Association of Down syndrome and segmental tracheal stenosis with ring tracheal cartilages: a review of nine cases.

Four patients with Down syndrome and midtracheal stenosis, three with proven absence of the midtracheal pars membranacea ("hourglass trachea"), are reported. Five previously reported patients who had Down syndrome and tracheal stenosis of this type are summarized. Respiratory difficulty and stridor were the reported clinical features of all but one of the patients whose clinical story is available. That approximately half the patients with tracheal stenosis with hourglass trachea and midtracheal absence of the tracheal pars membranacea reported had Down syndrome suggests that the association of this pattern of congenital tracheal stenosis with Down syndrome is, although infrequent, significant.

Left-lobe hepatic transplants: spectrum of normal imaging findings.

The limited availability of suitable size-matched donor whole organs has been a major cause of morbidity and mortality in infants and children requiring liver transplantation. Techniques for transplanting segments of the liver have successfully addressed this problem. The surgical anatomy of the left hepatic lobe transplant is unique in three primary respects: the cut edge, the presence of an enteric Roux loop for biliary drainage, and the alteration in the position and number of hepatic vessels. The spectrum of normal imaging findings in 17 left-lobe allografts is illustrated.

Hepatobiliary scintigraphy in pediatric liver transplant recipients.

The clinical usefulness of hepatobiliary scintigraphy was evaluated in pediatric liver transplant recipients. One hundred fifteen hepatobiliary scintigraphic studies were performed in 30 patients who received 22 whole liver and 16 segmental grafts. Parameters that were useful in predicting an adverse outcome were failure to visualize excreted radiopharmaceutical at or beyond the biliary anastomosis on a study performed within 24 hours after transplant, and persistent or increasing delay in the time of visualization of excreted radiopharmaceutical. Abnormalities of liver uptake and excretion were seen in rejection, but they were also seen in patients who remained well without rejection or parenchymal disease. Significant biliary leaks were identified in the three cases in which they were known to be present. In liver transplant recipients, hepatobiliary imaging is useful in predicting graft survival and identifying biliary leaks.

Magnetic resonance imaging of the pediatric abdomen.

In many ways, MRI of the pediatric abdomen is in its infancy. Motion will be a problem with most of the current pulse sequences, but the problem appears manageable given many of the newer software options for motion suppression. Faster pulse sequences (approaching CT scan times) will serve to improve image quality and reduce the need for sedation. Identification of a reliable enteric contrast agent probably will expand the current utility of MRI beyond evaluation of solid abdominal viscera. Although preliminary results appear promising, controlled comparative studies are necessary to determine the exact role of MRI in diagnosis and staging of common pediatric abdominal malignancies. The addition of MRI intravenous contrast agents and MR spectroscopy are anticipated to add depth and specificity to the diagnostic potential of this exciting modality.

MR imaging of double chambered left ventricle.

Double chambered left ventricle is a very rare anomaly within the broader category of "subdivision of the left ventricular cavity," which includes the relatively more common congenital aneurysms and diverticulae. We report a case of an adolescent with double chambered left ventricle that MR clearly delineated, resulting in successful surgical correction.

Magnetic resonance imaging of pediatric atraumatic musculoskeletal lesions.

MRI has become a useful study for evaluating the musculoskeletal system. This article discusses the technique of pediatric musculoskeletal MRI and its practical applications. MRI appearances of various pathologic entities, including infection, neoplasms, marrow disorders, and joint diseases, are also reviewed.

MR imaging of early fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva is a rare disorder characterized by progressive heterotopic bone formation in connective tissue and muscle in association with congenital skeletal anomalies. The disease usually leads to extensive immobility and eventual disability. We report the magnetic resonance findings in a boy prior to ossification of his soft tissue masses.