Heparin and the risk of intraventricular hemorrhage in premature infants.

OBJECTIVE: This study was carried out to determine whether the routine use of low-dose heparin in umbilical catheter infusates increases the risk of intraventricular hemorrhage or alters the coagulation profile in premature infants. METHODS: In a randomized, blinded trial, 113 infants born at less than 31 weeks' gestation were assigned to receive, in their umbilical catheter infusate, either 1 unit of heparin per milliliter (n = 55) or no heparin (n = 58). Prothrombin time, activated partial thromboplastin time, fibrinogen concentration, and antithrombin III activity levels were determined at the start and the completion of the study. Cranial ultrasonography was performed during the first week of life. RESULTS: There was no difference in the incidence of intraventricular hemorrhage between the heparin and no heparin groups, 35.8% and 31.5%, respectively (p = 0.6). Similarly, no difference was detected in the incidence of severe intraventricular hemorrhage (grades III/IV). Prothrombin time, activated partial thromboplastin time, and fibrinogen levels were not significantly different between the two groups. However, the use of heparin was associated with a lower antithrombin III activity level. Antenatal indomethacin use was associated with a 2.9 increased risk of intraventricular hemorrhage (95% confidence interval, 1.15 to 7.17). CONCLUSION: A low dose of heparin added to umbilical catheter infusates does not increase the incidence or severity of intraventricular hemorrhage or significantly alter the coagulation profile in premature infants.

Spontaneous resolution of an asymptomatic arachnoid cyst.

The authors describe the spontaneous resolution of an intracranial arachnoid cyst. The asymptomatic cyst was incidentally diagnosed at 4 years of age. Resolution of the cyst was noted 7 years later without any history or evidence of trauma.

MR imaging of symptomatic neurocutaneous melanosis in children.

Neurocutaneous melanosis is a syndrome consisting of cutaneous nevi and melanocytosis of the leptomeninges. Over a 5-year period (1989-1994) we evaluated with MR imaging the central nervous system of five children with a confirmed histologic diagnosis of neurocutaneous melanosis. The children ranged in age from 7 to 10 years and consisted of two girls and three boys. They all had multiple pigmented skin lesions (cutaneous nevi) and presented with seizures, signs of raised intracranial pressure, cranial nerve palsies and/or myelopathy. The MR studies were performed with T1-weighted, T2-weighted and T1-weighted post-gadolinium images of the brain in addition to T1-weighted post-gadolinium images of the entire spine. The MR findings in all the children consisted of marked, diffuse enhancement of thickened leptomeninges surrounding the brain and spinal cord which was only demonstrated on the post-gadolinium T1-weighted images and mild to moderate hydrocephalus. We present our MR findings and compare these findings with other imaging findings in the literature. Our findings represent part of a spectrum of imaging abnormalities seen in patients with neurocutaneous melanosis.

Persistent respiratory failure due to low cervical cord infarction in newborn babies.

Perinatal infarction of the spinal cord is described in two premature babies who survived for several months. In both cases, there was bilateral, multisegmental infarction at the lower cervicothoracic arterial zone (inferior cervical sector), predominantly within the territory of the anterior spinal artery. Clinically, both infants had acute respiratory failure, diaphragmatic respiration, intercostal paralysis, bell-shaped deformity of the thorax, and bilateral arm paresis. Intubation and ventilator support were required for weeks. Eventual extubation was followed by recurrent respiratory failure, atelectasis, and pneumonia. At the level of the infarction, multiple, scattered scars were found in central, perimedullary, and intramedullary arteries, but not in their parent vessels. The etiology of the angiopathy was not determined. These cases demonstrate that discrete arterial infarction of the cord can occur in premature babies, and that such a lesion should be considered in the differential diagnosis of neonatal respiratory failure.

MR and CT imaging patterns in post-varicella encephalitis.

The aim of the investigation was to determine the patterns of cerebral involvement on computed tomography (CT) and magnetic resonance (MR) imaging in post-varicella encephalitis. Four children between the ages of 2 and 11 years presented over a 5-year period with a diagnosis of post-varicella encephalitis. Their imaging studies and clinical data were reviewed retrospectively. The medical histories of all four children were noncontributory except for recent bouts of chickenpox 1 week to 3 months prior to hospitalization. Three children presented with parkinsonian manifestations. Bilateral, symmetric hypodense, nonenhancing basal ganglia lesions were found on CT. These areas showed nonenhancing low signal intensity on T1-weighted images and high signal intensity on T2-weighted images on MR. One child presented with diffuse, multiple gray and white matter lesions of similar imaging characteristics; some lesions, however, did enhance. This child had no gait disturbances. Post-varicella encephalitis can produce two patterns of dramatic CT and MR findings. With an appropriate history and clinical findings, varicella as a cause of bilateral basal ganglia or diffuse cerebral lesions can be differentiated from other possible etiologies which include trauma, anoxia, metabolic disorders and demyelinating diseases.

Single forebrain ventricle without prosencephaly: agenesis of the corpus callosum with dehiscent fornices.

Coalescence of the cerebral ventricles with formation of a single forebrain ventricle is described in an unusual case of agenesis of the corpus callosum with dehiscent fornices and severe hydrocephalus. The cerebral hemispheres were fully cleaved. The detached fornices were widely separated from the thalami. The membrana tectoria was retroverted over the midbrain and cerebellum, where it joined the fornices and merged with a curved membranous ependymal dome which, at a great distance, circumvented the thalami as it extended toward the anterior commissure. Other anomalies included arhinencephaly, multiple subependymal heterotopias, and Dandy-Walker malformation. Similar malformations have been described as interhemispheric cysts, as (holo)prosencephaly, or as "midline telencephalic dysgenesis." The basic features of prosencephaly and agenesis of the corpus callosum are reviewed and compared to the present case.

Three-dimensional computed tomography imaging in the evaluation of craniofacial abnormalities.

A retrospective study was undertaken to assess the value of three-dimensional computed tomography (3-D CT) in evaluating craniofacial abnormalities. Over a 2-year period, 145 children evaluated with routine two-dimensional (2-D) CT had 3-D CT reformatted images assessing a variety of anomalies including craniosynostosis, midface clefts, trauma, and craniofacial syndromes. There were 62 females and 83 males ranging in age from 1 month to 17 years (median: 5 months). Both soft-tissue and bone algorithms were used. In all 145 cases, the 3-D CT images confirmed or provided additional information of depth perception, contours, volumes, and extent of an abnormality. This was especially important and useful in the settings of complex anomalies and preoperative planning. We concluded that 3-D CT reconstructed images in conjunction with routine 2-D CT should be an integral part of the examination in evaluating craniofacial abnormalities.

Intraspinal Wilms' tumor metastases.

BACKGROUND: Intraspinal Wilms' tumor metastasis is rare, and is associated with a high mortality rate. METHODS: The authors reviewed the clinical course of two patients with Wilms' tumor in whom extradural metastasis developed. In addition, a review of the literature and of patients entered in the National Wilms' Tumor Studies was performed to determine the clinical presentation, treatment, and outcome of other patients with Wilms' tumor with intraspinal metastases. RESULTS: Both of the patients initially had abdominal pain without neurologic deficits. Despite therapy, paraplegia secondary to cord compression from recurrent epidural metastases developed in one patient, although a third remission has been achieved with further chemotherapy. the second patient remains in disease-free remission 25+ months after surgical resection of the extradural spinal tumor, adjuvant chemotherapy and radiation therapy, and autologous bone marrow transplantation. Review of the literature and of the patients entered in the National Wilms' Tumor Studies revealed an additional 27 patients with Wilms' tumor with this pattern of metastasis. Only four were disease-free at the time of this report. CONCLUSIONS: The authors' experience stresses the importance of early recognition and treatment of this complication of Wilms' tumor and demonstrates that intensive multimodality therapy can result in long-term disease-free remission.

MR of pediatric intracranial meningiomas.

PURPOSE: To assess MR and pathologic characteristics of childhood intracranial meningiomas, comparing the radiographic findings with those observed in adult intracranial meningiomas. METHODS: Clinical records, MR and CT scans, and histology of eight children with intracranial meningiomas presenting for a period of 7.5 years were retrospectively reviewed. RESULTS: Boys equaled girls but predominated from ages 4 to 11 years. The ages ranged from 4 to 18 years. Two patients had radiation-induced meningiomas. Two children had multiple lesions; neither had neurofibromatosis. All solitary lesions were supratentorial. Other characteristics included: dural-based attachment (n = 6); large size (> 5 cm) (n = 4); cystic components (n = 3); and intraventricular location (n = 1). Histologic subtypes were: meningothelial (n = 4); transitional (n = 3); and fibroblastic (n = 1). Preoperative diagnoses of meningiomas were made in six cases based on overall imaging characteristics. CONCLUSIONS: Diagnosis of childhood intracranial meningiomas does not differ from that of adults radiographically, with the exception of larger tumor sizes.

The role of bone scintigraphy in detecting child abuse.

This review of diagnostic imaging in cases of suspected child abuse characterizes the significant differences between bone scintigraphy and x-ray evaluation, describes the advantages and disadvantages of each modality, postulates on the specific mechanisms of injury that produce the characteristic scintigraphic findings, and emphasizes the influences that scintigraphic studies have on the medical, social, and legal aspects of child abuse. The major advantages of bone scintigraphy are its increased sensitivity (25% to 50%) in detecting evidence of soft tissue as well as bone trauma in child abuse. Furthermore, it is postulated that the specific mechanisms of inflicting the trauma relate to the patient's size and are characterized by bone scintigraphy. During fits of anger or frustration, the perpetrator of child abuse grasps the small infant or child by the thorax during the shaking activity. This produces characteristic rib injuries. The older and heavier child is more likely to be grabbed by the extremities, which produces periosteal injuries manifested as characteristic abnormal localizations in the diaphyses of the extremities. The roentgenograms of these injuries are frequently normal. The importance of bone scintigraphy is its complementary nature in defining and characterizing the extent and severity of trauma from child abuse. Such findings have direct bearing on the medical, social, and legal outcomes for the abused child. The quality of scintigraphic imaging is important, requiring the use of magnification techniques in the infant. The interpretation of the scintigraphic images depends on an understanding of the mechanisms by which the radionuclide localizes in bone. The same traumatic incident can lead to decreased, normal, or increased localization at the trauma site. Radionuclide scintigraphy is a complementary rather than competitive imaging modality to X-ray evaluation in the diagnosis and management of physical child abuse.

Carboplatin-induced regression of an optic pathway tumor in a child with neurofibromatosis.

Optic pathway tumors are common in children with neurofibromatosis-1 (NF-1). The optimal management of these tumors is unknown, particularly when the optic chiasm and other brain structures are involved. We report the dramatic response to carboplatin in a 10-year-old girl with NF-1 and a progressive optic pathway tumor. Tumor shrinkage was accompanied by striking improvement in visual fields, return of color discrimination, and marked improvement in visual acuity. No significant toxicity was observed. One year following completion of chemotherapy the glioma remains as small or smaller than it was at the conclusion of therapy, and there has been no deterioration of vision. Carboplatin is a promising agent for the treatment of optic pathway tumors in children with NF-1.

The radiological evaluation of the child with a myelomeningocele.

Seven hundred fifty-five children with myelomeningoceles were evaluated radiologically at the Children's Memorial Hospital in Chicago. From our material, we propose a diagnostic radiologic model to accurately evaluate the neurological problems in the myelomeningocele child. This model is based on the clinical symptoms in these children and the radiologic modalities of magnetic resonance imaging (MRI), computed tomography (CT), ultrasound, myelography, and plain radiographs. We found MRI to be the best modality to evaluate the posterior fossa and total spine. Computed tomography and ultrasound are used to evaluate ventricular size. At times MRI may not adequately diagnose subtle cases of tethering of the spinal cord, cord infarction, arachnoid cysts, or diastematomyelia. In these cases, further evaluation may be necessary with real time ultrasound to look at cord pulsations and water soluble myelography with follow through CT to differentiate cord infarction, arachnoid cyst, localized hydromyelia, or diastematomyelia. If MRI is not adequate to completely visualize the cord because of the severe nature of the scoliosis, then water soluble myelography with CT is indicated.

Radiographic manifestations of congenital anomalies of the spine.

Although the foregoing review of embryologic development and congenital anomalies of the spine in infants and children is necessarily brief, the most commonly encountered abnormalities have been reviewed, and when possible, an attempt has been made to cite the stage of embryologic development at which the various abnormalities originate. As noted, congenital abnormalities of the spine are relatively uncommon but may be of profound clinical significance. During the past decade, the most significant developments in the diagnosis and treatment of these abnormalities have been ultrasonography, CT scanning, and MR imaging. In the neonate, the spinal cord and neural outflow can be evaluated by ultrasonography until the osseous elements begin to fuse. Thereafter, MR imaging is the procedure of choice because it permits evaluation of the spine and spinal cord in all planes of imaging and provides detailed evaluation of the effect of osseous abnormalities on neural structures. Finally, plain radiographs of the spine for evaluation of neonates who have any of a spectrum of sacral dimples are rarely helpful, and in the presence of significant cutaneous or subcutaneous abnormalities, ultrasonography is the preferred modality for evaluation.