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OBJECTIVE. We review the cross-sectional imaging findings of six cases of pathologically proven renal medullary carcinoma in patients with sickle cell trait. MRI findings were available in three of the patients. To our knowledge, only one previous report has addressed MRI features of this rare disease.CONCLUSION. In young patients with sickle cell trait, an infiltrative renal mass with associated retroperitoneal adenopathy and caliectasis are characteristic findings of renal medullary carcinoma on CT and MRI.

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare, often fatal condition. Infants present with a functional obstruction of the gastrointestinal tract (GI), malrotation, microcolon, and a large nonobstructed bladder. Several features common to both MMIHS and Eagle-Barrett or prune belly syndrome (PBS) include hydronephrosis, bladder distension and laxity of the abdominal wall musculature. Additionally, MMIHS and PBS have been reported in the same family, suggesting the possibility of a common pathogenesis. MMIHS usually presents in female infants. We present a male infant diagnosed with both MMIHS and PBS. This is a unique case in which both MMIHS and true PBS are present in the same infant.

Juvenile idiopathic inflammatory myopathy is a rare yet potentially debilitating condition. MRI is used both for diagnosis and to assess response to treatment. No study has evaluated the performance of US elastography in the diagnosis of this condition in children. To assess the performance of compression–strain US elastography in detecting active myositis in children with clinically confirmed juvenile idiopathic inflammatory myopathy and to compare its efficacy to MRI. Children with juvenile idiopathic inflammatory myopathy underwent non-contrast MR imaging as well as compression–strain US elastography of the quadriceps muscles. Imaging findings from both modalities were compared to each other as well as to the clinical determination of active disease based on physical examination and laboratory data. Active myositis on MR was defined as increased muscle signal on T2-weighted images. Elastography images were defined as normal or abnormal based on a previously published numerical scale of muscle elastography in normal children. Muscle echogenicity was graded as normal or abnormal based on gray-scale sonographic images. Twenty-one studies were conducted in 18 pediatric patients (15 female, 3 male; age range 3–19 years). Active myositis was present on MRI in ten cases. There was a significant association between abnormal MRI and clinically active disease (P = 0.012). US elastography was abnormal in 4 of 10 cases with abnormal MRI and in 4 of 11 cases with normal MRI. There was no association between abnormal elastography and either MRI (P > 0.999) or clinically active disease (P > 0.999). Muscle echogenicity was normal in 11 patients; all 11 had normal elastography. Of the ten patients with increased muscle echogenicity, eight had abnormal elastography. There was a significant association between muscle echogenicity and US elastography (P

The objective of this study is to assess the impact of on-site attending radiologist overnight coverage on resident education during transition to 24/7 attending coverage. The study was exempted from IRB review. An anonymous survey was sent to 9 second year radiology residents who completed their first night call rotation (NC) with an attending radiologist (group 1) and 18 residents who completed their first NC prior to overnight attending coverage (group 2). This addressed anxiety level prior to NC, work pace, autonomy and confidence, and attending feedback, with responses graded on a five-point scale. Statistical analysis was performed using Spearman's rho correlation coefficient. Diagnostic Radiology In-Training (DXIT(TM)) exam scores were collected prior to and following completion of the NC rotation, and results were compared. Case volume before and after the transition was recorded. p value0.05 indicated statistical significance. Eight out of nine residents in group 1 and 16 out of/18 residents in group 2 completed the survey. Group 1 was more likely to report working at a comfortable pace (p = 0.008) and receiving attending feedback (p = 0.004) than group 2. A non-significant trend towards reduced anxiety prior to NC was present in group 1 (p = 0.077). No difference in independence (p = 0.918), autonomy (p = 0.635), or confidence during (p = 0.431) or after NC (p = 1.00) was identified. DXIT(TM) scores were not significantly different between the two groups (p = 0.396). While overall case volume dictated by residents increased, fewer plain radiographs were dictated. Overnight attending coverage provides a more comfortable pace of study interpretation and increased attending feedback without decreasing resident independence or DXIT(TM) scores. Plain radiograph interpretation may need to be further emphasized.

We present a 17-year-old boy with benign acute childhood myositis (BACM) who presented with acute onset of right calf pain, swelling, and difficulty walking. The MR findings are reviewed. MR may be useful in diagnosing BACM and in differentiating it from other causes of myositis.

Objective. The objective of this study was to review the problem of lumbar gibbus in children with storage diseases and bone dysplasias utilizing plain films and MR imaging. Materials and methods. Clinical histories and radiographic images in five patients with storage diseases [four mucopolysaccharidosis (MPS) and one mucolipidosis] and two with achondroplasia were reviewed. The International Skeletal Dysplasia Registry (Los Angeles, Calif.), surveyed for all patients with lumbar gibbus and skeletal dysplasias, provided 12 additional cases. Results. All patients had localized gibbus of the upper lumbar spine, characterized by anterior wedging and posterior displacement of the vertebrae at the apex of the curve, producing a beaked appearance. The curve, exaggerated in the sitting or standing position, was most severe in the two patients with MPS-IV (one of whom died). Both developed severe neurologic signs and symptoms requiring surgical intervention. In four patients, MR images demonstrated the apex of the curve to be at or below the conus. Two patients demonstrated anterior herniation of the intervertebral discs at the apex of the curve, though the signal intensity of the intervertebral discs was normal. Conclusion. Lumbar gibbus has important neurologic and orthopedic implications, and is most severe in patients with MPS. The etiology of the gibbus with vertebral beaking is multifactorial and includes poor truncal muscle tone, weight-bearing forces, growth disturbance and anterior disc herniation. The curve is generally at or below the conus. Neurologic complications are unusual, although orthopedic problems can arise. Due to their longer survival, patients with achondroplasia or Morquio's disease are more vulnerable to eventual gibbus-related musculoskeletal complications.

Acute appendicitis is the most common condition requiring emergency surgery in children. Differentiation of perforated from nonperforated appendicitis is important because perforated appendicitis may initially be managed conservatively whereas nonperforated appendicitis requires immediate surgical intervention. CT has been proved effective in identifying appendiceal perforation. The purpose of this study was to determine whether perforated and nonperforated appendicitis in children can be similarly differentiated with ultrasound.This retrospective study included 161 consecutively registered children from two centers who had acute appendicitis and had undergone ultra-sound and appendectomy. Ultrasound images were reviewed for appendiceal size, appearance of the appendiceal wall, changes in periappendiceal fat, and presence of free fluid, abscess, or appendicolith. The surgical report served as the reference standard for determining whether perforation was present. The specificity and sensitivity of each ultrasound finding were determined, and binary models were generated.The patients included were 94 boys and 67 girls (age range, 1-20 years; mean, 11 ± 4.4 [SD] years) The appendiceal perforation rate was significantly higher in children younger than 8 years (62.5%) compared with older children (29.5%). Sonographic findings associated with perforation included abscess (sensitivity, 36.2%; specificity, 99%), loss of the echogenic submucosal layer of the appendix in a child younger than 8 years (sensitivity, 100%; specificity, 72.7%), and presence of an appendicolith in a child younger than 8 years (sensitivity, 68.4%; specificity, 91.7%).Ultrasound is effective for differentiation of perforated from nonperforated appendicitis in children.

Ultrasound elastography allows assessment of tissue elasticity. To the best of our knowledge, the elastography appearance of muscles in normal children has not been described. To determine the US elasticity of muscles in children at rest and following exercise. Cine elastography of biceps brachii and rectus femoris muscles was obtained at rest and after exercise in 42 healthy children (23 males, 19 females; mean: 11.2 ± 4.4 years, range: 2–18 years). Elastography scores were assigned to each clip based on a five-point color scale. Mean elastography scores and standard deviations were calculated and resting and postexercise elastography scores were compared. Resting muscle elasticity was lower in the biceps brachii than in the rectus femoris (P = 0.008), and higher in the dominant than in the nondominant biceps brachii (P

To determine the prevalence of Baker cyst in a general orthopedic population and its association with effusion, internal derangement, and degenerative arthropathy.Reports of 400 knee magnetic resonance imaging examinations were reviewed. Presence of Baker cyst, effusion, internal derangement (meniscal and/or anterior cruciate ligament tears), medial collateral ligament injury, and degenerative arthropathy was recorded. Uni- and multivariate logistic regressions were used to evaluate associations between Baker cyst and these conditions. Probabilities of having a Baker cyst given these conditions were also calculated.No association was found between Baker cyst and anterior cruciate ligament tear or medial collateral ligament injury. There were significant associations (P.001) with effusion, meniscal tear, and degenerative arthropathy. There were also significant associations (P.01) for effusion, meniscal tear, and degenerative arthropathy, independent of one another. Probability of having Baker cyst given the presence of any one variable was .08-.10; any two variables, .19-.21; and all three variables, .38.The association between Baker cyst and joint effusion was confirmed. A relationship with meniscal tear and degenerative joint disease independent of effusion was also demonstrated. The probability of having a Baker cyst increases as the number of these associated conditions increases.

The authors discuss a case of hereditary angioneurotic edema in a child presenting with recurrent episodes of colocolic intussusception, each successfully reduced by air enema. Although additional manifestations of vasogenic edema are potential risks of the procedure, these were not encountered.

We report three patients with imperforate anus and other stigmata of the VATER syndrome who were diagnosed as having tracheoesophageal (TE) fistula, H-type, with intact esophagus. The fistula was diagnosed in two of the three patients only after recurrent respiratory infections.

Objective.To determine sensitivity and specificity of the physical examination (PE) for identifying synovitis in the knee and ankle joints of children with juvenile idiopathic arthritis (JIA), and to identify cases in which ultrasound (US) screening augments the PE.Methods.Nineteen patients with JIA were referred for US. Both knees and ankles were examined using US with and without power Doppler. Active arthritis on PE was defined as (1) non-bony swelling or (2) limitation of motion with either pain on motion or tenderness to palpation. Active arthritis on US was defined as synovial hyperplasia, effusion, or increased vascularity on power Doppler scan.Results.There was agreement between US and PE in 75% of cases. PE was 64% sensitive and 86% specific for identifying active arthritis. PE was 100% specific if (1) the patient was positive for both PE criteria or (2) if arthritis was present on PE in the knees. When the PE was negative and the US was positive, 21.4% developed active disease on PE within 6 months. In cases where the PE was positive and US was negative, the joint involved was most often the ankle and frequently the subtalar joint.Conclusion.PE is neither highly sensitive nor specific for identifying active synovitis when compared to US, and screening with US can identify subclinical disease. In joints with both non-bony swelling and limitation of motion with pain on motion or tenderness, and in the knee joint, little additional information is gained by US. This has implications for classification and treatment of JIA.

A child with acute lymphoblastic leukemia and pseudomonas sepsis rapidly developed left foot pain and swelling. A diagnosis of ecthyma gangrenosum was made. The clinical and imaging features of this unusual entity are discussed. To our knowledge, this is the first description of the MR imaging findings of ecthyma gangrenosum.

Narrowing of the abdominal aorta and renal arteries (mid-aortic syndrome) after treatment for neuroblastoma has been attributed to radiation therapy. We present a child with neuroblastoma, successfully treated without radiation therapy, who developed mid-aortic syndrome and hypertension. MR imaging demonstrated encasement of the aorta at presentation. Following successful treatment, long-segment aortic narrowing and narrowing of the proximal renal arteries were present. Hypertension has improved during a 5-year period although renal artery stenosis persists. The incidence of mid-aortic syndrome in children with neuroblastoma is unknown. Etiologies include vascular injury from radiation or chemotherapy, the effect of catecholamines secreted by the tumor on arterial growth, arterial injury at the time of surgery or a constitutional predisposition to the development of both neuroblastoma and mid-aortic syndrome.

One hundred twenty consecutive patients who had breast-conserving surgery for cancer and were referred for definitive radiation therapy (RT) had a mammogram performed before starting RT. This was done to determine the presence of residual neoplasm or other abnormalities that may alter or delay the planned RT and are undetected by other means. It also was performed to provide a baseline for the diagnosis of postoperative changes and recurrence of disease on follow-up studies. In six (5%) patients, calcifications or masses were found that proved to be residual tumors

The purpose of our study was to compare chest ultrasound and chest CT in children with complicated pneumonia and parapneumonic effusion.We retrospectively compared chest ultrasound and chest CT in 19 children (nine girls and 10 boys; age range, 8 months-17 years) admitted with complicated pneumonia and parapneumonic effusion between December 2006 and January 2009. Images were evaluated for effusion, loculation, fibrin strands, parenchymal consolidation, necrosis, and abscess. In the subset of patients who underwent surgical management, imaging findings were correlated with operative findings.Eighteen of 19 patients had an effusion on both chest ultrasound and chest CT. The findings of effusion loculation as well as parenchymal consolidation and necrosis or abscess were similar between the two techniques. Chest ultrasound was better able to visualize fibrin strands within the effusions. Of the 14 patients who underwent video-assisted thoracoscopy, five had surgically proven parenchymal abscess or necrosis. Preoperatively, chest ultrasound was able to show parenchymal abscess or necrosis in four patients, whereas chest CT was able to show parenchymal abscess or necrosis in three.In our series, chest ultrasound and chest CT were similar in their ability to detect loculated effusion and lung necrosis or abscess resulting from complicated pneumonia. Chest CT did not provide any additional clinically useful information that was not also seen on chest ultrasound. We suggest that the imaging workup of complicated pediatric pneumonia include chest radiography and chest ultrasound, reserving chest CT for cases in which the chest ultrasound is technically limited or discrepant with the clinical findings.

In 1971, the Swedish radiologist Ulf Rudhe wrote a provocative paper on right middle lobe emphysema in infants with left-to-right shunts in which he suggested cardiac surgery rather than lung resection. At the time, this was counter to accepted medical practice. Earlier diagnosis and better medical management of ventricular septal defect in infants has proved Rudhe correct. However, two current cases of large left-to-right shunts in infants with emphysema of the right middle lobe prompt this historical review of what seemed a closed-episode in pediatric cardiac surgery.

To evaluate the dose of scatter radiation to infants in a NICU in order to determine the minimal safe distance between isolettes.Dose secondary to scattered radiation from an acrylic phantom exposed to vertical and horizontal beam exposures at 56 kVp was measured at 93 cm and 125 cm from the center of the phantom. This corresponds to 2 and 3 ft between standard isolettes, respectively. For horizontal exposures, the dosimeter was placed directly behind a CR plate and scatter dose at 90-degrees and 135-degrees from the incident beam was also measured. Exposures were obtained at 160 mAs and the results were extrapolated to correspond to 2.5 mAs. Four measurements were taken at each point and averaged.At 125 cm and 93 cm there was minimal scatter compared to daily natural background radiation dose (8.493 microGy). Greatest scatter dose obtained from a horizontal beam exposure at 135 degrees from the incident beam was still far below background radiation.Scatter radiation dose from a single exposure as well as cumulative scatter dose from numerous exposures is significantly below natural background radiation. Infants in neighboring isolettes are not at added risk from radiation scatter as long as the isolettes are separated by at least 2 ft.

OBJECTIVE. The purpose of this study was to illustrate the spectrum of solitary gluteal lesions in children. The distinctive anatomic and radiologic features are discussed.CONCLUSION. Pathologic entities in the gluteal region reflect the diversity of tissue types present. Ultrasound is valuable for imaging of infants and young children and for evaluating superficial lesions. Cross-sectional imaging is most useful for defining the extent of disease and may show pathognomonic features, enabling a precise diagnosis.

The spectrum of congenital anomalies of the male urethra is presented. The embryologic basis of each anomaly, when known, is discussed. Clinical and imaging features of each entity are presented.

The optimal treatment of perforated appendicitis remains controversial, but there is a trend toward nonoperative management. CT scanning might be helpful in determining which patients could benefit from this treatment option. To determine the value of CT imaging in predicting clinical success or failure in children with nonoperative management of perforated appendicitis. Admission CT scans of 34 children with perforated appendicitis treated nonoperatively between January 2003 and June 2006 were retrospectively reviewed. All children were given intravenous antibiotics. Clinical outcome was correlated with imaging findings including the maximal area, number and complexity of collections, presence of an appendicolith or extraluminal air, and extent of intra-abdominal disease outside the right lower quadrant. Patients with an accessible simple collection were drained if their clinical condition did not improve. Successful nonoperative management was achieved in 20 patients; 14 patients failed nonoperative therapy. The presence of collections in three or more sectors (defined as the pelvis and four abdominal quadrants) correlated strongly with clinical failure (P

Dideoxyinosine (ddI) is a widely used antiretroviral agent in treatment of HIV infection. Pancreatitis is a serious side effect. Two cases are reported, one with rapid development of a pseudocyst.

Foreign body ingestion is a common occurrence in children. Morbidity associated with ingestion is rare and is dependent on the type of foreign body ingested. We report a case of a 9-year-old boy who developed intestinal obstruction and perforation following ingestion of magnetic-backed earrings.

We report a patient with congenitally short trachea (CST), defined as a decreased number of tracheal rings and high carina, who had respiratory distress and overaeration of the left lung secondary to extrinsic narrowing of the distal mainstem bronchus by ligamentum arteriosum compression.

Deferoxamine chelation therapy (widely used to reduce iron overload in hypertransfused thalassemic patients) has been implicated in causing skeletal growth abnormalities (rachitic-like changes in the long bones and vertebral body flattening), particularly when used in early infancy and at high dose levels. Radiographs of seven hypertransfused and well-chelated patients with thalassemia were reviewed. For two patients, serial films of the spine from the early 1970s to the present revealed a sequence of changes in the vertebral bodies, beginning with normal bodies that became bulbous and subsequently flattened. These two patients had begun deferoxamine chelation therapy early in infancy. The bone changes, though slightly reminiscent of post-radiation changes, are milder and result in a final Scheuermann-like picture.

A 13 1/2-year-old boy, who was a competitive gymnast, had pain in both wrists; the pain was worse in the left wrist than in the right. The patient had worked out as much as several hours a day, 5 days a week, over the previous 2 years. The pain primarily was dorsal and radial and was exacerbated most by hanging from the rings and high bar. Physical examination showed full range of motion, pain on fullforced dorsiflexion and palmar flexion, and slight tenderness over the radial aspect of the wrist. Radiographs obtained at another institution were considered normal (Fig. 1A), The bone age was 1 year behind the chronologic age. The patient continued his exercise routine with the use of splints. He returned 3 months later with continued pain, despite treatment with nonsteroidal antiinflammatory drugs. Plain radiographs (Fig. 1 B) showed widening and irregularities of the distal radial physes, which were more prominent in the left radius than in the right. Simiar, but less extensive, changes were present in the distal ulnar physes. The wrist joints and carpal bones had no abnormalities. Bone age still was slightly delayed. Ti-weighted (600/17) MR images corroborated widening ofthe growth plate and the irregularity of the bordering metaphysis (Fig. 1C). On T2weighted gradient echo (600/22, 35 flip angle), most of the metaphyseal irregularity had low signal intensity. However, an additional fine, irregular line near the open growth plate had the same high signal intensity as the growth plate (Fig. 1D). The MR imaging had been requested by the orthopedist in view of the continuing pain and seemingly normal plain films (Fig. 1A) that were obtained 3 months previously (the MR image actually was obtained before the repeat plain films) (Fig. 1B). The patient was advised to discontinue wrist weight-bearing activities. Radiographs ofthe wrist obtained 6 months later showed interval resolution of the growth plate abnormalities. Repeat MR images were not obtained. The patient planned to return to gymnastic training.

Three children with adult cadaveric bone allografts for the treatment of bone malignancies are presented. Follow-up magnetic resonance (MR) imaging demonstrated decreased signal on T1-weighted imaging and increased signal on T2-weighted imaging in the allograft without clinical evidence of recurrent disease. These signal characteristics appear to be a normal finding in cadaveric bone allografts and should not be mistaken for recurrence. The finding may reflect persistent marrow necrosis within the allograft marrow cavity.

Thoracolumbar fracture with listhesis (FL) is an uncommon manifestation of child abuse (increasingly known as nonaccidental trauma), with only six prior reports in the literature.This article seeks to call attention to FL of the thoracolumbar spine in abused children and infants.We reviewed plain films, CT and MR images in seven new cases of FL of the thoracolumbar spine in abused children ages 6 months to 7 years, two of whom became paraplegic from their injuries.Findings varied from subtle listhesis of one vertebra on another to frank vertebral dislocation, most commonly at L1/2. Paravertebral calcification was present in all but one case. In two children, thoracolumbar FL was the only radiographic sign of abuse.Radiographic findings of FL of the thoracolumbar spine may be subtle and may be erroneously interpreted as due to a congenital or neoplastic cause. While other signs of child abuse should be sought, spinal injury may be the sole sign of abuse. Recognition of this entity is important to pursue the diagnosis of abuse.

A choledochal cyst is an uncommon anomaly of the biliary system requiring surgical intervention. A case of a choledochal cyst imaged by computed tomography following oral administration of cholangiography contrast material (Telepaque) is reported. Telepaque-enhanced computed tomography is an easy, noninvasive method to demonstrate pertinent preoperative anatomy in cases of choledochal cysts, and is especially useful in the pediatric patient.

An unusual case of a juxtabronchial lymphoepithelial cyst in an HIV-positive child with post-obstructive pneumonia is presented. The pathogenesis and similarity with parotid lymphoepithelial cysts is discussed.

Salt losing nephropathy, occurring predominantly in male infants, has been reported in association with a spectrum of urologic diseases including obstructive uropathy and massive, infected vesicoureteral reflux (VUR). This has been called pseudo-hypoaldosteronism (PHA) or alternatively, pseudo salt-losing congenital adrenal hyperplasia (CAH), and is thought to reflect a tubular unresponsiveness to aldosterone. We report our experience with six cases, discuss one case in detail and review the 39 cases previously reported. A one month old male infant presented with a left upper quadrant mass. Signs and symptoms included vomiting, dehydration, hyponatremia and hyperkalemia. This suggested the diagnosis of CAH for which therapy was instituted. Ultrasonographic examination subsequently revealed the mass to be a urinoma in an infant with posterior urethral valve (PUV) and obstructive hydronephrosis.

Pediatric oncology patients who undergo intensive chemotherapy develop anemia caused by myeloid suppression that necessitates transfusions that, in turn, cause iron deposition in the reticuloendothelial system. We describe MR imaging of iron overload in pediatric patients who underwent such chemotherapy and who have solid and hematologic tumors.The MR appearance of the liver, spleen, and bone marrow was evaluated in 13 children with both solid (n = 10) and hematologic (n = 3) malignant lesions using known criteria for the presence of iron deposition. Findings were correlated with transfusional history, chemotherapeutic regimens, and ferritin levels.MR imaging obtained after chemotherapy and transfusional therapy revealed signs of iron deposition in the liver and spleen, particularly on T2-weighted images. Bone marrow signal intensity varied among patients. Pancreatic signal intensity was normal. Ferritin levels were elevated in all patients.Reticuloendothelial system iron deposition present in follow-up MR imaging of pediatric solid and hematologic malignant lesions reflected the intensity of the chemotherapeutic regimen, the degree of myeloid suppression, and the resultant transfusional requirements. Such iron deposition appeared to have no effect on cardiac, liver, or pancreatic function.

Purpose. We studied six patients with sickle cell disease (SSD), five homozygous for sickle cell anemia and one with sickle betathalassemia, in whom rounded intrasplenic masses proved to be preserved functioning splenic tissue.Materials and methods. Available images including computed tomography, ultrasonography, bone scans (Tc-99m MDP), liver spleen scans (Tc-99m sulfur colloid), and MRI were evaluated.Results. The masses were low density on CT (in an otherwise calcified spleen), hypoechoic relative to the echogenic spleen on US, and had the imaging characteristics of normal spleen on MRI. They failed to accumulate Tc-99m MDP but did demonstrate uptake of Tc-99m sulfur colloid.Conclusion. In a patient with SSD and intrasplenic masses, proper correlation of multiple imaging modalities will establish the diagnosis of functioning splenic tissue and avoid mistaken diagnosis of splenic abscess or infarction.

An HIV-positive child presented with a pneumothorax secondary to cavitary Pneumocystis carinii pneumonia (PCP). Lymphocytic interstitial pneumonitis had been evident on earlier radiographs but had resolved, concurrent with a decrease in her CD4 counts, before the radiographic changes of PCP became evident. As immune function declines in HIV-positive children, the chest radiograph may paradoxically clear. In such a setting, development of focal lung disease, including pneumothorax, may herald Pneumocystis carinii infection.

To correlate pathologic, computed tomographic (CT), and ultrasound (US) characteristics of nephrogenic adenofibromas and embryonal adenomas (uncommon pediatric renal tumors) in children.Medical records and imaging and pathologic findings were reviewed in three children (aged 6 1/2, 7, and 11 years) with adenomatous renal tumors and polycythemia. Specimens were reviewed at the National Wilms Tumor Study Pathology Center (Loma Linda, Calif).All tumors were smaller than 3 cm in greatest dimension. They were hyperechoic on US scans and had high attenuation on unenhanced CT scans. Two patients underwent nephrectomy for initial diagnosis of Wilms tumor. The third underwent local excision. At pathologic examination, embryonal-appearing adenomatous epithelial cells were found to form tubules and papillae with abundant psammomatous calcifications. Two masses were classified as embryonal adenomas and one as nephrogenic adenofibroma.Increased attenuation on CT scans and increased echogenicity on US scans of renal adenomatous tumors are distinctive findings that may reflect the presence of tubulopapillary structures and psammomatous calcifications.

To characterize the skeletal and bone marrow magnetic resonance (MR) imaging changes during and after treatment of childhood soft-tissue tumors.Three boys with soft-tissue sarcomas of the popliteal fossa underwent surgery, radiation therapy, and chemotherapy. Plain radiographic and MR imaging findings were correlated with the effect of treatment.After radiation therapy, MR images revealed findings that resembled those of rickets at sites of irradiation in the three patients. These findings included metaphyseal sclerosis, metaphyseal fraying, and epiphyseal plate widening. Bone marrow imaging changes were temporally related to therapy. During chemotherapy, reconversion to hematopoietic marrow was noted in nonirradiated areas in two patients, but after cessation of all treatment, these areas converted back to fatty marrow. Irradiated areas of bone marrow remained fatty throughout therapy in the three patients.Awareness of the MR imaging findings related to antineoplastic treatment of soft-tissue tumors is important to distinguish these changes from progression of primary disease.

Twenty-four patients with repaired diaphragmatic hernia had upper gastrointestinal series to assess the rotation and fixation of the bowel. A spectrum of rotational abnormalities was present in almost all patients, although those with repaired right-sided hernias demonstrated a more obvious anomaly of rotation than those with repaired left-sided hernias. No patient developed a midgut volvulus despite the presence of malfixation. Postoperative adhesions likely limit the occurrence of volvulus.

Magnetic resonance (MR) marrow signal in the axial and appendicular skeleton of 13 transfusion-dependent and chelated pediatric patients with sickle cell anemia (SSD) was compared with marrow signal in six non-transfusion-dependent patients with SSD. Hepatic, pancreatic, and renal MR signal were also evaluated. Indication for hypertransfusion therapy was primarily prior history of stroke. Transfusion-dependent patients had evidence of iron deposition throughout the imaged marrow and the liver, despite deferoxamine chelation therapy. Non-transfusion-dependent patients did not demonstrate grossly apparent signs of iron overload. Red marrow restoration was present in the spine, pelvis, and long bones and, in some patients, within the epiphyses. Marrow edema secondary to vaso-occlusive crises was evident in the metaphyses and diaphyses of long bones in areas of both red and fatty marrow and was best seen using fat-saturated T2-weighted imaging techniques.

The magnetic resonance bone marrow patterns in thalassemia were evaluated to determine changes produced by transfusion and chelation therapy. Thirteen patients had T1- and T2-weighted images of the spine, pelvis and femurs. Three received no therapy (age range 2.5-3 years). Three were hypertransfused (transfused to maintain a hemoglobin greater than 10 g/dl) and not chelated because of age (age range 6 months-8 years). Seven were hypertransfused and chelated (age range 12-35 years). Signal characteristics of marrow were compared with those of surrounding muscle and fat. Fatty marrow (isointense with subcutaneous fat) was compared with red marrow (hypointense to fat and slightly hyperintense to muscle). Marrow hypointense to muscle was identified as iron deposition within red marrow. The untreated group demonstrated signal consistent with red marrow throughout the central and peripheral skeleton. Hypertransfused but not chelated patients demonstrated marked iron deposition in the central and peripheral skeleton. Hypertransfused and chelated patients demonstrated iron deposition in the central skeleton and a mixed appearance of marrow in the peripheral skeleton. The MR appearance of marrow in thalassemia is a reflection of the patient s transfusion and chelation therapy. Iron deposition occurs despite chelation therapy in sites of active red marrow. As red marrow retreats centrally with age, so does the pattern of iron deposition. The long-term biological effects of this iron deposition are unknown.

Barium esophagrams were obtained in two patients with respiratory problems whose underlying congenital heart disease was pseudotruncus arteriousus type I. The esophagrams revealed vascular indentations on the esophagus, one posteriorly, and the other anteriorly. Both types of indentations were secondary to aorto-pulmonary communicating arteries from the descending aorta.

Sir, Recently, an article about Williams-Beuren syndrome [1] appeared in Pediatric Radiology. Characteristic facies, intellectual disability, as well as supravalvular aortic stenosis (SVAS) are typical of Williams syndrome (WS) (OMIM #194050). Familial aortic stenosis, or Eisenberg syndrome [2] (OMIM#185500), represents a separate group of patients with autosomal-dominant SVAS in which affected members have normal facies and average intelligence. The history of Eisenberg-type familial SVAS and WS, once considered part of a single condition, is an interesting one. Because of the commonality of the cardiac lesion in these two groups, advances in the genetic basis of Eisenbergtype familial SVAS led to the discovery that both WS and familial SVAS result from an abnormality in the elastin gene (ELN). In 1993 Keating [3], a molecular geneticist, used linkage analysis to suggest that ELN, located at 7q11.23, was the candidate gene for SVAS. Later publications identified several families with familial SVAS in whom the ELN gene was disrupted by either a small deletion or translocation. Based on the identification of the genetic abnormality present in familial SVAS, fluorescent in situ hybridization (FISH) analysis of patients with the WS phenotype was performed, and complete deletion of the ELN genewas identified. Haploinsufficiency of additional genes has been implicated in the other abnormalities characteristic of WS. The history of familial SVAS and its relationship toWS has been largely forgotten. Recent interest in the genetic cause of WS is directly related to the genetic breakthroughs in patients with isolated familial SVAS. Because of the commonality of the cardiac lesion in these two groups, advances in one disease led to discoveries in the second.

In the absence of hydrops or sepsis, a pericardial effusion is a rare occurrence in the neonate. We report a case of a neonate with a pericardial effusion in which there was an associated intracardiac hemangioma. Our literature review found 32 cases of pericardial effusion with-out hydrops in infants under 3 months of age; twelve of these cases were associated with intracardiac and pericardial tumors; 20 others were discovered to be randomly associated with other problems.

We present a case of a newborn with congenital rickets and radiographic evidence of secondary hyperparathyroidism in which the infant's mother had severe, longstanding untreated renal insufficiency.