The intelligence of hydrocephalic children.

In a group of 78 children with hydrocephalus in the first months of life, the level and pattern of intelligence were considered in relation to various parameters and symptoms of their condition. These included demography (age, sex, handedness); early developmental status; symptoms (visual, motor, and seizure); formative pathology; type of hydrocephalus; site of CSF obstruction; extent and configuration of cortical thinning; and shunt treatment. The common outcome of early hydrocephalus is an uneven growth of intelligence during childhood, with nonverbal intelligence developing less well than verbal intelligence. The origin of this selective cognitive deficit is in neither the hydrocephalic condition itself nor its treatment, but rather in the developmental brain anomalies and symptoms to which the hydrocephalic child is prone: In children with aqueduct blocks and intraventricular hydrocephalus, a selectively thin vertex and occipital lobe; in any hydrocephalic child, ocular abnormalities, motor deficits, and seizures.

Bilateral dissecting aneurysms of the intracranial internal carotid arteries in an 8-year-old boy.

Nontraumatic intracranial dissecting aneurysms have rarely been reported as the cause of acute infantile and childhood hemiplegia. The present case is unique because dissecting aneurysms occurred bilaterally in two clinically distinct episodes. A recent dissecting aneurysm of the right intracranial internal carotid artery was present with a healed dissecting aneurysm of left internal carotid artery in an 8-year-old boy.

Craniotelencephalic dysplasia in sisters: further delineation of a possible syndrome.

We describe two sisters with a complex of anomalies involving the cranium and brain. The changes in the former are consistent with those previously described as craniotelencephalic dysplasia and those in the latter indicate primary developmental abnormalities of the central nervous system including septo-optic dysplasia, absent olfactory nerves, agenesis of the corpus callosum, and lissencephaly. Per se, these cerebral malformations are causally heterogeneous, but their occurrence in association with craniotelencephalic dysplasia suggests that this combination is a distinct, probably autosomal recessive, syndrome.

Diastematomyelia: hemicord and meningeal sheaths; single and double arachnoid and dural tubes.

Metrizamide computed tomographic myelography demonstrates well: partial- and full-thickness diastematomyelia; the presence, shape, and (a)symmetry of the two hemicords; focal persistence of two anterior spinal arteries, one supplying each hemicord; the single or double subarachnoid space(s), arachnoid tubes, and dural tubes which encompass the hemicords; the dural septum formed by the medial walls of the two dural tubes; the dural cleft situated between the two walls of the dural septum; the bone spur within the dural cleft; and the presence of any tethering dorsal fibrous bands or aberrant dorsal nerve roots.

Radiologic characteristics of primitive neuroectodermal tumors in children.

Radiographic findings are reviewed in 31 children with primitive neural ectodermal tumors seen at the Hospital for Sick Children from 1962 through 1983. Seventeen children were initially evaluated with computed tomography (CT). Ten of these had both CT studies and angiography. The tumors were large, irregular, typically iso- to hyperdense, and showed dense, heterogeneous contrast enhancement. Cysts were present in 65% and calcifications in 71% of cases. The angiographic findings were nonspecific, ranging from avascular to markedly vascular. Although these tumors were usually found in the cerebral hemisphere, particularly the frontal lobes, two cases are included of tumors arising elsewhere: one in the pineal and one in the suprasellar region. Epidemiologic data are reviewed.

Contrast enhancement of the irradiated spinal cord in children.

Four children are reported in whom marked contrast enhancement of the spinal cord and roots was demonstrated by computed tomography months to years after relatively low dose therapeutic irradiation of paraspinal tumors, the radiation field including the cord. This phenomenon, previously unreported, probably represents subclinical radiation injury. None of the children had any neurologic abnormalities.

Iatrogenic embolization: complication of pediatric cerebral angiography.

Fourteen cases of inadvertent iatrogenic embolization of cerebral vessels occurring in a consecutive series of 3,731 angiographies in infants and children were studies. The incidence of embolization(0.9% of all patients ond 0.4% of all angiographic examinations) was about the same as has been reported in adults, but the clinical consequences were much milder, only one patient having documented transient neurologic symptoms. Also the angiographic appearance of the emboli differed from those described in adults. These differences may in part be due to the technique (general anesthesia with hyperventilation) but also to different reactions towards ischemia and to different types of emboli in children.

Choroid plexus size in young children with Sturge-Weber syndrome.

PURPOSE: To assess the size of the choroid plexus in young children with unilateral and bilateral Sturge-Weber syndrome. METHODS: Subjects included 15 children 4 years old or younger with Sturge-Weber syndrome. Eleven cases were unilateral and four were bilateral. Unilateral or bilateral involvement was determined by the distribution of abnormal leptomeningeal enhancement on MR images. The diameters of the choroid plexus were measured on contrast-enhanced axial MR images. The choroid plexus of the affected and unaffected sides in these cases were compared with those of 15 age-matched children without Sturge-Weber syndrome who were being examined for seizures. RESULTS: Our results show a wide variation in the size of the choroid plexus in children with Sturge-Weber syndrome; however, plexus associated with a hemisphere affected by Sturge-Weber syndrome were significantly larger than those on the unaffected side and in the age-matched control group. The size of the choroid plexus was positively correlated with the extent of leptomeningeal involvement as demonstrated by abnormal contrast enhancement. CONCLUSION: The choroid plexus is enlarged early in the course of Sturge-Weber syndrome in both unilateral and bilateral cases. There is a positive correlation between choroid plexus size and extent of leptomeningeal involvement in children with Sturge-Weber syndrome.

Benign suprasellar cysts: the CT approach.

Preoperative diagnosis of intracranial cysts has been simplified and made more rapid and accurate with computed tomography (CT). By means of conventional CT and CT metrizamide ventriculography, the position and communication of intracranial cysts with the ventricular system and subarachnoid space or cisterns can be demonstrated. Suprasellar arachnoid cysts can produce significant neurologic and endocrinologic abnormalities due to their position. They are a surgically curable cause of hydrocephalus. Preoperative differentiation from aqueduct stenosis or other causes of a large third ventricle is important. The usefulness of coronal CT and CT metrizamide ventriculography in the investigation of these lesions is illustrated in six patients.

Evaluation of the infant spine by direct sagittal computed tomography.

Direct sagittal computed tomography (CT) and metrizamide myelography, in addition to standard axial CT, have proven most useful in evaluation of complex anomalies of the infant spine. Direct sagittal CT was performed by placing the entire infant sideways and supine within the gantry after metrizamide was injected. This technique was performed in six infants with diagnoses of lipoma with dysraphism, lipomyelomeningocele, lipomyelocystocele, lumbosacral agenesis with cord regression, capillary hemangioma, and vertebral osteomyelitis. The technique showed the relation and/or extension of lesions in the dorsal ventral plane, particularly the presence or absence of subarachnoid, enteric, or genitourinary communication. Spinal and paraspinal anatomic detail was also demonstrated beautifully.

Primary meningeal tumors in children: correlation of clinical and CT findings with histologic type and prognosis.

PURPOSE: To identify the radiologic features that might help in preoperative differentiation of the meningiomas from the remaining primary meningeal tumors, in particular the malignant tumors. METHODS: The clinical and computed tomographic features of 21 children with histologically proved primary meningeal tumors were analyzed. FINDINGS: Benign tumors (meningiomas) are more likely to occur in older children, to have longer symptom duration, and to have CT appearances similar to the "typical" adult meningioma. Atypical CT features suggest a malignant meningeal tumor, such as meningeal sarcoma, melanoma, or meningeal primitive neuroectodermal tumor. The recent identification of a new subtype of meningioma (a "sclerosing" group) is discussed. This is common in children and the CT and clinical features are similar to those seen in other meningiomas. It is frequently mistaken histologically for an intraaxial tumor, or for an atypical or malignant meningioma. These sclerosing meningiomas may also show brain invasion but despite this, in the short term, the prognosis is no different from other meningiomas. CONCLUSION: The bad reputation previously ascribed to childhood primary meningeal tumors should be confined to that small group that are malignant. Meningiomas have a more favorable outlook.

Abnormal ocular enhancement in Sturge-Weber syndrome: correlation of ocular MR and CT findings with clinical and intracranial imaging findings.

PURPOSE: To estimate the prevalence of abnormal ocular enhancement in children with Sturge-Weber syndrome as detected with MR imaging and CT and to correlate this with the clinical, fundoscopic, and intracranial imaging findings. METHODS: Fifteen children, 4 years old or younger, with Sturge-Weber syndrome were examined with enhanced CT and MR imaging. Eleven children had unilateral intracranial involvement and 4 had bilateral involvement, for a total of 19 abnormal hemispheres and related orbits. The presence of ocular enhancement was compared with the fundoscopic findings independently. Ocular enhancement was correlated with the extent of leptomeningeal disease, the severity of the cutaneous lesion, and the presence of glaucoma by the calculation of likelihood ratios and 95% confidence limits. RESULTS: Seven of the 15 patients had abnormal ocular enhancement, which was present in 10 (53%) of the eyes associated with the 19 abnormal hemispheres. MR imaging showed choroidal hemangioma in 7 of 8 patients in whom hemangiomas were shown at fundoscopy. The likelihood of ocular enhancement was increased with the presence of bilateral disease, extensive facial nevi, and glaucoma; there was no significant correlation with the extent of hemispheric involvement. CONCLUSION: Both enhanced MR imaging and CT can show diffuse choroidal hemangioma in patients with Sturge-Weber syndrome. However, MR imaging is more sensitive and is recommended to aid in the detection of abnormalities with preventable late complications.

Melanotic neuroectodermal tumor of infancy: clinical, radiologic, and pathologic findings in five cases.

Five pathologically proved melanotic neuroectodermal tumors of infancy are reported. These rare neoplasms of infancy exhibit a distinct predilection for the maxillary bone. Three tumors originated in the maxilla, one in the calvaria, and one in the cerebellar vermis. Those occurring in bone did not metastasize but were locally invasive, as reflected in their radiologic appearance. Bone erosion, expansion, hyperostosis, and osteogenesis can occur in the same neoplasm and were appreciated best on CT. MR imaging showed the soft-tissue component and extent of the neoplasm better than CT did. The pathologic findings from all five cases (and one possibly related melanotic tumor of the face) revealed abundant melanin. MR imaging of two melanotic tumors showed isointense T1-weighted and slightly hyperintense T2-weighted signals. This appearance is contrary to that of most melanin-containing tumors, which exhibit enhanced T1 and T2 relaxation, and indicates that variables other than the absolute amount of melanin may determine the MR signal. Clinically, rapid neoplastic growth and excessive melanin production by the tumor cells caused facial disfigurement and visible blue black discoloration. All five melanotic neuroectodermal tumors were resected and the vermian tumor was also irradiated. Four of five children were well and free from disease 1 month to 7 years after resection. The calvarial tumor was incompletely resected and involved the underlying brain, eventually causing death. The clinical, radiologic, and pathologic features of melanotic neuroectodermal tumors of infancy are reviewed. Melanotic neuroectodermal tumors of infancy that involve bone can be diagnosed from the clinical and radiologic findings. Prompt diagnosis and surgical resection are essential for cure.

Benign intrinsic tectal "tumors" in children.

A specific group of intrinsic dorsal midbrain tumors was identified in six children by computerized tomography (CT) and magnetic resonance (MR) imaging. Each patient presented with raised intracranial pressure as a result of hydrocephalus due to obstruction of the sylvian aqueduct. No patient had brain-stem signs referable to the tectal tumor initially or subsequently. All six children underwent cerebrospinal fluid (CSF) diversionary procedures. The radiological features were consistent and specific, with all patients showing tectal calcification or primary increased attenuation of the tectal plate on CT scans. In addition, lack of contrast enhancement was noted initially in four patients and eventually in all six patients. In all patients MR imaging showed a focal tectal tumor distorting the collicular plate with no cystic component and increased signal intensity on T2-weighted images. There has been no evidence of progression in these six patients in the follow-up period ranging from 8 months to 17 years (8 months and 2 1/2, 4 1/2, 8, and 17 years). Diversion of CSF has been the only surgical treatment and no patient underwent deep x-ray therapy. Five patients have had normal intellectual development. In contrast to the majority of previously described periaqueductal and tectal tumors, this group of lesions appeared to be truly benign. The authors suggest that patients presenting with these clinical and radiological features may be managed by CSF diversion, serial examination, and MR imaging.

Pathology of temporal lobectomy for refractory seizures in children. Review of 20 cases including some unique malformative lesions.

Significant pathological abnormalities were encountered in a series of 20 temporal lobectomies in children with intractable complex partial seizures. In particular, "dual pathology" (mesial temporal sclerosis with other lesions) was found rather than mesial temporal sclerosis as the only lesion. Unusual pathological findings included capillary penetration of neurons in a neuronal heterotopia in one patient, and foci of extensive cortical disorganization in some cases of mixed tumors and gangliogliomas. A high proportion of neuronal migration disorders was also seen with overlapping pathological features between cortical dysplasia and tuberous sclerosis. In this correlative clinical, radiological, electroencephalographic, and pathological study, some of the pathological lesions in children did not fit the classical categories of neoplasia and malformation and transitional forms were rarely encountered.

Pediatric spinal neoplasms.

Magnetic resonance imaging (MRI) is an accurate and noninvasive tool in the evaluation of children with spinal neoplasm. Localization and definition of site and extent of disease for treatment planning, the most important goals of neuroimaging in spinal neoplastic disease, have become more precise with MRI, because all spinal compartments can be seen without the need for subarachnoid puncture. Epidural and contiguous soft tissue disease, and associated vertebral abnormalities such as marrow replacement by metastatic processes, are readily confirmed during the same examination. In children, as in adults, gadolinium-diethylenetriaminepentaacetic acid aids in separating intramedullary neoplasms from associated edema or syrinx, and is essential in the evaluation of intradural-extramedullary spread of neoplasm. MRI is also useful in the exclusion of compressive lesions in children with systemic neoplasms and cord infarction or treatment-related transverse myelopathy.

Magnetic resonance imaging evaluation of delayed myelination in Down syndrome: a case report and review of the literature.

Magnetic resonance imaging has been found to be useful in assessing brain myelination and provides information on brain maturation. The normal pattern of brain myelination conforms to a fixed sequence, with good pathologic and MRI correlation. Neuropathologic analysis of myelination has shown delayed central myelination in Down syndrome. Delayed myelination on MRI in Down syndrome has not previously been reported. We report a case of Down syndrome with a significant delay in myelination as demonstrated on MRI. This 18-month-old infant had brain myelination equivalent to that expected for an 11-month-old infant. To determine the relative incidence, extent of delayed myelination, and time for recovery to full myelination in Down syndrome, more cases require examination and assessment. Magnetic resonance imaging has the advantage of serial assessment of myelination during brain maturation.

The "empty sella" in childhood.

The "empty sella" (ES), a common entity in adults, is so named because a cerebrospinal fluid-filled arachnoid hernia fills the pituitary fossa and compresses the pituitary gland, creating the illusion of an "empty" sella. We report our experience of ES in childhood. Our four cases represent associations with ES that have not been previously described in childhood: case 1 was associated with central precocious puberty; case 2 with secondary ES resulting from shrinkage of a prolactinoma during bromocriptine therapy; case 3 with pseudotumor cerebri; and case 4 with no significant pathology. Together, they account for approximately 1 percent of cases of radiographically enlarged sella tursica investigated here. This brings the number of cases of ES reported in children to 27. Review of these 27 cases reveals seven with local skull dysplasia and/or a familial inheritance. The remainder lacked clinical homogeneity or similarity to the common adult variety of ES. Children with ES did not exhibit the higher female preponderance reported in adults with ES. They showed a higher frequency of secondary cases (4/27), and of associated endocrine (10/15) and visual (8/17) abnormalities than is seen in adults with the "empty sella."