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Biomedical subjects

L N Sutton

Publications and source records attributed to L N Sutton.

At least 127 records · Page 7Linked to original sources

Symptomatic Arnold-Chiari malformation: review of experience with 22 cases.

Twenty-two patients with closed myelomeningoceles, shunted hydrocephalus, and symptomatic Arnold-Chiari malformations were studied retrospectively. Seventeen of the 22 patients were aged 6 months or younger; five patients were aged 3 to 23 years. Patients in the younger group presented with stridor, apnea, and/or feeding difficulty; those in the older group presented with hemiparesis, quadriparesis, oscillopsia, nystagmus, or opisthotonos. Fourteen of the 17 younger patients underwent surgical decompression of the Arnold-Chiari malformation: 10 within 18 days following the onset of symptoms and four on Day 19 or later. Of the 10 infants with early treatment, five eventually died secondary to continued symptoms and five survived. Of those surviving, three were asymptomatic and two had continuing symptoms but were improved over their preoperative state. Among the four patients undergoing surgery later, two died, one had lessening of stridor, and one had complete relief of symptoms. Of the three infants not undergoing decompression, two died and one eventually became asymptomatic. All five of the older patients underwent decompression and all had complete resolution of their preoperative symptoms and signs. The authors conclude that while decompression of a symptomatic Arnold-Chiari malformation may be effective in children and adults, this treatment does not always improve the clinical condition of infants. This may be the result of ischemic/hypoxic effects on the infant's brain stem, which may not be organized normally at birth.

Adult↗

Effects of increased ICP on brain phosphocreatine and lactate determined by simultaneous 1H and 31P NMR spectroscopy.

In order to study the metabolic events surrounding ischemia induced by the graded increase of cerebrospinal fluid (CSF) pressure, the technique of simultaneous phosphorus-31- and hydrogen-1-enhanced nuclear magnetic resonance spectroscopy was applied to five cats as intracranial pressure (ICP) was gradually raised by the instillation of mock CSF. Threshold lactate rose at an average cerebral perfusion pressure (CPP) of 49 torr, and, in general, preceded a threshold decrease in phosphocreatine, which was observed at an average CPP of 29 torr. There was considerable variation among cats in the CPP at which failure of brain energy metabolism occurred, however, suggesting differences in the autoregulatory curves. It is concluded that, with elevated ICP, there is no universally "safe" CPP at which brain energy metabolism may be assumed to be uncompromised.

Animals↗

Neurosurgical considerations in cranio-orbital surgery.

The neurosurgeon is an integral member of the craniofacial team. The role of the neurosurgeon in the preoperative planning, intraoperative management, and postoperative follow-up of patients with craniofacial malformations is reviewed.

Brain↗

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↗

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↗

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↗

Brain stem necrosis after preradiation high-dose methotrexate.

Both cranial radiation therapy (RT) and high-dose systemic methotrexate (MTX) are used to treat intracranial neoplasmas, but both may cause neurologic damage. MTX may be less neurotoxic if given before rather than after radiotherapy. We cared for a 5-year-old girl with a pineocytoma who had progressive brain stem dysfunction 4 months after MTX therapy, followed by local radiation therapy. CT studies were consistent with radiation necrosis that was confirmed at autopsy. MTX used in conjunction with cranial irradiation can result in severe neurotoxicity, even if the drug is given first.

Brain Diseases↗

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↗

Pituitary enlargement mimicking pituitary tumor.

Primary hypothyroidism can result in reactive enlargement of the pituitary gland which is indistinguishable from primary pituitary lesions on computerized tomography (CT) scans. The presenting symptoms may be due to pituitary gland enlargement, as in two of the three cases reported here. Therefore, the diagnosis of pituitary hypertrophy or hyperplasia secondary to hypothyroidism must be based on the endocrinological work-up. Following treatment of primary hypothyroidism, the diminution in size of the pituitary gland can be demonstrated with CT.

Adolescent↗

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↗

Re-operation for recurrent brain tumors in children.

In order to determine the efficacy of re-operating on recurrent brain tumors, we did a retrospective analysis of 39 children who underwent such surgery between 1975 and the present. A total of 52 procedures was performed for recurrence of both benign and malignant tumors. 9 patients are neurologically normal and 12 are independent but left with some sort of neurologic deficit for an average of 31 months and 32 months, respectively, postoperatively. 1 child presented severely handicapped and remains so 18 months after his most recent surgery. 17 patients died. 11 improved after re-operation and lived an average of 12 months. 4 were unimproved with an average survival of 10 months. There were 2 surgical deaths for an operative mortality of 4%. We feel that re-operation is a useful therapy for both benign and malignant recurrent brain tumors, and prolongs both the quantity and quality of life in appropriately selected patients.

Astrocytoma↗

Resistance of the auditory brain stem response to high barbiturate levels.

In the comatose patient the auditory brain stem response may be one of the few indices of CNS function available. Because barbiturates may be administered to control intracranial pressure and its sequelae, demonstration of the effect of barbiturates on the brain stem response is desirable. In cats preloaded with 63 mg/kg of pentobarbital, intravenous injection of an additional 60 mg/kg reduced, but did not abolish, the later waves of the response. One cat that survived a cumulative dose of 183 mg/kg continued to show a well-defined brain stem response. Even doses of barbiturate nearly incompatible with survival do not abolish this response.

Animals↗