Vesicoureteral reflux: diagnosis and grading with echo-enhanced cystosonography versus voiding cystourethrography.

PURPOSE: To evaluate the usefulness of echo-enhanced cystosonography compared with voiding cystourethrography (VCUG) for detecting and grading vesicoureteral reflux (VUR). MATERIALS AND METHODS: Two hundred sixteen pediatric patients underwent cystosonography enhanced with SH U 508A, a galactose-based echo-enhancing agent. Sonograms of the kidneys and bladder were obtained before filling, during bladder filling, and during voiding. This examination was followed immediately with VCUG. Each kidney or portion of a kidney with its own complete collecting system was considered separately, for a total of 440 kidney units. RESULTS: VUR was detected in 123 kidney units at cystosonography and in 104 at VCUG. In 401 kidney units, there was concordance between results at cystosonography and at VCUG regarding the presence or absence of VUR. Ninety-four kidney units showed VUR with both methods. Seventy-seven of the 94 refluxing units were depicted with the same grade of VUR with both modalities, and in 17 the VUR grade was greater at cystosonography than at VCUG. Twenty-nine of the 94 units showed VUR at only cystosonography, and 10 units at only VCUG. The McNemar test showed that cystosonography depicted a significantly (P = .003) higher number of units with VUR. By patient, VUR was depicted with both studies in 67 and with only one study in 25. VUR was seen at only cystosonography in 16 patients and at only VCUG in nine. The McNemar test for patients showed no significant difference between the two tests in detection of VUR. CONCLUSION: Cystosonography with SH U 508A appears comparable to VCUG in the depiction of VUR.

Sickle cell anemia.

Sickle cell anemia (SCA) is a disease caused by production of abnormal hemoglobin, which binds with other abnormal hemoglobin molecules within the red blood cell to cause rigid deformation of the cell. This deformation impairs the ability of the cell to pass through small vascular channels; sludging and congestion of vascular beds may result, followed by tissue ischemia and infarction. Infarction is common throughout the body in the patient with SCA, and it is responsible for the earliest clinical manifestation, the acute pain crisis, which is thought to result from marrow infarction. Over time, such insults result in medullary bone infarcts and epiphyseal osteonecrosis. In the brain, white matter and gray matter infarcts are seen, causing cognitive impairment and functional neurologic deficits. The lungs are also commonly affected, with infarcts, emboli (from marrow infarcts and fat necrosis), and a markedly increased propensity for pneumonia. The liver, spleen, and kidney may experience infarction as well. An unusual but life-threatening complication of SCA is sequestration syndrome, wherein a considerable amount of the intravascular volume is sequestered in an organ (usually the spleen), causing vascular collapse; its pathogenesis is unknown. Finally, because the red blood cells are abnormal, they are removed from the circulation, resulting in a hemolytic anemia. For the patient with SCA, however, the ischemic complications of the disease far outweigh the anemia in clinical importance.

Autosomal recessive polycystic kidney disease: radiologic-pathologic correlation.

Autosomal recessive polycystic kidney disease is a heritable but phenotypically variable disorder characterized by varying degrees of nonobstructive renal collecting duct ectasia, hepatic biliary duct ectasia and malformation, and fibrosis of both liver and kidneys. In the kidney, the dilated collecting ducts and interstitial fibrosis, when severe, may significantly impair renal function and result in hypertension and renal failure. Imaging typically shows large but reniform kidneys, diffusely increased renal parenchymal echogenicity at ultrasonography, and a striated nephrogram after contrast material administration. In the liver, periportal fibrosis accompanies the malformed and dilated bile ducts; this may result in portal hypertension. The liver may appear normal or may show intrahepatic biliary dilatation; once portal hypertension develops, splenomegaly and varices are usually evident. The relative degrees of kidney and liver involvement tend to be inverse: Children with severe renal disease usually have milder hepatic disease, and those with severe hepatic disease tend to evidence mild renal impairment. Presently, treatment consists of supportive management and control of hypertension. Replacement therapy for renal failure (dialysis or kidney transplantation) and control of portal hypertension (portal circulatory diversion or liver transplantation) may be necessary.

Pancreatoblastoma: imaging findings in 10 patients and review of the literature.

PURPOSE: To describe the features of pancreatoblastoma at magnetic resonance (MR) imaging, computed tomography (CT), and ultrasonography (US). MATERIALS AND METHODS: Imaging and surgical findings in 10 patients (age range, 2-20 years; mean age, 6.8 years) with pathologically proved pancreatoblastoma were reviewed for tumor size, organ of origin, definition and quality of tumor margins, tumor heterogeneity, calcification, enhancement, ascites, biliary and/or pancreatic ductal dilatation, local invasion, adenopathy, vascular invasion, vascular encasement, metastases, and signal intensity on MR images. Results from 10 CT, seven US, and three MR imaging examinations were reviewed. RESULTS: Five of the 10 tumors were pancreatic; four others appeared to be pancreatic or hepatic. Most had well-defined margins (nine of 10), were heterogeneous (nine of 10), and enhanced (10 of 10). Other findings included calcification (two of 10), biliary and pancreatic ductal dilatation (one of 10), and ascites (three of 10). Hepatic (two patients) and pelvic (two patients) metastases were present. Adenopathy (two patients) and vascular invasion (one patient) were not identified radiologically. Tumors had low to intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. CONCLUSION: Pancreatoblastoma is typically a heterogeneous tumor with well-defined margins that may appear to arise from the pancreas or liver. It may behave aggressively, with localized vascular or bowel invasion or with widespread metastatic disease. Although it is rare, it should be considered in the differential diagnosis of an upper abdominal mass in a child.

From the archives of the AFIP. Infiltrative renal lesions: radiologic-pathologic correlation. Armed Forces Institute of Pathology.

Most renal masses exhibit an expansile growth pattern characterized by radial tumor enlargement that displaces normal renal parenchyma and forms spherical, often exophytic, lesions. These expansile masses have pushing margins that impress adjacent normal renal parenchyma but do not infiltrate it; this behavior results in a well-defined, encapsulated appearance at both radiologic and gross pathologic examination. In contrast, certain disease processes involve the kidney in an infiltrative fashion by using the normal renal architecture as scaffolding for interstitial growth. These infiltrative renal lesions lack a sharp border of demarcation with the normal parenchyma and therefore demonstrate ill-defined zones of transition between the lesion and normal parenchyma. Although infiltrative lesions frequently enlarge the kidney, its reniform shape is usually maintained. Cross-sectional imaging can often help distinguish infiltrative from expansile growth patterns through analysis of the parenchymal interface between the process and the kidney, the effect of the lesion on the collecting system and renal sinus, and the overall renal morphology. A wide variety of neoplastic and inflammatory conditions characteristically involve the kidney by cellular infiltration. Although considerable overlap of the imaging features exists among the various infiltrative processes, the correct diagnosis may be suspected when the clinical data and associated radiologic findings are considered together.

Adrenocortical neoplasms in children: radiologic-pathologic correlation.

Primary neoplasms of the adrenal cortex are rare in children and differ significantly in epidemiology, clinical characteristics, and biologic features from their counterparts in adults. In children, the inclusive term adrenocortical neoplasm is applied because adrenal adenoma and adrenal carcinoma may be difficult to distinguish histopathologically. Pediatric adrenocortical neoplasms typically occur before 5 years of age, affect young girls more commonly than boys, and are associated with hemihypertrophy and Beckwith-Wiedemann and Li-Fraumeni syndromes. Most children with an adrenocortical neoplasm present with signs and symptoms of endocrine abnormality, including virilization and Cushing syndrome. Cross-sectional imaging studies typically demonstrate a large, circumscribed, predominantly solid suprarenal mass with variable heterogeneity due to hemorrhage and necrosis. Calcification is not uncommon. Local invasion and metastases to the lungs, liver, and regional lymph nodes may be present at diagnosis. When friable tumor thrombus extends into the inferior vena cava, it poses a high risk of pulmonary embolization. The finding of increased retroperitoneal fat due to hypercortisolism on computed tomographic and magnetic resonance images of children with an adrenal mass favors the diagnosis of adrenocortical neoplasm. Surgical resection is the mainstay of therapy, with chemotherapy used for patients with metastases or persistent elevated hormone levels following surgery. Patients younger than 5 years with aggressive adrenocortical neoplasms fare better than older children.

Pulmonary disease in the immunocompromised child.

Immune deficiency states in children may be related to primary immunodeficiency syndromes or secondary disorders of the immune system. The secondary immunodeficiencies in children include human immunodeficiency virus-associated acquired immunodeficiency, as well as immunosuppression secondary to antineoplastic chemotherapeutic agents, bone marrow transplantation, and drugs given to prevent transplant rejection. This article discusses the common primary and secondary immunocompromised states of childhood, with emphasis on their attendant infectious, lymphoproliferative, and neoplastic complications.

Nephrogenic rests, nephroblastomatosis, and associated lesions of the kidney.

The fetal kidney is formed by the development of nephrons from fetal metanephric blastema surrounding the ureteric bud. The fetal renal tissue matures into normal renal parenchyma during gestation, but, occasionally, fetal tissue persists into infancy as microscopic foci called nephrogenic rests. Nephrogenic rests are found in approximately 1% of infant kidneys at autopsy. Nephrogenic rests are associated with an increased risk of Wilms tumor, and it is theorized that nephrogenic rests undergo neoplastic change into Wilms tumor. Fortunately, this transformation occurs in less than 1% of young children with nephrogenic rests. Nephrogenic rests are associated with many syndromes, including Beckwith-Wiedemann syndrome, hemihypertrophy, and sporadic aniridia. Children with identifiable syndromes, once diagnosed, should be screened for the development of Wilms tumor. Nephrogenic rests are associated with other lesions such as multilocular cystic nephroma and multicystic dysplasia, usually without malignant complications.

Childhood pyelonephritis: comparison of gadolinium-enhanced MR imaging and renal cortical scintigraphy for diagnosis.

PURPOSE: To compare gadolinium-enhanced inversion-recovery magnetic resonance (MR) imaging with renal cortical scintigraphy in the diagnosis of childhood pyelonephritis. MATERIALS AND METHODS: Thirty-seven patients with fever-producing urinary tract infection underwent gadolinium-enhanced inversion-recovery MR imaging and technetium-99m renal cortical scintigraphy. Each study was read in double-blind fashion by two radiologists. The kidney was divided into three zones, and each was graded as positive, equivocal, or negative for pyelonephritis. RESULTS: Seventy kidneys (210 zones) were imaged. Twenty-six kidneys (54 zones) had evidence of pyelonephritis at both MR imaging and scintigraphy. Twenty-four kidneys (100 zones) were negative on both studies. Twelve kidneys (42 zones) were positive at MR imaging but negative at scintigraphy, and four kidneys (seven zones) were negative at MR imaging but positive at scintigraphy. The results of MR imaging for pyelonephritis were not equivalent to the results of scintigraphy (P = .001 for renal zones). The proportion of positive agreement between readers for the presence of pyelonephritis was 0.85 and 0.57 for MR imaging and scintigraphy, respectively. The proportion of negative agreement was 0.88 and 0.80 for MR imaging and scintigraphy, respectively. CONCLUSION: Gadolinium-enhanced inversion-recovery MR imaging enabled detection of more pyelonephritic lesions than did renal cortical scintigraphy and had superior interobserver agreement.

From the archives of the AFIP. Genitourinary rhabdomyosarcoma in children: radiologic-pathologic correlation.

Rhabdomyosarcoma is the most common tumor of the lower genitourinary tract in children in the first 2 decades of life. Most cases of genitourinary rhabdomyosarcoma are of the embryonal histologic subtype and include tumors of the bladder, prostate, testes and paratesticular sites, penis, perineum, vagina, and uterus. The natural history, pattern of metastatic spread, treatment, and prognosis of childhood rhabdomyosarcoma vary with the anatomic site of the lesion. In children with rhabdomyosarcoma of the bladder or prostate, presenting signs and symptoms include urinary or fecal retention, dysuria, urinary tract infection, and hematuria. Paratesticular rhabdomyosarcoma produces painless scrotal swelling, which may be ignored until the tumor has reached a large size. Vaginal tumors may manifest as a prolapsing mass in the introitus. Radiologic studies of children with genitourinary rhabdomyosarcoma reflect the nonspecific gross features of the tumor, which may be ill defined with infiltrative margins or well circumscribed by a pseudocapsule of compressed tissue. The botryoid variant of embryonal rhabdomyosarcoma results when submucosal tumor produces a polypoid mass resembling a cluster of grapes within a hollow structure. Botryoid morphology is characteristic, but not specific, for rhabdomyosarcoma within the vagina or urinary bladder, since yolk sac tumor and "tumoral" cystitis may have a similar appearance. Invasion of adjacent structures by the primary tumor may make the precise anatomic origin of genitourinary rhabdomyosarcoma difficult to determine on cross-sectional images. Recent refinements in multidisciplinary therapeutic regimens combining chemotherapy, radiation therapy, and surgery have dramatically improved outcome for children with genitourinary rhabdomyosarcoma. Diagnostic imaging plays an important role in monitoring response to therapy.

Experimental pyelonephritis in piglets: diagnosis with MR imaging.

PURPOSE: To compare findings at magnetic resonance (MR) imaging with those at histopathologic examination in the detection of experimentally induced pyelonephritis in piglets. MATERIALS AND METHODS: MR imaging was performed in 23 piglets with and nine piglets without experimentally induced pyelonephritis. Escherichia coli were injected into the bladder of the 23 piglets with surgically created vesicoureteral reflux. Imaging was performed with unenhanced and contrast material-enhanced T1-weighted and fast multiplanar inversion-recovery (IR) and fast spinecho T2-weighted sequences. MR images and pathologic findings were reviewed independently by two pediatric radiologists and a pathologist, respectively, in a blinded fashion. RESULTS: Sixty-four kidneys and 192 renal zones were evaluated. Coronal gadolinium-enhanced fast multiplanar IR imaging was the only sequence that was sensitive and specific for the diagnosis of pyelonephritis. For the two reviewers, respectively, sensitivity was 85% (n = 75) and 92% (n = 81) of 88 histopathologically positive zones and specificity was 95% (n = 99) and 94% (n = 98) of 104 pathologically negative zones. Findings at gadolinium-enhanced fast multiplanar IR imaging were not statistically different from findings at histopathologic examination in the detection of pyelonephritis. Interobserver reproducibility for the contrast-enhanced fast multiplanar IR sequence was excellent (kappa statistic = 0.82 and 0.90, respectively, for interpretation of a renal zone and of a kidney). CONCLUSION: Contrast-enhanced fast multiplanar IR imaging is a sensitive and specific test for detection of experimental pyelonephritis in this piglet model.