David L. Kelly

Natural history of arteriovenous malformations of the brain: A clinical study

The natural history of intracranial arteriovenous malformations (AVMs) was studied in 131 patients. The 83 patients managed nonsurgically and the 48 patients treated surgically were followed for an average of 8 years. Hemorrhage occurred in 61.8% of all patients. A second hemorrhage occurred in 67.4% of the survivors of the first hemorrhage. The mortality associated with recurrent hemorrhage did not increase significantly with successive episodes of hemorrhage. The rate of rebleeding was 17.9%/year initially, but declined to 3%/year after 5 years and then to 2%/year after 10 years. Among patients treated nonsurgically, the prognosis was more favorable for patients presenting with seizures than for patients presenting with hemorrhage. Patients in the seizure group had a 26.9% incidence of hemorrhage causing an 11.6% mortality; 40.5% of the patients in the hemorrhage group died. The prognosis was poor for patients with posterior fossa AVMs; the mortality was 66.7% with the first hemorrhage. Recurrent posterior fossa hemorrhage was the rule in survivors, and most of those hemorrhages were fatal. The prognosis for children with AVMs was no different from that for adults.

Biological Features of Meningiomas That Determine the Production of Cerebral Edema

Although meningiomas are known to cause varying degrees of cerebral edema, the relative importance of their location, size, histological subtype, and other histological features in the production of cerebral edema has not been studied adequately. Therefore, we undertook a retrospective analysis of 43 meningiomas excised between 1975 and 1980. The results indicate that histological subtype has no relationship to the production of cerebral edema, with one exception. Meningiomas containing partly or completely a hemangiopericytic component were the only histological subtype associated consistently with cerebral edema. The location of a meningioma per se may not determine the production of cerebral edema. A relationship between size, aggressive histological features, vascular proliferative changes, and the production of cerebral edema was seen. The need for and the nature of further studies required to explain the cerebral edema that may be associated with small meningiomas are discussed.

The vascular component in meningiomas associated with severe cerebral edema.

Cerebral edema is usually a complication of the later growth stages of intracranial neoplasms. Three patients with small meningiomas presented with unusually several cerebral edema out of proportion to the size of tumors. All three tumors exhibited benign meningothelial components, the formation of pseudopsammoma bodies, and striking vascular mural proliferation of small dark cells. In two tumors ultrastructural examination of these cells showed features of pericytes. The vascular pericytic component in these tumors may grow more actively than the meningothelial component and thus may cause the production of severe cerebral edema. The clinical, radiological, operative, and light and electron microscopic findings are presented, and the relationship to angioblastic meningiomas is discussed.

Correlation of admission fibrin degradation products with outcome and respiratory failure in patients with severe head injury.

It has been suggested that concentrations of fibrin degradation products (FDPs) after head injury reflect the extent of brain tissue destruction. In addition, elevated FDP concentrations have been associated with the development of the adult respiratory distress syndrome (ARDS). Coagulation variables, including a FDP screen, were measured on the admission of 33 patients with severe closed head injury (Glasgow coma score 8 or less) to define the prognostic value of FDP concentrations. A Glasgow outcome score was assigned to each patient 6 months after injury, and those patients with higher FDP concentrations had poorer functional outcomes. This correlation of outcome with admission FDP concentration was statistically significant, as was the correlation between outcome and the admission Glasgow coma score. Of 22 patients with FDP concentrations of less than 64 micrograms/ml, only 1 developed early respiratory failure, whereas 9 of 11 patients with FDP concentrations of 64 micrograms/ml or more developed ARDS. These findings were not affected by the presence of multiple system trauma. FDP concentrations seem to have prognostic value similar to the Glasgow coma score and seem to identify those patients with head injury who are at high risk for ARDS.

Left atrial myxoma with cerebral emboli

Infarction of the central nervous system secondary to embolism from left atrial myxoma is a recognized phenomenon. However, myxoma as the source of an embolus may be overlooked if an index of suspicion is not present during the evaluation of the patient with a stroke without a known cause. We present a case report that illustrates some of the pitfalls and characteristic findings present in the evaluation of these patients. The magnetic resonance image of multiple cerebral aneurysms and infarction associated with this condition is presented along with a unique, arteriographically proven resolution of a fusiform cerebral aneurysm after the removal of the cardiac tumor. A review of published case reports suggests that, if the cardiac tumor is recognized and treated quickly, the ultimate prognosis, while uncertain, is usually good. However, because the potential exists for recurrence of the cardiac tumor, for enlargement of the cerebral lesions, or for late development of cerebral lesions, long term follow-up is mandatory and a vigorous work-up must be pursued if the patient again becomes symptomatic or develops central nervous system manifestations for the first time.

Relationship between Flow Cytometric Features and Clinical Behavior of Meningiomas

Meningiomas have a wide range of biological potential and clinical behavior. Histological findings are helpful in recognizing the malignant potential of a given tumor, but often fail to correlate with gross features, liability of recurrence, and extent of associated cerebral edema. To find alternate approaches to improve the correlation between biological and clinical behavior, 20 meningiomas were studied by flow cytometry (FC), an assessment that has been applied to meningiomas previously. Such FC features as DNA index (DI) and proliferative index (PI, %G2 + %S) were correlated with size, location, brain invasion, associated edema, and recurrence. Tumors with severe edema had significantly higher PIs (19.5 +/- 4.1) than those with moderate (12.6 +/- 4.5) or minimal (8 +/- 0) edema (P less than 0.05). The PI was greater than 16 in those tumors that recurred (n = 3) or invaded the brain (n = 3). Six tumors were aneuploid (DI, 1.33 +/- 0.17; PI, 17.1 +/- 5.3). These were uniformly large when compared with the diploid tumors, which were more variable in size. All of the aneuploid tumors were associated with moderate to severe cerebral edema. Two partly psammomatous tumors with high PIs and foci of high cellularity suggesting recent growth were associated with severe edema. One of these exhibited brain invasion. These preliminary data indicate that FC may have a potential use in the clinical management of meningiomas.

Diastematomyelia: a 40-year experience

Diastematomyelia is a rare but potentially devastating spinal dysraphism classically characterized as a bony or fibrous spur separating two hemispinal cords. This study reviews our experience with diastematomyelia over a 40-year period from 1947 through 1987, and suggests that the neurologic and orthopedic deficits of diastematomyelia remain stable during extended follow-up after surgery.

Surgical repair of aneurysms of the posterior inferior cerebellar artery—A clinical series☆

Aneurysms of the posterior inferior cerebellar artery are relatively rare. Fourteen such aneurysms (10 vertebral, 4 peripheral) are reported, and their clinical presentation, surgical therapy, and outcome are discussed. All 14 patients had suffered a subarachnoid hemorrhage; 93% (n = 13) underwent direct clipping of the aneurysm while 7% (n = 1) underwent coating of the aneurysm. Two patients died in the perioperative period (one from vasospasm and one from rebleeding). Full activity was achieved by all but one (92%) of the remaining patients in extended follow-up.

Brain Stem Auditory Evoked Potentials in Arnold-Chiari Malformation: Possible Prognostic Value and Changes with Surgical Decompression

Symptomatic Arnold-Chiari malformation (Chiari II) is a recently described clinical entity, the natural history and surgical management of which remain controversial. The brain stem auditory evoked potentials (BAEPs) have been described as abnormal in 50 to 86% of children with myelomeningocele. We present the case of a full-term infant with myelomeningocele, shunted hydrocephalus, and Chiari II malformation who was surgically treated by decompression when she was 3 weeks old. BAEPs recorded immediately after decompression showed dramatic improvement over BAEPs recorded immediately before decompression, an improvement that could not be attributed to maturation of the central nervous system. It is postulated that in further large series the degree of abnormality in BAEPs may be of value in predicting the clinical course of these patients. The current status of BAEPs as they apply to the evaluation of posterior fossa anomalies is reviewed.

Conservative management of aplasia cutis congenita: case report.

A male newborn with a large scalp and skull defect and a cervical meningocele was transferred to the neonatal intensive care unit of the North Carolina Baptist Hospital. The scalp defect measured 8.5 x 9.5 cm, and the sagittal sinus was exposed. Because the defect was considered too large to be covered with a rotation skin flap, it was treated thrice daily with the topical application of bacitracin ointment and circumferential dressings. By the end of 3 months, the skull defect was covered with skin. The cervical meningocele was then removed without incident. At the 9-month follow-up examination, the scalp defect was well healed and the skull defect was closing slowly; the neurological findings were still normal for the patients age.