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Biomedical subjects

R B Glass

Publications and source records attributed to R B Glass.

At least 19 recordsLinked to original sources

The role of magnetic resonance cholangiography in the management of children and young adults after liver transplantation.

We reviewed the results of 50 magnetic resonance (MR) cholangiograms to evaluate their usefulness in directing clinical management in young patients after liver transplantation (LTx). Thirty-two patients underwent 50 MR cholangiograms on a 1.5-T unit. Studies were performed from 1 week to 16 yr after LTx. Indications included biochemical abnormalities with (n = 19) or without (n = 16) biopsy evidence for chronic rejection, sepsis (n = 14), and intractable ascites (n = 1). Original interpretations were compared to laboratory and ultrasound findings, and clinical outcome. Of 19 studies performed on 14 patients with biopsy evidence of chronic rejection, 16 were abnormal on MR (but only one was abnormal on ultrasound), resulting in corrective surgery (n = 1), re-Tx (n = 1), and endoscopic dilatation (n = 1). Of 16 studies on 16 patients with biochemical abnormalities without evidence of chronic rejection on biopsy, 14 were abnormal on MR (but only five of 13 on ultrasound), leading to corrective surgery (n = 3) and re-listing for Tx (n = 3). Thirteen of 14 studies on six patients with sepsis were abnormal on MR (five of nine were abnormal on ultrasound), identifying surgically correctable strictures (n = 2), and leading to re-Tx (n = 1) and percutaneous biliary drainage procedures (n = 2). The one patient with ascites had a normal study. We advocate usage of MR cholangiography for the detection of biliary complications after LTx, particularly in those patients who present with biochemical abnormalities that are not easily explained by acute cellular rejection or viral infection and in those with biliary sepsis.

Adolescent↗

Radiologic changes in infancy in McKusick cartilage hair hypoplasia.

Cartilage hair hypoplasia (CHH), or metaphyseal dysplasia McKusick type, classically comprises short stature and scant fine hair. In this skeletal dysplasia there is a high incidence of immune deficiency and Hirschsprung disease, as well as a higher rate of malignancy. Clinical findings may be subtle in young children, and radiographic changes may be elusive. We present four children below age 2 in whom the clinical diagnosis of CHH was confirmed radiographically. We emphasize radiologically and clinically discernable anterior angulation of the entire sternum, a sign not previously described in this dysplasia.

Cartilage↗

MR cholangiography in children and young adults with biliary disease.

OBJECTIVE: Our objective was to describe the MR cholangiography findings for young patients with suspected biliary disease who underwent half-Fourier acquisition fast spin-echo technique with respiratory triggering. SUBJECTS AND METHODS: Twenty-eight MR cholangiography studies were performed in 22 patients on a 1.5-T MR unit. Ten of these 22 patients had undergone liver transplantation. RESULTS: MR cholangiography revealed abnormalities of both the extrahepatic and the intrahepatic major and minor bile duct systems, despite the small diameter of the duct system in this group of patients. Four patterns of biliary disease were shown: global dilatation of extrahepatic or intrahepatic ducts (n = 7); segmental, uniform dilatation of central or peripheral intrahepatic ducts (n = 9); segmental, nonuniform dilatation of central or peripheral intrahepatic ducts (n = 2); and fusiform ectasia with segmental, irregular intrahepatic dilatation and bile lakes (n = 2). The findings of eight studies were interpreted as normal. The four patterns of abnormalities were correlated with the results from percutaneous transhepatic cholangiography, T-tube cholangiography, and liver biopsy and with clinical and surgical information, as available. CONCLUSION: MR cholangiography is a noninvasive technique for evaluation of biliary disease. The improved resolution afforded by respiratory triggering permits evaluation of both major and minor bile ducts, even in young, uncooperative subjects. Four patterns of abnormalities were prospectively identified, correlated with other information, and used to direct clinical treatment.

Adolescent↗

Postnatal maturation of the sacrum and coccyx: MR imaging, helical CT, and conventional radiography.

OBJECTIVE: The purpose of this paper is to provide a detailed radiologic description of the postnatal developmental anatomy of the sacrum and coccyx as revealed by MR imaging, helical CT, and conventional radiography. MATERIALS AND METHODS: One hundred ten imaging examinations of the sacrococcygeal spine were performed in patients who were newborn to 30 years old. Imaging included conventional radiography (n = 63), three-dimensional gradient-recalled echo MR imaging (n = 10), and helical CT with sagittal and angled coronal reformations (n = 37). A detailed analysis was performed of the ossification and fusion of the primary and secondary ossification centers. RESULTS: The sacrum and coccyx were noted to develop from 58 to 60 sacral ossification centers and eight coccygeal centers, respectively. These centers were noted to ossify and fuse in an organized temporal pattern from the fetal period to the age of 30. CONCLUSION: The sacrum and coccyx are formed by a complex process that fuses primary and secondary ossification centers. Because the maturation process can be asymmetric, an understanding of this process may prove useful for distinguishing physeal plates from fracture lines.

Adolescent↗

Acampomelic campomelic dysplasia: further radiographic variations.

Acampomelic campomelic dysplasia (ACD) is a rare genetic syndrome affecting bone and connective tissue. This syndrome is a variant of the more commonly encountered campomelic dysplasia but is characterized by the absence of long bone curvature (acampomelia). Affected children have a characteristically flat facial profile and present with respiratory distress. They all have markedly hypoplastic scapulae. We present two sisters with ACD between whom there were some clinical and radiographic differences and also variations from the classic CD. We describe shallow orbits, a radiographic finding that has not been previously documented in this dysplasia.

Bone and Bones↗

Frontometaphyseal dysplasia: neonatal radiographic diagnosis.

Frontometaphyseal dysplasia (FMD) is an uncommon genetic syndrome affecting bone and connective tissue. This condition is characterized by hyperostosis of the skull and prominence of the supraorbital ridges; long bones have flared metaphyses. Frontometaphyseal dysplasia is an X-linked dominant trait with more severe manifestations in males and with extreme variability in females. Diagnosis in the neonatal period is difficult. We present 2 newborn boys with the radiographic findings of FMD.

Abnormalities, Multiple↗

Variable prenatal appearance of osteogenesis imperfecta.

Osteogenesis imperfecta is a heterogeneous group of disorders of type I collagen with both lethal and nonlethal forms. Prenatal sonographic findings in affected fetuses are variable and depend on the severity of the disease. Six cases of osteogenesis imperfecta in which prenatal sonography had been performed were reviewed. Two cases of lethal type II osteogenesis imperfecta revealed short femurs at 16 to 17 weeks' gestation with development of bowing and fractures by 19 weeks' gestation. Four fetuses with the nonlethal type III or IV had femoral bowing with or without shortening in the late second or third trimester with grossly normal mineralization. Fractures in this latter group did not develop until 1 to 12 months after delivery. Understanding the progressive nature and variability of osteogenesis imperfecta is crucial in the prenatal diagnosis and management of this disease.

Adolescent↗

Lumbar spine injury in a pediatric population: difficulties with computed tomographic diagnosis.

Thirty-five children with lumbar spine injuries were evaluated with computed tomography and plain radiography following blunt trauma. The majority of these children (31) were injured in motor vehicle crashes; most of them (27 of 35, 77%) were restrained by lap-styled safety belts. The other mechanisms of injury included motor vehicle crashes involving a pedestrian (2), a fall (1), and a crush (1). The types of injuries encountered were subluxation or distraction combined with fracture in 18; fracture only in 13; and distraction only in four. Abnormalities were not detected with thick-section computed tomographic (CT) scans in 20 (57%) of the cases. Children involved in motor vehicle crashes are at high risk for lumbar spine injuries, because the sudden deceleration forces may cause hyperflexion, resulting in vertebral body compression or interspinous ligament disruption. Lumbar spine radiographs are necessary in all cases with suspected lumbar spine injury because the abnormality may be missed by thick-section CT scanning and may not be detected even with complementary thin sections.

Accidents, Traffic↗

Bone changes associated with cystic fibrosis.

Musculoskeletal symptoms are known to occur frequently in patients with cystic fibrosis. Radiographs of the hands and wrists, and of the tibia and fibula of 56 patients with cystic fibrosis were reviewed. No radiographic joint abnormalities were detected. However, it was noted that the fourth metacarpal was shortened in 5 of 56 patients (9%), a finding that has not been previously documented. Hypertrophic pulmonary osteoarthropathy was present in 3 patients (5.5%).

Adolescent↗

Delayed spontaneous rupture of augmented bladder in children: diagnosis with sonography and CT.

With the increasing use of augmentation enterocystoplasty to treat patients with small-capacity, noncompliant bladders, an increase in the number of cases of delayed spontaneous intraperitoneal rupture of the augmented bladder has been reported. Although patients with a ruptured bladder usually will have an acute abdomen, these clinical signs and symptoms may be masked in spina bifida patients because of their neurologic deficit. Cystography and sonography were performed in four spina bifida patients with delayed spontaneous rupture of an augmented bladder. One patient also had isotope cystography. Two patients were examined with CT. Cystographic findings were abnormal in only one case. Peritoneal fluid was identified sonographically in all four cases and also was seen in both CT studies. Our study reveals that enhanced cystography will frequently fail to show leakage from an augmented bladder. Sonography and CT are reliable in detecting free intraperitoneal fluid, a finding that can significantly aid in the diagnosis of ruptured bladder after enterocystoplasty. Therefore, cystography with normal findings must be followed by sonography or CT in order to detect extravasated urine.

Adolescent↗

Clear cell sarcoma of the kidney: CT, sonographic, and pathologic correlation.

The sonographic and computed tomographic findings in 12 children aged 1-6 years with clear cell sarcoma of the kidney were reviewed retrospectively. Tumor size, calcification, and internal architecture were characterized and correlated with the gross pathologic findings. All tumors were unilateral and large (8.5-16 cm in diameter). Except for one, all masses were predominantly solid, and all contained some well-defined portions of low attenuation or hypoechogenicity that represented tumor necrosis. In addition, seven tumors contained uncomplicated fluid-filled cysts with diameters ranging from a few millimeters to 5 cm. Extension into the inferior vena cava was not noted. The radiologic features of clear cell sarcoma of the kidney are common to all malignant renal neoplasms. Some cases, however, may have features that simulate those of benign conditions, such as multilocular cystic nephroma or segmental cystic dysplasia. No pattern was discerned that would permit discrimination between clear cell sarcoma of the kidney and the most common renal neoplasm of childhood, Wilms tumor.

Child↗

Mastoid abnormalities in Down syndrome.

Hearing loss and otitis media are commonly associated with Down syndrome. Hypoplasia of the mastoids is seen in many affected children and sclerosis of mastoid bones is not uncommon in Down syndrome. Awareness and early recognition of mastoid abnormality may lead to appropriate and timely therapy, thereby preserving the child's hearing or compensating for hearing loss; factors which are important for learning and maximum development.

Adolescent↗

The expanded spectrum of limb anomalies in the VATER association.

The radiographs of 230 children who had undergone neonatal surgery for imperforate anus and/or esophageal atresia/tracheoesophageal fistula were reviewed. Of the 31 children with limb anomalies thus detected, the 24 who had no radiologic or laboratory evidence of chromosomal abnormality form the basis of this report. In 16 children the limb anomalies fell within the commonly described spectrum of the VATER association. In the other 8 children and in 3 of the children with typical VATER limb anomalies additional anomalies were encountered: Sprengel deformity [2], hypoplasia of the humerus [3], radioulnar synostosis [1], midline anomalies of the hand [1], absence of the pubis, femur, tibia, and fibula and two rays of the foot [1], and other foot deformities. Subtle anomalies of the hand were common and included: clinodactyly, syndactyly, shortening of the middle phalanx of the fifth digit, and rotary malposition of the digits.

Abnormalities, Multiple↗

Gaucher disease of the liver: CT appearance.

We present a child with Gaucher disease with hepatic involvement that caused portal hypertension. Computerized tomography (CT) showed distortion of liver parenchyma and central necrosis of the liver.

Ascites↗

Ectopia cordis and the radiographic changes of a new surgical repair technique.

Preliminary repair of the midline thoracic defect in a newborn with ectopia cordis entailed the use of a prosthetic silo and fluid-filled tissue expanders placed under the lateral chest wall. On post-operative radiographs these expanders simulated the appearance of massive subcutaneous oedema of the chest wall.

Female↗

Uroradiographic manifestations of Burkitt's lymphoma in children.

The radiological studies of 18 children with biopsy proved Burkitt's lymphoma were analyzed retrospectively. Before therapy the genitourinary tract was evaluated in 15 children by excretory urography, sonography, computerized tomography and/or gallium citrate scintigraphy. Genitourinary abnormalities were detected in 9 children. Changes due to tumor included renal or ureteral displacement in 4 children, hydronephrosis in 3 and intraparenchymal masses in 4. Extrinsic compression of the bladder causing no compromise of function was seen in only 2 children. Gonadal involvement occurred in 2 boys and 1 girl. The modality of choice for evaluating the genitourinary tract in patients with Burkitt's lymphoma has been excretory urography. Since ultrasound and computerized tomography provide more direct information about tumor deposits within the kidney and retroperitoneum, either should be performed in this population before initiation of chemotherapy.

Adolescent↗

Leg-length determination with biplanar CT scanograms.

Computed tomography (CT) scanograms provide an accurate method for evaluation of leg-length discrepancy. Flexion of a leg may be missed on a single frontal projection, and the measurements obtained will be erroneously shortened. When performing CT sonography, lateral views of the limbs may be easily and rapidly obtained without having to change the position of the patient. This permits assessment of limb flexion.

Humans↗