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Biomedical subjects

R I Markowitz

Publications and source records attributed to R I Markowitz.

At least 55 records · Page 3Linked to original sources

Congenital lobar emphysema. The roles of CT and V/Q scan.

An infant with congenital lobar emphysema of the left upper lobe presented with unusually severe mediastinal shift and underwent computed tomography (CT) and radionuclide V/Q scans. The V/Q scan confirmed the non-functioning nature of the hyperinflated lobe while the CT scan depicted the abnormal anatomy as well as the normal morphologic characteristics of the remaining lung thus allowing for safe and appropriate surgical management.

Emphysema↗

Trisomy 18 associated with ectopia cordis and occipital meningocele.

A premature infant presented with ectopia cordis, occipital meningocele, and manifestations of trisomy 18; cytogenetic analysis confirmed a 47,XX,+18 chromosome constitution. While most cases of ectopia cordis appear as isolated, sporadic defects, careful evaluation is warranted to detect evidence of an associated chromosome abnormality.

Abnormalities, Multiple↗

Gallbladder opacification in infants following high dose angiocardiography.

Seven infants in congestive heart failure underwent high dose angiocardiography for diagnosis of severe congenital heart disease and subsequently displayed delayed opacification of the gallbladder. Biliary excretion of sufficient volume to opacify the gallbladder occurred despite structurally normal kidneys and no evidence of renal failure. Decreased renal clearance of contrast due to generalized diminution of glomerular filtration is postulated. The high doses of contrast and slow renal clearance allowed a relatively increased rate of hepatobiliary excretion and subsequent observation of the opacified gallbladder on abdominal radiographs. This phenomenon may not be as uncommon as is generally thought but its timing and location often do not allow an opportunity to make this observation.

Angiocardiography↗

Hyperinflation of the lungs in infants with large left-to-right shunts.

Infants with significant left-to-right shunts due to ventricular septal defects and atrioventricular canal defects commonly present with respiratory symptoms, such as shortness of breath while feeding, tachypnea, and wheezing. Radiographs show hyperinflated lungs as well as cardiomegaly and increased vascularity. Enlarged vessels adjacent to small compressible airways as well as peribronchial interstitial edema may cause diffuse air trapping. In this study, using an automated planimetric device, we measured the total thoracic, cardiomediastinal, and lung volumes in a group of patients with large left-to-right shunts as well as in a group of normal controls and found that, as expected, all volumes were significantly increased in the abnormal group. We also tried to correlate these volumes (corrected for patient size) with the degree of left-to-right shunt and found that there was no significant correlation between the cardiac or lung volumes and shunt size as estimated by cardiac catheterization.

Heart Septal Defects↗

Aneurysms of the right ventricular outflow tract after tetralogy of Fallot repair: role of radiology.

Eight patients with aneurysms of the right ventricular outflow tract patch following tetralogy of Fallot repair were found to have residual distal obstructions. The site of such an obstruction must be carefully documented preoperatively because failure to relieve significant distal obstruction may result in recurrent aneurysm formation. Chest radiography; echocardiography, including Doppler analysis; and cardiac catheterization, including angiocardiography, each have a role in the detection and evaluation of this complication.

Angiocardiography↗

The anterior junction line: a radiographic sign of bilateral pneumothorax in neonates.

The anterior junction line is a normal anatomic landmark on chest radiographs of healthy adults and older children caused by the visceral and parietal pleurae of the two lungs meeting anteriorly at the midline. It is not seen on chest radiographs of healthy infants. When this sign is identified on the supine frontal view of a neonate, it signifies bilateral pneumothorax. In this situation the line is formed by the meeting of the medial parietal pleurae on each side as they herniate anteriorly in front of the thymus and heart. The sign is best seen when the patient is rotated slightly to the left. It is not seen when there is unilateral pneumothorax or a concomitant pneumomediastinum. Although the anterior junction line is not a highly sensitive indicator of bilateral pneumothorax, it is highly specific and its recognition can promote faster diagnosis.

Female↗

Single, mediastinal, unilobar lung--a rare form of subtotal pulmonary agenesis.

We describe a case of a full-term infant with severe respiratory failure and pulmonary insufficiency caused by an anomaly consisting of a single, unilobar lung arising from the trachea and situated in the middle mediastinum. Plain film, echocardiogram, and surgical aspects will be described and correlated with the post mortem findings and embryologic considerations. We were unable to find a similar case reported in the literature and conclude that this rare lesion represent an intermediate form between total and unilateral pulmonary agenesis.

Echocardiography↗

Familial apple peel jejunal atresia: surgical, genetic, and radiographic aspects.

Fifty-seven cases of apple peel jejunal atresia have been reported in the English literature. Patients with this anomaly have a high incidence of prematurity (70%), malrotation (54%), short gut syndrome (74%), multiple atresias (15%), complications (63%), and mortality (54%). Mortality has decreased from 63% to 47% since 1970. We report three new cases of apple peel jejunal atresia, including two from one family. Radiographic evidence of high small bowel obstruction and a malrotated microcolon on preoperative roentgenogram with barium enema should suggest this diagnosis. Five families, including ours, have been reported in which more than one child was affected, and it has been suggested that this disorder is transmitted by an autosomal recessive gene. However, the occurrence of conventional intestinal atresia in other siblings, the association with multiple atresias, and discordance in a set of apparently monozygotic twins indicate that there may be a more complex spectrum of genetic transmission. Subsequent siblings are at increased risk for apple peel atresia or related malformations. Prenatal ultrasound can facilitate early diagnosis and treatment.

Anastomosis, Surgical↗

Appendicitis in children. Accuracy of the barium enema.

The barium enema (BE) may be useful in the diagnosis of atypical appendicitis in children. We analyzed our experience with 18 children in whom appendicitis was suspected and BE was performed. All of the children underwent surgical exploration. Nonfilling of the appendix with cecal indentation, extravasation of barium from the appendix, or both, were considered positive signs of an inflamed appendix on BE. Using these criteria, 12 of 14 cases of proved appendicitis were true positive and two were equivocal. Four children were proved not to have appendicitis; one of these patients had a true-negative BE, two had equivocal BEs, and there was one false-positive BE (Schönlein-Henoch purpura). Extravasation of barium into the peritoneal cavity was noted in one patient; this was a rare complication.

Adolescent↗

Bronchial compression by a patent ductus arteriosus associated with pulmonary atresia.

Two infants with pulmonary atresia are presented, wherein a large, vertically oriented patent ductus arteriosus, acting as the only source of pulmonary blood supply, compressed the left bronchus, causing significant respiratory distress. Hyperinflation of the left lung was the clue to this circumstance. After bronchographic confirmation, surgical repair was accomplished by placement of aortopulmonary shunts and division of the patent ductus. Other causes of airway compression in congenital heart disease are briefly reviewed. The embryology of the ductus arteriosus is discussed, emphasizing its anatomic configuration and physiologic role in pulmonary atresia.

Airway Obstruction↗

Communicating hydrocephalus secondary to superior vena caval obstruction. Occurrence after Mustard's operation for transposition of the great arteries.

Communicating hydrocephalus was observed on computed tomographic (CT) scans in two infants who had previously undergone Mustard's operation for transposition of the great arteries. Obstruction of the superior limb of the intracardiac baffle developed in these children, resulting in venous obstruction to the head and upper extremities that resulted in cranial enlargement. Radiologic clues included a bulging superior vena cava and a prominent azygous system on chest roentgenogram, split cranial sutures on skull films, and enlarged subarachnoid spaces and dilated ventricles on CT scans. Two-dimensional echocardiography showed the obstructed baffle, as well as signs of redirected blood flow. Angiography and measurement of venous pressures confirmed the obstruction, and surgical correction was successful. Follow-up CT scans showed resolution of the hydrocephalus. Increased venous pressure, leading to decreased CSF absorption and increased intraventricular and subarachnoid CSF accumulation in an infant with an expandable cranium, is a possible explanation for the development of cranial enlargement and hydrocephalus under these circumstances.

Echocardiography↗

A preoperative x-ray scoring system for risk assessment of newborns with congenital diaphragmatic hernia.

The survival rate for newborn infants with congenital diaphragmatic hernia (CDH) is about 50%. The preoperative x-rays of 34 babies with CDH, presenting during the first 12 hours of life were reviewed to determine whether or not the 16 survivors (47%) might be identified. A scoring system using five roentgen findings having a significant correlation with survival (side of diaphragmatic hernia, location of stomach, presence of pneumothorax, relative volume of aerated ipsilateral and contralateral lung) were summed to obtain a total x-ray score. Cumulative scores ranged from 2 to 9 with 4 of 16 survivors (25%) and 16 of 18 (89%) non-survivors scoring above 6. Twelve of 16 (75%) survivors and 2 of 18 non-survivors (11%) (P less than 0.005), scored 6 or less. Individual x-ray findings were less specific in predicting outcome than the total score. Careful examination of the preoperative chest x-ray may give the surgeon an additional method for predicting outcome following repair of CDH during the first 12 hours of life.

Hernia, Diaphragmatic↗

Hirschsprung disease: accuracy of the barium enema examination.

To determine the relative accuracy of the various radiologic signs of Hirschsprung disease (HD), we retrospectively reviewed both radiographs obtained after a barium enema and the medical records of 62 children who had surgery to prove or exclude the diagnosis of HD. The visualization of a rectosigmoid transition zone was highly predictive of HD, but nonvisualization did not rule out HD. A false positive transition zone at the splenic flexure was seen in four neonates who had small left colon syndrome rather than HD. Retention of barium seen on radiographs obtained 24 hours after a barium enema was not a specific sign, but it was the only sign of HD in seven neonates, including two who had total colonic aganglionosis. Anal manipulation prior to the barium enema examination did not affect the diagnostic value of that procedure. We conclude that the single most reliable radiographic sign of HD is the presence of a rectosigmoid transition zone. Statistically, the use of three radiographic features combined (rectosigmoid transition zone, retention of barium, and stool mixed with barium) correlated better with the presence or absence of HD than did any of these features alone. A comparison of 24 and 48 hour postevacuation radiographs may help to differentiate HD from meconium plug syndrome.

Barium Sulfate↗