drainage. Their coexistence is extremely rare. We report the imaging findings of a patient who had coexisting but completely separate intralobar and extralobar sequestrations at the left lower lung. We elucidated the complex vascular anatomy using three dimensional volume rendering and multiplanar reconstructions from a 64-detector helical CT scanner.