McCune-Albright syndrome (polyostotic fibrous dysplasia) with intracranial frontoethmoid mucocele.
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Adolescent↗
Biomedical subjects
B S Carson
Publications and source records attributed to B S Carson.
Why would you remove half a brain? The outcome of 58 children after hemispherectomy-the Johns Hopkins experience: 1968 to 1996.
PURPOSE: To report the outcomes of the 58 hemispherectomies performed at Johns Hopkins between 1968 and January 1996. METHODS: Charts were reviewed of the 58 hemispherectomies performed at Johns Hopkins Medical Institutions by the Pediatric Epilepsy Group during the years 1968 to 1996. Twenty-seven operations were done for Rasmussen's syndrome, 24 operations for cortical dysplasias/hemimegalencephalies, and 7 for Sturge-Weber syndrome or other congenital vascular problems. Seizure control alone did not seem to adequately describe the outcomes of the procedure. Therefore, a score was constructed that included seizure frequency, motor disability, and intellectual handicap. This burden of illness score better described the child's handicap before and after surgery. RESULTS: Perioperative death occurred in 4 out of 58 children. Of the 54 surviving children, 54% (29/54) are seizure-free, 24% (13/54) have nonhandicapping seizures, and 23% (12/54) have residual seizures that interfere to some extent with function. Reduction in seizures was related to the etiology of the unilateral epilepsy. Eighty-nine percent of children with Rasmussen's, 67% of those with dysplasias, and 67% of the vascular group are seizure-free, or have occasional, nonhandicapping seizures. All operations were considered by the parents and the physicians to have been successful in decreasing the burden of illness. In 44 the procedure was very successful, in 7 it was moderately successful, and in 3 it was minimally successful. Success was related to the etiology, and early surgery was preferable. CONCLUSION: Hemispherectomy can be a valuable procedure for relieving the burden of seizures, the burden of medication, and the general dysfunction in children with severe or progressive unilateral cortical disease. Early hemispherectomy, although increasing the hemiparesis in children with Rasmussen's syndrome, relieves the burden of constant seizures and allows the child to return to a more normal life. In children with dysplasias, early surgery can allow the resumption of more normal development.
Adolescent↗
Third ventricular choroid plexus papilloma with psychosis. Case report.
This 9-year-old boy with a history of behavioral problems and worsening psychosis responded initially to treatment with haloperidol. However, a magnetic resonance image obtained as part of his psychiatric evaluation revealed an anterior third ventricle tumor and mild-to-moderate hydrocephalus. The resected tumor was found on pathological examination to be a choroid plexus papilloma. The patient had an uneventful postoperative course and remained free of psychosis or mood disorder at 1-year follow-up examination.
Cerebral Ventricles↗
Giant congenital nevocellular nevus overlying an encephalocele.
Discussion regarding the diagnosis, prognosis, and management of giant congenital nevocellular nevi (GCNN) has permeated the literature because of concerns regarding malignant potential. As a derivative of dysgenetic neural crest development, GCNN histology in the neonate may be quite variable and not easily discernible from malignant melanoma, presenting a diagnostic dilemma to both the seasoned pathologist and surgeon. Here we present an interesting case of GCNN arising from a large encephalocele in a neonate. In spite of a clinically aggressive course, histology repeatedly failed to meet the criteria for melanoma until the diagnosis of malignant melanoma was made at autopsy. Clinical suspicion for malignancy must be high when evaluating atypical large nevi, and prompt surgical management is necessary to protect against the risk for malignant melanoma.
Brain Neoplasms↗
Hemispherectomy: a hemidecortication approach and review of 52 cases.
Between 1975 and 1994, 52 hemispherectomies, of which two were anatomical and 50 hemidecortications, were performed at Johns Hopkins Medical Institutions. Eighteen patients were 2 years old or less. There were three perioperative mortalities and one patient died 9 months later from causes not related to surgery. One patient developed hydrocephalus 6 years postsurgery and has been treated effectively. Seizure control and the functional status of each patient were measured as outcome variables. Forty-six (96%) of the surviving patients were seizure free or had reduced seizures as of their last follow-up examination. Twenty-one individuals (44%) were participating in age-appropriate classes or working independently, 18 were classified as semiindependent, and nine children will likely depend on a lifetime of assisted living. The relationships between the outcome variables and the patient's age at surgery, the interval to surgery, and the etiology of the disease were compared. The authors' clinical experiences strongly suggest the importance of a multidisciplinary approach to patient selection and follow-up care. Moreover, anesthetic management of infant surgery is a major component of success.
Adolescent↗
Excellence in clinical subspecialties.
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Economics, Medical↗
The effects of topical dexamethasone on experimental brain tumors and peritumoral brain edema.
To determine if topical dexamethasone administered to brain tumor beds would not only control peritumoral edema and suppress tumor growth but also prevent systemic steroid complications, we studied experimental brain tumors produced in 102 rabbits by implanted VX2 carcinoma cells. We separated 58 animals into three groups: 1) untreated rabbits (n = 15), 2) systemic dexamethasone-treated (4 mg/kg/day) rabbits (n = 18), and 3) topical dexamethasone-treated (2.5 microliters/h, osmotic pump) rabbits (n = 25). We administered systemic or topical dexamethasone from the third day or from the seventh day after tumor implantation, and sacrificed the animals on the 13th day. We compared survival in these three groups with that of another 44 rabbits, beginning treatment on the seventh day. We measured brain water content in the white matter of the sacrificed rabbits by the specific gravity method. We measured the length and width of the brain tumors of all the rabbits and estimated tumor volume. Systemic and topical dexamethasone administered from the third day produced statistically significant inhibition of tumor volume as well as a mean reduction in peritumoral brain edema in most tested sites. Systemic and topical dexamethasone treatment resulted in a statistically significant increase in survival relative to the untreated group. These results suggest that topical dexamethasone is efficacious in a brain tumor model and its administration to brain tumor beds constitutes a new therapeutic modality.
Administration, Topical↗
Intracranial compliance in infants: evaluation with Doppler US.
PURPOSE: To test graded fontanelle compression during Doppler ultrasound (US) scanning to identify and monitor infants with altered cranial compliance. MATERIALS AND METHODS: An ophthalmodynamometer exerted pressure on the anterior fontanelle during Doppler US scanning of the middle cerebral artery. Sixty examinations were performed in 43 infants--13 full-term and 11 premature healthy control subjects, 10 with increased intracranial volume, and nine with suspected abnormal cranial compliance but without increased intracranial volume. Resistive index (RI) and angle-corrected time-averaged mean velocities (TAV) of blood flow were compared at three different pressures. RESULTS: Baseline RI values in healthy full-term infants were significantly lower than in healthy premature infants and infants with abnormal compliance (P < .05). Values for healthy premature infants and infants with abnormal compliance were indistinguishable (P > .5). Neither RI nor TAV changed significantly in healthy infants, but both changed significantly with compression in infants with abnormal cranial compliance. CONCLUSION: This procedure may be more useful than measurement of RI of the anterior cerebral artery alone to evaluate infants with altered cranial compliance.
Brain Diseases↗
Lambdoid synostosis.
Recent increases in presentation of occipital deformities have presented the craniofacial team with new challenges in diagnosis and treatment. As presented in this article, they appear to have a functional abnormality of the suture that results in a localized growth deformity. The growth inhibition in severe cases can result in changes in the local bone and brain, along with the entire skull and cranial base. Histologic analysis supports the theory that this is true lambdoid synostosis. Surgical correction is indicated in moderate to severe cases, and the authors have presented their technique and new data on outcomes.
Cranial Sutures↗
Folliculo-stellate cells in the human anterior pituitary express cytokeratin.
Recent studies have suggested that the anterior pituitary is derived not from Rathke's pouch, but instead from neuroectoderm. To address this controversy and the differentiation of the adenohypophysis, we evaluated folliculo-stellate cells from 7 human pituitaries using a panel of immunohistochemical stains. The panel included antibodies to a low molecular weight cytokeratin (CK-8), a broad spectrum of cytokeratins (AE 1/AE 3), S-100 protein, vimentin, and glial fibrillary acid protein. The most intense immunostaining was observed with the antibody to broad spectrum cytokeratins. Less intense immunohistochemical staining of folliculo-stellate cells was observed with the antibodies against glial fibrillary acidic protein and S-100 protein. Immunoblotting of anterior pituitary tissue obtained from 3 autopsied patients showed that cytokeratins identified in the anterior pituitary were of low molecular weight (40 to 56 Kd). These results indicate that folliculo-stellate cells show epithelial differentiation, and support the hypothesis that the anterior pituitary is derived from Rathke's pouch.
Blotting, Western↗
The occipital bar and internal osteotomies in the treatment of lambdoidal synostosis.
Lambdoidal synostosis results in unilateral or bilateral deformities. Patients with this diagnosis often present with moderate to severe deformities at a relatively late period in development. This late presentation limits the options for treatment and methods for correction. Occipital bar advancement and internal osteotomies provide an accurate, reliable, and quantitative method to treat the deformity. This technique addresses the variable degree of asymmetry and the large flattened bony segments. It was performed in 12 patients over 18 months, with good to excellent results.
Age Factors↗
Therapeutic effects of local delivery of dexamethasone on experimental brain tumors and peritumoral brain edema.
To determine if dexamethasone administered by osmotic pump directly to brain tumors would control peritumoral edema and at the same time suppress tumor growth and prolong survival, the authors studied experimental brain tumors produced in 102 rabbits by implanting VX2 carcinoma cells. Of these, 58 animals were separated into three treatment groups: Group 1 included 15 untreated rabbits; Group 2 included 18 rabbits treated with systemic dexamethasone (4 mg/kg/day); and Group 3 included 25 rabbits treated with local dexamethasone (0.24 mg/day) delivered by osmotic pump. Systemic or local dexamethasone was administered from Day 3 or Day 7 after tumor implantation, and animals were sacrificed on Day 13. A survival study was performed with 44 rabbits separated into the same treatment groups, beginning drug delivery on Day 7. Brain water content in the white matter of sacrificed animals was measured by the specific gravity method. The length and width of the brain tumors in all animals were measured and the tumor volume estimated. Findings showed that systemic and local dexamethasone administered from Day 3 or Day 7 was associated with a significant (5% level) inhibition of tumor volume as well as a mean reduction of brain edema in most tested sites. Systemic and local dexamethasone therapy also resulted in a significant (5% level) increase in survival time relative to the untreated group. These short-term results suggest that locally delivered dexamethasone may constitute a clinically important therapeutic modality.
Animals↗
Disposition and respiratory effects of intrathecal morphine in children.
BACKGROUND: The extent and duration of respiratory depression after opioid administration are poorly defined in infants and children. METHODS: The disposition and respiratory effects of intrathecal morphine were studied in ten patients (ages 4 months-15 yr) after repair of craniofacial defects. Morphine, 0.02 mg/kg, was administered intrathecally before the end of surgery. Postoperatively, we determined the minute ventilation (VE) in response to increasing partial pressure of end-tidal carbon dioxide (PETCO2) during carbon dioxide rebreathing. The slope (VE/PETCO2) and intercept (VE at PETCO2 60 mmHg, VE 60) of the carbon dioxide response curve were calculated at 6, 12, and 18 h after morphine administration. Cerebrospinal fluid (CSF) and blood were analyzed for morphine concentration by radioimmunoassay. RESULTS: Mean VE/PETCO2 decreased from a preoperative value of 35.1 +/- 3.7 to 16.3 +/- 2.8 ml.kg-1 x min-1 x mmHg-1 at 6 h after morphine, and remained depressed to 23.4 +/- 2.9 and 23.5 +/- 3.3 ml.kg-1 x min-1 x mmHg-1 at 12 h and 18 h, respectively, compared to preoperatively). The infants' (n = 3) VE/PETCO2 at 6 h were 21, 4, and 27 ml.kg-1 x min-1 x mmHg-1. Mean VE 60 decreased from 874 +/- 125 to 276 +/- 32 ml x kg-1 x min-1 at 6 h, but then recovered at 12 and 18 h to 491 +/- 68 and 567 +/- 82 ml.kg-1 x min-1, respectively. The infants' VE 60 at 6 h were 350, 142, and 245 ml.kg-1 x min-1. Mean CSF morphine concentration was 2,860 +/- 540 ng/ml at 6 h, and decreased to 640 +/- 220 and 220 +/- 150 ng/ml at 12 and 18 h, respectively. CONCLUSIONS: Intrathecal morphine, 0.02 mg/kg, depressed the ventilatory response to carbon dioxide for up to 18 h concomitant with increased CSF morphine concentrations. Infants (4-12 months of age) did not exhibit greater ventilatory depression than did children (2-15 yr of age).
Administration, Inhalation↗
Intrathecal 4-hydroperoxycyclophosphamide: neurotoxicity, cerebrospinal fluid pharmacokinetics, and antitumor activity in a rabbit model of VX2 leptomeningeal carcinomatosis.
Dissemination of tumor to the leptomeninges and cerebrospinal fluid represents a common pattern of metastasis for many cancers; however, few chemotherapeutic agents are available for intrathecal (i.t.) use and treatment results are often poor. We studied the neurotoxicity and pharmacokinetics of i.t. 4-hydroperoxycyclophosphamide (4-HC) in the rabbit and the activity of i.t. 4-HC in a VX2 rabbit model of leptomeningeal carcinomatosis to evaluate the potential use of 4-HC in the treatment of leptomeningeal tumors. Toxicity studies examined 4-HC doses ranging from 0.5 to 6.0 mumol administered by intraventricular injection weekly for 4 to 8 weeks. Clinical or histological neurotoxicity was not observed in rabbits treated with < 1.0 mumol 4-HC for 4 weeks. Clinical toxicity, characterized by lethargy, weight loss, seizures, or death, was apparent at doses > 2.0 mumol. Vasculitis of superficial arteries was observed in rabbits treated with > 1.0 mumol 4-HC. In cerebrospinal fluid pharmacokinetic studies, the mean drug half-life after intraventricular or intralumbar administration was 24.3 and 18.2 min. Regional inequities in drug exposure were apparent as area under the clearance curve values for cerebrospinal fluid distant from the injection site were lower than those of proximate sites (P < 0.001). Weekly intraventricular treatment of VX2 leptomeningeal tumor-bearing rabbits with 0.5 or 1.0 mumol of 4-HC resulted in an increased life span of 22.5 and 35%, respectively. These results indicate that i.t. 4-HC, at doses lower than those producing neurotoxicity in the rabbit, is effective treatment for VX2 leptomeningeal carcinomatosis.
Animals↗
Complications and early outcome of anterior craniofacial resection.
OBJECTIVE: To evaluate the complications of anterior craniofacial resection and correlate their impact with tumor control status. DESIGN: We conducted a retrospective review of 32 consecutive, operable patients' records seen over a 6-year period, requiring 35 procedures. SETTING: Academic tertiary referral medical center. PARTICIPANTS: Twenty-six patients (81%) had malignant lesions (esthesioneuroblastoma, squamous cell carcinoma, and a group of miscellaneous malignant tumors). Six patients had various benign neoplasms. INTERVENTION: The surgical approach involved bifrontal craniotomy coupled with lateral rhinotomy in 19 cases (61%), facial degloving in 10 cases (32%), a total rhinectomy in one case, and endoscopic sinusectomy without facial incision in two cases. OUTCOME MEASURE: Clinically noted complications and oncologic outcome. RESULTS: There was one avoidable perioperative death indirectly associated with the patient's procedure. Nine patients suffered significant intracranial neurological complications such as tension pneumocephalus and delayed epidural abscess. All of these complications were managed successfully. Of patients with malignant tumors, 13 (52%) are alive with no evidence of disease and one is alive with recurrence after a mean follow-up period of 28.9 months. The 10 patients who succumbed to disease had a mean postoperative survival of 22.9 months. CONCLUSIONS: In contrast to the perspective of only a decade ago, we conclude that craniofacial resection is a relatively safe, versatile, and effective procedure for surgical management of tumors involving the anterior skull base.
Adult↗
Colloid cysts of the third ventricle: immunohistochemical evidence for nonneuroepithelial differentiation.
The histogenesis of colloid cysts (CCs) of the third ventricle has been a subject of controversy. We examined, using immunohistochemical techniques, four CCs for the presence of cytokeratins (CKs), glutathione S-transferase isoenzymes (GST-pi, GST-mu), and glial fibrillary acidic protein. Antibodies to both low molecular weight CKs (anti-CK8) and to a mixture of CKs (AE1/AE3) were used. For comparison, normal fetal and adult choroid plexus, ependyma, and nasal mucosa were also examined. The epithelium lining all four CCs showed positive immunostaining for the CKs and GST-pi but not for GST-mu or glial fibrillary acidic protein. Fetal and adult nasal mucosa showed a pattern of immunohistochemical staining almost identical to that of CCs. In contrast, fetal and adult choroid plexus tissue showed positive immunostaining for GST-pi and low molecular weight CKs but not for the CK mixture (AE1/AE3). Fetal and adult ependyma were negative for both CKs and GST-pi. These results suggest that CCs differentiate along nonneural lines distinct from the neuroepithelial differentiation of the choroid plexus and ependyma.
Adult↗
Chromosome abnormalities in low-grade central nervous system tumors.
Ependymomas, oligodendrogliomas, and low-grade astrocytomas are slow-growing central nervous system (CNS) tumors that occur in both adults and children, whereas craniopharyngiomas and choroid plexus papillomas occur predominantly in children. We examined karyotypes of 32 of these low-grade tumors, including ten oligodendrogliomas, six ependymomas, 11 low-grade astrocytomas, four craniopharyngiomas, and one choroid plexus papilloma. Only normal karyotypes were obtained from 6 oligodendrogliomas. The rest had normal stemlines; three tumors had 45,X,-Y sidelines and one tumor had a sideline of monosomy 22. The most frequent abnormalities in the ependymomas were +7 (three tumors), -21 (two tumors), -22 (two tumors), and del(9)(p22) (two tumors). Gains of chromosome 7 and deletions of 9p were found more often in high-grade gliomas. Seven low-grade astrocytomas had normal stemlines, two had chromosome 7 abnormalities, a pilocystic astrocytoma had +der(15), and one tumor had a -Y sideline. The four craniopharyngiomas and one choroid plexus tumor were all apparently normal. The cytogenetics of low-grade CNS tumors differ from higher grade gliomas in that most low-grade tumors show little deviation from the normal karyotype.
Adolescent↗
Simple partial seizures: clinicofunctional correlation--a case report.
A 12-year-old girl developed simple partial motor and sensory seizures due to a right perirolandic astrocytoma. Subdural EEG recording and functional stimulation disclosed close correlation between EEG-clinical manifestations of focal seizures and functional responses to cortical stimulation. This case supports the idea that responses in the perirolandic area to endogenous epileptogenic activity and to cortical stimulation reflect common underlying physiologic mechanisms.
Brain↗