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Biomedical subjects

W E Berdon

Publications and source records attributed to W E Berdon.

At least 19 recordsLinked to original sources

Three pediatric patients with extension of prostatic embryonal rhabdomyosarcoma anterior to the bladder into the space of Retzius.

Invasive embryonal rhabdomyosarcoma of the prostate often extends superiorly into the bladder base requiring partial cystectomy as part of excision of the primary tumor. Three patients were studied with ultrasonography CT and MRI and showed the extension of the tumor superiorly and anterior to the bladder in the prevesical space of Retzius. Complete excision with bladder sparing was successful in two of the patients. The imaging of pelvic tumors in children (although well studied with ultrasonography, CT) is helped by MRI in the sagittal plane and/or lateral films of intravenous pyelograms and cystograms to establish the relation of the tumor to the bladder.

Child

Malignant endobronchial lesions of adolescence.

Rare primary endobronchial malignancies were diagnosed in four adolescents: atypical bronchial carcinoid, mucoepidermoid carcinoma, bronchogenic (squamous cell) carcinoma, and non-Hodgkin's lymphoma. Metastatic disease was evident in each case. Three of the four patients died within one year of diagnosis. This paper describes each of these entities, illustrates their plain film and CT abnormalities (bronchial "cut-off", hilar mass, local congestive edema, lobar collapse, hyperinflation), and discusses the utility of CT in their diagnosis.

Adolescent

The diagnosis of abdominal neuroblastoma: relative roles of ultrasonography, CT, and MRI.

Abdominal neuroblastoma is reviewed in terms of its diagnosis, including in utero, at birth, and through infancy into childhood. Age at diagnosis remains the best predictor of survival, with infants under 1 year of age having almost 100% cure. Ultrasonography and magnetic resonance imaging (MRI) are the recommended diagnostic modalities to stage the abdominal primary, although computed tomography (CT) (including myelography) is still widely and efficiently used. Examples are given of each stage.

Abdominal Neoplasms

Benign gaseous distension of the bowel in premature infants treated with nasal continuous airway pressure: a study of contributing factors.

Continuous positive airway pressure (CPAP) administered as a mixture of oxygen and compressed air via nasal prongs has dramatically improved survival rates and lessened the frequency of barotrauma and bronchopulmonary dysplasia in the premature infant with respiratory distress syndrome. Associated with the increased use of nasal CPAP has been the development of marked bowel distension (CPAP belly syndrome), which occurs as the infant's respiratory status improves and the baby becomes more vigorous. To identify contributing factors, we prospectively compared 25 premature infants treated with nasal CPAP with 29 premature infants not treated with nasal CPAP. Infants were followed up for development of distension, defined clinically as bulging flanks, increased abdominal girth, and visibly dilated intestinal loops. We evaluated birth weight, weight at time of distension, method of feeding (oral, orogastric tube), and treatment with nasal CPAP and correlated these factors with radiologic findings. Of the infants who received nasal CPAP therapy, gaseous bowel distension developed in 83% (10/12) of infants weighing less than 1000 g, but in only 14% (2/14) of those weighing at least 1000 g. Only 10% (3/29) of infants not treated with nasal CPAP had distension, and all three weighed less than 1000 g. Presence of sepsis and method of feeding did not correlate with occurrence of distension. Neither necrotizing enterocolitis nor bowel obstruction developed in any of the patients with a diagnosis of CPAP belly syndrome. Our study shows that nasal CPAP, aerophagia, and immaturity of bowel motility in very small infants were the major contributors to the development of benign gaseous bowel distension.

Gases

Colonic diverticulitis in young patients with chronic renal failure and transplantation.

The association of colonic diverticulitis with chronic renal failure is well known. In those patients with "adult" autosomal dominant polycystic kidney disease, colonic diverticulitis is an especially common complication. We present two young patients (one teenager and one mid-twenties) who developed intra-abdominal abscess several years after renal transplantation. Neither patient had autosomal dominant polycystic disease nor a known history of gastrointestinal problems but both proved to have underlying, previously unsuspected colonic diverticular disease with abscess formation.

Abscess

Salt losing nephropathy simulating congenital adrenal hyperplasia in infants with obstructive uropathy and/or vesicoureteral reflux--value of ultrasonography in diagnosis.

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.

Adrenal Hyperplasia, Congenital

Cystic adrenal masses in the neonate associated with hemihypertrophy and the relation to the Beckwith-Wiedemann syndrome.

We report 2 cases of unilateral, multilocular, cystic adrenal masses in neonates. Both patients demonstrated somatic hemihypertrophy, which is considered to have the same implications as the Beckwith-Wiedemann syndrome in terms of tumor risk. Pathological examination showed multiple, adrenal cysts without evidence of tumor or routine adrenal hemorrhage. This finding appears to represent a previously unreported association between the spectrum of hemihypertrophy/Beckwith-Wiedemann syndrome and benign cystic adrenal disease.

Adrenal Gland Diseases

MR imaging of diffuse bone marrow replacement in pediatric patients with cancer.

In the magnetic resonance (MR) imaging examinations of three children with tumors (two neuroblastoma, one rhabdomyosarcoma) and three with leukemia, the marrow demonstrated a diffuse, uniform pattern of hypointensity on T1-weighted images and hyperintensity on T2-weighted images. The authors observed that this reversal ("flip-flop") of the usual MR characteristics of fatty marrow was seen in the epiphyses, metaphyses, and diaphyses. The purpose of this study was to establish the radiographic and clinicopathologic correlates of this MR finding on the basis of findings from plain radiographs, bone scans, and bone marrow aspirates. Plain radiographs and bone scans demonstrated either normal findings or changes limited to the metaphyses. In all patients, analysis of bone marrow aspirates demonstrated metastases. The authors concluded that even in the absence of evidence of discrete bone metastases on a plain radiograph or a bone scan, this diffuse and uniform "flip-flop" pattern reflects diffuse marrow replacement by tumor cells.

Bone Marrow

Parotid enlargement in children seropositive for human immunodeficiency virus: imaging findings.

We investigated the clinical, immunologic, and imaging features of parotid enlargement in 10 children seropositive for human immunodeficiency virus (HIV) who were infected prenatally. In seven patients, sonography revealed many small hypoechoic areas suggestive of lymphoid infiltration. In three older children, large anechoic areas suggesting lymphoepithelial cysts were present. The lesions probably represent generalized lymphoid infiltration akin to pulmonary lymphoid hyperplasia, although only two of our patients had radiologic evidence of pulmonary lymphoid hyperplasia. Four of five of the children with parotid enlargement who had immunologic studies were phenotypically HLA-DR5, an immunophenotype associated with improved immune response, occurring in less than one in four in the normal population. This suggests that painless parotid enlargement may be a good prognostic sign in HIV-seropositive children. Parotid enlargement was found in 10 HIV-positive children from 6 months to 11 years old. All except one were infected prenatally.

Antibodies, Viral

Benign hemorrhagic adrenocortical macrocysts in Beckwith-Wiedemann syndrome.

A new pseudotumorous lesion found in the adrenal cortex of six infants with Beckwith-Wiedemann syndrome is described. These cystic masses were discovered either prenatally by using sonography or early in the neonatal period as palpable flank masses. Imaging studies, including sonography and CT, could not confidently exclude malignancy. After the masses were removed surgically, histologic examination showed them all to be benign hemorrhagic macrocysts within the capsule or permanent cortex (in contrast to neonatal adrenal hemorrhage, which usually occurs more centrally in the fetal cortex). The cysts were as large as 8 cm in diameter, and in one case a solitary cyst was predominant. Hemihypertrophy was present in all cases. Four of the six lesions were right-sided, and there was a male-female ratio of 5:1. Benign hemorrhagic adrenocortical macrocysts are a cause of abdominal mass in the fetus and neonate with Beckwith-Wiedemann syndrome.

Adrenal Cortex Diseases

Contemporary imaging approach to pediatric urologic problems.

This article has tried to do two things: review the more commonly used imaging modalities and apply them to the topics of obstruction and reflux. Increasingly, sonography is the initial diagnostic tool, obviously the only one usable in utero. The VCUG is the next most important method, especially in working out the often confusing sonographic images of the upper urinary tracts. Nuclear medicine remains drastically underused; it is the best way to detect parenchymal inflammatory disease and offers a method of examination and follow-up that requires low dosages and allows quantification of data. It will be interesting 10 years from now to review this subject and see what remains of the old and current methods and what will emerge.

Child, Preschool

Congenital pyriform sinus fistula: a cause of acute left-sided suppurative thyroiditis and neck abscess in children.

Acute bacterial thyroiditis or neck abscesses in children can be caused by infection through pyriform sinus fistulae which usually originate from the tip of the left pyriform sinus. They are thought to be remnants of either the third or fourth pharyngeal pouches. CT, ultrasound and gastrointestinal contrast studies are all useful in clarifying the pathological process and showing the extent.

Abscess

Therapeutic and diagnostic implications of acquired choledochal obstruction in infancy: spontaneous resolution in three infants.

Three infants aged 2 days to 11 weeks with conjugated hyperbilirubinenemia, had sonographically documented dilated common hepatic bile ducts, and echogenic material in the gallbladder. A 2-day-old infant, born to a diabetic mother, had none of the classic predisposing factors for cholelithiasis, and two infants had received total parenteral nutrition (TPN) and TPN plus furosemide. The first infant after receiving 4 1/2 weeks of TPN and furosemide, developed common duct obstruction with increasing bilirubin and hepatic duct caliber over a 12-day period. An operation was scheduled; however, on the following day the bilirubin dropped abruptly and surgery was canceled. The experience with this infant encouraged conservative management in two subsequent infants with similar clinical and sonographic findings. Spontaneous resolution occurred 9 days after the onset of common duct obstruction in one infant and after 16 days in the other.

Cholelithiasis

Anatomic and functional abnormalities of the esophagus in infants surviving congenital diaphragmatic hernia.

As more infants with congenital diaphragmatic hernia (CDH) survive by means of extracorporeal membrane oxygenation (ECMO), new clinical observations are being made. We report anatomic and functional abnormalities of the esophagus in these infants. Twenty-two of 30 infants with CDH survived. Three of 7 non-ECMO-treated infants and 14 of 17 ECMO-treated infants had an air- or fluid-filled mediastinal mass that was confirmed by an upper gastrointestinal series as ectatic esophagus. Contrast studies demonstrated severe gastroesophageal reflux. Intra-esophageal pH monitoring confirmed significant reflux (Euler scores greater than 50) in 69% of tested patients. Prolonged acid clearance in most patients implied abnormal peristalsis. Hydramnios was correlated to the observed esophageal abnormalities, being present in 13 of 16 infants with an available amniotic history. All patients had symptomatic gastroesophageal reflux managed without antireflux surgery and were discharged 36 +/- 2 days after initiation of feeding. Follow-up (range: 7 to 56 months; mean: 32 months) showed that weight gain in affected children was slower than in those not affected. All but one are asymptomatic. Repeat upper gastrointestinal series shows persistent ectasia but less gastro-esophageal reflux. Gastroesophageal reflux/ectasia is a new observation with CDH. The associated hydramnios may result from proximal foregut obstruction by kinking the gastroesophageal junction with CDH. The radiographic appearance can be quite unusual. Clinical manifestations of gastro-esophageal reflux are manageable without surgery but may account for the observed compromised growth.

Dilatation, Pathologic

Congenital neuroblastoma: evaluation with multimodality imaging.

The authors retrospectively evaluated 12 patients with congenital (neonatal) neuroblastoma to assess the utility of newer imaging modalities. Findings at prenatal ultrasound (US), performed in four patients, were nonspecific (hydramnios and hydrops fetalis) in two and consistent with a suprarenal mass (one solid, one cystic) in the other two. Postnatal US helped accurately detect adrenal tumors (solid or complex, with one exception) but was less accurate in the diagnosis of metastatic disease to the liver. Computed tomography accurately depicted all primary tumors and liver metastases. Magnetic resonance (MR) imaging helped establish the correct diagnosis in three patients. This study again confirmed the benign course of neonatal neuroblastoma, with 50% of the patients classified with stage IV-S disease and two deaths occurring in the series, both due to complications. Therefore, aggressive diagnostic imaging is less desirable, and US is therefore very useful, despite its limitations. The prenatal detection and solid appearance of a suprarenal mass makes the diagnosis of neuroblastoma very likely, as does the presence of liver lesions. In the absence of these characteristic findings, US should be repeated to exclude adrenal hemorrhage. MR imaging seems to be a good alternative in some instances.

Adrenal Gland Neoplasms