Biomedical subjects
Publications and source records attributed to H E James.
We present a newborn infant with paraplegia and contractures of the lower limbs, consistent with neurologic injury rather than malformation. The mother was involved in a severe motor vehicle accident during the sixth month of pregnancy. We propose that this infant's injuries are a result of that accident.
We here present a hereto previously undescribed association of occult spinal disorders of the distal spinal cord/filum in the Vater association. We present 6 patients who were diagnosed as having the Vater association who subsequently presented with occult spinal disorders, primarily lipomas of the filum/conus complex with or without subcutaneous lipomas. Uniformly the spinal cord was tethered to the lipoma/filum complex. Progressive neurological dysfunction was noted in those in whom the diagnosis was made late in the disease. The importance of neuroimaging of the distal spinal canal, in children in whom the diagnosis of the Vater association has been made, is emphasized to prevent insidious neurological damage if not already present.
Hydrocephalus is marked by the excessive accumulation of cerebrospinal fluid within the ventricles. The disorder is characterized by an imbalance in the production and reabsorption of cerebrospinal fluid. Congenital hydrocephalus is usually the result of an intrauterine infection or maldevelopment of the aqueduct of Sylvius. Acquired hydrocephalus can be caused by infection, neoplasm or hemorrhage. In infants, hydrocephalus usually presents as progressive head enlargement. The presenting symptoms in children are irritability, headache, nausea, vomiting and lethargy. Diagnosis is made with ultrasonography, computed tomography or magnetic resonance imaging. The majority of patients are treated with cerebrospinal fluid shunt procedures, most commonly the placement of ventriculoperitoneal or lumboperitoneal shunts. The outcome of hydrocephalus is determined by the etiology, the presence or absence of associated anomalies, and the timeliness of diagnosis and treatment.
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We report an infant with a craniopharyngioma which was detected in utero. Maternal uterine ultrasonography, done at 27 weeks because of polyhydramnios, revealed a 4 cm midline mass near the base of the fetal skull. At 31 weeks, magnetic resonance imaging of the maternal abdomen confirmed the presence of a mass in the region of the third ventricle and revealed hydrocephalus. Two days post-partum a computed tomography (CT)-guided needle biopsy of the mass was performed and recovered tissue which was histologically consistent with a craniopharyngioma. The infant's postnatal period was complicated by seizures, which were treated with phenobarbital, and by progressive hydrocephalus, necessitating placement of a ventriculo-peritoneal shunt. He also received therapy for central hypothyroidism and diabetes insipidus. The infant's parents refused permission for attempted resection of the tumour and he died at 8 weeks of age. This represents the second reported case of an antenatally detected craniopharyngioma. Four other cases of different intracranial tumours have been detected in utero using ultrasound, with no reported survivors past 3 days of age. There is a uniformly poor prognosis of such infants, but earlier diagnosis and intervention may change this result.
Experimental cryogenic brain oedema was created in albino rabbits, and intracranial pressure (ICP), cerebral blood flow (CBF), EEG, blood pressure, central venous pressure, were subsequently studied at a constant PaCO2. Upon completion the brain water content of the gray and white matter was analyzed by gravimetry. The findings were compared to controls and sham-operated. Two subsets of elevated ICP following cryogenic injury were identified: one with a mean of 6.2 +/- 3.3 torr (n = 7) and the other of 19.3 +/- 9 (n = 5) (p less than 0.005). Both these subsets had similar white and gray matter gravimetry values indicating that the magnitude of cerebral oedema was comparable and could not explain the difference in ICP. There was however a significant difference in the CBF of the left hemisphere between these subsets, with Subset A at 49.1 +/- 9 ml/100 g/min and Subset B with 70 +/- 9.1 (p less than 0.001). We conclude that the ICP elevation in the cryogenic oedema model may be due to not only increased brain water of the injured hemisphere, but also to increased cerebral blood volume due to increased CBF.
Out of a total 157 hospitalized head-injured children, twelve years of age and under, fifteen were considered to be severe, three of whom died within 72 hours of admission. Nine children with closed head injuries who were in coma for at least 24 hours (did not open eyes, speak, or follow commands), with absent or impaired oculocephalic reflex, impaired pupil reactivity to light, and who were decerebrating for at least twelve hours, were studied. Five were given high dose dexamethasone therapy (1 mg/kg) within six hours of injury, repeated at six hours, and then maintained at 1 mg/kg/day for eight days, and four either received none or were treated with a low dose regimen (0.25 mg/kg/day). In those receiving high dose therapy, intracranial pressure waves were noticeably less, peak intracranial pressure was lower, and intensive care and hospital stay were shorter. It was also noted that in the high dose therapy group spontaneous eye opening and speech returned sooner, and all were considered to have returned to their premorbid status by six months following injury. Of the no steroid or low dose group, one died, and of the remainder at six months one was aphasic and still decerebrating, another was aphasic and severely handicapped, and the third returned to school seven months after injury.
Sixty-two patients, age twelve years and under, were treated for incontinence due to neurogenic bladder secondary to spina bifida (58) or trauma (4) with a follow-up period of three to thirty-two months (mean, ten months). In early life, patients were managed with the Credé maneuver (18 patients). Older children were managed with intermittent clean catheterization (44 patients) with or without pharmacologic agents. Continence was achieved with intermittent clean catheterization in 77 per cent, thus enabling these children to be more acceptable to their peers and in many cases attend schools from which they had previously been excluded because of urinary incontinence. With the Credé maneuver, renal deterioration was noted in 11 per cent on follow-up IVP, and in 2 per cent of the patients on intermittent clean catheterization. Recurrent asymptomatic bacteriuria occurred in 33 per cent of those with the Credé maneuver and in 55 per cent of the patients using intermittent clean catheterization. The use of antibiotic bladder irrigations reduced this incidence to 5 per cent. All patients with renal deterioration except one responded to intermittent clean catheterization; this patient was treated with cutaneous vesicostomy. Urodynamic assessment was performed in 15 patients, and this diagnostic tool assisted in the therapeutic management of these children.
10 children with Dandy-Walker syndrome are presented to discuss their clinical assessment and therapy. The presence on physical examination of a large posterior fossa was the most reliable clinical finding and was diagnostic in 9 out of the 10 patients. The different diagnostic confirmatory studies are discussed. The most satisfactory treatment in this series was the combination of a shunt from the lateral ventricle and the posterior fossa to the peritoneum.
The various stages and therapeutic modalities in the management of children with moderate and severe head injuries is reviewed. Emphasis on aggressive management upon arrival to the emergency room, and continuing until brain swelling has definitely resolved, is stressed. Management includes prompt endotracheal intubation and hyperventilation, arterial and venous lines and bladder catheter insertion. Computed tomography to assess underlying pathology, intracranial pressure monitoring for appropriate therapy should follow. In this way mannitol, other diuretics, hypothermia and barbiturate coma can be decided on, and the response assessed. The role of high dose steroids is discussed.
The mechanical activity of the anal sphincter can be translated into electrical activity and recorded on graph paper or an oscilloscope. The activity of the anal sphincter may be extrapolated to activity of the external urethral sphincter because both are striated muscles innervated by the pudendal nerve that arises from S-2, S-3, and S-4. Stimulation of these nerves causes contraction of the sphincter muscles, and a deflection of the recording device occurs. This technique was employed intraoperatively in monitoring operations on the conus medullaris and sacral nerve roots in 10 patients with spinal dysraphism (age range, 3 weeks to 15 years). Their diagnoses were tethered conus, 4; lipomeningocele, 3; spinal hamartoma, 1; syringocele, 1; and sacral arachnoiditis, 1. With general anesthesia, and the patient in the prone position, an electrode-containing anal plug was inserted or two needle electrodes were inserted into the anal sphincter muscle. The electrodes were connected to the electromyography recording stylus of the urodynamic bladder diagnostic unit. During the spinal operation, whenever a structure could not be identified clearly, it was stimulated with the disposable electrical stimulator and, if oscillations of the stylus occurred (indicating contraction of the anal sphincter), the structure was preserved. This technique permitted spinal operations in these 10 patients without changes in neurological or urological function.
Experimental vasogenic cerebral edema was created in rabbits with a cold-induced left occipital cortical lesions. Intracranial pressure (ICP), intracranial elastance (Em), water content, hemispheric brain tissue volume, electrolytes, electroencephalograms, behavior, and gross pathology were studied. Various therapeutic modalities were employed alone or in combination to reduce ICP acutely: acetazolamide, furosemide, mannitol, pentobarbital, lorazepam, and dexamethasone. All therapies except dexamethasone were effective in reducing ICP. Peak ICP reduction occurred at 27 +/- 9.8 (SD) minutes with mannitol and at 71.4 +/- 15.5 minutes with acetazolamide, with the remaining agents and combinations falling between these two extreme values. Em improved by 31.7 +/- 17.02% in all therapuetic trials except those employing acetazolamide and lorazepam. With therapy, there was a reduction in the water content of the hemispheres, but the difference from that in the untreated, lesioned animals was not statistically significant. In the lesioned left hemisphere, sodium content was increased by acetazolamide (p less than 0.005), furosemide (p less than 0.025), pentobarbital (p less than 0.05), and the combination of dexamethasone, pentobarbital, and mannitol (p less than 0.005). Significant reduction was noted in the lesioned group for the potassium content of the left hemisphere in the dexamethasone (p less than 0.05), pentobarbital (p less than 0.025), and combination groups containing these agents (p less than 0.005 to 0.025). (Neurosurgery, 5: 656--665, 1979).
Thirteen patients with intracranial lesions were submitted to a twist drill needle biopsy under computerized tomographic (CT) control, with sedation and local anesthesia. (The patients' ages ranged from 12 to 81 years.) The final diagnoses were glioblastoma in 7 patients and 1 case each of anaplastic astrocytoma, low grade astrocytoma, thrombosed arteriovenous malformation, cerebral infarct, 3rd ventricular epidermoid, and degenerative disease of the brain. Definitive diagnosis was obtained in all but 2 patients with this technique. Appropriate therapy was subsequently instituted in 11 patients without further operation. Transiently increased weakness of the previously affected limbs was the only untoward effect (4 patients). Intracranial hematoma after this procedure was seen in 1 patient in this series, as detected by the postprocedure CT scan, but there was no change in the clinical course. All patients were treated with dexamethasone for 24 to 48 hours before and for several days after the procedure to avoid decompensation of intracranial dynamics because of edema. The procedure, including appropriate level CT scans of the lesion area, was performed in approximately 1 hour in all patients. (Neurosurgery, 5: 671--674, 1979).
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The effect of positive end-expired pressure ventilation (PEEP) on mean systemic arterial pressure, mean pulmonary arterial pressure, mean pulmonary wedge pressure, mean intracranial pressure (ICP), and cerebral perfusion pressure (CPP), in a case of neurogenic pulmonary oedema (NPO) is presented. PEEP improved oxygenation, reduced, and finally stopped clinical pulmonary oedema. In this patient PEEP produced severe systemic hypotension. As a consequence of this, CPP was impaired.
Arrested hydrocephalus probably results from improvement of CSF circulation during growth. Although a generally accepted condition, its mode of onset, its presence in a given patient, and time required to establish the absence of progressive hydrocephalus have not been defined. Five patients are presented with ostensibly arrested hydrocephalus due to: aqueductal stenosis, communicating hydrocephalus, and Dandy-Walker malformation. In a period ranging from 4 to 13 years, they presented clinical signs of decompensation with intracranial hypertension. One had never had a shunt procedure. Another two were performing well in school until they suddenly deteriorated. The diagnosis of arrested hydrocephalus requires close follow-up well into adolescence, with periodical neurological and psychomotor evaluations. Sequential observation of the ventricular size with computed tomography (CT) is recommended.
The effect of therapy with methylprednisolone sodium succinate (5.33 mg/kg/day) and dexamethasone sodium phosphate (1 mg/kg/day) on grey matter of rabbits rendered edematous by a metabolic blocker, 6-aminonicotinamide, is presented. Methylprednisolone was observed to significantly reduce the water content of the grey matter (p less than 0.001), whereas dexamethasone had little effect. Both agents, however, were equally effective in reducing intracranial pressure (p less than 0.001) and improving intracranial elastance, when compared to untreated animals. However, in the dexamethasone-treated group, there was improved behavior and EEG findings in 50% of the animals when compared to the untreated controls, and similar improvement was present in less than 15% of the methylprednisolone group. The disparity between the effects on water content, behavior and EEG supports the thesis that in cerebral edema these agents have a metabolic effect out of proportion to their effect on tissue water.
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