Search

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.

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.

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.

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.