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Biomedical subjects

J M Drake

Publications and source records attributed to J M Drake.

At least 73 records · Page 4Linked to original sources

Functional obstruction of an antisiphon device by raised tissue capsule pressure.

The proper function of an antisiphon device (ASD) requires that it be exposed externally to atmospheric pressure and that its mobile membrane be free to move. A 4-year-old boy who had an ASD placed for postshunt subdural hematomas presented with functional obstruction of the ASD-symptomatic ventriculomegaly despite evidence of patency of the shunt system by radionuclide flow study. At surgery, the distal shunt system including the ASD was infused with normal saline before and after surgical exposure of the device. The pressures required to maintain a flow rate of 5 ml/h and 50 ml/h were 27 mm Hg and 30 mm Hg, respectively. After surgical exposure, these pressures fell to 0 mm Hg and 5 mm Hg, respectively, for the same infusion rates. Surgical exposure removes the effects of the tissue capsule, including the overlying skin and a collagenous sheath, restoring the external pressure to atmospheric pressure. Functional obstruction of ASDs occurs because of raised ambient pressure from the tissue capsule acting to depress the mobile membrane of the ASD, increasing its resistance to flow. This case confirms previously reported effects of subcutaneous implantation of ASDs in experimental animals and is the probable explanation for reported functional obstruction of ASDs in other patients.

Astrocytoma↗

Lumboperitoneal shunting: a retrospective study in the pediatric population.

There is a shortage of data concerning the long-term follow-up of patients with lumboperitoneal (LP) shunts, especially in the pediatric population. A retrospective study of 143 patients who underwent LP shunting between 1974 and 1991 was therefore performed. The mean age at the time of shunt insertion was 3.3 years (range, 18 d to 17.8 yr), and the indication for shunting was: hydrocephalus (81%), cerebrospinal fluid fistula (12%), and pseudotumor cerebri (7%). The mean follow-up time was 5.7 years (range, 5 d to 17.5 yr), and during this period, there were five deaths of which one was shunt related (2.5 yr post-shunt insertion). Of the types of LP shunt used during the study period, the T-tube shunt (101 patients) fared significantly better (P = 0.003) than the percutaneous type (42 patients), and the overall survival characteristics for the T-tube shunt approximated those seen for ventriculoperitoneal shunts, with a 50% probability of remaining free of malfunctions for 5 years. A high rate of migration (19%) was partially responsible for the poor performance of the percutaneous-type shunts. By the end of the study, 40 patients (28%) had been converted to ventricular shunts, and this rate of conversion was similar for both shunt types. LP shunts have certain advantages over other forms of cerebrospinal fluid diversion and were successfully used for various clinical conditions during this study.(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗

Primary leptomeningeal melanoma: an unusually aggressive tumor in childhood.

Primary malignant melanoma of the leptomeninges of the central nervous system is a rare and aggressive tumor in children. We report our experience from 1964 to 1990 with this tumor in eight children. The mean age at diagnosis was 4.9 years (range, 1.3 to 13 yr). Five children presented with signs and symptoms of raised intracranial pressure from hydrocephalus secondary to tumoral obliteration of the basal cisterns, but the time from the initial symptomatology to diagnosis was frequently delayed. Three patients in this series had hairy nevi in association with their leptomeningeal melanoma. Cerebrospinal fluid (CSF) analysis typically showed raised opening pressures, decreased glucose, and increased protein concentrations. Malignant melanoma cells were found in the CSF in three patients. Confirmatory radiographic examinations included air encephalography, myelography, and computed tomographic and magnetic resonance scanning. Four patients were treated with lumboperitoneal shunts, and one patient was treated with a ventriculoperitoneal shunt for hydrocephalus. Two patients underwent craniotomies and subtotal excisions of their tumors. In seven patients, a definitive diagnosis of leptomeningeal melanoma was made by pathological examination of tissues sent at surgery or at post mortem. In one case, the diagnosis was established by a detailed cytological analysis of the CSF. Four children died of fulminant disease and tumor spread before treatment could be instituted. The four children who received treatment had a combination of radiation therapy and chemotherapy. One child received intrathecal methotrexate. The two children with the longest survivals (2 and 3 yr, respectively) received cisplatinum and dimethyltriazenoimidazole carboxamide in addition to craniospinal irradiation.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Management and outcome of low-grade astrocytomas of the midline in children: a retrospective review.

Low-grade astrocytomas of the midline of the brain can be difficult to manage because of their location. To evaluate treatment and outcome, we performed a retrospective study of children with midline low-grade astrocytomas admitted to The Hospital for Sick Children between 1976 and 1991. Eighty-eight children with biopsy-proven low-grade astrocytomas were identified. Forty-three tumors occurred in the optic pathways or hypothalamus, 13 in the thalamus, 7 in the pineal region, 14 in the midbrain, and 11 in the medulla. Patient follow-up ranged from 6 months to 15 years, with a mean of 4 years, 9 months. Overall outcome was related to the extent of resection, histological type, and location. Partial resections were often associated with involution of the tumor. Response to radiation was variable, and serious sequelae were observed. Thirty-three patients experienced recurrence, often with a good response to subsequent surgery; however, 12 of these patients died. The probability of survival was calculated to be 96% at 1 year, 91% at 5, and 80% at 10 years. Our study suggests that resection should be considered in all patients, both at presentation and recurrence.

Adolescent↗

Tonsillar herniation: the rule rather than the exception after lumboperitoneal shunting in the pediatric population.

Although the development of tonsillar herniation (acquired Chiari malformation) in association with lumboperitoneal (LP) shunting is well recognized, it has previously been considered rare. In order to ascertain the incidence of this complication after LP shunting, the authors undertook a retrospective study of all patients in whom this form of shunt had been inserted between 1974 and 1991 at The Hospital for Sick Children, Toronto. In the 143 patients, the mean age at insertion was 3.3 years and the indications for shunt placement were hydrocephalus (81%), pseudotumor cerebri (7%), cerebrospinal fluid fistula (6%), and posterior fossa pseudomeningocele (6%). The mean follow-up period was 5.7 years, during which time there was one shunt-related death due to unsuspected tonsillar herniation. Five other patients developed symptomatic tonsillar herniation treated by suboccipital decompression. Review of all computerized tomography (CT) scans not degraded by artifact showed evidence of excess soft tissue at the level of the foramen magnum in 38 (70%) of 54 patients so studied. In order to confirm that this CT finding represented hindbrain herniation, sagittal and axial magnetic resonance (MR) images were obtained for 17 asymptomatic patients and revealed tonsillar herniation (range 2 to 21 mm) in 12 (70.6%). In addition, some of these asymptomatic patients had evidence of uncal herniation and mesencephalic distortion. Similarities and distinctions are drawn between the morphological changes occurring after LP shunting and those seen in association with the Chiari I and II malformations. Although less than 5% of this study population required treatment for tonsillar herniation, the incidence of this complication was high in asymptomatic patients; MR imaging surveillance for patients with LP shunts is therefore recommended.

Adolescent↗

A comparison of the protective effect of dexamethasone to other potential prophylactic agents in a neonatal rat model of cerebral hypoxia-ischemia.

It has recently been reported that pretreatment with a single dose of dexamethasone (0.1 mg/kg) 24 hours before hypoxia in 7-day-old rat pups is protective against an hypoxic-ischemic insult (unilateral carotid artery occlusion followed by 3 hours of hypoxia in 8% O2). The authors now examine whether pretreatment 6 hours before insult is equally effective and compare other agents potentially suitable for prophylaxis in neonatal hypoxia-ischemia, including the calcium antagonists flunarizine (30 mg/kg pretreatment), nimodipine (0.5 mg/kg pretreatment), and the 21-aminosteroid U-74389F (10 mg/kg pre- and posttreatment). For each active agent, there was also a vehicle-treated control group. Comparison of the mean area of ipsilateral infarction on brain coronal sections showed that there was no statistically significant difference between the various control groups (mean area of infarction 66% +/- 4%). Pretreatment with dexamethasone 6 hours prior to hypoxia offered complete protection with no infarction. A beneficial effect was seen following pretreatment with flunarizine (mean area of infarction 33.6% +/- 7.8%), although this degree of damage was still significantly different from that seen with dexamethasone pretreatment. Pretreatment with nimodipine or U-74389F offered no protection (mean area of infarction 77.5% +/- 4% and 59% +/- 10%, respectively). Unlike findings in adult animals and clinical studies, the current studies show that dexamethasone may have a role in the treatment of neonatal hypoxia-ischemia and deserves reappraisal.

Animals↗

Ventriculostomy for treatment of hydrocephalus.

Ventriculostomy for hydrocephalus can either obviate completely or reduce considerably the complications of cerebrospinal fluid (CSF) shunts. Renewed interest in this procedure has followed the development of flexible and steerable neuroendoscopes, high-quality video cameras, endoscopic surgical instruments, including lasers, and methods of providing computer-guided stereotactic assistance. Ventriculostomy is the preferred treatment for patients with congenital aqueduct stenosis that requires CSF diversion. It is less successful in patients with acquired aqueduct stenosis but is still a treatment option. Interventriculostomy for treatment of intracranial cysts or loculated regions of the ventricles also can obviate or reduce the need for CSF shunts.

Humans↗

Prepontine cyst lined by respiratory epithelium with squamous metaplasia: immunohistochemical and ultrastructural study.

An 8.5-year-old female presented with multiple episodes of aseptic meningitis and was found to have a cystic lesion in the prepontine region. Microscopic examination revealed a respiratory-type epithelium with squamous metaplasia. Like its normal analogue in the respiratory tract, the epithelium of these intracranial cysts can undergo squamous metaplasia when chronically irritated. The ultrastructural and immunohistochemical characteristics of the transition from ciliated epithelium to squamous metaplasia are described.

Brain Diseases↗

Detection and management of gangliogliomas in children.

Forty-two children treated for gangliogliomas were reviewed to identify the best methods of detection and management. Thirty-two of the tumors were supratentorial, four infratentorial, and six were in the spinal cord. Twenty-five patients presented with seizures; the mean duration of symptoms was 5 years in contrast to 1 year in non-seizure patients. Of 31 children studied by computed tomography (CT), calcification in the tumor lesion was found in 19. Magnetic resonance imaging (MRI) showed abnormal high-signal intensity on T2 imaging in six of eight patients. All patients underwent surgical resection and were diagnosed pathologically. Twenty-four patients had total resection, and 14 underwent temporal lobectomies including hippocampectomy. The management of this tumor remains surgical resection without the need for any adjuvant therapy.

Adolescent↗

Focal midbrain tumors in children.

The clinical and neuroradiological features of focal midbrain tumors in 12 children are described, and the results of their surgical management are presented. Patients with a focal midbrain tumor usually exhibit either symptoms and signs of raised intracranial pressure caused by an obstructive hydrocephalus (50%) or symptoms and signs caused by pressure on the tegmentum and cerebral peduncles. The lesions are confined to the tectal plate or tegmentum with possible extension upward to the thalamus and downward to the pons, displacing but not invading these structures. The edges of the tumor are well defined, and the large majority have a solid consistency with intense regular enhancement after intravenous contrast. Radical resection is hardly ever feasible in brain stem tumors, but in this series, significant reduction of the tumor mass was obtained in 75% of the patients, with no surgical mortality and minimal surgical morbidity and with the majority of patients showing clinical improvement postoperatively. All tumors were nonpilocytic, low-grade astrocytomas. Six patients received adjunctive radiotherapy. The mean follow-up period is 2.5 years, and all patients are alive and doing well. We conclude that focal midbrain tumors in children appear to be a distinct subgroup of brain stem tumors and are very amenable to surgical resection with an excellent long-term prognosis.

Adolescent↗

Death from chronic tonsillar herniation in a patient with lumboperitoneal shunt and Crouzon's disease.

A 2.5-year-old girl with Crouzon's disease, hydrocephalus, and a lumboperitoneal shunt died as a result of chronic tonsillar herniation (acquired Chiari 'malformation'). The possible synergistic role of the cranial dysmorphism and the lumboperitoneal shunt in the development of this anomaly is discussed. The literature is reviewed and it is argued that if hydrocephalus occurs in infants in whom cephalocranial disproportion is likely to develop, then it is inadvisable to insert a lumboperitoneal shunt.

Cerebellar Diseases↗

Aggressive surgical management of craniopharyngiomas in children.

The cases of 50 patients with craniopharyngioma operated on at The Hospital for Sick Children in Toronto between January, 1975, and December, 1989, are reviewed. All patients were under 18 years of age (mean 9.39 years). Headaches, endocrine deficiencies, and visual deficits were the most common symptoms on admission. Forty-five patients underwent what was considered by the surgeon to be total excision of their tumor, and five had subtotal excision. Tumors recurred in 17 patients (mean time of recurrence 32.6 months after surgery). One patient died in the postoperative period and three have been lost to follow-up study. Of the remaining 46 patients, 28 are leading a normal or nearly normal life, although all are receiving endocrine replacement and some have required help to overcome mild deficits in memory or visual acuity. Twelve patients are able to function reasonably well and attend school despite being hampered by intellectual or visual deficits or problems with weight control; four have a significant handicap, and two have died.

Actuarial Analysis↗

Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele.

Between January, 1981, and July, 1991, 17 infants under 1 month of age were admitted to The Hospital for Sick Children with the signs and symptoms of a Chiari II malformation. These patients' presentation included swallowing difficulty (71%), stridor (59%), apneic spells (29%), aspiration (12%), weakness of cry (18%), and arm weakness (53%). Decompression of the Chiari II malformation was performed in all patients, with a time interval between onset of symptoms and surgery ranging from 1 to 121 days. Fifteen patients (88%) remain alive, all of whom have shown a complete recovery. The mean follow-up period in this group of patients was 65 months. Two patients died, one due to respiratory arrest 8 months after decompression and the other because of shunt infection and peritonitis 7 years after decompression. These results support the concept that compressive forces, rather than a primary intrinsic disorder of the brain-stem nuclei, play a crucial etiological role in the development of a symptomatic Chiari II malformation. Early recognition of the symptoms of Chiari II malformation should be followed by immediate decompressive laminectomy in order to promote a prompt and full neurological recovery.

Arnold-Chiari Malformation↗

Suprasellar and sellar tumors in childhood and adolescence.

Suprasellar and sellar region tumors in children constitute a diverse group of lesions. We advocate an aggressive surgical approach to these tumors for diagnostic and therapeutic purposes. With detailed knowledge of the microneurosurgical anatomy of this region, the neurosurgeon can choose from a number of operative approaches on the basis of tumor size and location. The benefits of radiation therapy for craniopharyngiomas and optic chiasmatic/hypothalamic tumors must be carefully weighted against the substantial long-term risks associated with irradiation of the developing brain in this region. During the next decade, the role of primary chemotherapy for germinoma and adjuvant chemotherapy for optic pathway gliomas will be determined. Also, the role of stereotactic radiation therapy, of great value to some children with recalcitrant tumors in the suprasellar region, may be better defined.

Adolescent↗

Arteriovenous malformations of the brain in children: a forty year experience.

Despite the great capacity for the pediatric brain to recover from stroke, the morbidity and mortality in children who harbor an arteriovenous malformation (AVM) remains high. This study examines the clinical data and management experience with 132 patients with brain AVM from 1949 to 1989. Although the high tendency for a childhood AVM to present with hemorrhage (79%) remained constant for the forty year study period, the associated morbidity and mortality of hemorrhage changed. The mortality rate from hemorrhage for the entire series was 25%, which was reduced from 39% to 16% after the introduction of computed tomography. The mortality from AVM hemorrhage since 1975 was dependent on location; 8 of 14 patients (57%) with a cerebellar AVM died from hemorrhage while only 2 of 44 patients (4.5%) with a cerebral hemisphere AVM died (p less than 0.0001). Sixteen children (12%) presented with a chronic seizure disorder. Surgical excision of the malformation resulted in complete seizure control off anti-convulsant medication in 73% of patients. Although 21% of patients were treated non-operatively (many with terminal poor-grade hemorrhage), 79% had a surgical procedure with total AVM excision achieved in 70 patients (53.1%). Complete AVM resection was followed by a normal neurological outcome in 47 children (67%). Most partial excisions (n = 9) and clipping of feeding arteries (n = 7) were performed in the early years of this study, and did not provide protection from rehemorrhage. Although conservative management has been advocated for selected non-hemorrhagic AVMs, we conclude that essentially all children with an AVM should be treated in order to eliminate the risk of hemorrhage.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebral Hemorrhage↗

Chemical and biological microstructures as probed by dynamic processes.

The dynamic process of electronic energy transfer is shown to be an important tool for probing the microstructure of molecular systems, particularly those in which donors and acceptors occupy specifically labeled sites of spatially confining host matrices. Special attention is given to analyzing the temporal behavior of the direct energy transfer reaction for systems in which the dipolar coupling is between a donor and randomly distributed acceptors. This dynamic process is dependent on two competing lengths when the donor and acceptor distribution is determined by the microstructure of the confining system: Rp, the dominant length characterizing the size of the confinement, and R0, which scales the strength of the dipolar coupling. When energy transfer processes are viewed in the context of these two competing lengths, a picture emerges of the microstructure of the confinement that is consistent with and corroborated by other structural probes.

Energy Transfer↗