Congenital unilateral pulmonary venous atresia: definitive diagnosis and treatment.

Three cases of unilateral right-sided pulmonary venous atresia were evaluated over an 18-year period. These bring the total number of cases to 25 in the literature. The clinical presentation of all these patients was similar and consisted of recurrent pulmonary infections, asthma-like symptoms, and exercise intolerance. The patients presented in 1982 (patient 1, a 12-year-old boy), 1994 (patient 2, a 9-year-old girl), and 1999 (patient 3, a 13-year-old boy). All patients were evaluated with a chest roentgenogram, and patients 1 and 2 had a ventilation and perfusion scan. Patients 1 and 3 also had cardiac catheterization and pulmonary angiography. Patient 2 had a magnetic resonance imaging study of the chest. Only patient 3 had wedge pulmonary angiography. Although a rare congenital defect, this diagnosis should be strongly suspected based on the typical clinical presentation and the preliminary studies, such as the chest roentgenogram and ventilation and perfusion scan. However, for definitive diagnosis, cardiac catheterization with wedge pulmonary angiography is necessary. Anastomosis of the atretic pulmonary veins to the left atrium is a theoretical consideration. However, this may not be feasible due to pulmonary venous anatomy or significant pulmonary dysfunction with pulmonary vascular changes. In these circumstances, we recommend performing pneumonectomy to remove the nidus for repeated bouts of pulmonary infections, to eliminate the left-to-right shunt, and to eliminate the dead space contributing to exercise intolerance.

Radiologic interpretation of congenital heart disease.

Radiology in the evaluation of congenital heart disease has changed over the years but still has an important role to play. Although we can rarely make the definitive diagnosis of the intracardiac abnormalities, we can direct the clinicians' attention when cardiac disease is unsuspected and we can assist in the evaluation of complications of the disease or its treatment.

Multiple pelvoureteric diverticulosis in a 1-month-old infant with a del(10p) chromosomal abnormality presenting with UTI and VUR.

A 1-month-old girl with a del(10p) chromosomal abnormality presented with a febrile urinary tract infection. Radiographic voiding cystourethrography demonstrated bilateral vesicoureteral reflux and multiple pelvoureteric diverticula ranging in size from 2 to 5 mm. Pandiverticulosis of the urinary tract in a child of this age has not been previously reported.

A comparison of the radiographic interpretation skills of pediatric transport nurses and pediatric residents.

OBJECTIVE: To compare the radiograph interpretation skills of pediatric/neonatal transport nurses to those of 3rd-year pediatric residents. DESIGN: A validation study of radiograph interpretation. METHODS: Twelve pediatric transport nurses and nine senior pediatric residents were asked to interpret 20 radiographs or sets of radiographs. These films had been previously selected and interpreted by a panel of pediatric radiologists. The subjects' interpretations were compared with those of the radiologists, and a score was assigned for each film or set of films. Five points were awarded for an interpretation in complete agreement with the radiologists' interpretation; 2.5 points were awarded for partial agreement. No points were awarded for complete disagreement. Subjects also answered questions about prior training and experience in radiograph interpretation. RESULTS: The 3rd-year residents' mean score was 66.34 (range 57.5-82.5), while the nurses had a mean score of 33.75 (range 17.5-47.5). This difference was significant (P < 0.001). The residents had a mean of 133.33 of formal radiology instruction, while the nurses had less than 10 hours of instruction. The nurses had received focused training in the identification of pneumothoraces. The nurses had higher mean scores than the residents on the two sets of films demonstrating pneumothoraces, but this difference was not significant. CONCLUSIONS: The 3rd-year residents had significantly higher mean scores on a test of radiograph interpretation. The major difference between the groups appears to be the amount of formal training afforded the residents. The nurses' performance on the films demonstrating pneumothoraces suggests that focused training may be an effective means by which to gain skills important to transport.

Cardiopulmonary and thoracic tumors of childhood.

Cardiopulmonary and thoracic tumors in children are a diverse group of neoplasms. Unfortunately, presenting signs and symptoms are nonspecific. Imaging determines the anatomic site of origin, characterizes the mass, and defines its extent. This article reviews the appearance of cardiopulmonary and thoracic malignancies of childhood utilizing routine radiography, CT scan, MR imaging, and echocardiography.

Contrast enema findings of fibrosing colonopathy.

PURPOSE: Our objective was to describe characteristic contrast enema findings of fibrosing colonopathy. MATERIALS AND METHODS: We performed barium enemas on 18 children with cystic fibrosis who had received greater than 4500 U of lipase per kg body weight per meal and who had distal intestinal obstruction syndrome unresponsive to medical management. RESULTS: Fifteen patients had abnormal enema results. The most common findings included stricture, loss of haustra, and colonic shortening. Ten patients required surgery, nine underwent colon resection, and one had lysis of adhesions. Pathologists confirmed fibrosing colonopathy in eight of nine speci- mens. CONCLUSION: Colonic stricture, abnormal haustra, and longitudinal shortening are characteristic barium enema findings of fibrosing colonopathy.

Further delineation of spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type, with emphasis on diagnostic features.

Further delineation of a generalized bone dysplasia which we call spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type is presented. This dwarfing condition has several serious complications, with the most common cause of death being spinal cord damage secondary to atlantoaxial instability. It is a heritable condition with an autosomal recessive mode of transmission. Radiologic diagnostic criteria are developed on the basis of studies in 8 patients with the oldest being between 4 and 5 years old. The condition is clinically and radiographically apparent neonatally or in early infancy, and it is probable that all or almost all affected individuals will come to medical attention in the age range screened by this study.

Improvement of cortical morphology in infantile hydrocephalic animals after ventriculoperitoneal shunt placement.

As a sequel to our previous descriptions of the pathological changes induced by hydrocephalus in the infantile cerebral cortex, the study presented here has evaluated the effects of surgical decompression on cortical cytology and cytoarchitecture. Hydrocephalus was induced in 14 kittens by the intracisternal injection of kaolin at 4 to 11 days of age. Nine of these hydrocephalic animals received low-pressure ventriculoperitoneal shunts at 9 to 15 days after kaolin injection; these animals were monitored preoperatively and postoperatively by ultrasound and were killed at various postshunt intervals up to 30 days. Five normal or saline-injected animals served as age-matched controls. At the time of shunt placement, the ventricular index confirmed that all recipient animals had attained moderate or severe degrees of ventriculomegaly. Within 3 days after shunt placement, the size of the lateral ventricles had decreased to control levels and was accompanied by rapid and dramatic improvements in behavior and skull ossification. When the animals were killed, gross inspection revealed that about half of the animals exhibited mild to moderate ventriculomegaly, with cortical mantles 50 to 80% their normal thickness. Tissue from frontal (primary motor), parietal (association), and occipital (primary visual) cortical areas was processed for light microscopic analysis. Pyknotic or dark shrunken neurons, which are found typically in hydrocephalic brains, were observed only occasionally in the cortex of shunted animals. Gliosis and mild edema were prevalent, however, in the periventricular white matter. The laminae of the cerebral cortex could be identified in all shunted animals. In those animals with mild residual ventriculomegaly, the entire cortical mantle was somewhat compressed, as evidenced by an increased packing density of neurons. Furthermore, the somata of some neurons were disoriented. Overall, these results indicate that most of the morphological characteristics of the cerebral cortex are preserved after surgical decompression and suggest that ventriculoperitoneal shunts may prevent neuronal damage and/or promote neuronal repair.

Combined liver-kidney transplantation in a child with primary hyperoxaluria.

A 3.5-year-old boy presented with end-stage renal disease and bilateral nephrocalcinosis. Renal biopsy demonstrated marked parenchymal calcium oxalate deposition and a diagnosis of primary hyperoxaluria (PH) was made. Following 2 years of hemodialysis he received two renal allografts which were lost at 7 and 11 months, respectively, due to biopsy-proven recurrent oxalosis. Combined liver-kidney transplantation was then performed, after which renal and hepatic function initially stabilized. The patient died on the 28th postoperative day, of infectious complications and progressive respiratory insufficiency. However, comparisons between the patterns of urinary oxalate excretion noted after the isolated renal and liver-kidney transplants indicated that, following the latter, successful biochemical correction of the enzyme defect responsible for type 1 PH had occurred.

Cytological and cytoarchitectural changes in the feline cerebral cortex during experimental infantile hydrocephalus.

While many reports have documented the effects of hydrocephalus on the ependyma and periventricular white matter, primarily in adult animal models, little is known about alterations specific to neurons. The present study has evaluated qualitatively the effects of hydrocephalus on the neurons and vasculature of the cerebral cortex in a neonatal animal model. The cisterna magna of 4 to 11-day-old kittens was injected with a solution of 25% kaolin to induce hydrocephalus. Ultrasonographic evidence of hydrocephalus was noted within 3-5 days of injection. Hydrocephalus progressed until day 18-25 postinjection when the animals were sacrificed. The cytologic and cytoarchitectural changes within the cortical mantle of affected animals were compared with control age-matched counterparts who had undergone intracisternal saline injections. Areas 4 (primary motor), 22 (association) and 17 (primary visual sensory) were examined light microscopically. Neurons from hydrocephalic brains exhibited 3 types of pathological response. Pyknotic somata were shrunken, disoriented and so hyperchromatic that neither nuclei or nucleoli could be delineated. Reactive somata were also shrunken and hyperchromatic, but nuclei and nucleoli could still be observed. Many neurons contained an abundance of vacuoles, giving their somata a flocculent appearance; these cells were termed 'spongy' neurons. Both normal and pathological neurons were smaller and disoriented, with a considerable decrease in neurons noted in areas 22 and 17 from severely hydrocephalic animals. The deeper cortical layers were more affected than the more superficial laminae in that more reactive and pyknotic neurons were present in layers V and VI. As the ventriculomegaly became more severe, changes could be observed in neurons within layers II and III. Furthermore, the cerebral vasculature exhibited a decrease in the number of vessels and a preponderance of profiles oriented parallel to the meningeal surface. The severity of these effects followed a rostral to caudal gradient, such that the occipital cortex demonstrated the most damage. These results suggest that both the motor deficits and the subtle cognitive deficiencies seen with hydrocephalus may be attributed to perturbation of neuronal and vascular elements in the cerebral cortex.

Pyomyositis in a child with acquired immunodeficiency syndrome. Patient report and brief review.

Although common in tropical regions, pyomyositis is rare in the continental United States. Fewer than 50 cases have been reported to date. It is characterized by suppuration of large muscle groups that can, if not quickly and appropriately treated, lead to sepsis and death. Diagnosis can be difficult secondary to the atypical appearance of the abscess process early on. Almost all cases have occurred in otherwise healthy people. The simultaneous occurrence of pyomyositis and immunodeficiency is rare. A recent report of a case in an adult with the acquired immunodeficiency syndrome (AIDS) is not, however, unexpected. We describe the first documented occurrence of pyomyositis in a child with AIDS. A brief review of the topic is included. Pyomyositis should be included in the list of unusual infections that can occur in children with AIDS.

Effects of hydrocephalus and surgical decompression on cortical norepinephrine levels in neonatal cats.

Norepinephrine (NE) changes during hydrocephalus, and the effects of surgical decompression on these changes, were studied using a new model of neonatal hydrocephalus. Kittens 4 to 10 days old received intracisternal injections of a sterile solution of 25% kaolin. Control kittens were injected similarly with sterile injectable saline. Ultrasonography was used to follow the progression of ventriculomegaly and the initial effects of the shunts. A subgroup of hydrocephalic animals was shunted using a cerebrospinal fluid lumbar-peritoneal catheter. Hydrocephalic animals were killed at approximately 25 days of age (16-21 days after kaolin injection). Surgical decompression was performed at 12, 16, and 17 days after kaolin injection; these animals were killed 30 days after the shunts were inserted. Control animals were killed at 29 and 53 days of age, to correlate with the ages of the hydrocephalic and shunted animals, respectively. Cortical samples equivalent to Brodmann's areas 4, 22, and 17 were measured for NE using high-performance liquid chromatography. Hydrocephalus caused NE levels to decrease significantly in all cortical areas. These alterations followed a rostrocaudal gradient in severity, with mean reductions of 65.8, 83.9, and 95.8% in areas 4, 22, and 17, respectively. Partial recovery occurred in animals that received shunts 16 and 17 days after kaolin injection, such that NE reductions of 75.7, 56.2, and 81.6% were noted in areas 4, 22, and 17, respectively. Shunting at 12 days after kaolin injection produced complete recovery in areas 4 and 22, with only a 67.7% decrease in area 17. These results suggest that the projection fibers from the locus ceruleus are damaged by the direct effects of hydrocephalus. Axotomy or neuropraxia of these fibers could result in decreases in NE throughout the cerebral cortex. In addition, there appears to be a period of time during which surgical decompression will allow neuropraxic fibers to recover with partial restoration of NE levels. Earlier insertion of a shunt appears to allow for more recovery than later decompression.

Lipid aspiration pneumonia due to gastroesophageal reflux. A complication of nasogastric lipid feedings.

The development of lipid aspiration pneumonia after chronic nasopharyngeal installation of mineral oil was first described in 1925 by Laughlin. Since that time this entity has been well recognized and numerous substances have been identified or implicated as the aspirated material. The classic radiographic appearance of severe chronic lipid aspiration pneumonia has been described as consisting of intense perihilar infiltrates. However, the radiographic findings are more often non-specific and usually consist of varying degrees of diffuse interstitial infiltrates that tend to be more prominent in the perihilar regions and the right lung. We are reporting a case of biopsy-proven lipid aspiration pneumonia in an infant with known gastro-esophageal reflux (GER) who had medium-chain triglyceride oil administered via nasogastric tube. Serial roentgenograms demonstrated a changing pulmonary pattern from diffuse bilateral interstitial infiltrates initially to a diffuse alveolar pattern at the time of the lung biopsy. Modern medicine has developed new methods for providing nourishment to sick newborns and infants to improve their nutritional status and help them to grow. One such method involves the administration of medium-chain triglycerides (MCT oil) into the GI tract via a nasogastric or nasoenteral tube. The purpose of this report is to describe a significant complication of this method of providing nutrition to an infant with gastroesophageal reflux (GER) and the diagnostic dilemma it presented.

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.

The "keyhole": a sign of herniation of a trapped fourth ventricle and other posterior fossa cysts.

When a cystic structure in the posterior fossa increases in size, the accompanying increase in pressure may cause it to herniate upward through the tentorial hiatus. In our experience this happens most commonly with a dilated trapped fourth ventricle secondary to infection or intraventricular hemorrhage. However, herniation of an arachnoid cyst or a Dandy-Walker malformation through the tentorium may also occur. When herniation occurs, the cystic structure assumes a "keyhole" configuration, indicating that it is trapped and that surgical intervention is necessary. Five cases are presented that illustrate this point, including two patients with dilatation of the fourth ventricle secondary to hemorrhage, two patients with Dandy-Walker malformation, and one patient with an arachnoid cyst.

The wide spectrum of renal osteodystrophy in children.

When chronic renal failure was a routinely fatal condition little attention needed to be paid to the recognition and management of the associated bone disease. The improved medical and surgical management have prolonged the lives of children with chronic renal disease, but in so doing it has also changed the nature of their biochemical environment. These two factors contribute to an increase in the spectrum of abnormalities we see in the bones of children with renal failure, some of which are still unexplained. It has become even more important for the radiologist to recognize subtle as well as overt alterations in the bony architecture because these will influence the clinical management. This is especially true in children because of the severe growth impairment as well as the bony deformities that result from long-standing renal disease.

Chest masses in children: the plain film revisited.

Mass lesions in the chest can usually be best evaluated by conventional means, primarily plain chest film examination. Fluoroscopy is often useful, especially to differentiate normal thymus from abnormal anterior mediastinal masses and to evaluate possible vascular masses. It makes sense to do a barium swallow for most middle mediastinal masses before resorting to the more expensive CT or MRI. In addition to the added expense, the marked increase in radiation dose should also be considered when CT is contemplated.