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Biomedical subjects

D A Bruce

Publications and source records attributed to D A Bruce.

At least 55 records · Page 3Linked to original sources

Management of trigonocephaly.

Trigonocephaly, resulting from premature fusion of the metopic suture, is one of the least common forms of craniosynostosis referred to our institution. We describe a method of treating this condition that has been satisfactory in our hands. A bicoronal scalp flap is reflected down to the glabella and supraorbital ridges and a "gull-wing" bone flap is then formed in the frontal region, hinged forward on the metopic ridge. The midline strut in the area of the fused metopic suture is then burred down using a high-speed drill, but is not removed. We feel that this method results in an excellent immediate cosmetic result and is technically easier then the morselization procedures which have previously been described. We have found this to give a satisfactory result in 13 patients who underwent this procedure.

Congenital Abnormalities↗

Single stage composite resection and reconstruction of malignant anterior skull base tumors.

Operative morbidity, including osteomyelitis and meningeal and cerebral sepsis, remains high among patients undergoing the composite (bony and overlying soft tissue) resection of malignant skull base tumors. This is in part because of the poor wound healing afforded by free skin grafts and the poorly vascularized pericranial flaps routinely used to resurface the operative defect. In an effort to reduce the operative morbidity, single stage resection and reconstruction with distant vascularized flap tissue was performed in five consecutive patients who presented with malignant skull base tumors. We think that immediate flap reconstruction will allow skull base lesions previously considered unresectable or associated with a prohibitive complication rate to be safely resected. Case presentations illustrate operative techniques. The potential disadvantage of masking a tumor recurrence with immediate flap coverage is discussed.

Aged↗

Effects of hydrocephalus and increased intracranial pressure on auditory and somatosensory evoked responses.

Recent studies in human and animal subjects have suggested a relationship between intracranial pressure (ICP) and ventricular dilatation and multimodality evoked responses which, if substantiated, would be of value to clinical practice as a noninvasive way of assessing the need for shunting in selected patients in whom computed tomography (CT) is not definitive. In an attempt to better define these changes, auditory evoked response (BAER) and somatosensory evoked response (SER) were performed on 16 cats as a base line, after which they were made hydrocephalic by the cisternal injection of kaolin. Nine cats survived, and CT or magnetic resonance scans were performed on them 4 to 6 weeks later. In those animals in which ventricular dilatation was noted, repeat evoked responses were recorded. In the 6 hydrocephalic cats, the ventricle was punctured to measure ICP, which in all cases was less then 5 mm Hg. The lumbar spinal dural sac was then ligated, which resulted in periodic plateau waves up to 75 to 100 mm Hg after 4 to 6 hours, lasting up to 10 minutes. In neither group of cats was any change in either BAER or SER observed until preterminally, when ICP was in the range of 75 to 100 mm HG and cerebral perfusion pressure was compromised. This suggests that the BAER and SER are not sensitive to either ventricular dilatation or intracranial hypertension.

Animals↗

Magnetic resonance imaging of spinal cord disease of childhood.

Correct diagnosis of spinal cord disease in childhood is often delayed, resulting in irreversible neurologic deficits. A major reason for this delay is the lack of a reliable means to noninvasively visualize the spinal cord. Magnetic resonance imaging (MRI) should be useful in the evaluation of diseases of the spinal cord. A 1.5 Tesla MRI unit with a surface coil was used to study 41 children, including eight patients with intrinsic spinal cord lesions, eight patients with masses compressing the cord, 12 patients with congenital anomalies of the cord or surrounding bony structures, three patients with syrinxes, and three patients with vertebral body abnormalities. Intrinsic lesions of the cord were well seen in all cases as intrinsic irregularly widened, abnormally intense cord regions. MRI was helpful in following the course of disease in patients with primary spinal cord tumors. Areas of tumor were separable from syrinx cavities. Extrinsic lesions compressing the cord and vertebral body disease were also well visualized. Congenital anomalies of the spinal cord, including tethering and lipomatous tissue, were better seen on MRI than by any other radiographic technique. MRI is an excellent noninvasive "screening" technique for children with suspected spinal cord disease and may be the only study needed in many patients with congenital spinal cord anomalies. It is also an excellent means to diagnose and follow patients with other forms of intra- and extraspinal pathology.

Adolescent↗

Tumor staging for pineal region tumors of childhood.

The central midline location of the pineal gland, its intricate contact with cerebrospinal fluid pathways, and the generally large size of tumors in this area before they produce symptoms raise problems in the use of TNM classification. Biopsy was performed on 30 consecutive pineal region tumors seen in children detected over an 8-year period. The pathologic features did not conform to previous autopsy data. Germinomas comprised only 7% of tumors, whereas 23% were malignant germ cell tumors. Neither computerized tomography, magnetic resonance imaging scan, nor markers were shown to be diagnostic of pathologic type. It was concluded that the significant factors affecting outcome are tumor cell type and presence or absence or mitoses. Surgical biopsy is recommended for all pineal region tumors in children. At the current time, the data base is insufficient to devise a staging classification specific for pineal region tumors and the TNM system appears largely inappropriate. Thus, it is reasonable to develop a data base for tumors of this region that then may lead to a specific staging system.

Brain Neoplasms↗

Ependymomas. Factors in clinical and diagnostic staging.

In ependymoma, there is evidence that indicates that age, sex, histologic patterns, functional status, location, duration of symptoms, size, and accessibility to surgical removal influence survival. The role of neuron-specific enolase and other biologic markers must await the accumulation of further information. Further advances in diagnostic imaging, immunodiagnostics, and intraoperative staging should lead to a better understanding of the disease and pinpoint prognostic factors more precisely. A profile that contains important information is suggested; this should eventually lead to the development of a staging system.

Adolescent↗

Oligodendroglioma of the posterior fossa in childhood.

Oligodendrogliomas are uncommon intracranial tumors of childhood, especially when these tumors arise in the posterior fossa. Oligodendrogliomas are usually treated with local radiation therapy. The authors report four children, median age 7.5 years, cared for over a 7-year period, who had oligodendrogliomas (all histologically malignant) of the cerebellar region. Three patients received local radiation therapy and all had recurrent disease at a median of 11 months posttreatment outside their radiation field; at the time of relapse, disease at the primary tumor site was stable. One child treated with craniospinal and local radiation therapy is disease-free 15 months after diagnosis. These results suggest that oligodendrogliomas of the posterior fossa should be considered potentially malignant lesions and treated with local plus presymptomatic craniospinal radiation therapy.

Adolescent↗

Leptomeningeal dissemination of primary central nervous system tumors of childhood.

Presymptomatic craniospinal radiation therapy improves the rate of survival for children with brain tumors, which frequently metastasize to the leptomeninges. Radiotherapy may cause neurological damage and should be used only in patients considered to be at highest risk for leptomeningeal dissemination (LMS) at either the time of initial diagnosis or onset of disease relapse. We reviewed 314 consecutive patients with brain tumors to determine the incidence, timing, and importance of LMS. LMS occurred in 60 (19%) children. LMS occurred before diagnosis in 30 patients, as the only site of relapse or simultaneously with local first disease recurrence in 17 patients, and after local disease recurrence in 13 patients. Children with primitive neuroectodermal tumors, anaplastic gliomas, and ependymomas most frequently had LMS. Patients with primitive neuroectodermal tumors and posterior fossa anaplastic gliomas frequently had LMS before diagnosis or at the onset of relapse, whereas patients with ependymomas had LMS after local disease relapse. Both myelography and cerebrospinal fluid cytological examination are required to diagnose LMS.

Arachnoid↗

Brainstem gliomas of childhood: magnetic resonance imaging.

We compared magnetic resonance imaging (MRI) and CT on 16 children with brainstem gliomas. MRI demonstrated masses of decreased signal intensity, which enlarged and distorted brainstems in all patients with active disease and showed brainstem abnormalities in 21 of 23 studies (91%). In one-half of the patients, MRI showed more extensive disease than did CT. Exophytic portions of tumors were shown well on MRI. MRI was more sensitive than CT in demonstrating disease relapse.

Adolescent↗

Discrepancy between computed tomography and magnetic resonance imaging in a case of medulloblastoma.

In a 2-year-old girl with signs and symptoms of a posterior fossa tumor, the computed tomographic scan showed what appeared to be a brain stem glioma. However, magnetic resonance imaging (MRI) demonstrated the lesion to be extrinsic to the brain stem. Posterior fossa exploration disclosed a medulloblastoma filling the 4th ventricle. This discrepancy in imaging techniques points out the usefulness of MRI in the evaluation and treatment of posterior fossa lesions.

Brain Stem↗

Familial pineoblastoma. Report of two cases.

Two cases of pineoblastoma are presented. One occurred in a 12-year-old girl and the other, 20 months later, in her 43-year-old mother. A possible genetic basis and mutual exposure to an environmental factor responsible for the expression of a tumor gene are postulated in the discussion.

Adult↗

Chiasmatic optic glioma treated with chemotherapy. A preliminary report.

Chiasmatic optic glioma is a rare tumor with an erratic natural history, usually seen in young children. A prior study from this institution demonstrated that these lesions were frequently lethal, despite initial clinical stabilization following radiation therapy, and that visual, intellectual, and late endocrinological disabilities were prevalent. A novel approach was developed in 1977, when an initial clinical response to vincristine was recorded in a child with a recurrent optic glioma. Since then, all children with recurrent optic glioma and all children aged 6 years old and under with newly diagnosed optic glioma have been offered a program of initial therapy with vincristine and actinomycin D for six cycles over 18 months. The four children with recurrent tumor who were treated with that regimen remain clinically stable 13 to 115 months after chemotherapy. Twelve children (eight under 24 months old) with newly diagnosed optic glioma have been treated with this program, and three are still on therapy. Four developed progression while on therapy, and five remain stable from 1 to 60 months posttherapy. The four children who developed progressive disease have been treated with radiation therapy and remain stable. Six of the 12 children showed shrinkage of their tumor on computerized tomography while receiving chemotherapy. This program may serve as an alternative to initial radiation therapy in young children.

Child, Preschool↗

Management of the newborn with myelomeningocele: time for a decision-making process.

The relationship between time of surgical intervention and eventual outcome was examined in 110 newborns with myelomeningocele. Numerous earlier reports have cited a significant increase in mortality and morbidity associated with delay of surgery beyond 48 hours. Within the study population of infants, 52 infants (47%) had "early" surgery within the first 48 hours of life, 32 infants (29%) had "delayed" surgery between 3 and 7 days of age, 12 infants (11%) had "late" surgery between 1 week and 10 months of age, and 14 infants (13%) never had surgery by parental decision. Survival rates were similar between those with early, delayed, or late surgery as 92%, 94%, and 100%, respectively, were alive at age 10 months. Also, no significant association existed between time of surgery and development of ventriculitis, developmental delay, or worsening of paralysis. From these observations, it is concluded that there is no urgency in surgical intervention for the initial management of newborns with myelomeningocele. Rather, there is time for comprehensive discussions, counseling, and emotional support for those parents in need of a decision-making process before establishing consent for or against surgical management of their newborn.

Bacterial Infections↗

Magnetic resonance imaging of lesions of the posterior fossa and upper cervical cord in childhood.

Magnetic resonance imaging (MRI) promises to be an effective, noninvasive means of visualizing intracranial pathology. It should be especially useful in the evaluation of posterior fossa and cervical spinal cord disease of childhood; computed tomographic (CT) evaluation is frequently suboptimal in this region. MRI results are reported for 46 consecutively seen children with posterior fossa and/or cervical spinal cord disease (28 had brain malignancies; seven had congenital anomalies; three had cerebrovascular accidents). MRI was performed primarily by the partial saturation on a .12 Tesla resistive proton unit. All patients underwent concurrent CT evaluation. MRI demonstrated abnormalities in 96% of scans in patients with structural CNS disease (48 of 50). CNS malignancies were visualized in 100% (28 of 28) of children studied. MRI was especially useful in demonstrating the full extent of infiltrating gliomas and the anatomic location of other mass lesions. MRI frequently demonstrated disease to be more extensive than seen on CT. MRI was more sensitive than CT in documenting response to treatment and disease relapse in patients with infiltrating tumors. Cystic regions within tumors were poorly seen on MRI. Congenital anomalies were demonstrated in all patients evaluated and were better delineated using MRI than CT. MRI is sensitive in the evaluation of posterior fossa and cervical spinal cord disease of childhood and it has obvious advantages over CT; however, its specificity in such evaluations has yet to be proven.

Brain↗

Intracranial embryonal cell carcinoma.

Intracranial embryonal cell carcinoma (ECC) is a germ cell tumor most frequently found in the pineal region. However, little is known about the incidence, pattern of growth, or response to treatment of ECC. Between 1975 and 1983, 16 consecutive patients younger than age 18 with tumors of the pineal region have had biopsies performed prior to treatment, and 5 (31%) have had primary intracranial ECC. One other child had ECC in the suprasellar region. The clinical, radiographic, and histologic features of the six patients with ECC are presented and contrasted with the findings in children with other pineal region neoplasms. Pathologic confirmation is necessary to distinguish ECC from other intracranial tumors. Three patients were treated with radiation therapy (RT) plus adjuvant chemotherapy; two patients were treated with chemotherapy followed by RT; and one was treated with RT only. All patients initially responded to therapy, but only one has survived for longer than 1 year. It is concluded that biopsy is necessary for the diagnosis of ECC; that ECC is more common than currently believed; and that current treatment for intracranial ECC is unsatisfactory. Biopsy of pineal region tumors is necessary if progress is to be made in their management.

Adolescent↗

Vascular steal associated with vein of Galen aneurysm.

Seven patients presented with neonatal arteriovenous malformation and vein of Galen aneurysms. Six had a large degree of vascular steal demonstrated by cerebral angiography and all had significant parenchymal loss on initial CT. Angiographically, the steal affected the ophthalmic artery and branches of the middle and anterior cerebral arteries. Repeat CT in two patients, one of whom was untreated for 7 months, revealed pronounced atrophy. Patients with neonatal vein of Galen aneurysms present with parenchymal loss on CT which is related to vascular steal and may be progressive.

Cerebral Angiography↗