Erin C. Prenger

Stroke in Children Within a Major Metropolitan Area: The Surprising Importance of Intracerebral Hemorrhage:

Our objective was to determine the incidence rate of stroke and stroke subtypes in children. We reviewed the medical records, autopsy records, and brain imaging studies of all children with a possible stroke within the Greater Cincinnati metropolitan area population of nearly 1.3 million during 1988 and 1989. Traumatic brain hemorrhages and germinal matrix hemorrhages were excluded. Of the 295,577 children in Greater Cincinnati, medical records of 178 children were screened. Sixteen cases (13 whites and three blacks) less than age 15 years fit strictly defined criteria for first-ever stroke. The incidence rate for cerebral infarction was 1.2 cases per 100,000 (95% confidence interval, 0.3 to 2.0). The combined incidence rate for intracerebral hemorrhage and subarachnoid hemorrhage was 1.5 cases per 100,000 children (95% confidence interval, 0.4 to 2.3). The incidence rate of all stroke in white children was 2.6 cases per 100,000 (95% confidence interval, 1.2 to 4.1), compared to 3.1 per 100,000 in black children (95% confidence interval, 0 to 6.6). The combined 30-day mortality for intracerebral hemorrhage and subarachnoid hemorrhage was 22% (two of nine) compared to 14% (one of seven) for cerebral infarction. We conclude that in contrast to the picture in adults, hemorrhagic stroke among infants and children is at least as common as ischemic infarction. (J Child Neurol 1993;8:250-255).

Acquired chiari malformations : Incidence, diagnosis, and management

The acquired descent of the cerebellar tonsils radiographically indistinguishable from Chiari I malformations has been previously reported. The relationship between lumbar shunting procedures for hydrocephalus and symptomatic Chiari malformations has been established. We report the cases of 10 children with lumboperitoneal shunts in whom previous radiographic studies had confirmed a normal hindbrain configuration. Seven of the 10 patients acquired tonsillar descent into the foramen magnum, detected by magnetic resonance imaging, whereas the others remained normal. Four of seven patients were symptomatic; two underwent the removal of the lumboperitoneal shunt and conversion to a ventriculoperitoneal shunt, and two underwent posterior fossa decompression. Further magnetic resonance imaging revealed that one of the two patients who underwent conversion shows ascent of the cerebellar tonsils. All four patients became asymptomatic less than 6 months after treatment. In this article, we discuss seven cases of acquired Chiari malformations and the complete reversal of an acquired Chiari malformation after surgical treatment, as documented by magnetic resonance imaging. Cases of asymptomatic acquired Chiari malformations are reported, including those resulting from shunting for intracranial hypertension without hydrocephalus. We conclude that a craniospinal pressure gradient creates a potential for cerebellar tonsil descent and recommend that ventriculoperitoneal shunting be performed in children with communicating hydrocephalus to avoid this potential complication. We also recommend annual surveillance of the cervicomedullary junction in children with lumboperitoneal shunting. Finally, if symptomatic tonsillar descent occurs from lumbar shunting, a trial conversion to ventriculoperitoneal shunting may eliminate the need for posterior fossa decompression.

Ethanol endovascular management of brain arteriovenous malformations: Initial results

OBJECTIVE The goal was to determine the safety and efficacy of absolute ethyl alcohol treatment in the management of intra-axial brain arteriovenous malformations (AVMs). METHODS Seventeen patients (eight female and nine male patients; mean age, 41 yr) underwent ethanol endovascular therapy for treatment of their brain AVMs. Superselective amytal testing preceded all procedures. Neuroleptic intravenous anesthesia was used for 16 patients, and general anesthesia was used for 1 patient. Follow-up monitoring consisted of clinical evaluations, magnetic resonance imaging, and arteriography. RESULTS In follow-up evaluations (mean follow-up period, 13 mo) after embolization of brain AVMs, neither vascular recanalization nor the neovascular recruitment phenomenon was observed in any patient. Progressive AVM thrombosis at arteriographic follow-up evaluation was a constant feature. Seven patients were cured of their AVMs with ethanol endovascular therapy alone. Three patients were cured of their lesions with ethanol embolization plus surgical resection. One patient was cured of his lesion with ethanol embolization and radiation therapy of the residual nidus. Three patients underwent only partial therapy, with significant improvement in symptoms. Three patients are currently undergoing ethanol endovascular therapy. Complications occurred with 8 of 17 patients, most of which were transient. Two patients died because of late subarachnoid hemorrhages, one patient 4 months and one patient 14 months after partial therapy. CONCLUSION Progressive and permanent AVM occlusion is a common finding in arteriographic follow-up evaluations. In no patients did arterial recanalization or the neovascular recruitment phenomenon occur. Our initial results indicate that ethanol has a permanence that is seldom encountered with other embolic agents. With aggressive decadron therapy, the complications related to swelling in the brain are largely reversible.

Acquired Chiari MalformationsIncidence, Diagnosis, and Management

ABSTRACTTHE ACQUIRED DESCENT of the cerebellar tonsils radiographically indistinguishable from Chiari I malformations has been previously reported. The relationship between lumbar shunting procedures for hydrocephalus and symptomatic Chiari malformations has been established. We report the cases of

Review of the embryologic development of the pituitary gland and report of a case of hypophyseal duplication detected by MRI

We describe the clinical manifestations, associated abnormalities, MRI appearances and pathologic significance of a case of hypophyseal duplication. A 16-year-old girl presented with delayed sexual development and history of midline craniofacial anomalies. MRI revealed paired infundibula extending inferiorly to two small pituitary glands, a midline hypothalamic mass, and a midline cleft in the basisphenoid. Twelve cases of pituitary duplication have previously been described. The suggested pathogenesis is duplication of the prechordal plate and anterior end of the notochord during early embryologic development.

Sigmoid sinus thrombosis after closed head injury in children.

In the literature, clinical descriptions of sigmoid sinus thrombosis occurring after closed head injury in children are rare. One to 5 days after trauma to the back of the head, five children (aged 1 to 7 yr) presented with gait ataxia, vomiting, and headache. Trauma was mild in four children. Computed tomography of all the children, performed within 5 days after the injury, showed focal hyperdensity in the region of the left sigmoid sinus. Four children had extra-axial hyperdense collections along the left transverse sinus, and three had skull fractures adjacent to the left sigmoid sinus. Magnetic resonance imaging (MRI) of all the children, performed 2 to 6 days after injury, showed left sigmoid-sinus thrombosis and decreased flow or thrombosis within the lateral third of the left transverse sinus. All the children had MRI scans 4 to 6 weeks after their diagnosis and were followed up for 1 to 12 months. In four children whose symptoms subsided completely within 2 to 10 weeks, MRI showed recanalization of the sigmoid sinus within 4 to 6 weeks after injury. In one child whose symptoms resolved after 6 months, sigmoid-sinus thrombosis persisted with the formation of collateral flow. We conclude that traumatic sigmoid-sinus thrombosis should be suspected when a child has persistent or delayed gait ataxia and vomiting after injury to the back of the head. Computed tomography characteristically demonstrated focal hyperdensity within the sigmoid sinus that we term the dense sigmoid-sinus sign. Because the sinus recanalized and the symptoms subsided in most children within 6 weeks, we conclude that prophylactic medical or surgical intervention is not indicated.

Seizure outcome and complications following hypothalamic hamartoma treatment in adults: endoscopic, open, and Gamma Knife procedures

OBJECT This study aimed at identifying outcomes with respect to seizures, morbidity, and mortality in adult patients undergoing resective or Gamma Knife surgery (GKS) to treat intractable epilepsy associated with hypothalamic hamartoma (HH). METHODS Adult patients undergoing surgical treatment for HH-related epilepsy were prospectively monitored at a single center for complications and seizure outcome by using a proprietary database. Preintervention and postintervention data for patients 18 years of age and older, and with at least 1 year of follow-up, were analyzed, with specific attention to seizure control, complications, hormonal status, and death. RESULTS Forty adult patients were found in the database (21 were women). The median HH volume was 0.54 cm(3). In 70% of patients, it was located inside the third ventricle, attached unilaterally and vertically to the hypothalamus (Delalande Type II). Most patients (26) underwent an endoscopic resection, 10 patients had a transcallosal or other type of open (pterional or orbitozygomatic) resection, and 4 patients chose GKS. Twenty-nine percent became seizure free in the long term, and overall a majority of patients (55%) reported at least > 90% seizure improvement. Only 3 patients were ultimately able to discontinue anticonvulsants, whereas most patients were taking an average of 2 antiepileptic drugs pre- and postoperatively. The only factor significantly correlated with seizure-free outcome was the absence of mental retardation. The HH volume, HH type, and amount of resection or disconnection were not correlated to seizure freedom. A total of 4 patients (10%) died, 2 immediately after surgery and 2 later. All of them had undergone a resection, as opposed to GKS, and still had seizures. Postoperatively, persistent neurological deficits were seen in 1 patient; 34% of patients had mild hormonal problems; and 59% experienced weight gain of at least 6.8 kg (average gain 12.7 kg). CONCLUSIONS Surgical or GKS procedures in adults with HH provided seizure freedom in one-third of patients. The only significant favorable prognostic factor was the absence of mental retardation. The overall mortality rate was high, at 10%. Other important morbidities were persistent hormonal disturbances and weight gain.

Therapeutic alternatives in the treatment of life-threatening vasoformative tumors

Although classified as benign vasoformative tumors, a small percentage of hemangiomas and lymphangiomas follow an unpredictable biologic course. Rapid growth and the involvement of vital structures can lead to a variety of life-threatening complications. Aggressive treatment using a variety of therapeutic modalities is required in these situations. Our clinical experience with three children with life-threatening benign vasoformative tumors is presented, followed by a discussion of the diagnostic and therapeutic considerations bearing on such aggressive lesions.

Magnetic resonance imaging of fourth ventricular choroid plexus neoplasms in childhood. A report of two cases.

Choroid plexus neoplasms (CPN) are rare tumors occurring with a relative incidence of 0.5% of intracranial neoplasms in all age groups and 1.5-6.4% of all pediatric brain tumors. In children, the most common site of origin is the atria of the lateral ventricles where the CPN may represent either a carcinoma or a benign papilloma. CPN arising in the fourth ventricle are more common in adults, and are more likely to represent a benign papilloma. We present 2 children with CPN arising from the inferior fourth ventricle with similar features on MRI, one of which represented a benign papilloma and the other a malignant carcinoma. CPN should be included, even in young children, in the differential diagnosis of any infratentorial, intraventricular tumor.