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Biomedical subjects

R J Hudgins

Publications and source records attributed to R J Hudgins.

17 recordsLinked to original sources

Decision making in primary surgical repair of myelomeningoceles.

A 5-year review of 43 consecutive patients presenting to Scottish Rite Children's Medical Center with an open myelomeningocele defect was undertaken. The aim of the present study was to analyze the myelomeningocele defects, dimensions, and area to better define those factors that dictate the need for plastic surgical consultation for wound closure. Of the 43 patients identified, two were excluded because they first presented as older children; the remaining 41 all had their myelomeningoceles repaired within the first 36 hours of life. Of these, 31 underwent repair by the Neurosurgical Service, whereas for 10 patients (24.4%), the Plastic Surgery Service was asked to assist with closure. Comparison showed the mean (+/- standard deviation) area in the referred patients was 27.4 (7.6) cm2 versus 17.6 (7.9) cm2 in the patients not referred for closure (p = 0.002). A trend analysis predicting referral as a function of myelomeningocele area showed that 0 of 10 (0%) with an area of less than 15 cm2, 2 of 13 (15.4%) with an area equal to 15 to 20 cm2, 3 of 7 (42.9%) with an area of 21 to 25 cm2, and 4 of 9 (44%) with an area greater than 25 cm2 were referred (p = 0.001). Data from the interpretation of maximum myelomeningocele dimension also showed statistically significant trends in referral. Using multiple logistic regression, it was found that the odds of referral increased by a factor of 3.3 for every 1 cm increase in maximum dimension.

Decision Making

Surgical correction of severe scaphocephalic deformities.

Sagittal synostosis may result in severe skull deformities. Characteristic components of the deformity include extreme elongation, frontal and occipital bossing, temporal pinching, and angulatory apical skull deformation. Conventional strip craniectomy often fails to correct these complex problems completely in severe early or late cases of sagittal synostosis. Techniques for total calvarial vault reconstruction have previously been reported, but a single large series has not been presented. Eighteen consecutive patients ranging in age from 3 months to 5 years (mean = 12 months) with severe early and late scaphocephalic skull deformities underwent total calvarial vault reshaping. All children required transfusions ranging from 250 to 1,100 mL. Operative times averaged 6 hours, and hospital stay ranged from 4 to 7 days. There was no perioperative mortality. Two patients experienced transient syndrome of inappropriate secretion of antidiuretic hormone, which responded to fluid restriction. One patient was noted to have a 2-cm parietal craniectomy defect 9 months after operation. Microscrews, which were used in all 18 patients, had to be removed in 2 patients when they became palpable. Excellent aesthetic results were noted in all 18 patients up to 36 months of follow-up.

Child, Preschool

Treatment of intraventricular hemorrhage in the premature infant with urokinase. A preliminary report.

Hydrocephalus secondary to intraventricular hemorrhage in the premature infant is common and is secondary to the presence of blood in the subarachnoid space. Rapid clearance of the blood may reduce the incidence of permanent hydrocephalus and mitigate the need for shunt placement. We have treated 4 premature infants with posthemorrhagic hydrocephalus with urokinase, a thrombolytic agent, instilled into the ventricles via a surgically paced ventricular access device. The infants were treated with 10,000 IU of urokinase twice a day for 1 week. No adverse reactions have occurred and none of the infants has required shunt placement. In a group of historical controls, the shunt rate was 87%.

Birth Weight

Delayed intracranial hemorrhage in children after suboccipital craniectomy.

OBJECTIVE: The purpose of this study was to report the clinical and imaging findings of seven children who developed extraaxial, intracranial hemorrhage 3-12 years after suboccipital craniectomy for neoplasia. We attempt to explain the hemorrhages based on a previously reported hypothesis of neomembrane formation associated with dural substitutes used to repair large dural defects. MATERIALS AND METHODS: Clinical charts (seven patients), surgical and pathologic findings (four patients), and imaging studies (CT scans and MR images in four; CT scans, MR images, and angiograms in one; and CT scans only in two patients) were reviewed retrospectively. Hemorrhage occurred 3-12 years after suboccipital craniectomy for tumor (ependymoma in two, medulloblastoma in three, astrocytoma in one, and ganglioglioma in one). Silastic dural substitute was used to repair the surgical wound in six cases and human dural graft in one case. Hematomas were spontaneous in four and occurred after minor head trauma in three. Four patients had multiple hemorrhagic episodes. RESULTS: CT scans and MR images showed acute extraaxial hemorrhages at the craniectomy site without contiguous residual or recurrent neoplasia in all patients. No intraaxial or intratumoral hemorrhage was detected. Findings on cerebral angiograms in one patient were normal. Four patients underwent surgical exploration of the hematoma and craniectomy site; no macroscopic source of bleeding was detected. The hematomas were not associated with recurrent tumor pathologically. CONCLUSION: Delayed, benign extraaxial hematomas may occur in children who have undergone craniectomy for tumors of the posterior fossa and have had dural substitute used to repair large defects. Fragile vessels associated with nonmembranes have been proposed as the source of hemorrhage.

Brain Neoplasms

Total calvarial reconstruction for sagittal synostosis in older infants and children.

Premature closure of the sagittal suture is the most common form of craniosynostosis, but this condition occasionally goes unrecognized until the child is too old to undergo procedures that depend upon continued calvarial growth for success. As the entire calvaria is affected and thus misshapen by sagittal synostosis, late correction involves total calvarial reconstruction. The extensive nature of this undertaking has precluded its utilization despite the presence of significant deformities. Adapting the techniques and experience gained from craniofacial surgery, the authors performed total calvarial reconstruction on nine children with sagittal synostosis and subsequent scaphocephaly diagnosed after the age of 1 year. In each case the goals of shortening the anteroposterior length, widening the biparietal diameter, and reducing frontal and occipital deformities were met. Morbidity consisted of acute blood loss, postoperative hyponatremia, and in one case a residual skull defect. The rationale for this procedure and the techniques utilized are discussed.

Blood Loss, Surgical

Pineocytomas.

Six cases of histologically proven pineocytoma are reported. The diagnosis was established by surgery in five cases and at autopsy in one. All patients received focal radiation therapy (4500 to 5400 cGy). Two patients had local recurrences at 1 and 48 months and subsequently died of tumor. A third patient died of Alzheimer's disease 29 years after the initial presentation. Three patients are alive with no evidence of disease at 21+, 52+, and 84+ months after treatment. Tumor dissemination occurred after a local recurrence in one patient. We found no evidence of recurrence outside the irradiated field unless there was an initial recurrence at the primary tumor site. We recommend postoperative staging for all patients with pineocytoma and focal radiation therapy if local disease alone is found. Craniospinal radiation therapy appears to be justified only if tumor dissemination is documented on staging tests.

Adolescent

The proliferative potential of human ependymomas measured by in situ bromodeoxyuridine labeling.

Twelve patients with ependymomas received a 30- to 60-minute intravenous infusion of bromodeoxyuridine (BrdU), 150 to 200 mg/m2 at surgery, to label tumor cells in the DNA synthesis phase. Labeled cells were detected in excised tumor specimens by indirect immunoperoxidase staining using anti-BrdU monoclonal antibody as the first antibody. The BrdU labeling index (LI, defined as the percentage of labeled cells in relation to the total number of cells scored) was calculated for each specimen. All four spinal cord ependymomas had a BrdU LI of less than 1%, which is consistent with our clinical experience that most such tumors grow slowly and have an excellent prognosis. Five of the eight intracranial ependymomas also had a low BrdU LI of approximately 1% or less, and three had a BrdU LI of 3.2%, 3.4%, and 4.8%. The latter three tumors, only one of which was diagnosed as a malignant ependymoma at the time of study, were either recurrent or recurred within 2 years after gross or subtotal removal. Cytologic analysis of cerebrospinal fluid (CSF) was performed in five cases; CSF seeding of tumor cells was found in only one patient, who had a malignant ependymoma. A high BrdU LI did not always correlate with CSF seeding. Measurement of the LI using BrdU and anti-BrdU monoclonal antibodies can provide more accurate information on the proliferative potential of individual tumors and may lead to a more rational grading system of ependymomas. The results of such studies do not always predict the potential for CSF seeding.

Adult

Radiotherapy of primary intracranial germinomas: the case against routine craniospinal irradiation.

A retrospective study was performed on all patients with biopsy-proven intracranial germinomas and unbiopsied suprasellar or pineal region tumors treated during the past 30 years in the Department of Radiation Oncology, University of California, San Francisco. A total of 33 patients were treated: 13 with biopsy-proven germinomas, and 20 others who were unbiopsied. All patients were treated with megavoltage equipment; total dose varied between 40-55 Gy. Only two patients were treated with prophylactic spinal irradiation. No patient received initial or adjuvant chemotherapy. Follow-up times for biopsy-proven patients ranged from 0.5 to 16.7 years with a median 5.3 years. No biopsy-proven patient had a recurrence of the tumor or died; thus, actuarial relapse-free and determinate survivals at 5 years were 100%. Although only one patient in this group received prophylactic spinal irradiation, no patient failed in the spinal axis. The 20 unbiopsied patients had follow-up times ranging from 0.1 to 27.5 years with a median of 5.5 years. Six unbiopsied patients died: two from recurrent disease at the primary site, one from distant peritoneal metastases, two from complications of treatment, and one from intercurrent disease. For this group, actuarial relapse-free survival at 5 years was 72%; the corresponding determinate survival was 73%. Nineteen unbiopsied patients were treated without craniospinal irradiation. Only one developed spinal metastases. The results from this and other series indicate that the risk of spinal metastases from intracranial germinoma is too low to warrant routine prophylactic spinal irradiation. However, patients with gross tumor spill causing contamination of the CSF, malignant CSF cytology, or documented subependymal or subarachnoid metastases presumably are at higher risk for leptomeningeal failure. Craniospinal irradiation is recommended for these patients.

Brain Neoplasms

Pineal region tumors in children.

The authors believe that the preferred treatment for pineal region tumors in children requires definitive surgery with a histological diagnosis and that a conservative approach consisting of shunting and radiation therapy no longer seems to be appropriate. The results are reported of a retrospective review of the presentation, treatment, and outcome of 36 children under the age of 18 years treated between 1974 and 1986. Eleven children had germinomas (two-cell type), seven had astrocytomas, and the remaining 18 had 15 histologically different tumor types. Surgery was performed on 30 patients; there were no deaths, but a 10% rate of persistent morbidity was found. The median follow-up period was 4 years. Nine (82%) of 11 patients with germinomas are alive without evidence of recurrence; one child died from recurrent tumor in the pineal region and another is presently being treated for recurrent tumor of the spinal cord. Six (86%) of the seven patients with astrocytoma are well after biopsy and radiation therapy. Of the remaining 18 children, five (28%) died from tumor progression. The cerebrospinal fluid (CSF) tumor markers alpha-fetoprotein and beta-human chorionic gonadotropin were helpful in determining the presence of malignant germ-cell tumors, particularly those with a poor prognosis. Magnetic resonance imaging was useful for diagnosis and for planning the operative approach. Magnetic resonance images showed the presence of pineal region tumors in four children with hydrocephalus who had no evidence of tumor on computerized tomography scans. Because the great variety of tumor types found in the pineal region must be treated in different ways and because improved microsurgical and stereotaxic surgical techniques have made mortality and morbidity rates acceptably low, a biopsy diagnosis should be obtained in all patients. Preoperative assessment of CSF tumor markers and cytology is useful for the identification of patients who have a poor prognosis.

Adolescent

Natural history of fetal ventriculomegaly.

The natural history of in utero ventriculomegaly was defined by a retrospective review of the outcome of 47 fetuses evaluated during a 5-year period by the Fetal Treatment Program at the University of California. In 20 fetuses, a diagnosis of ventriculomegaly associated with other severe abnormalities was made early in pregnancy. Termination of pregnancy was elected in 19 of 20 cases, and no fetus survived. In five fetuses, the diagnosis was made late in pregnancy and was associated with severe abnormalities. Fetuses were handled in a routine obstetric fashion and none survived. Of the other 22 fetuses 19 had stable and two had progressive ventriculomegaly; in one case, ventriculomegaly resolved in utero. Nineteen of these fetuses have survived, 13 with normal intellectual development and six with moderately to severely delayed development. Associated abnormalities were detected with ultrasonography in 74% of fetuses; there was a 20% false-negative rate of detection. Ventriculomegaly was isolated and progressive in two fetuses. In both cases, fetuses were delivered at term, and postnatally a shunting procedure was performed. Both children are neurologically normal. From our results and a review of the literature, which supports our findings, we were unable to define a group of fetuses with in utero ventriculomegaly that would benefit from in utero shunting.

Abortion, Therapeutic

Magnetic resonance imaging and management of a pineal region dermoid.

We report the case of a 19-year-old man harboring a pineal region dermoid, of which only eight other cases have been reported. A diagnosis was made with magnetic resonance imaging (MRI), which showed a marked high signal intensity on both T-1 and T-2 weighted images that is consistent with the lipid character of the lesion. Residual tumor could be seen on postoperative MR images. MRI is an accurate, noninvasive modality for the diagnosis and follow-up evaluation of these tumors.

Adult

Management of infratentorial brain tumors.

Because 60-70% of brain tumors in children occur in the posterior fossa, the pediatric neurosurgeon must be familiar with the specialized techniques necessary for successful surgery in this region. We discuss the preoperative evaluation, surgical techniques, and postoperative management for children harboring infratentorial brain tumors. Using this approach to treatment, we have had no operative mortalities, a 2% rate of infection, a 15% rate of transient neurologic deficit, and a 5% rate of permanent neurologic deficit.

Child

Craniometaphyseal dysplasia associated with hydrocephalus: case report.

Craniometaphyseal dysplasia is a rare bone disorder that may cause a variety of neurological abnormalities; hydrocephalus has not been observed with this disorder, however. A case of craniometaphyseal dysplasia associated with hydrocephalus is reported, and possible causes are discussed.

Bone Diseases, Developmental

Aneurysms of the posterior inferior cerebellar artery. A clinical and anatomical analysis.

The clinical and anatomical features of 21 surgically treated saccular aneurysms of the posterior inferior cerebellar artery (PICA) are analyzed. Seventeen of these lesions originated from the PICA-vertebral junction, and four arose from distal PICA branching sites. Twelve lesions arose from the left PICA, nine were right-sided, and all were small (less than 12.5 mm). Most of these aneurysms occurred in females (16 of 21) and presented as classic subarachnoid hemorrhage. The lack of specific focal deficits prevented an accurate pre-angiographic determination of aneurysm location in most instances. Clinically significant vasospasm and aneurysm multiplicity occurred with approximately equal frequency as at other locations. The angiographic and surgical features of these lesions are determined by the course of the vertebral artery and PICA; that is, they occur at branching sites and at curves in the parent vessel, and point in the direction in which flow would have continued if the curve at the aneurysm's origin had not been present. Aneurysms at the PICA-vertebral junction usualthese lesions are determined by the course of the vertebral artery and PICA; that is, they occur at branching sites and at curves in the parent vessel, and point in the direction in which flow would have continued if the curve at the aneurysm's origin had not been present. Aneurysms at the PICA-vertebral junction usualthese lesions are determined by the course of the vertebral artery and PICA; that is, they occur at branching sites and at curves in the parent vessel, and point in the direction in which flow would have continued if the curve at the aneurysm's origin had not been present. Aneurysms at the PICA-vertebral junction usually occur at least 1 cm above the foramen magnum level, arise distal to the PICA origin in the angle between the two vessels, and are best approached by a paramedian incision with the patient in the lateral recumbent position. Isolated clipping of the aneurysm neck is essential in this instance, as trapping may compromise vital perforating arteries of the brain stem. More distal (retromedullary) PICA aneurysms are sometimes associated with another vascular anomaly (two cases in this series), and are best handled through a bilateral suboccipital craniectomy. Clipping of the neck is the preferred treatment, but trapping is usually safe, if necessary.

Adult

Pediatric neurosurgical implications of the amniotic band disruption complex. Case reports and review of the literature.

Defects caused by the amniotic band disruption complex (ABDC) may vary from simple malformations caused by digital constriction to major scalp, craniofacial, and visceral malformations. ABDC may cause 7-14% of stillbirths. The etiology is unclear, but the most commonly accepted mechanism involves rupture of the amnion followed by fetal malformation, deformation, and compression. This mechanism does not adequately explain all anomalies such as hydrocephalus and holoprosencephaly that are seen in the ABDC. The use of prenatal ultrasound has allowed the diagnosis of the ABDC in utero. Since 1980, four children with the ABDC who required neurosurgical intervention were seen at the University of California, San Francisco; the presentation and subsequent surgical treatment of 2 of these children are discussed. A combined craniofacial team approach to the management of these children can maximize reconstructive and neurologic outcome.

Abnormalities, Multiple