McComb Jg

Factors affecting intellectual outcome in pediatric brain tumor patients

A prospective study utilizing repeated intellectual testing was undertaken in 73 children with brain tumors consecutively admitted to Childrens Hospital of Los Angeles over a 3-year period to determine the effect of tumor location, extent of surgical resection, hydrocephalus, age of the child, radiation therapy, and chemotherapy on cognitive outcome. Forty-three patients were followed for at least two sequential intellectual assessments and provide the data for this study. Children with hemispheric tumors had the most general cognitive impairment. The degree of tumor resection, adequately treated hydrocephalus, and chemotherapy had no bearing on intellectual outcome. Age of the child affected outcome mainly as it related to radiation. Whole brain radiation therapy was associated with cognitive decline. This was especially true in children below 7 years of age, who experienced a very significant loss of function after whole brain radiation therapy.

To shunt or to fenestrate: which is the best surgical treatment for arachnoid cysts in pediatric patients?

The treatment options for intracranial arachnoid cysts are either craniotomy and fenestration of the cyst into the cerebrospinal fluid spaces or shunting of the cyst contents extracranially. Fenestration may eliminate the need to shunt, but it is a major operative procedure and is not always successful. To determine which treatment provides the greatest benefit with the fewest complications, the records of 31 patients with 34 arachnoid cysts treated at the Childrens Hospital of Los Angeles between 1976 and 1986 were reviewed. The mean age of the patients was 4.4 years, with a range of 0 to 15.5 years. The most common location was the middle fossa (14 cases), followed by the posterior fossa (7 cases), the suprasellar region (5 cases), and hemispheric (5 cases) and other locations (3 cases). Signs and symptoms were related to abnormally rapid head growth in infants and to increased intracranial pressure and seizures in older children. The initial treatment of 29 cysts was fenestration. Twenty-two (76%) procedures were successful, with no additional treatment needed for the cyst. The other 7 cysts required the subsequent placement of a cystoperitoneal shunt. In 5 cases, the cysts were treated initially with cystoperitoneal shunts. Of the total 12 cystoperitoneal shunts, 5 have required revisions on one or more occasions. No significant difference in morbidity was noted between the two treatment options. Because we consider shunt independence to be a major goal of therapy, we suggest that patients with arachnoid cysts be divided into two categories, those presenting with associated hydrocephalus and those without hydrocephalus.(ABSTRACT TRUNCATED AT 250 WORDS)

Unusual presentations of cerebral solitary fibrous tumors: report of four cases.

OBJECTIVE AND IMPORTANCEFour rare cases of central nervous system solitary fibrous tumors (C-SoFTs) are described. This tumor has not previously been reported in children or in deep cortical structures. Three of these tumors occurred in the posterior fossa. Only four cases in the posterior fossa have been described previously. Nine years after its debulking from the posterior fossa, one tumor disseminated to the spine, lung, and liver. Only one such aggressive C-SoFT has been described previously. CLINICAL PRESENTATIONA 7-year-old child had had 1 year of right-sided weakness at presentation. Magnetic resonance imaging scans revealed a left basal ganglia lesion. A 49-year-old woman and a 30-year-old man presented after experiencing headaches for months. Magnetic resonance imaging scans in these patients revealed a tumor in the fourth ventricle and right jugular foramen, respectively. A 55-year-old man had spinal, liver, and lung dissemination of a previous posterior fossa tumor at presentation. INTERVENTION AND TECHNIQUEAll four patients underwent craniotomy for resection or subtotal removal of the tumor. Intraoperative observations noted solid well-encapsulated tumors. Immunohistochemistry confirmed C-SoFTs in all four cases. CONCLUSIONC-SoFTs are rare central nervous system, typically dural-based, tumors. They frequently are overlooked in the differential diagnosis of solid central nervous system tumors. Our findings suggest that these tumors can occur at any age and in most locations, regardless of proximity to the meninges (basal ganglia and ventricle), suggesting that the cells of origin are not meningothelial, but rather the mesenchyme of the cerebral vasculature. T1- and T2-weighted magnetic resonance imaging was notable for areas of hypointensity and of hyperintensity best described as patchy or a ying-yang appearance in all cases. Delayed extracranial metastasis may be noted.

Diffuse arteriovenous malformations: a clinical, radiological, and pathological description.

In a review of our series of patients with arteriovenous malformations (AVMs), a group with atypical angiographic and histopathological characteristics was discovered. Unlike the typical AVM, these lesions contained normal cerebral tissue between the abnormal vessels. We call these lesions diffuse AVMs, and think that this AVM represents one end of the AVM spectrum from a tight nidus to a diffuse lesion. The mean age of these patients was 18.1 years. Eight patients presented with an intracerebral hemorrhage, two with seizures, one with headache without hemorrhage, and one with ischemic symptoms compatible with vascular steal. Cerebral angiography revealed three AVMs to be 2 to 4 cm in diameter, four were 4 to 6 cm in diameter, and five were > 6 cm in diameter. Characteristic angiographic features included multiple small arterial feeders, small ectatic vessels in the malformation itself, multiple small draining veins, and a diffuse, puddling appearance of the contrast dye. Despite 16 operations in 11 patients, complete resection of the AVM was accomplished in only 8. The four patients with residual disease have received radiation therapy. Histopathology of the surgical specimens found AVM vessels interspersed among normal appearing neurons and white matter. Leptomeningeal angiodysplasia was noted when the cerebral cortex was involved. Gliosis was noted in some cases. Diffuse AVMs represent a difficult surgical challenge and recognition of the lesion aids in surgical planning.

Management of Hydrocephalus Secondary to Intraventricular Hemorrhage in the Preterm Infant with a Subcutaneous Ventricular Catheter Reservoir

To control hydrocephalus resulting from massive intraventricular hemorrhage in premature neonates with respiratory distress syndrome, we inserted a specially designed low profile subcutaneous ventricular catheter reservoir (reservoir) by the 12th day of life (average; range, 3 to 30 days) in 20 neonates whose mean birth weight was 1110 +/- 270 g (28.7 +/- 1.6 weeks of gestation). The reservoir was repeatedly aspirated over 10 to 48 days. No cerebrospinal fluid infection, reservoir obstruction, or breakdown of the skin overlying the reservoir occurred. Serial computed tomographic scans documented control of the hydrocephalus and an increase in the thickness of the cortical mantle of the survivors. No mortality was associated with placement of the reservoir or its subsequent conversion, if necessary, to a ventriculoperitoneal shunt. However, only 7 of the 20 infants survived. On follow-up 3 to 5 years later, 2 of the 7 have normal intellectual and motor development. Two infants are normal intellectually, but have a motor deficit. The remaining 3 patients have both significant intellectual and motor developmental delay. The use of the reservoir is offered as a safe and effective alternative to repeated ventricular punctures, external ventricular drainage, or initial shunting. Aggressive management of hydrocephalus secondary to intraventricular hemorrhage may improve neurological function in some surviving neonates.

Intracranial pressure monitoring in severe pediatric near-drowning

Between April 1979 and April 1981, 20 near-drowned children admitted to Childrens Hospital of Los Angeles with a Glasgow coma score of 3 underwent intracranial pressure (ICP) monitoring and brain resuscitative therapy. By the 3rd hospital day, 14 patients had developed ICP elevation above 20 torr, and 11 of these had sustained intracranial hypertension above 30 torr. Ten of these children died, and 4 remain in a persistent vegetative state. Of 6 patients whose ICP never exceeded 20 torr, 3 recovered completely, 1 died, and 2 remain without any cognitive function. The salvage rate for patients with normal ICP after near-drowning accidents is significantly better than that for patients in whom the ICP is elevated (P = 0.017). Intracranial hypertension is associated with a uniformly bad outcome and is frequent in patients who die or suffer permanent, severe central nervous system damage after near-drowning. Language: en

Treatment of Spinal Cord Compression by Epidural Malignancy in Childhood

Epidural spinal cord compression by a malignant tumor is a rare occurrence in children. Both the tumors involved and the extent of involvement of the vertebral column are different in children and adults. Often, the epidural tumor in a child is identified before significant spinal canal compromise has occurred, and these children frequently can be managed by radiation therapy and/or chemotherapy. There is a group of children, however, who have severe spinal canal encroachment by a tumor, as evidenced by a near complete or complete block on myelography. In this study, we report a group of patients with severe spinal cord compression, as documented by imaging studies. We compared the results of a decompressive laminectomy and subtotal tumor resection followed by adjuvant therapy with the results obtained with radiation therapy and/or chemotherapy alone. Thirty-three patients met the criteria for inclusion in the study. Twenty-six were treated with a laminectomy and adjuvant therapy, and 7 were treated without surgical intervention. With surgical therapy, 25 of 26 epidurals were either improved or stable, whereas 4 of 7 nonsurgical patients deteriorated. Especially notable was a decrease in pain in the operative patients immediately after their procedure. There was no surgical mortality or morbidity. The results of this study indicate that children with severe spinal cord compression as evidenced by a near complete or complete block on myelography or filling of 50% or more of the spinal canal on magnetic resonance imaging are best treated by a combination of surgical decompression and tumor removal followed by adjuvant therapy.

A prospective population-based study of pediatric trauma patients with mild alterations in consciousness (Glasgow Coma Scale score of 13-14).

OBJECTIVE Considerable controversy surrounds the appropriate evaluation of children with mild alterations in consciousness after closed head trauma (Glasgow Coma Scale [GCS] score of 13-14). The objective of the current study was to determine the incidence of intracranial lesions in pediatric patients with a field GCS score of 13 or 14 after closed head injuries. METHODS The current study is a population-based, multicenter prospective study of all patients to whom emergency medical services responded during a 12-month period. The setting was urban Los Angeles County, encompassing a patient population of 2.3 million children, 13 designated trauma centers, and 94 receiving hospitals. RESULTS In the pediatric age group (<15 yr old), 8488 patients were transported by emergency medical services for injuries. Of these, 209 had a documented field GCS score of 13 or 14. One hundred fifty-seven patients were taken to trauma centers, and 135 (86%) underwent computed tomography. Forty-three patients (27.4%) had abnormal results on computed tomographic scans, 30 (19.1%) had an intracranial hemorrhage, and 5 required an operative neurosurgical procedure for hematoma evacuation. Positive and negative predictive values of deteriorating mental status (0.500 and 0.844, respectively), loss of consciousness (0.173 and 0.809), cranial fracture (0.483 and 0.875, and extracranial injuries (0.205 and 0.814) were poor predictors of intracranial hemorrhage. CONCLUSION Pediatric patients who have mild alterations in consciousness in the field have a significant incidence of intracranial injury. The great majority of these patients will not require operative intervention, but the implications of missing these hemorrhages can be severe for this subgroup of head-injured patients. Because clinical criteria and cranial x-rays are poor predictors of intracranial hemorrhage, it is recommended that all children with a GCS score of 13 or 14 routinely undergo screening via non-contrast-enhanced computed tomography.

Neuropsychological Outcome after Severe Pediatric Near-Drowning

Between April 1979 and August 1983, 49 nearly drowned children in serious condition were admitted to Childrens Hospital of Los Angeles (CHLA) with Glasgow coma scores of 3, 4, or 5 and underwent intracranial pressure monitoring and brain resuscitative therapy. Of the 49 patients, 29 (59%) died in the hospital 1 day to 3 months after admission, 13 (27%) were discharged in vegetative states, and 7 (14%) made good recoveries. No patient made a partial neurological recovery. The sustained mean highest intracranial pressure was significantly higher and the sustained lowest cerebral perfusion pressure was significantly lower for those who died than for survivors (P less than 0.05), but these data did not significantly distinguish between intact and vegetative survivors. Pupillary reactivity noted on arrival at CHLA also significantly discriminated survivors and fatalities (P less than 0.05), but not between intact and vegetative survivors. The presence of any motor activity after arrival at CHLA, even posturing or twitching, indicated a significant chance for intact survival (P less than 0.05), although such activity did not discriminate between death and vegetative survival. Extensive neuropsychological testing indicated that the apparently intact recovered patients generally showed nearly average levels of cognitive functioning, with mild residual gross motor and coordination deficits.

Surgical treatment of rathke cleft cysts in children.

OBJECTIVERathke cleft cysts (RCCs) are cystic epithelial lesions in the sellar and suprasellar regions that are often discovered incidentally. They require surgical fenestration and drainage in a small proportion of patients who develop symptoms or demonstrate progressive enlargement. Our aim was to review our experience with pediatric patients treated surgically for RCCs. METHODSA retrospective review was conducted of all patients treated surgically for RCCs at Childrens Hospital Los Angeles between 1999 and 2007 after approval by the institutional review board. Clinical notes, operative reports, radiological studies, and pathology reports were reviewed. The median follow-up period was 34 months. RESULTSTen patients undergoing surgical treatment of an RCC were identified, making up 20% of the 51 patients with RCCs followed clinically over the same time period. The mean age was 13 years (age range, 2–17 years). There were 6 females and 4 males. Patients requiring surgery presented with the following clinical symptoms: headache (8 patients, 80%), endocrine insufficiency (6 patients, 60%), meningitis followed by visual loss (1 patient, 10%), and incidental finding (1 patient, 10%). The mean cyst diameter was 13.6 mm (range, 8–18 mm). Four patients had strictly sellar lesions, 4 patients had suprasellar extension of an RCC, and 2 patients had primarily suprasellar RCCs. Nine of 10 patients underwent transsphenoidal surgery, and 1 patient underwent a pterional craniotomy. Complete cyst drainage on radiography was noted in 9 of 10 patients (90%), all of whom underwent transsphenoidal surgery. One patient experienced a symptomatic recurrence 6 years after complete surgical drainage. Headaches improved in 7 of 8 patients after surgery. Two patients had complete resolution of a hormonal axis deficit, whereas 3 patients developed new anterior pituitary axis deficits. Two patients developed persistent diabetes insipidus after surgery. CONCLUSIONRCCs are an infrequent cause of symptoms in pediatric patients. The transsphenoidal approach offers an effective means of achieving complete cyst drainage for lesions requiring surgery. Fenestration and aspiration of the cyst are usually sufficient to achieve total resolution of symptoms and signs caused by RCCs. Clinical symptoms such as headaches improved in the majority of patients, whereas hormonal disturbances typically persisted. Patient selection remains of paramount importance when considering surgery for pediatric patients with RCCs.