Developmental and neurologic status of children after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass.

BACKGROUND: Deep hypothermia with either total circulatory arrest or low-flow cardiopulmonary bypass is used to support vital organs during heart surgery in infants. We compared the developmental and neurologic sequelae of these two strategies one year after surgery. METHODS: Infants with D-transposition of the great arteries who underwent an arterial-switch operation were randomly assigned to a method of support consisting predominantly of circulatory arrest or a method consisting predominantly of low-flow bypass. Developmental and neurologic evaluations and magnetic resonance imaging (MRI) were performed at one year of age. RESULTS: Of the 171 patients enrolled in the study, 155 were evaluated. After adjustment for the presence or absence of a ventricular septal defect, the infants assigned to circulatory arrest, as compared with those assigned to low-flow bypass, had a lower mean score on the Psychomotor Development Index of the Bayley Scales of Infant Development (a 6.5-point deficit, P = 0.01) and a higher proportion had scores < or = 80 (i.e., 2 SD or more below the population mean) (27 percent vs. 12 percent, P = 0.02). The score on the Psychomotor Development Index was inversely related to the duration of circulatory arrest (P = 0.02). The risk of neurologic abnormalities increased with the duration of circulatory arrest (P = 0.04). The method of support was not associated with the prevalence of abnormalities on MRI scans of the brain, scores on the Mental Development Index of the Bayley Scale, or scores on a test of visual-recognition memory. Perioperative electroencephalographic seizure activity was associated with lower scores on the Psychomotor Development Index (P = 0.002) and an increased likelihood of abnormalities on MRI scans of the brain (P < 0.001). CONCLUSIONS: Heart surgery performed with circulatory arrest as the predominant support strategy is associated with a higher risk of delayed motor development and neurologic abnormalities at the age of one year than is surgery with low-flow bypass as the predominant support strategy.

Cystic retrocerebellar malformations: unification of the Dandy-Walker complex and the Blake's pouch cyst.

Twenty-six cases of developmental retrocerebellar cyst (RCC) formation are studied with respect to determining the usefulness and anatomic relevance of separate terms currently in use, including Dandy-Walker complex, Dandy-Walker malformation, Dandy-Walker variant, mega-cisterna magna (MCM), and Blake's pouch cyst. An anatomic and embryological continuum between Dandy-Walker complex and Blake's pouch cyst is proposed. A method for the useful assessment of RCC is outlined. The patency or closure of the aqueduct is crucial to the evaluation and management of hydrocephalus associated with RCC formation.

Comparison of the effectiveness of potential marrow donors recruited from apheresis donors or whole blood donors and through appeals to the general public.

Marrow transplants with phenotypically HLA-matched, unrelated donors have been used effectively to treat a number of diseases. Many blood centers have recruited HLA-typed apheresis blood donors into marrow donor registries. However, to build larger registries so that more patients may be treated with unrelated donor marrow transplants, whole blood donors and people who do not donate blood have been added to the registries. The marrow donor program at our blood center had 2844 potential donors, of whom 1725 were also apheresis donors, 608 were whole blood donors, and 511 were recruited from the general public as a result of community appeals for marrow donors for a specific patient. Over a 9-month period, 297 potential donors were asked to donate blood samples for HLA-DR typing or mixed lymphocyte culture (MLC) testing or to participate in an informational session, undergo a medical evaluation, and sign a statement indicating an intention to donate marrow for a specific patient. Overall, these requests were successfully completed by 75.5 percent of apheresis donors, 87.2 percent of whole blood donors, and 78.1 percent of the potential donors recruited through community appeals. Furthermore, there was no difference among the three groups in the portion of people who donated blood samples for HLA-DR typing or MLC testing. Fifteen of 18 apheresis donors who were found to match a specific donor signed a statement of intent to donate marrow, 2 apheresis donors were deferred for medical reasons, and 1 decided not to donate.(ABSTRACT TRUNCATED AT 250 WORDS)

HLA class I and II antigen phenotypes of North American Indians from Minnesota: implications for marrow transplants using unrelated donors.

As a result of an appeal for a bone marrow donor for a North American Indian (Native American) patient, 261 Native Americans from our community were typed for HLA-A,B,DR antigens, and 51 were typed for HLA-A,B antigens only. The HLA antigen frequencies of the Native Americans were compared with those of 12,881 white bone marrow donors and were found to differ markedly. To investigate the implications these differences in HLA antigen frequencies would have for the location of unrelated bone marrow donors, the HLA types of 12 Native American bone marrow transplant patients from our institution were used to search among 5389 HLA-A,B,DR-typed white donors in the National Marrow Donor Program file and the file of 261 HLA-A,B,DR-typed Native American donors. In the white donor file, at least two donors were found that matched at all HLA-A,B,DR antigen loci of one Native American patient (8%). Using the Native American donor file, which was less than one-twentieth the size of the white donor file, and HLA-A,B,DR-matched donor was also found for one (8%) of the patients. These results suggest that although donors for nonwhites can be identified in a file of HLA-typed white volunteers, the probability of finding a suitably matched donor for such individuals is enhanced if donors representing racial or ethnic minorities are included in unrelated donor registries.

Percutaneous biopsy of pyogenic infection of the spine in children.

Three boys had percutaneous biopsies of pyogenic infections of the spine guided by computed tomography (CT). All had typical features of chronic vertebral osteomyelitis, i.e., narrowing of disc space, destruction of the contiguous vertebral endplates, sclerosis of vertebral bodies, and soft tissue swelling as noted on radiographs, CT, and magnetic resonance imaging (MRI). All three had back pain; one was asymptomatic at the time of the biopsy. Culture of the biopsy material grew Staphylococcus aureus in two cases and Salmonella group B in one. If blood cultures are negative, a biopsy of the vertebral body is recommended in order to determine the activity of the process and the type of organism, and its susceptibility to antibiotics. CT-guided percutaneous biopsy is an alternative to open surgical biopsy.

Surgical division of craniopagi.

The world's surgical experience on the division of craniopagi is reviewed, and a new case is reported. The problems, including ethical considerations, that must be addressed in selecting patients for operation and in preparing for the procedure are discussed in detail. Hemodynamic stability and the problem of intraoperative gravitational distortion of the brain are addressed. The importance of careful planning is stressed. Survival of the 31 craniopagi after operation is discussed in relation to types of junction.

Facial skeletal changes following hypertelorbitism correction.

This is a retrospective study of skeletal changes in 19 patients with corrected hypertelorism. A favorable outcome, defined as relapse less than 5 mm, occurred in patients with an average interorbital distance of 32 mm, whereas patients with an average interorbital distance of 40 mm tended to relapse over 5 mm. Neither age, interorbital configuration, nor diagnosis affected the stability of orbital translocation. At last evaluation (mean 6.7 years postoperatively), the mean interorbital distance was 22.4 mm in the favorable outcome group and 28.3 mm in the unfavorable category. This study suggested that the standard hypertelorism operation may interfere with anterior facial growth. Unless psychosocial factors predominate in a child with mild deformity, repair should be postponed until late adolescence. In a young child with gross telorbitism, nasoethmoidal resection and transmaxillary osteotomies or facial bipartition is justified. Another long-term skeletal problem was resorption of the reconstructed nasal complex. Technical and biological explanations for this are given. The correction of hypertelorism is surgery of the nose and of the midface.

Radiation therapy for pineal and suprasellar germ cell tumors.

Radiation therapy (XRT) was used in the treatment of 25 patients with tumors of the pineal and suprasellar locations. A tissue diagnosis was obtained before XRT in 5 patients, and 20 were irradiated without histologic verification. The overall survival rate is 80% (76% with no evidence of disease [NED]). Megavoltage XRT was delivered to the entire neuraxis in 22 patients, and 86% (19/22) are alive from 4 to 88 months (median, 30 months) after treatment. In two of three patients treated only to local fields, tumor recurred in the spine; both are dead of disease. Biopsy-proven germinomas and multiple midline tumors responded favorably to XRT, whereas solitary pineal tumors and teratomas with marker positivity (human chorionic gonadotropin, alpha-fetoprotein) did not respond as well. The endocrinologic presentation, tumor marker status, and early response to radiation measured on computed tomography are useful means for selecting patients for radiation therapy.

Congenital hemihypertrophy and abnormalities of the cerebral vasculature. Report of two cases.

Two patients are described with congenital hemihypertrophy and vascular abnormalities of the brain on the side of the hypertrophy and in the posterior fossa. The abnormalities observed included giant aneurysm, capillary hemangioma, and arteriovenous malformation. Vascular anomalies in the affected limbs are common in congenital hemihypertrophy, and neurological abnormalities and hypertrophy of the brain have been reported. The presence of vascular abnormalities of the brain in this condition may provide an opportunity to further the understanding of the development of cerebrovascular malformations.

Childhood hemangiomas and vascular malformations: angiographic differentiation.

Clinical and cellular differences between hemangiomas and vascular malformations in children have been defined. Hemangiomas are benign endothelial cell neoplasms that appear in infancy and usually have a natural history of proliferation and involution. Vascular malformations are errors of vascular morphogenesis that are present at birth, grow with the child, and never involute but often expand. The authors reviewed the preoperative angiograms of 14 children who had cellular analyses of resected vascular lesions. Hemangiomas could be distinguished from vascular malformations by the presence of a well circumscribed mass demonstrating intense tissue staining, usually organized in a lobular pattern. The vascular malformations, although angiographically variable depending on the predominant vascular channel type, were diffuse lesions consisting entirely of vessels without intervening tissue stain. These angiographic differences between hemangiomas and vascular malformations corroborate clinical and laboratory studies.

Melanotic neuroectodermal tumor of the neurocranium in infancy.

Melanotic neuroectodermal tumors of the neurocranium are a rare but life-threatening disorder of infancy. 11 previously reported cases are reviewed in terms of clinical presentation, radiological diagnosis, and management. A twelfth case, a 4-month-old infant who developed three discrete sites of tumor unilaterally in the neurocranium is presented. Several hypotheses for the mechanism of formation of these tumors are reviewed. The authors propose that the mechanism of formation involves a dysontogenesis of neural crest tissue and that these tumors form, at least in part, from fragments of melanin-containing arachnoid villi which are displaced during embryonic development.

Computed tomography of Sturge-Weber syndrome in infants.

Computed tomographic findings in two infants with Sturge-Weber syndrome include calcification of the brain not visible on plain radiographs. In both cases, the choroid plexus was very prominent, and, in one, a hemangioma of the choroid plexus was found at surgery. In the other, especially dense deposits of mineral were identified at surgery to be calcified branches of the middle cerebral artery.

Diastematomyelia and structural spinal deformities.

Sixty patients with diastematomyelia were seen over a thirty-year period and congenital scoliosis was found in 60 per cent. All of the patients had associated vertebral abnormalities and most (87 per cent) had a neural deficit. Myelography was helpful in the diagnosis, particularly prior to any procedure that might cause traction on the spinal cord. Laminectomy for removal of the spur was indicated when neural deficits were progressive or before corrective surgery on the spine, and in ten patients the operation alleviated neural sequelae. Observation of patients with diastematomyelia who have no neural deficit or a stable, non-progressing deficit is recommended.

Arteriography in children using an isocentric parallelogram.

A modified U-am with parallelographic motion is readily usuable for serialographic neurological, abdominal, and peripheral angiographic studies in infants and children. Clinical experience with the apparatus has demonstrated dependable and mechanically simple operation, and confirmed that undistorted rotated and sagittally angled views can be obtained without the need for repositioning sedated pediatric patients.

Metrizamide ventriculography and computed tomography in lesions about the third ventricle.

Metrizamide ventriculography was used in 24 patients with suspected lesions in and near the third ventricle. The diagnoses were aqueduct stenosis (6), upper brain stem expansions (5), third ventricular tumors (6), craniopharyngiomas (2), subarachnoid cyst (1) and normal (1). Computed tomography was used as the guide to predict the likely causes of obstruction and to determine if ventricular shunting was appropriate before or after ventriculography. Several cases required pneumoencephalography in combination with ventriculography in order to determine whether an upper brain stem lesion was intrinsic or extrinsic.

Sequelae of metrizamide myelography in 200 examinations.

Twenty pediatric and 180 adult patients underwent myelography using metrizamide (Amipaque). All patients were examined and interviewed before and after the studies. New or exacerbated symptoms attributed to metrizamide myelography were graded according to severity. After the procedure, 51 of 200 patients were unchanged from baseline. Headache was the most common complaint, with an overall incidence of 62%. Nausea and/or vomiting occurred in 38%. Back or leg pain, neck stiffness, temperature elevation, and a variety of less common manifestations were also observed. The incidence of sequelae was higher than in comparable Scandinavian studies with this contrast medium.

Thoracic and lumbar dural ectasia in a two-year-old boy.

A two year old boy, admitted for evaluation of failure to thrive was found to have multiple paraspinal masses and a widened spinal canal. The masses proved to be lateral meningoceles, present varying degrees at every thoracic and lumbar level.

Acute posterior fossa epidural hematomas in children.

Epidural hematoma in the posterior fossa is a rare clinical entity requiring immediate surgical intervention to prevent rapid and fatal deterioration. This diagnosis should be considered in children with occipital head trauma who have altered sensorium and evidence of compression of structures within the posterior fossa. Such a clinical setting demands close neurologic observation in a facility where contrast studies and neurosurgical intervention are available. The absence of an occipital skull fracture or the presence of normal pulse rate and blood pressure should not influence the decision. Lumbar puncture is absolutely contraindicated.