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Biomedical subjects

L Schut

Publications and source records attributed to L Schut.

At least 109 records · Page 6Linked to original sources

Pineal region tumors of childhood.

The incidence, response to treatment, and outcome of children with pineal region neoplasms is poorly characterized. Since 1975, in one institution, 25 consecutive patients with pineal tumors have undergone biopsy prior to further treatment. This constituted 11% (25/234) of all brain neoplasms seen over this time period. Specific tumors diagnosed included pineal parenchymal tumors ( pineoblastomas , pineocytomas ) in eight patients (32%); germ cell tumors (embryonal cell carcinomas, teratomas, germinomas) in eight patients (32%); glial tumors (astrocytoma, ganglioglioma ) in eight patients (32%); and ganglioneuroblastoma in one patient (4%). Clinical parameters, computed tomographic findings and CSF markers (alphafetoprotein and human chorionic gonadotropin) were unreliable in discriminating between specific tumor types. Response to treatment and patterns of disease relapse were dependent on the type of tumor present. Five of eight children with pineal parenchymal tumors had disease recurrence, and in all leptomeningeal dissemination occurred prior to or concurrent with local relapse. Three of eight children with germ cell tumors and two of eight patients with glial tumors suffered a relapse; in all five children recurrence was initially local. Findings suggest that pineal region neoplasms are not infrequent in childhood; that these tumors vary greatly in histologic type; that contrary to other reports germinomas do not constitute the majority of pineal tumors; and that histologic confirmation is necessary prior to treatment for appropriate management.

Adolescent↗

Computed tomography in penetrating wounds of the orbit with retained foreign bodies.

Penetrating wounds of the orbit can have serious immediate and delayed effects on the eye and the CNS. Infection, vessel erosion, or interference with ocular function necessitates removal of the foreign object. Precise localization of the retained material and its relationship to the globe, extraocular muscles, optic nerve, and brain is essential, when surgery is contemplated. We describe two children with retained foreign bodies that required removal. Computed tomography (CT) was essential in one instance in planning the surgical approach. In the other patient, the retained foreign body and an associated brain abscess were recognized only by CT. The contribution of CT to the assessment of retained foreign bodies is discussed and its present limits are defined. Computed tomography is highly recommended as the single most informative diagnostic modality for this type of injury.

Brain Abscess↗

Central nervous system lesions and developmental scoliosis in myelomeningocele.

Hydrocephalus, spinal cord tethering, cord atresia, hydromyelia, syringomyelia and various manifestations of the Arnold-Chiari malformation occur in association with scoliosis in patients with myelomeningocele. While the causes of the cord lesions remain debatable, these lesions appear to be progressive and causally related to the scoliotic deformity. We have reviewed seventeen myelomeningocele patients with developmental scoliosis and have found that aggressive orthopaedic treatment combined with neurosurgical treatment can satisfactorily control spinal deformity in most cases. Before undertaking orthopaedic treatment of severe spinal deformities in patients with myelomeningocele a careful investigation of the central nervous system with myelography and cord and head CT scans should be carried out.

Arnold-Chiari Malformation↗

Cerebral gangliogliomas during childhood.

We have reviewed our experience with gangliogliomas treated in the post-computed tomography (CT) era at the Children's Hospital of Philadelphia. Of 234 newly histologically verified neoplasms seen at our institution since 1975, 10 (4.3%) were gangliogliomas of the cerebral hemispheres. The presenting complaint was seizures in 9 of 10 patients, and in 8 the seizures were poorly controlled despite increasing doses of anticonvulsant medication. At the time of diagnosis, only 2 patients had a focal neurological deficit and none had signs or symptoms of increased intracranial pressure. Learning disability and behavioral disturbances were common in this group of children. The CT appearance of these lesions was characteristic: most appeared as a cerebrospinal fluid density area that was located peripherally and often indented the skull. There was little contrast enhancement, and a few were diagnosed initially as arachnoid or porencephalic cysts. Despite the CT appearance, all but 2 of the lesions were found to be solid at operation. In one patient, the lesion appeared as an enhancing lesion of the thalamus, and this patient died. Operation resulted in symptomatic improvement. Eight of 9 children are alive, with a follow-up of 1 to 80 months (median, 19.5 months), and are free of progressive disease. Five are seizure-free while receiving anticonvulsant therapy and an additional 3 have improved seizure control as a result of operation. Two of the 3 children with intellectual difficulties preoperatively have shown improvement on testing after operation, probably the result of improved seizure control. One patient developed a disseminating malignancy and died. It is concluded that worsening seizures in the pediatric age group should warrant CT examination and that ganglioglioma should be included in the differential diagnosis of low density areas on CT.

Adolescent↗

Chiasmatic gliomas of childhood. A reappraisal of natural history and effectiveness of cranial irradiation.

Disagreement exists concerning the natural history and treatment of chiasmatic gliomas (CG) of childhood. We reviewed our experience in 21 cases of surgically verified CG with a median age of 4 years at time of diagnosis, followed for a median of 5.3 years (range 3-14.8 years). Initial treatment included radiation therapy (RT) in 18 patients, chemotherapy in 2, and observation in 1. Disease recurrence, defined as progressive visual or neurological deterioration, was documented in 10 children (48%), occurring at a median of 6 years after diagnosis. 5-year actuarial survival was 89%, but fell to 60% by 10 years. Visual improvement after RT was uncommon, occurring once. Intellectual deficits were noted in 5 of 17 survivors. We compared our results to that of other patients reported and concluded that: (1) CG may act aggressively independent of their location in the visual pathway at time of diagnosis; (2) the beneficial effects of RT are difficult to document; (3) progressive disease may occur late in the course of illness, and (4) intellectual sequelae are common in long-term survivors.

Astrocytoma↗

Hemorrhagic brain tumors in pediatric patients.

In an unselected pediatric series of 113 newly tissue verified intracranial tumors, intracranial hemorrhage was the initial presentation in 10% of the cases. Review of all cases of subarachnoid hemorrhage admitted to Children's Hospital of Philadelphia during the period of 1976--1979 revealed that intracranial tumors were present in 15%. The largest hemorrhagic pathological group was medulloblastoma-primitive neuroectodermal tumors. No particular age predominance was noted. The high incidence of intracranial tumor hemorrhage in this series of pediatric patients suggests that this may be a more common presentation than previously reported.

Adolescent↗

Improved relapse-free survival in medulloblastoma utilizing modern techniques.

Between 1969 and 1979, 22 patients with medulloblastoma were treated by the same surgical group and radiation therapy group. The patients were divided into two groups because of the clinical availability in December 1974 of the computed tomographic (CT) scanner and of the operating microscope used in the initial surgical procedure. There were 11 patients in each group. The percentage of patients with a relapse-free survival in the group treated between 1969 and 1974 (Group 1) was 38% at 4 years. The survival in the 11 patients treated between 1974 and 1979 (Group 2) was 84% at 4 years. This improvement is statistically significant (P less than or equal to 0.001). All patients received the same dose of radiation. Efforts to minimize the tumor burden by total surgical resection did not increase postoperative morbidity or mortality. These results are discussed, along with the relative impact of the CT scan, total resection at operation, and increased focus for radiation therapy on the improved outcome.

Cerebellar Neoplasms↗

Congenital craniofacial asymmetry: early treatment.

Congenital craniofacial asymmetry has two dominant causes: isolated synostosis and craniofacial clefts. Treatment considerations in these problems differ from those with isolated cranial or isolated facial defects. Isolated cranial defects are most frequently treated by the neurosurgeon with craniectomy alone. Isolated facial asymmetry when congenital in origin usually manifests as hemifacial microsomia and based on our experience with 40 such patients, is best treated in later childhood. Treatment timing of craniofacial asymmetry varies with the cause, but is best done in the first two years of life. Nasofrontal encephaloceles are usually best treated in the first few weeks of life; synostosis syndromes are treated at six months of age after the facial sutures have had time to stabilize sufficiently for adequate dissection and mobilization; and other craniofacial clefts at approximately two years of age following descent of the teeth and better homeostatic capability of the patient. Based on our series of 58 patients, 40 treated with isolated synostosis at less than one year of age, eight at more than one year of age, and ten patients with craniofacial clefts, the guidelines for timing and methods of treatment have evolved. Liberal use of craniectomy bone with expected regrowth is possible in the first year of life, and more limited use in the second year of life. This bone is used to hold the repositioned orbit, augment hypoplastic zygomas, and reconstruct noses, or for other uses. In isolated synostosis, repositioning provides a form of immediate catch-up growth then proceeds normally. In craniofacial clefts, repositioning puts structures into normal relations and growth likewise proceeds normally. The isolated synostosis syndromes treated at a later age are done with more difficulty, though may be effectively cared for. Complications other than incomplete structural correction have been nonexistent in the group two years of age and less.

Abnormalities, Multiple↗

Pediatric brain stem gliomas.

Sixty-two pediatric patients with brain stem glioma diagnosed between 1964 and 1978 have been reviewed. Posterior fossa eploration was performed on 53% of the patients. Pathology was obtained by biopsy in 58% of those explored. Fifty-four patients had definitive radiation therapy with a median dose of 5000 rads. The actuarial five-year survival for the entire group is 30%. A pathologic diagnosis was available from necropsy or biopsy on 36 of the 62 patients. One-half had malignant tumors, and none survived more than 16 months. The remaining patients with well-differentiated gliomas had five-year actuarial survival of 55%. The use of computed tomography (CT) has been found to be valuable in diagnosis and follow-up, as well as in the design of radiation therapy portals. The data demonstrate no dose response curve. We recommend local radiation therapy of 5000 to 5500 rads to the tumor area as defined by CT.

Actuarial Analysis↗

Recovery following brainstem hemorrhage.

Two patients made virtually complete recoveries following hemorrhage within the brainstem. Progressive clinical deterioration in each case was managed surgically by evacuation of the hemorrhagic lesion in 1 case and treatment of secondary hydrocephalus in both. Computerized tomographic scanning proved essential for nonsurgical diagnosis and later management.

Adult↗

Computed tomography of brain stem gliomas in children.

In 24 CT studies in children with brain stem gliomas, the CT appearance was seen to vary with the tumor's physical characteristics. Infiltrative tumors produce diffuse enlargement of the brain stem; they are isodense with brain parenchyma or of decreased density and may show partial enhancement. Some gliomas grow focally as an expansile mass or as a large exophytic lesion. These have heterogeneous density and show prominent enhancement. Early diagnosis depends on appreciation of displacement of the fourth ventricle and compression and distortion of the cisterns. The use of diagnostic studies and surgical and therapeutic results in the 24 cases are compared to 45 such cases diagnosed prior to the use of CT.

Adolescent↗

Computed tomography of pineal, parapineal, and histologically related tumors.

Thirty-one of 32 pineal and related tumors were diagnosed by computed tomography (CT). There were 7 germinomas, 4 primary pineal tumors, 4 embryonal cell carcinomas, one teratoma, one epidermoid, one primitive neuroectodermal tumor, 8 astrocytomas and 6 lipomas. Histologic confirmation was obtained in 22. The CT characteristics allow differentiation of benign tumors such as teratomas and epidermoids from malignant germinal lesions such as germinoma and embryonal cell carcinoma. Abnormal calcification occurs most frequently in the primary pineal tumors, pineoblastoma and pineocytoma. The CT characteristics and consideration of the patient's age and sex often lead to histologic prediction and better treatment planning.

Adolescent↗

The value of CAT scanning following pediatric head injury.

The CAT scan has proven invaluable in the management of severe pediatric head traumas. The most frequent findings are those of subarachnoid hemorrhage and diffuse swelling. Mass lesions are readily identified by the CAT scan, and it is easy to distinguish between hematoma and/or edema in the acute stages. Follow-up scans have produced a much better understanding of the long-term pathophysiologic effects of head trauma on the injured child.

Age Factors↗