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Congenital hydrocephalus results from a variety of causes, some of the most common include spina bifida (myelomeningocele), aqueductal stenosis, and Dandy-Walker malformation. In addition, a number of cases result from genetic causes, other malformations, postinfectious, or neoplastic conditions. Outcome varies with cause but can be favorable. Most cases still are managed with shunting, although endoscopic modalities also can be considered.

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An astroblastoma of high-grade type arising in the brain of a 3-year-old child is reported. The first description of the ultrastructural, immunohistochemical, and cytogenetic findings in this rare tumor variant are presented.

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BACKGROUND/PURPOSE: The aim of this study was to investigate driveway-related injuries in children, identify associated risk factors, and evaluate outcome compared with other mechanisms of blunt trauma. METHODS: A 6-year review (1991 to 1996) of pediatric (age less than 18 years) pedestrian injuries treated at two urban trauma centers was conducted: one regional pediatric trauma center and one level I trauma center with pediatric commitment. Five hundred twenty-seven children injured in pedestrian accidents were identified from the trauma registry; 51 children (10%) sustained traumatic injuries as a result of being struck in their driveway. Data are reported as mean +/- SEM. RESULTS: Children less than 5 years of age (n = 41) had an injury severity score (ISS) of 12.3+/-2.3, 15 (37%) sustained closed head injury, 13 (37%) had torso trauma, 19 (46%) skeletal trauma, and eight (20%) died. Children > or = 5 years old (n = 10) had an ISS of 10.7+/-2.4, three (30%) sustained closed head injury, four (40%) torso trauma, six (60%) skeletal trauma, and none died. In contrast, all other pediatric pedestrian accidents analyzed over the same time period had a mortality rate of only 2% (11 of 476). CONCLUSIONS: Pediatric driveway trauma carries a significant risk of head injury and a 10-fold increase in mortality in children under 5 years of age when compared with all other pediatric pedestrian accidents. More emphasis must be placed on injury prevention and public education to prevent this devastating mechanism of injury in these young, vulnerable children.

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Blunt carotid injury (BCI) is a rare entity which can have devastating neurologic consequences. Little has been reported on the mechanism of injury, presentation or management of these injuries in children. We present a series of 5 children with BCI. One patient died at presentation while the remainder developed delayed infarctions. Three surviving patients developed intracranial hypertension and required intracranial pressure (ICP) monitoring. Surgical resection of infarcted tissue was required to control ICP in 2 patients. All four surviving patients are impaired but ambulatory. We propose an aggressive management strategy for BCI aimed at early detection of deficit, early angiography, anticoagulation if appropriate, and active management of ischemia including hemodynamic treatment, ICP monitoring, and active use of medical and surgical means to monitor and control intracranial hypertension.

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An elevated serum carcinoembryonic antigen (CEA) without evidence of neoplasia was noted in an 84-year-old woman. She subsequently developed a lytic skull lesion, which at surgery proved to be an atypical meningioma. Immunohistochemical analysis demonstrated that this tumor was producing CEA. This aggressive meningioma required two further resections and adjuvant radiotherapy in the following 18 months. Serum elevation of CEA has been reported only twice in association with meningiomas; both of those tumors were of the secretory subtype.

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Ongoing research in the etiology of neural tube defects is increasingly being directed towards the molecular mechanisms at work in the formation of these complex lesions. We undertook to review the family history of patients in a large myelomeningocele/spina bifida clinic in an effort to identify genetic trends in these families, particularly as they relate to current research efforts and laboratory models. Surveys were received from 363 patients (35.5% of the clinic population) and analyzed. The myelomeningocele recurrence rate was 4.3%. Seven sets of twins were identified and all were discordant for their spinal lesions. A family history of spina was found to be evenly distributed between maternal and paternal relatives, rather than tending to follow through the maternal side. Epilepsy was more commonly found on the maternal side of the family, most likely reflecting the postulated causal relationship between maternal anticonvulsant use and the occurrence of spina bifida, although also possibly supporting the concept that a genetic predisposition for maternal epilepsy may also be associated with a higher frequency of birth defects among children of epileptics, independent of anticonvulsant use. Patients with spina bifida in the setting of Waardenburg syndrome and fragile X syndrome were also identified and will be discussed.

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We report 5 cases of complete, or nearly complete, agenesis of the lumbar and sacral spine. In 1 case pregnancy was terminated at 23 weeks of gestation, and the others were livebirths. The surviving 4 patients were all infants of diabetic mothers. Imperforate anus was present in 2 of 4 liveborn patients and was suspected in the abortus. All liveborn cases were paraplegic, and all exhibited the 'Buddha' deformity of the lower extremities. One patient had congenital obstructive hydrocephalus, probably representing a variant of holoprosencephaly, requiring a ventriculoperitoneal shunt. No other nervous system anomaly was identified in any patient, and the other patients are showing normal cognitive development. We propose that these patients have defects which occurred during germ cell formation (i.e., during gastrulation), with interruption of the primitive streak resulting in combined failure of primary and secondary neurulation.

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A case of intracranial mixed malignant germ cell tumor (GCT) in a patient with the Klinefelter syndrome (KS) is reported. Extragonadal GCTs, including those of intracranial origin, have previously been noted in KS patients. A review of the English literature suggests that although this phenomenon is rare, there appears to be more than a coincidental relationship between GCTs and a 47,XXY karyotype. This case represents the sixth reported case of intracranial GCT in KS but the first to be histologically confirmed to have mixed malignant germ cell elements. This occurrence of malignant cell types in a KS patient emphasizes the need for a histologic diagnosis prior to initiation of therapy.

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Pituitary adenomas are uncommon in childhood. Between 1975 and 1988, 36 patients underwent transsphenoidal resection at the Mayo Clinic before their 17th birthday. Fifteen (41.7%) had prolactin (PRL)-secreting tumors, 16 (44.4%) had adrenocorticotropic hormone (ACTH)-secreting tumors (including two patients with Nelson-Salassa syndrome), and three (8.3%) had tumors secreting growth hormone (GH); two patients (5.6%) had clinically nonfunctioning tumors. Of the 36 patients, 21 (58.3%) were girls, and girls predominated in every group except that with ACTH-secreting tumors, where they accounted for only 37.5% of the patients. The average age at the time of surgery was 14.7 +/- 2.2 years, the youngest patient being 7.3 years old. The ACTH-secreting tumors were associated with an earlier onset of symptoms than the other tumor types: six (37.5%) of 16 patients became symptomatic before 10 years of age, compared with two (10.0%) of the 20 other patients. Presenting symptoms primarily reflected endocrine dysfunction, with neurological presentation (visual field deficits) occurring in only two patients. There were no deaths in this patient group. Significant operative morbidity included steroid-induced psychosis in one patient and diabetes insipidus in three, which resolved in two. Plurihormonal tumors, broadly defined as tumors producing more than one hormone, were common, comprising five (36%) of 14 PRL-secreting tumors, all three GH-secreting tumors, and one of two clinically nonfunctioning tumors, or 25.7% overall. Macroadenomas accounted for 89% of plurihormonal tumors. Long-term follow-up review (median > 5 years) revealed good control of PRL-secreting tumors (although five of 15 patients had received postoperative radiotherapy), contrasted with a 25% late recurrence rate for ACTH-secreting tumors, which had an 80% initial remission rate. The majority of patients had good endocrine function, including reproductive function. It is concluded that: 1) although pediatric pituitary adenomas occur primarily in adolescence, Cushing's disease is found at any age; 2) transsphenoidal surgery is feasible and safe in this age group; 3) plurihormonal tumors occur more frequently in the pediatric age group than in adults; and 4) long-term control rates in PRL- and ACTH-secreting tumors are probably similar to those seen in adults.

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Cerebrovascular complications of sickle cell anemia are well known to be one of the more common causes of stroke in the pediatric age group. Although it is far less common, the association of stroke with sickle cell trait has been described in young adults and only twice in children in the English literature. In this report, we describe a case of a posterior circulation infarct occurring in a 12-year-old male who also had a normal variant vertebral artery, which may have been a contributing factor in his presentation. The literature is reviewed and other possible risk factors for stroke in sickle cell trait are discussed.

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We believe that children beyond 5 years of age with stable ventriculomegaly can be monitored if their intellectual performance is within the normal range and stable. The child 3 years old or younger with moderate or greater ventriculomegaly should be shunted. Close follow-up with objective data is essential for all other children with compensated hydrocephalus, as well as for all children with a shunt.

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The authors report a series of seven patients with myelopathy who were found to have spinal dural arteriovenous (AV) fistulas in which the nidus was located at some distance from the spinal cord. The nidus was intracranial in three cases and involved a sacral nerve root sheath in the other four; in each case, the arterialized draining vein led into the coronal plexus of medullary veins. A lack of normal draining radicular veins was noted in all cases. Magnetic resonance images were obtained in four patients and demonstrated spinal cord tissue changes only in the lower thoracic cord in three cases and in the cervical cord in one, all consistent with an ischemic process secondary to venous hypertension. Five patients were managed surgically by division of the draining vein, with improvement of the neurological deficit in all. One patient was treated by embolization alone and had stabilization of her deficit. The remaining patient in the series died of unrelated systemic disease before the spinal dural AV fistula could be treated. These cases support the theory that venous hypertension is the dominant pathophysiological mechanism involved in spinal dural AV fistulas independent of their location. In patients with a suspected spinal dural AV fistula, lumbar and thoracic spinal angiography will reveal the site of the fistula in the majority of cases (88% in this series). In the remaining patients, the possibility of a remote fistula must be considered. The lack of normal venous drainage of the cord following injection in the artery of Adamkiewicz is the most reliable indicator of venous hypertension in the cord and can be helpful in making the decision to proceed with a search for a cranial or sacral arterial supply.

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Two patients who had focal neurological deficits as the initial manifestation of a malignant non-Hodgkin's lymphoma involving the skull are described. Soft-tissue masses and variable bone destruction were the predominant computed tomographic findings, and magnetic resonance imaging studies revealed meningeal involvement in one case and sinus thrombosis in the other. Malignant non-Hodgkin's lymphomas initially appearing in the skull are rare, but these lesions should be considered in patients with a palpable scalp mass who have focal neurological signs.

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STUDY OBJECTIVE: To determine the frequency of baby walker use as a contributing factor in head injuries in children less than 2 years old. DESIGN: Retrospective clinical review. SETTING: Urban Level I trauma center and multispecialty clinic. TYPE OF PARTICIPANTS: All children less than 2 years old who were evaluated for a head injury during a three-year period. MEASUREMENTS AND MAIN RESULTS: 129 patients' cases were reviewed. Walker-related injuries occurred in 19 of 129 patients (14.7%). This represents the third most common mechanism of injury in this age group. Mean patient age at the time of injury was 8.7 months. Of the 19 accidents involving walkers, 18 (94.7%) involved falling down stairs. Nine children (47.4% of all walker-related injuries) suffered fractures of the cranial vault. No patients required surgical intervention, although one required treatment for post-traumatic meningitis. CONCLUSION: Baby walkers continue to be a frequent cause of head injury in this age group, and further efforts must be made to deal with these preventable injuries.

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Radiation-induced meningiomas are becoming increasingly well-recognized. We report a case of a 35-year-old man who developed a suprasellar meningioma 9 years after receiving a radiation dose of 4480 cGy for a pituitary adenoma. The literature is also reviewed.

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The technique of computer-assisted stereotactic resection of intra-axial neoplasms can also be used in the treatment of vascular malformations. We report a series of 12 pediatric patients with supratentorial lesions who underwent stereotactic resections between 1985 and 1988. There were 5 boys and 7 girls, with mean age 8 years (range 3-16). Epilepsy was the presenting symptoms in 8 children, hemorrhage in 3, and in 1 the lesion was incidentally diagnosed. Seven lesions were angiographically occult. All lesions were resected without mortality or morbidity. Seizures resolved in 7 of 8 cases, with the remaining patient experiencing a reduction in seizure frequency. Illustrative cases are presented.

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