Faculty Bibliography

A multidisciplinary team approach using a staged transcatheter embolization and neurosurgical protocol was applied to 22 patients with neonatal presentation of vein of Galen malformations over a 12 year period. Aggressive medical therapy was combined with interventions including: ventricular shunting, transcatheter embolization, retrograde transtorcular embolization, and neurosurgical obliteration. There was a high frequency of high output cardiac failure, multiple organ system dysfunction, seizures, hydrocephalus, visual, developmental and neurological disability. Of the first 11 patients, five survived; four with seizures and three with marked retardation. Of the last 11 patients, six survived; five with seizures but only one with retardation. Despite persistently high morbidity and mortality, our continuously evolving protocol offers these otherwise hopeless patients some chance of survival

A technique of wound closure following tethered cord correction is presented that significantly reduces the incidence of cerebrospinal fluid collections in the subcutaneous space. In over 60 cases, the described method of fascia and skin closure has lessened wound problems to a minimal level. Patient hospitalization time has also been greatly diminished

Chronic headaches in a shunt-dependent patient with small ventricles has long been treated with little or no regard to intracranial pressure. In this study, pressure monitoring on 12 such patients demonstrated that they fell into three distinct categories: 3 had headaches caused by intracranial hypertension, 2 had headaches from hypotension, and 7 showed no relation of symptoms to pressure. As therapeutic procedures for treating these three categories are entirely different and sometimes opposing, it is clear that intracranial pressure monitoring is essential to successful management of this complaint

Intrinsic focal tumor of the medulla oblongata is an uncommon entity. Of the 51 cases presenting for treatment of a brainstem glioma between 1984 and 1990, only 7 were confined to the medulla. Radical excision was performed on each patient. Four of the first 5 patients who were extubated in the recovery room had CO2 retention, with associated respiratory arrest and hypoxia. Three suffered permanent cranial nerve deficits as a result. It is the purpose of this report to describe the serious complications of medullary surgery and to make recommendations as to how they may be avoided

Two cases of aplasia cutis congenita with associated bony defects are presented to highlight the dangers of delayed coverage of exposed dura. Management of one case was complicated by repeated local and systemic sepsis and the other by repeated, life-threatening hemorrhage. Early, definitive coverage of these defects using full thickness skin flaps avoids the risks of hemorrhage and should, if the recipient bed is clean, lessen the incidence of complicating sepsis

Sixteen children underwent 18 operations for radical resection of chiasmatic-hypothalamic tumors. The clinical presentation correlated with age: infants under 1 year of age presented with macrocephaly, failure to thrive, and severe visual failure; children aged 1 to 5 years predominantly had precocious puberty with mild visual deficits; and older children (greater than 5 years old) had slowly progressive loss of vision. All three infants had biologically aggressive tumors in spite of low-grade histology, and died from progressive tumor growth. Eleven of the 13 children aged 1 year or over are alive and well, without clinical or radiographic evidence of disease progression, 4 months to 4 1/2 years following surgery. Six of these patients, with a follow-up period of 10 months to 4 1/2 years (mean 27 months), have had no adjuvant therapy following radical surgical resection. The authors conclude that: 1) radical surgical resection of chiasmatic-hypothalamic tumors can be performed with minimal morbidity; 2) radical resection may delay the time to disease progression in older children and postpone the need for irradiation; 3) resection of postirradiation recurrent tumors may provide neurological improvement and long-lasting clinical remission; and 4) chiasmatic-hypothalamic tumors of infancy are aggressive neoplasms that require multimodality therapy

171 patients with intramedullary spinal cord tumors were operated on, of which 25 patients (15%), mostly children, developed symptomatic hydrocephalus. Twenty patients (12%) had malignant tumors, with 13 of the 20 cases (63%) complicated by increased intracranial pressure and ventriculomegaly. Of the remaining 151 patients with benign tumors (89%), only 12 (8%) developed symptomatic hydrocephalus. In an effort to understand the relationship between hydrocephalus and intramedullary spinal cord tumor, the authors analyze the level and histology of the neoplasm, as well as its association with spinal cysts. A review of the neurosurgical literature reveals that 34 similar cases of hydrocephalus associated with intramedullary spinal cord tumors have been reported to date. The authors note that the presence of hydrocephalus in patients with malignant intramedullary astrocytomas is associated with a shorter rate of survival than in those patients with high-grade lesions but without hydrocephalus, apparently due to rapid tumor progression. The ventriculomegaly seen with benign spinal cord gliomas has no statistically significant effect upon long-term prognosis