Imaging of cerebrovascular injury in infants treated with extracorporeal membrane oxygenation.
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Biomedical subjects
Publications and source records attributed to C R Fitz.
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One hundred forty-six neonates treated with extracorporeal membrane oxygenation (ECMO) were evaluated with cranial sonography and CT at different times in the course of their disease. Cranial sonography was performed during treatment with ECMO, and CT was done 3-14 days after cessation of ECMO bypass. In 66 (45%), intracranial abnormalities were detected with either sonography or CT. Thirty neonates had hemorrhages only, 24 had nonhemorrhagic abnormalities, and 12 had combined lesions. CT provided additional information in 44 (67%) of the 66 neonates. Sonography was normal in 37 neonates, showed fewer lesions in 10 neonates, and was concordant with CT findings in 14 neonates. It cannot be established whether these lesions went undetected with sonography during ECMO, or occurred after cessation of ECMO bypass. Neurodevelopmental evaluation was performed in 71 of 90 survivors 1 year of age or older. Forty-three infants (61%) were normal; developmental delay was suspected in 12 (17%) and present in 16 (23%). The severity of intracranial abnormality as determined by a neuroimaging score correlated well with short-term developmental outcome. Mean neuroimaging scores were significantly higher, with increasing degree of neurodevelopmental delay (p less than .001 by Kruskal-Wallis rank order test). Although cranial sonography remains the primary technique for the detection of life-threatening hemorrhage during ECMO, cranial CT after ECMO is important in defining the total degree of intracranial injury present in these high-risk neonates.
Neonates treated with extracorporeal membrane oxygenation are at high risk for the development of intracranial hemorrhage and infarction. The appearance of these lesions on cranial sonography is often unusual and may be confusing. We compared the findings at autopsy with premorbid cranial sonograms in 17 nonsurviving neonates to define better the anatomic basis for the sonographic appearance of these lesions. Macroscopic abnormalities were identified at autopsy in 13 of the 17 neonates. Five neonates had multifocal hemorrhagic white-matter infarcts, three had large parenchymal hemorrhages with adjacent areas of parenchymal necrosis, three had hemorrhagic infarcts of the cerebellum, one had a germinal matrix and intraventricular hemorrhage, and one had bilateral periventricular cysts with surrounding gliosis. All 17 neonates had abnormalities on microscopic examination. Although sonography was accurate in the detection of macroscopic lesions (11 of 13 lesions detected with sonography), the nature and extent of these abnormalities were difficult to judge because of the variable echogenicity of unclotted blood and the presence of focal areas of abnormal echogenicity associated with microscopic calcification and gliosis. Sonography is excellent for the detection of acute cerebrovascular complications during extracorporeal membrane oxygenation, but the appearance of these lesions is variable and nonspecific.
Serial tumor volume measurements were performed on computed tomographic (CT) scans or magnetic resonance (MR) images of 12 children treated with radiation therapy for high-grade (three children) or low-grade (nine children) astrocytomas of the optic nerve, thalamus, hypothalamus, cerebellar-pontine angle, and pons. Three types of initial tumor response were observed: Tumor volume increased in four children, remained unchanged in one, and decreased immediately after completion of radiation therapy in the others. High-grade tumors regressed maximally within 6 months and low-grade tumors within 10-25 months. All high-grade tumors recurred within 12 months. Three low-grade tumors recurred 3, 6 1/2, and 10 years after diagnosis, respectively. The tumor-volume halving time was 60-78 days for high-grade tumors and 108-330 days for low-grade tumors. The tumor doubling time was 48-60 days for high-grade tumors. The posttreatment increase in tumor volume has implications for radiation treatment planning. It did not indicate a poor prognosis.
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Findings at neuroimaging in 100 consecutive infants treated with extracorporeal membrane oxygenation (ECMO) are presented. Imaging in these infants consisted of pretreatment cranial ultrasonography (US), daily US studies while on ECMO, and follow-up cranial computed tomography (CT) after treatment. There were findings of abnormalities in 43 patients. Thirty had intracranial bleeding, often of unusual extent and distribution. Thirteen additional infants had nonhemorrhagic abnormalities alone. Bleeding considered to be major was seen in 12% of infants. Large parenchymal hemorrhages and infarcts, cerebellar hemorrhages, and diffuse edema were the most significant abnormalities, with a 50% mortality (eight of 16 patients). No lateralization was noted with respect to distribution of bleeding sites or areas of nonhemorrhagic abnormalities. US was a sensitive but imperfect screening tool for intracranial abnormalities. Abnormalities missed with US included peripheral and small parenchymal lesions, subarachnoid hemorrhage, cerebral atrophy, and sagittal sinus thrombosis.
A retrospective review was done of intracranial images obtained within the newborn period in 46 infants who had been treated with extracorporeal membrane oxygenation (ECMO). A neuroimaging score was determined on the basis of the extent and severity of findings on both ultrasound and computed tomographic scans. Presence of abnormality at neuroimaging and the neuroimaging score were correlated with the findings of neurodevelopmental evaluations (Bayley scales) performed in infants who were at a mean age of 11.8 months (range, 6-16 months). A significant inverse correlation was found between the neuroimaging score and mental and psychomotor development indexes of the Bayley scale (P less than .005). Mean neuroimaging scores in infants with normal development were significantly lower than those in infants with delayed development (P = .002). Although individual outcomes cannot be predicted with neuroimaging, the neuroimaging score can be a useful adjunct in assigning patients who survive with ECMO treatment to risk categories for developmental outcome.
CT scans on 110 children with a diagnosis of tuberous sclerosis have been compared with the clinical features of the disease. Patients with definite radiological as well as definite or suggestive clinical features accounted for only 80% of cases. 16% had positive radiological and negative clinical features and 4% had positive clinical and negative radiological features. There is little relationship between the mental development and the incidence of periventricular tubers or cortical and white matter lesions. In 15 patients older than 7 years the diagnosis was only made at the time of CT. Cerebral atrophy was not a prominent feature. Giant cell astrocytomas were present in 14%, one third of whom presented for the first time.
The author reviews the variety of radiological modalities that may be used to investigate spinal disease and then discusses their application in specific disease states.
For asphyxiated term infants, prediction of neurological outcome based on clinical criteria alone is not always reliable. We have used changes in tissue density on CT scan (known to represent hypoxic-ischaemic injury) as an adjunct to clinical examination to assess the severity of cerebral injury. The study population comprised 56 term newborns with hypoxic-ischaemic encephalopathy. Scans were performed with a General Electric 8800 scanner and classified as Normal, Patchy, Diffuse or Global according to the extent of areas of decreased density (DD). These were correlated with neurological outcome (i.e. Normal, Minor handicap, Major handicap, Indefinite or Death). Death or major handicap occurred in only two of 14 infants in the combined Normal or Patchy DD groups but in 26 of 29 in the combined Diffuse or Global groups. This correlation was highly significant (p less than 0.0005). 13 classified as Indefinite were normal at age three to 10 months but were excluded from analysis because of the short follow-up. The data demonstrate that CT scanning is a valuable adjunct to neurological examination for assessment of cerebral injury in the asphyxiated term infant.
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Two children presented with acute spinal cord compression by primary and metastatic intraspinal mesenchymal chondrosarcoma, a rare pediatric malignancy. Patients with the primary intraspinal tumor usually present early and often respond well to combined surgery, irradiation and chemotherapy. Patients with intraspinal metastases present late in the course of their disease and their prognoses are poor.
Ultrasound brain scans sometimes demonstrate increased echogenicity or cysts, or both, in the periventricular white matter, superolateral to the ventricle, in the most common site of periventricular infarction. Over 33 months, 23 preterm infants dying after 20 or more days of life were entered into this study. Superolateral echogenicity or cysts were found in 13 (57%) cases. Periventricular infarction was present at autopsy in 12 (52%) cases. Ultrasound accurately diagnosed the size, site, and extent of periventricular infarction in 78% of scans. Interpretive errors were made with poor-quality scans and with early and late studies. We conclude that sector ultrasound brain scans accurately diagnose major periventricular infarction. Hemorrhage into the site of infarction is not a prerequisite for diagnosis of periventricular infarction by ultrasound.
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Seven of fourteen children with spinal cord ependymoma had myxopapillary tumors of the filum terminale. These tumors made up 15.9% of all primary spinal neuroectodermal tumors in children (44 cases) seen during a 62-year period (1919 to 1981). Their clinical presentation, radiological features, pathological findings, treatment, and outcome are reported. Six of the seven patients were known to be alive at the time of writing. The seventh patient was lost to follow-up after 3 years without tumor recurrence. Of 5 patients whose primary mode of treatment was operation alone. 3 had intraspinal or intracranial recurrences. Despite tumor recurrences, 2 patients were long term survivors after further operation and irradiation, whereas the third patient recently received craniospinal irradiation for intracranial tumor recurrence. The 2 patients who did not have tumor recurrence after operation alone had been followed for 3 and 7 years, respectively. Two children with subtotal tumor resection and spinal irradiation had no recurrences at 1 and 17 years, respectively. Our data suggest that this unusual subtype of spinal ependymoma is not uncommon during childhood and has a good prognosis. All patients with this tumor require prolonged follow-up for tumor recurrence after operation and irradiation.
Among 848 cases of primary intracranial malignancy seen during a 63-year period at a pediatric hospital, there were 2 cases of primary pineal choriocarcinoma. The clinical and laboratory findings of these 2 cases were similar to those of 33 cases of intracranial choriocarcinoma reported in the literature. In 1 patient with precocious puberty, the diagnosis was confirmed by labeling human chorionic gonadotropin (HCG) within the tumor, which had been stored for 25 years. In the other patient, who is alive and well 18 months after diagnosis, the response to cranial irradiation and intensive chemotherapy was monitored with serial measurements of serum, cerebrospinal fluid, and urinary HCG and with computed tomography. We conclude that intensive chemotherapy, radiation, and tumor resection, if feasible, offer the best chance of curing this otherwise fatal disease.
A patient with Menkes' syndrome is reported. This patient has survived to the age of six years without mental retardation, and shows previously unreported computed tomographic (CT) findings of tortuous vessels and unusual osseous manifestations of undertubulation of long bones and posterior vertebral body scalloping.
Holoprosencephaly is a brain anomaly of varying severity with associated extracranial, symptomatic abnormalities in only a minority of cases. The class of brain defects known as holoprosencephaly represents a continuum usually divided into three types: alobar, semilobar, and lobar. Each has both distinctive radiological characteristics and some similarities. Typical facial anomalies are found in the severe forms. Absent septum pellucidum and septo-optic dysplasia, possibly of similar embryological origin, have some resemblances to lobar holoprosencephaly, but are clinically and radiologically separate in most instances. Agenesis of the corpus callosum, which is superficially like holoprosencephaly, should not be confused with the prosencephalic defects.