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R enal medulla carcinoma is a recently described rare, aggressive tumor of the kidney occurring in patients younger than those in whom renal cell carcinoma occurs. To our knowledge, renal medulla carcinoma has been reported only in individuals of African descent carrying the sickle cell trait or afflicted by hemoglobin SC disease [1, 2]. We report what is, to our knowledge, the first case of renal medulla carcinoma occurring in a white child without a history of sickle cell disease or trait.

The case presented is of an infant who developed a radial artery pseudoaneurysm following arterial puncture and was subsequently diagnosed with hemophilia. A discussion of radial artery pseudoaneurysms follows.

Background: Otolaryngologists and anesthesiologists have described a maturational descent of the epiglottis that occurs in infancy and childhood. Objective: To investigate the changing level of the epiglottis to confirm and characterize this phenomenon more completely. Design: A survey of 500 images with 338 images selected for the study. Setting: A tertiary care facility. Patients: Asymptomatic children aged 1 day to 18 years. Main Outcome Measure: The position of the tip of the epiglottis was correlated with the cervical vertebral level. Results: Data indicate that maturational descent of the epiglottis starts in infancy and continues into adolescence. These results are statistically significant ( P Conclusion: Maturational descent of the epiglottis occurs in a predictable pattern. Understanding this phenomenon may facilitate laryngoscopic, as well as clinical and radiologic, evaluation of the airway in children. Arch Otolaryngol Head Neck Surg. 1997;123:627-628

We studied a patient with Klippel–Feil anomaly and subclavian steal due to isolation of the right subclavian artery. Other anomalies included hypoplasia of the right clavicle and right vertebral artery, low bifurcation of the left common carotid artery and left choanal atresia. The patient was mentally retarded with corticospinal, cerebellar, and brain stem signs. The entire brain and cervical spinal cord were small; there was no central nervous system malformation, hydroceph-alus, or bony impingement on neural structures. Embryologic vascular disorders have been found to be responsible for various congenital systemic and neurologic anomalies such as intestinal atresia and hydra-nencephaly. Subclavian artery supply disruption sequence has been hypothesized to result in Klippel-Feil, Poland, and Mobius anomalies. In this case the vertebral and facial abnormalities may well be related to anomalous subclavian and carotid supply. An embryonic vascular “steal” has been thought to cause the sirenomelia anomaly. We postulate that the subclavian steal, during the embryonic period, may have been responsible for the small size of this patient's cerebral hemi-spheres, brain stem, cerebellum, and cervical spinal cord.

The Doppler spectrum of the normal inferior vena cava (IVC) shows a majority of flow toward the right atrium, with wide variations in flow velocity and direction owing to effects of the cardiac and respiratory cycles. Seventeen subjects underwent duplex sonography: nine children after liver transplantation, two children with right upper quadrant neuroblastoma, and six healthy control subjects. In six children the spectrum obtained from the IVC bore a strong resemblance to the unidirectional, low-velocity, continuous-flow pattern associated with the portal vein; these children included two children with biliary atresia and cirrhosis, two liver transplant recipients, and two children with IVC compression by neoplasm. In healthy children and adults, a pseudoportal Doppler signal in the IVC can be generated by subcostal right upper quadrant compression. The pseudoportal IVC appears to be a sign of partial IVC obstruction. Knowledge of this potential pitfall and meticulous morphologic imaging can help prevent mistaking the IVC for a patent portal vein.

An initial attempt to reduce a hip dislocation in a 15-month-old by palpation under general anesthesia led to the subsequent discovery of a posterior subluxation by CT. During the second attempt, sonographic guidance in the operating room was used to show concentric reduction both before and after the application of a spica cast. Successful reduction was later confirmed by CT. Imaging by real-time sonography can be useful in monitoring infant hip reduction.

The base of the proximal esophageal pouch in babies born with esophageal atresia varies in location from the low cervical region to the upper third of the mediastinum, but the relevance of this finding to the VATER association is not known. The initial radiographs and hospital records of 42 newborns with esophageal atresia and a distal tracheoesophageal fistula treated in the Newborn Special Care Unit of the Yale-New Haven Hospital between 1977 and 1986 were examined to (1) determine the location of the base of the upper esophageal pouch on its superimposed vertebral body; (2) identify coexisting VATER associated anomalies; and (3) detect the presence of vertebral, rib, or sternal anomalies. Ten of 11 babies with the upper esophageal pouch at the first thoracic vertebra or above had additional VATER defects including vertebral, rib, or sternal anomalies. These patients also required staged repair or circular myotomy for wide gap esophageal atresia and were at greater risk of anastomotic leak, stricture, or gastroesophageal reflux requiring fundoplication. High proximal pouch esophageal atresia accompanied by vertebral, rib, and sternal anomalies constitutes a heretofore unrecognized high risk group with the VATER association.

In recent years, the use of real-time ultrasonography (US) has enabled dynamic evaluation of the infant hip through a range of motion and stress. Preliminary experience has suggested that a certain amount of instability in the hips of newborns is normal, but no standards have been established. In this study, a group of term neonates whose physical examinations were normal were examined with US on the 1st and 2d days of life. Each hip was imaged in the transverse plane in nonstressed and stressed positions, and movement of the femoral head under stress was quantitated. This displacement under stress was used to establish a normal range of hip instability in neonates. Patterns of hip laxity in boys and girls are identical, and in most infants hip instability diminishes between the 1st and 2d days of life. Our method of quantitating hip instability produces consistent results, with intraobserver 95% confidence intervals of +/- 1.2 mm for each measurement.

Sacrococcygeal teratomas are uncommon neonatal tumours occurring in 1 of every 35 000 live births (Altman et al, 1974). Pre-operative examination has traditionally included plain radiography, excretory urography, voiding cystourethrography, contrast enema and occasionally ultrasonography (Schey et al, 1977). Smith et al (1980) have also emphasised the value of arteriography of these tumours. We describe a different approach in which a large sacrococcygeal teratoma was assessed by intravenous digital subtraction angiography (DSA) and direct sagittal computed tomography (CT).

Congenital absence of the portal vein with systemic visceral venous return in a 8-year-old girl with oculoauriculovertebral dysplasia (Goldenhar Syndrome) had been previously reported following its discovery during preoperative evaluation of a liver mass which was diagnosed as focal nodular hyperplasia after open biopsy. Subsequently, an enlarging diffuse hepatic neoplasm developed with associated elevated alpha feto-protein levels. Repeat biopsy and imaging showed the tumor to be a hepatoblastoma involving both lobes of the liver. The patient was treated by hepatic resection and orthotopic liver transplantation and is doing well at 18 months follow-up.

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 authors describe the first case of giant intracranial aneurysm in a child with tuberous sclerosis. The subject of vascular disease in this condition is reviewed.

We recently observed three neonates with transient oliguric acute renal failure associated with characteristic ultrasonographic findings. Their clinical courses, laboratory values, and ultrasonographic findings are discussed. The purpose of this report is to alert those who care for newborn infants to this entity of transient renal dysfunction and its characteristic ultrasonographic findings in the kidney. CASE REPORTS Case 1 A full-term girl weighed 3,770 g at birth and was delivered by vacuum extraction because of shoulder dystocia. Her mother's pregnancy was complicated by premature rupture of membranes of greater than 24 hours' duration and maternal fever with amnionitis at delivery. At birth the baby had a weak cry, decreased tone, and Apgar scores of 6 and 8, at one and five minutes, respectively.

Plastic bronchitis (PB) is a potentially life-threatening disorder found in patients after total cavopulmonary connection (TCPC). Although lymphatic abnormalities have been postulated to play a role in the disease process, to date, the etiology and pathophysiology of this complication remains

Background Feature tracking strain (FTS) is a new technique to evaluate myocardial deformation from routinely acquired cardiac magnetic resonance (CMR) cine images, however, it has not been validated in single ventricle patients. The purpose of this study is to validate FTS against myocardial tagged harmonic phase (HARP) images, considered the reference standard in non-invasive deformation analysis.

Background Existing cardiac magnetic resonance sequences are frequently suboptimal in the measurement of the aortic arch, particularly in the case of arch obstruction. Navigated three-dimensional (3-D) sampling perfection with application optimized contrast using different flip angle evolution (SPACE) is a novel turbo spin echo dark blood cardiac magnetic resonance sequence that yields a robust 3-D dataset and gates in systole in order to demonstrate the maximal aortic caliber. Additionally, it utilizes susceptibility for optimal blood suppression by orienting blood flow in the readout direction, thereby enhancing imaging in the setting of turbulence. We report our experience measuring the aortic arch using this sequence.