Hereditary tyrosinemia type I (chronic form): pathologic findings in the liver.

Hereditary tyrosinemia type I presents with either acute hepatic failure in the neonatal period or later in infancy with progressive liver dysfunction secondary to cirrhosis. The inevitably fatal outcome in those children with the chronic form has been transformed with the advent of liver transplantation. Native livers from five children who received allografts were studied pathologically and compared with earlier hepatic biopsies in two of these patients that had been performed several years before transplantation. Our findings support the conclusion that a sequence of morphologic changes from the initial micronodular cirrhosis through an intermediate mixed cirrhotic pattern to macronodular cirrhosis occurs. The micronodular phase is transitory, over a period of only a few months, since mixed micronodular macronodular cirrhosis was already present in the livers of children who received transplants by 11 months of age. Focal hepatocellular dysplasia was present in one of the livers with mixed cirrhosis but was not identified in the other two cases. Macronodular cirrhosis accompanied two cases of hepatocellular carcinoma in this study. In order to preclude the latter complication, liver replacement is necessary before the age of 2 years.

Correlation of clinical and pathologic findings in early onset neonatal group B streptococcal infection with disease severity and prediction of outcome.

This study analyzed the clinical characteristics of 69 neonates who were admitted to the University of Minnesota Hospital between January, 1972, and June, 1984, with early onset Group B streptococcal infection (EOGBS) and determined those features associated with fatal infection. The incidence of EOGBS was 1.6 cases/1000 live births among 7960 inborn infants; the mortality rate for inborn and outborn infants was 28%. Multivariate analysis identified five features adequately predicting fatal outcome: birth weight less than 2500 g, absolute neutrophil count less than 1500 cells/mm3, hypotension, apnea and a pleural effusion on the initial chest radiographs. With these five variables and an initial blood pH less than 7.25, a clinical score was constructed that correctly predicted outcome in 93% of patients in this study (87% sensitivity, 95% specificity). Autopsy findings in 16 of 19 infants with fatal EOGBS suggested that surfactant deficiency respiratory distress syndrome was common in preterm infants with EOGBS and contributed to their higher mortality compared with term infants.

Symposium on Nonpulmonary Aspects in Chest Radiology. The thymus.

In this article, the normal radiographic patterns, normal variants, and biologic behavior of the thymus are reviewed. The imaging of thymic pathology is also described.

Ultrasound and computed tomographic evaluation of renal transplantation.

Ultrasound and computed tomography are very useful in the diagnosis of parenchymal and urologic abnormalities after renal transplantation. The increased use of Doppler duplex sonography will further enhance the role of ultrasound in the evaluation of patients with kidney transplants. Computed tomography is of particular value in the setting of suspected posttransplant malignancy. Both imaging techniques can be used to guide diagnostic and therapeutic interventional radiologic procedures.

MRI in organ transplantation.

Experience with MRI in transplant patients is limited. The normal transplant kidney is characterized by a sharply defined corticomedullary border on T1 weighted images. Loss of CMC is seen in transplant rejection and in some patients with ATN. Presence of CMC does not rule out rejection, however. The spectrum of changes in cyclosporine toxicity is unclear, as only a few patients have been reported. For heart, liver, and pancreas transplantation magnetic resonance spectroscopy and imaging may prove useful in the evaluation of organ viability preoperatively and in early detection of rejection. Carefully designed prospective studies are needed to better define the role of MRI in organ transplantation.

Ultrasound and computed tomographic evaluation in hepatic transplantation.

Ultrasound and CT are valuable in the non-invasive evaluation of complications of hepatic transplantation. Ultrasound is particularly helpful in detecting biliary obstruction and in documenting vascular patency. As with renal transplantation, the value of US in the assessment of rejection will likely increase with time. Computed tomography is most valuable in identifying large parenchymal abnormalities and abdominal fluid. Both US and CT can be used to guide diagnostic and therapeutic percutaneous procedures in hepatic transplant recipients.

Grading of reflux by radionuclide cystography.

Over a three-year period, radionuclide cystography (RC) was performed on 145 patients. Of these, 31 nephroureteral units in 25 patients demonstrated vesicoureteral reflux by RC and also by radiographic cystography (XC). The RC and XC studies were performed within three months of one another. The grading of reflux was determined by RC and then compared with that of XC using the criteria for grading as established by the International Reflux Study Committee (IRSC). Of 18 nephroureteral units in 14 patients with grade II reflux by XC, 14 were similarly graded by RC (78%). Of five nephroureteral units in five patients with grade III reflux by XC, the correlation by RC was 100%. However, when grades II and III were combined into a single category of low-grade reflux, the correlation achieved between the two cystographic techniques was 100%. Of five nephroureteral units in four patients with grade IV reflux by XC, the correlation was 60%, and in three units in two patients with grade V reflux the correlation was 100%. When grades IV and V are combined into a category of high-grade reflux, a correlation of 100% is achieved. When the reflux is graded into the five IRSC categories, the overall unequivocal correlation between the two studies is 80%. However, when only two categories are used (low grade = grades II and III, and high grade = grades IV and V), a correlation of 100% is obtained.

Extravasation of contrast material from unused bladders during voiding cystourethrography.

Extravasation from unused bladders in three children during the voiding phase of voiding cystourethrography is described. In these cases, extravasation was not a clinically significant problem and did not indicate irreversible nondistensibility of the small-capacity bladders.

Aortic arch in neonates with esophageal atresia: preoperative assessment using CT.

Preoperative localization of the aortic arch is important in infants with esophageal atresia to prevent morbidity and death from an ipsilateral surgical approach. Computed tomography is an accurate, noninvasive technique for determining the position of the aortic arch.

Abdominal complications in pediatric bone marrow transplant recipients.

Abdominal problems and catastrophes often complicate the clinical course after bone marrow transplantation (BMT) in children. These complications can be grouped into categories of infection, chemotherapy and radiation toxicity, graft-versus-host disease (GVHD), recurrent or de novo malignancy, and miscellaneous complications and can involve the hepatobiliary system, pancreas, spleen, gastrointestinal tract, and urinary tract. Infection is common after BMT: the causative organism depends on the changing immunologic state of the recipient and even on environmental factors such as recent construction, humidity, and antibiotic use. Chemotherapy and radiation therapy can cause hepatic veno-occlusive disease, pancreatitis, nephritis, and hemorrhagic cystitis. GVHD is a process in which donor lymphoid cells produce damage to recipient target organs, especially skin, liver, and intestinal mucosa. Recurrent or de novo disease or malignancies, particularly B-cell lymphomas, may develop in chronically immunocompromised children. Other problems include stone disease, splenic and renal infarction, and complications of hyperalimentation therapy. Abdominal imaging, including plain radiography, contrast material-enhanced studies of the bowel, real-time and duplex sonography, and computed tomography, is essential in diagnosing these problems and evaluating response to therapy.

Pulmonary emphysema in a neonate with Marfan syndrome.

A premature infant with arachnodactyly and congenital cyanotic heart disease had severe pulmonary emphysema, a little-known manifestation of Marfan syndrome. The possible etiologies of emphysema in this syndrome are discussed.

Pneumatosis intestinalis after bone marrow transplantation.

The clinical and pathologic data in 18 patients in whom pneumatosis intestinalis developed after bone marrow transplantation were reviewed to determine the significance of this finding. The colon, predominantly the right side, was involved in 17 of the 18 cases. Pneumatosis intestinalis developed earlier in the 14 symptomatic patients than in the four asymptomatic patients. Symptoms included diarrhea (12 patients), abdominal pain (six patients), rectal bleeding (two patients), and abdominal distension (two patients). Factors contributing to the development of pneumatosis intestinalis included pretransplantation chemotherapy and radiotherapy, steroid therapy, infectious colitis, graft-versus-host disease, and septic shock. Intestinal disease contributed to the deaths of seven patients with pneumatosis intestinalis, necessitated right hemicolectomy in another patient, and resolved with conservative treatment in 10 patients. In summary, bone marrow transplant recipients with pneumatosis intestinalis may follow either a benign or fatal course, depending on the underlying condition of the patient. Clinical correlation is important in determining the significance of this finding.

Primary pulmonary rhabdomyosarcoma of the lung in children. Report of two cases presenting with spontaneous pneumothorax.

This report describes two cases of pulmonary rhabdomyosarcoma occurring in children, ages 21 and 30 months at diagnosis. Both patients presented with spontaneous pneumothorax and had cystic changes in the affected lung by chest x-ray. Ultrastructural and immunohistochemical studies supported the myogenic phenotype of these neoplasms. The authors were unable to confirm that either of these tumors had unequivocally originated in a congenital cyst although previous authors have reported this association. An apparent morphologic spectrum of embryonic appearing neoplasms including pulmonary blastoma and a malignant mesenchymoma have been observed in the lungs of children.

Radiological aspects of primary hyperoxaluria.

Primary hyperoxaluria is a rare metabolic disorder characterized by excessive synthesis and urinary excretion of oxalate. Nephrocalcinosis with or without calcium oxalate nephrolithiasis leads to renal failure in infancy through young adulthood. Oxalosis is the condition in which the highly insoluble calcium oxalate crystals are deposited in extrarenal tissues including bone, blood vessels, heart, and the male urogenital system. The radiographic abnormalities in 14 patients with primary hyperoxaluria are described. These abnormalities include nephrolithiasis, nephrocalcinosis, dense vascular calcifications, abnormal bone density, and characteristic metaphyseal abnormalities. Changes of renal osteodystrophy and pathologic fractures are common. Radiographic bone abnormalities are dependent on the age of the patient when renal failure occurred and the degree of success of renal transplantation. Characteristic skeletal changes are present in six of seven patients who developed renal failure when less than 7 years of age.

Imaging of the kidney, liver, and pancreas transplant.

Imaging of the renal, hepatic, and pancreas allograft is essential for evaluation of postoperative complications. Surgical complications after organ transplantation can generally be specifically diagnosed. These complications include obstruction, leakage, peritransplant fluid collections, and vascular problems such as vascular stenosis or thrombosis, arteriovenous fistula, and pseudoaneurysm. Using computed tomography (CT) or ultrasound guidance, interventional procedures can often be performed to treat various surgical complications. On the other hand, medical causes of allograft transplant dysfunction such as rejection, acute tubular necrosis, drug toxicity, ischemia, infection, inflammation, or the development of recurrent disease are frequently associated with nonspecific imaging findings. Allograft biopsy is still usually necessary for definitive diagnosis in these cases. In this article, we will briefly outline the spectrum of abnormalities that can be seen in the allograft recipient and show some examples of these complications.

CT demonstration of asymptomatic pulmonary emboli after bone marrow transplantation: case report.

Asymptomatic pulmonary emboli producing microcalcifications were observed 200 days after bone marrow transplantation on a routine surveillance CT in a child. The findings consisted of peripheral hazy, nodular infiltrates in the lower lung fields. To our knowledge this has not been recorded in the radiologic literature.

Inflammatory pseudotumor of the mesentery and small intestine.

Three cases of abdominal inflammatory pseudotumor or inflammatory myofibroblastic tumor of the small intestinal mesentery are presented with additional examples from the literature. An abdominal mass was detected in three preadolescent children who presented with fever, growth failure, hypochromic anemia, thrombocytosis and polyclonal hypergammaglobulinemia. The radiographic findings confirmed the presence of a mass. Following surgical resection, the systemic manifestations dramatically resolved. The solid mesenteric mass was composed of spindle cells with the features of myofibroblasts and mature plasma cells and small lymphocytes. Clinical follow-up confirmed the benign nature of this lesion with no signs of recurrence in any patient from 3 to 6 years after the initial surgery.