Biomedical subjects
Publications and source records attributed to K R Winston.
The incidence, embryology, anatomical features of the site of junction, associated abnormalities, and survival of the 79 craniopagi reported from 1496 to 1987 have been reviewed. All craniopagi upon whom sufficient information is available have been classified on the basis of the deepest shared anatomical structure.
The world's surgical experience on the division of craniopagi is reviewed, and a new case is reported. The problems, including ethical considerations, that must be addressed in selecting patients for operation and in preparing for the procedure are discussed in detail. Hemodynamic stability and the problem of intraoperative gravitational distortion of the brain are addressed. The importance of careful planning is stressed. Survival of the 31 craniopagi after operation is discussed in relation to types of junction.
A melanin-producing, encapsulated, histologically benign-appearing tumor of the trigeminal nerve was surgically removed from a 9-year-old boy. It recurred 6 months later and was then subtotally resected and treated with radiation therapy. The clinical and pathological features of this and 10 similar cases reported in the literature are reviewed. These tumors are best categorized as meningeal melanocytomas.
Ischemic necrosis of the occipital scalp followed embolization and surgery for a large convexity meningioma in one of our patients. This complication was successfully managed by free tissue transfer. Suggestions for the prevention of this serious complication are presented.
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Radiation therapy (XRT) was used in the treatment of 25 patients with tumors of the pineal and suprasellar locations. A tissue diagnosis was obtained before XRT in 5 patients, and 20 were irradiated without histologic verification. The overall survival rate is 80% (76% with no evidence of disease [NED]). Megavoltage XRT was delivered to the entire neuraxis in 22 patients, and 86% (19/22) are alive from 4 to 88 months (median, 30 months) after treatment. In two of three patients treated only to local fields, tumor recurred in the spine; both are dead of disease. Biopsy-proven germinomas and multiple midline tumors responded favorably to XRT, whereas solitary pineal tumors and teratomas with marker positivity (human chorionic gonadotropin, alpha-fetoprotein) did not respond as well. The endocrinologic presentation, tumor marker status, and early response to radiation measured on computed tomography are useful means for selecting patients for radiation therapy.
Between 1972 and 1981, 37 children with craniopharyngioma were cared for at Children's Hospital, Boston. In this paper, the results of treatment with radiation therapy after conservative operations are compared with those following an initial attempt to excise the tumor. Radiation therapy was equally, if not more, effective than attempted excision in controlling subsequent tumor growth. Although this was not a controlled study, the complications of each approach are indicated, and it is inferred that conservative operations combined with radiation therapy offer less risk for psychosocial impairment than does attempted tumor excision when patients are considered as a group. The ultimate effect that either approach might have on quality of life remains to be defined, and therapy must still be individualized to the particular clinical problem.
We examined 36 infants with myelodysplasia, using excretory urography, voiding cystourethrography, and urodynamic assessment in the newborn period and periodically thereafter to determine those at risk for decompensation of the urinary tract. Urodynamic evaluation showed 18 with dyssynergia of the detrusor and external sphincter, nine with synergic activity of the sphincter, and nine with no activity of the sphincter. Thirteen (72%) of the group with dyssynergia had or later were found to have hydroureteronephrosis, while this was the case in only two (22%) with synergy and one (11%) with absent activity. The conditions of these 16 patients improved after decompression by cutaneous vesicostomy or intermittent catheterization. Infants with dyssynergia of the detrusor-external sphincter are at high risk for deterioration of the urinary tract; they should be followed up closely, and intermittent catheterization should be started early.
One hundred two patients were evaluated for late effects of brain tumor in childhood by a medical record review and follow-up questionnaire. The patients had survived brain tumor for 5 to 47 years (median, 18 years). Among 30 patients who had received brain irradiation, 3 were successfully treated for second neoplasms that developed within the radiotherapy field 11, 16, and 29, years later, respectively. Moderate or severe functional deficits were present in 24 patients (24%), more commonly among those treated before 2 years of age and among those with cerebral astrocytoma. The other 78 patients (76%) had mild or no gross deficits, and all but 1 of them were attending school or were employed. Twenty-one patients in the series reported a total of 41 offspring, none of whom had cancer or mental retardation. Although markedly diverse performance levels were found among the 102 long-term survivors of childhood brain tumors, the majority had an acceptable quality of life.
A child with epidural hematoma presented with choreiform movements which promptly resolved following the evacuation of the hematoma.
The medical literature relating to spontaneous cervical epidural hematoma has been reviewed, and two new cases have been added. In total, 43 cases have been analyzed in detail, and the authors present some new hypotheses as to the pathophysiology of this disorder.
Tissue dehydration, including that of the brain, is a natural part of maturation. In the newborn it is accompanied by an acute salt water diuresis which is particularly pronounced in the premature infant. The decrease in cranial volume and fall in intracranial pressure to subatmospheric levels observed in premature infants after birth suggests that the brain shares in this process. Since fluid homeostasis is normally under hormonal control, the inordinate loss of fluid in the prematurely born may be attributable to a hormonal imbalance. The possibility that prolactin, a proven osmoregulatory hormone in submammalian species but not in mammals and primates, may have an important role in this process is suggested by recent evidence that prolactin regulates tissue water in fetal and newborn animals. If this hormone assists in the regulation of water and electrolyte content of the brain during the perinatal period then prolactin receptors might be expected at blood-brain and blood-CSF barriers. Our study with preterm, term and adult rabbits indicates that prolactin receptors are present and in much higher concentrations in the choroid plexus than in other tissues examined. Specific binding of prolactin to receptors on tissues normally is observed to increase with age. Since binding to the choroid plexus decreased with age it would suggest that the effect of prolactin on choroid plexus function may be more important during the perinatal than later stages of development.
The authors present neurological findings and data from psychophysiological tests administered during the follow-up period of 35 patients with craniopharyngioma. Group I patients who were treated by irradiation alone or by radiotherapy and conservative surgical procedures (including biopsy, cyst aspiration, and shunting), showed significantly less frontal lobe and visual perceptual dysfunction than Group II patients, in whom radical tumor resection was attempted by a subfrontal exposure. Frontal lobe dysfunction was demonstrated in sorting tests by perseverative responses, inflexibility of behavior, and lack of inhibitory control, while intelligence quotients remained relatively unaffected. Immediate memory defects and decreased manual dexterity were present, to different degrees, in both treatment groups. On the basis of these preliminary data and a maximum follow-up period of 10 years, the authors conclude that primary irradiation of a craniopharyngioma appears associated with a lower morbidity rate and may avoid the frontal lobe disorders seen in the patients with extensive tumor resection.
Caudal herniation of the hindbrain, indistinguishable from the Chiari I deformity, may occur after the establishment of spinal subarachnoid shunts and become symptomatic years after the procedure. Examples are presented and others are cited from the literature. It is proposed that the force responsible for the displacement is the difference in pressure between the cranial and spinal compartments. On the basis of these observations and other considerations as well, a similar process, disproportionate absorption of cerebrospinal fluid from the spinal region, might account for the spontaneous form of the Chiari I deformity.
This is a report of three children with spinal epidural abscess. The literature is reviewed and the features of this condition in children are noted. Because of the nonspecificity of presenting symptoms in children, the diagnosis may be delayed, resulting in a worse outcome, especially in children under 1 year of age. The extensive laminectomy advised for the treatment of spinal epidural abscesses in adults is undesirable in children because of the risk of spinal deformity and in most cases is probably not necessary.
Survival and quality of survival have been investigated in 79 patients operated for intracranial metastatic tumor during the decade 1967--1977. The 30-day mortality was 10%. Median survival varied between two and 14 months depending upon the type of primary tumor. The one-year survival was 22% and the two-year survival was 10%. Duration of survival depended strongly upon type of primary turmor and little upon preoperative condition, age, or sex. Fifty-three percent of neurologically impaired patients who survived for at least one month were in better condition after surgery and 5% were in worse condition.
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A method successfully used in closing a large skin defect in each of three children with myelodysplasia is described in detail. Full thickness skin closure was achieved in 7--10 days without skin grafts or rotation flaps.