Faculty Bibliography

Historically, the prognosis for a child diagnosed with a brainstem tumor has been bleak. The development of magnetic resonance imaging (MRI) has allowed for detailed visualization of tumors within the brainstem. This knowledge combined with the clinical history and neurological examination allows subcategorization of such tumors and delineation of children who might benefit from surgery. Surgical candidates include children with long clinical histories, focal neurological deficits and focal, cystic, exophytic or cervico-medullary tumors on the MRI scan. All children have the potential for multiple cranial nerve abnormalities, extremity weakness and swallowing and breathing difficulties. Nursing care of patients with brainstem tumors is complex and challenging

Hydrocephalic women with cerebrospinal fluid shunts are now surviving to reproductive age. Twenty-one pregnancies in 18 patients with shunts, including 11 from the present series and 10 from previous series, were analyzed for neurological, obstetrical, and perinatal outcome. Fourteen women had preexisting shunts, and 4 had the onset of symptomatic hydrocephalus and the placement of shunts during pregnancy. Neurological complications occurred in 13 of 17 (76%) pregnancies in patients with preexisting shunts, including symptoms of increased intracranial pressure (ICP) in 10 of 17 (59%) pregnancies, exacerbation of seizure disorder in 2 of 17 (12%) pregnancies, and severe headaches without increased ICD in 1 patient. In 7 of 11 (66%) of the symptomatic patients, symptoms spontaneously resolved postpartum. Four of 17 (23%) of these pregnancies were associated with shunt obstruction requiring antepartum or postpartum surgery. Four patients had a primary shunt placement, and one had a shunt revision during pregnancy without complications. There were no unusual obstetrical or perinatal complications in the series. The clinical management of pregnant patients with hydrocephalus should include preconception counseling and magnetic resonance imaging, as well as the use of serial antenatal magnetic resonance images, ICP monitoring, or the judicious use of radioisotope studies of shunt patency if signs of increased ICP appear. A cesarean section is recommended for the delivery of the neurologically unstable patient. For asymptomatic mothers, a vaginal delivery with a shortened second stage and prophylactic antibiotics are advised

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