Arteriovenous malformation and moyamoya disease.
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Arteriovenous Malformations↗
Biomedical subjects
R M Scott
Publications and source records attributed to R M Scott.
The use of clinical practice guidelines (CPGs) to evaluate practice and control costs in ventriculoperitoneal shunt management.
BACKGROUND: As a step toward maximizing the quality and cost-effectiveness of neurosurgical care, we designed clinical practice guidelines (CPGs) for the management of VP shunt malfunctions and infections at a tertiary care pediatric teaching institution. The detailed CPGs determine the use of radiographic studies, laboratory tests, and invasive procedures in the management of this problem. One purpose of the CPGs is to provide clear clinical guidelines for the medical trainee, thereby reducing variability in care and unnecessary utilization of resources. METHODS: The CPGs were developed in stages over a 2-year period. The practice patterns in our institution for the management of shunt malfunctions and infections were articulated. They were compared with those published in the neurosurgical literature, and areas of clinical decision-making variability were identified. Preliminary guidelines were formulated, and data regarding patient care were prospectively collected. Based on this data, final CPGs were formulated and implemented. Total and itemized hospital charges for patients managed according to the CPGs were compared with those for patients in the 3 years before CPG implementation. RESULTS: CPG-managed patients had generally lower total and itemized charges as compared with control patients. Decreased charges per hospital day and charges for shunt films in the CPG group were statistically significant. CONCLUSIONS: The process by which the CPGs were developed and implemented, as well as the CPGs themselves, are described. We also present the clinical, demographic, and financial data that were prospectively collected for all patients managed within the CPGs over an initial 1-year period and compare it with data obtained for control groups of shunt malfunction patients admitted during the 3 years before implementation of the CPGs. We find a trend toward reduction of charges after implementation of the CPG.
Boston↗
Synostotic frontal plagiocephaly: anthropometric comparison of three techniques for surgical correction.
Surgical correction of synostotic frontal plagiocephaly ("unilateral coronal synostosis") focuses on distortions of the forehead and orbits. Technical variations include unilateral versus bilateral fronto-orbital positioning. Surgical alignment of the deviated nasal root was introduced in our unit. Anthropometry was used to assess anatomic outcome, and results were compared in 22 children with synostotic frontal plagiocephaly who had either (1) unilateral fronto-orbital advancement ("canthal advancement") (n = 8), (2) bilateral fronto-orbital advancement/ modeling without nasal straightening (n = 7), or (3) bilateral fronto-orbital advancement/modeling with closing wedge nasal osteotomy (n = 7). Postoperative fronto-orbital asymmetry was most marked in the group I patients wherein the ipsilateral supraorbital rim was retruded 3.9 mm and elevated 2.6 mm, on average relative to the corneal apex, compared with the normal side. Group II children averaged 2-mm orbital retrusion and 2.2-mm elevation. Group III patients averaged 1.4-mm orbital retrusion and 2.9-mm elevation. These differences in orbital rim measurements among the three groups were not statistically significant. Postoperative nasal root angulation of 4 degrees or more was found in more than 50 percent of children who had either a unilateral or a bilateral procedure, without nasal correction. In contrast, primary nasal osteotomy resulted in a nasal cant of 3 degrees or less in all children. This difference in nasal angulation among the three groups was statistically significant (p = 0.035). Group III had a straighter nasal angle than groups II and I (in that order). Measurement of the distances from nasion to inner and to outer canthi also reflected persistent deviation of the nasal root. Group III children had a more central radix than either group I or II (p = 0.05). The data in this study support an operative strategy of bilateral (parallelogrammic) positioning of the forehead/ superior orbits with primary correction of nasal root angulation.
Cephalometry↗
Rectal diclofenac analgesia after abdominal hysterectomy.
Sixty-two patients undergoing total abdominal hysterectomy with or without salpingo-oophorectomy were randomly allocated to received either 100 mg rectally of diclofenac (n = 31) or a placebo (n = 31) at the end of the operation and 12-hourly for 72 hours postoperatively. The analgesic effect of the diclofenac was measured by a visual analogue scale pain score and morphine usage. Patients receiving diclofenac showed a morphine requirement of < 50% of the patients receiving placebo (p < 0.0001). There was no statistical evidence of any reduction in the incidence of nausea and vomiting with the use of diclofenac.
Analgesia↗
Spontaneous rapid resolution of an epidural hematoma associated with an overlying skull fracture and subgaleal hematoma in a 17-month-old child.
Acute traumatic epidural hematomas (EDH) constitute one of the most critical emergencies in neurosurgical management. The rapid spontaneous resolution (<24 h) of EDH is an extremely rare phenomenon. A 17-month-old patient fell from a height of 1.5 m and presented with a 8-mm temporal EDH, an overlying linear skull fracture, and a subgaleal hematoma without evidence of intraparenchymal injury or edema. The patient complained only of mild headache, harbored no neurological deficits, and was, therefore, managed conservatively in the intensive care unit with provision to proceed to surgical decompression in the event of neurological change. A repeat CT study 18 h later revealed near-complete resolution of the EDH with a coincident increase in the volume and spread of the subgaleal hematoma. This is the fourth reported case of rapid spontaneously resolving EDH and the youngest one to date. All 4 cases have coincided with an overlying linear skull fracture. We propose that unlike classical EDH, rapidly resolving EDH in the absence of elevated intracranial pressure (ICP) originates from elevated interstitial pressure in the subgaleal compartment transiently decompressing into the epidural space through a skull fracture and resolving as the pressure in the subgaleal compartment decreases below ICP.
Compartment Syndromes↗
Presentation and management of Chiari I malformation in children.
To determine the efficacy of operative treatment for children with Chiari I malformation, the medical records and magnetic resonance imaging (MRI) studies of 68 consecutive patients cared for at The Children's Hospital, Boston, Mass., USA, from December, 1988 to November, 1996 were retrospectively reviewed. All patients underwent suboccipital craniectomy, C1 laminectomy, and dural grafting. Bipolar coagulation to shrink and reduce the volume of the cerebellar tonsils was carried out in 40 patients. In 32 of 40 patients with associated syringomyelia, the procedure included placement of a IVth ventricle to cervical subarachnoid space shunt. Twenty-three patients with syringomyelia also had plugging of the obex. There was no operative mortality. Morbidity included a 22% incidence of nausea/vomiting and a 10% incidence of headache, both limited to the immediate postoperative period. Within the first postoperative month, all patients or their parents reported clear improvement in their presenting symptoms and 93% were found to have clear improvement in their presenting signs. In follow-up periods of 6-70 months, all patients had continued unequivocal symptom improvement and all patients were found on examination to have clear improvement in neurological signs. In patients with syringomyelia, MRI studies carried out at least 6 months postoperatively revealed near total or total syrinx resolution in 80% of the cases. This study demonstrates that a standard bony and dural decompression of the foramen magnum region with modifications designed to maximize the restoration of CSF circulation across the foramen magnum is a safe, effective operative treatment for Chiari I malformation in children.
Adolescent↗
A 14-year-old male with basal ganglia tumor.
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Adolescent↗
Optic chiasm astrocytomas of childhood. 1. Long-term follow-up.
We reviewed 61 patients seen from 1975 to 1994 with a biopsy-confirmed optic chiasm astrocytoma or a characteristic clinical and imaging presentation. The mean age at diagnosis was 72 months, including 30 who were less than 5 years old. The mean tumor diameter at presentation was 31 mm among 54 tumors measured. Tumors involved the optic nerve in 41, hypothalamus in 38, optic tract in 15, and optic radiations in 2. Four patients, all with neurofibromatosis, received no treatment. Forty patients received conventional radiation therapy at a mean age of 89 months. Chemotherapy was given to 19 children and 7 of these were followed for greater than 1 year. Six of these 7 had progressive disease which required subsequent surgery and/or radiation therapy. Thirty-six patients underwent surgery. We restricted our analysis of outcome to those 38 patients followed for longer than 60 months. Development was normal in 13%, mildly disabled in 53%, severely disabled in 21% and dead in 13%. Children were severely disabled or dead in 47% if younger than 5 years at diagnosis, and 21% if older. There was no endocrine dysfunction in 18% and new endocrine failure was seen in 61%. More posterior tumors did not portend a poorer prognosis. Children less than 5 years old had a poorer prognosis. Neurofibromatosis did not have a marked effect on outcome. Intracranial hypertension was an indicator of a poor prognosis. No specific treatment modality was clearly superior in terms of long-term survival or improvement in quality of life.
Adolescent↗
Optic chiasm astrocytomas of childhood. 2. Surgical management.
We presented our experience from 1975 to 1994 with emphasis on the long term follow-up of children with optic chiasm astrocytomas in part I. In part II we describe the surgical management of 20 patients with the diagnosis of an astrocytoma of the optic chiasm confirmed by biopsy. These patients underwent surgery by the senior author between 1988 and 1994. The patients and the tumors in this series are comparable in age, sex, and presentation and imaging characteristics to other series. Surgery was undertaken with the intent to reduce tumor volume and mass effect and in certain cases to confirm the diagnosis. The patients' mean age at the time of surgery was 81 (range 18-216) months. The surgical approaches used were pterional in 11 patients, transorbital/subfrontal in 8, and subfrontal in 1 patient. Greater than 50% of the tumor was resected in 8 patients, less than 50% was resected in 9, and biopsy only was achieved in 3 patients. Larger tumors were more likely to have magnetic resonance imaging documented debulking of tumor. Permanent morbidity included mild visual loss, diabetes insipidus, mild hemiparesis, and panhypopituitarism in 1 patient each. There was no surgical mortality. The likelihood of resecting greater than 50% of the tumor was higher in those with a diameter greater than 25 mm. The indications for craniotomy are (1) to debulk symptomatic tumors greater than 50 mm in diameter which are exophytic or cystic; (2) to relieve obstruction at the foramen of Monro, and (3) for diagnostic biopsy.
Astrocytoma↗
Elevation of cerebrospinal fluid levels of basic fibroblast growth factor in moyamoya and central nervous system disorders.
Moyamoya syndrome is a vaso-occlusive disease involving the intracranial vessels of the circle of Willis which is accompanied by an intense compensatory recruitment of new vessels. Angiogenic substances such as basic fibroblast growth factor (bFGF) present in the cerebrospinal fluid (CSF) have been proposed as possible mediators of the neovascular response. We analyzed CSF samples collected intraoperatively from predominantly pediatric patients with moyamoya and other conditions such as Chiari malformation (Ch), tethered cord (TC), arteriovenous malformation (AVM), brain tumor (BT) and hydrocephalus (HCP). We found that CSF bFGF was significantly elevated in patients with moyamoya (141 pg/ml, n = 37), Ch (56.7 pg/ml, n = 22), TC (55.1 pg/ml, n = 23), AVM (354 pg/ml, n = 5), and BT (208 pg/ml, n = 5) compared to patients with HCP (5.5 pg/ml, n = 7) and controls (1.6 pg/ml, n = 25; p < 0.05). There was no dependence of CSF bFGF on patient age or gender. Although CSF bFGF in the moyamoya group showed no correlation with the Suzuki radiographic stage at either pre- or post-operative (1-year follow-up) angiography, it showed a trend with the Matsushima angiographic score with increasing collateral vascularization from the synangiosis developing at higher levels of CSF bFGF. Our findings suggest that CSF bFGF may be playing a wide-ranging role in a number of central nervous system conditions associated with ischemia and hypervascularity. Although not a specific marker for moyamoya, elevated CSF bFGF may serve as a weak predictor of the extent of angiogenesis to be expected in indirect revascularization procedures.
Adolescent↗
Postoperative cerebral edema occurring in children with slit ventricles.
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Brain Edema↗
Short report: an outbreak of Japanese encephalitis in Kathmandu, Nepal.
We report the first proven outbreak of Japanese encephalitis (JE) occurring in the Kathmandu Valley of Nepal. During September and October 1995, we treated 15 patients with meningo-encephalitis. All of the patients were Nepalese, all but one lived in the Kathmandu Valley, and their overall mortality was 53%. Anti-JE virus (JEV) IgM in the cerebrospinal fluid was found in the two cases for whom it was tested. The two tested patients were similar to the other patients in clinical presentation and in home location. We recommend immunization against JEV for those traveling to Kathmandu during the months of August to October.
Aged↗
Late effects of early surgery on lipoma and lipomeningocele in children less than 1 year old.
PURPOSE: We attempted to assess the usefulness of urodynamic testing for diagnosing lipoma and lipomeningocele, and the late effects of early neurosurgical intervention in children less than 1 year old. MATERIALS AND METHODS: A total of 28 patients in whom spinal surgery was performed before age 1 year for correcting lipoma and lipomeningocele underwent neurological and urodynamic evaluation preoperatively, and within 3 months and up to 14.5 years postoperatively. RESULTS: Preoperatively 15 patients (54%) had an abnormal neurological examination and 7 had an abnormal urodynamic study. Of the 13 patients with a normal neurological examination 3 had an abnormal urodynamic study. Overall 10 patients (37%) had an abnormal urodynamic examination preoperatively. Immediately after surgery 8 patients (29%) had improvement (2 of 15 with an abnormal neurological examination and 6 of 10 with an abnormal urodynamic study), whereas the condition of 9 (32%) worsened, including 4 of the 13 who were neurologically and 6 of the 18 who were urodynamically normal (1 in both categories). With time an additional 7 patients (25%) had progressive deterioration, including 6 urodynamically and 5 neurologically (4 in both categories), 2 (7%) had improvement (1 each urodynamically and neurologically) and the condition of 19 (68%) remained stable. Six patients (21%) underwent secondary spinal surgery for recurrent tethering. CONCLUSIONS: Early surgical repair seems to provide a degree of protection against later spinal cord tethering, and subsequent neurological and/or urodynamic deterioration (25% of our patients versus a reported 80% of those followed expectantly).
Female↗
Angiographic changes after pial synangiosis in childhood moyamoya disease.
PURPOSE: To describe the angiographic changes accompanying the surgical treatment of moyamoya disease by pial synangiosis and to compare these changes with patient outcome. METHODS: The preoperative and postoperative cerebral angiograms, MR images, and clinical records of 13 children treated with pial synangiosis for moyamoya disease were reviewed. RESULTS: After synangiosis, 10 patients had significant neurologic improvement and three had minimal or no improvement. Postoperative MR images showed no new infarctions. Well-developed (grade A or B) transpial or transdural collaterals to the brain were present at the site of synangiosis in 84% of the surgically treated hemispheres. Cerebrovascular occlusive changes increased postoperatively in 76% of hemispheres. After synangiosis, moyamoya collaterals were increased in 48%, unchanged in 16%, and decreased in 36% of surgically treated hemispheres. All 10 patients with grade A or B collaterals bilaterally after synangiosis were asymptomatic or improved on follow-up. CONCLUSION: Pial synangiosis typically results in an increase in collaterals from the superficial temporal artery or middle meningeal artery to the brain. Synangiosis appears to result in stabilization or improvement in neurologic symptoms but does not prevent the angiographic progression of disease or the development of moyamoya collaterals. The angiographic demonstration of well-formed collaterals after synangiosis is associated with a favorable clinical outcome.
Adolescent↗
Magnetic resonance imaging changes after stereotactic radiation therapy for childhood low grade astrocytoma.
BACKGROUND: Stereotactic radiotherapy (SRT) is fractionated radiotherapy delivered under stereotactic guidance to produce highly focal and precise therapy. We studied the incidence of imaging changes that can mimic tumor progression after completion of SRT for childhood low grade astrocytoma. METHODS: Between June 1992 and September 1994, we prospectively treated 28 children with low grade astrocytomas with SRT. The patients ranged in age from 2 to 22 years (median: 10 yrs) and none had received prior radiation therapy or radiosurgery. Routine fractionation was employed (180-200 centigray[cGy]) to a total dose of 5220-6000 cGy over 5 to 6 weeks. All of the patients underwent initial and follow-up magnetic resonance imaging (MRI) according to protocol. RESULTS: Median clinical follow-up for the 28 patients was 24 months (range, 5-32 mos) with a median radiographic follow-up of 15 months (range, 3-26 mos). Fifteen patients had reduction in tumor size, one patient had stable disease. Twelve patients (43%) developed increased size of the lesion, increased signal intensity or enhancement, cysts or cavitations, and an increase in edema or mass effect on follow-up MRI. Most of these changes occurred between 9 and 12 months after the start of SRT and resolved or decreased by 15 to 21 months. All but one patient had normal or stable neurologic examinations. CONCLUSIONS: Treatment-related MRI changes are common after conventionally fractionated schedules using stereotactic radiation techniques for patients with low grade astrocytomas. These changes can be distinguished from tumor progression by their transient nature as well as the general absence of clinical symptoms.
Adolescent↗
The critical importance of stimulus intensity in intraoperative monitoring for partial dorsal rhizotomy.
During partial lumbosacral dorsal rhizotomy (PDR), intraoperative dorsal rootlet stimulation (drs) evokes motor responses, presumed to be reflexes, which are used to select rootlets for section. However, dr stimuli may also costimulate ventral root (vr) and evoke an M rather than a reflex response, the two being distinguishable only by comparison of response latencies after drs at two separate sites. In 15 consecutive spastic cerebral palsy patients undergoing PDR, we asked whether reflex and M responses were distinguishable on the basis of stimulus intensity (SI). For soleus H reflexes evoked by percutaneous tibial nerve stimulation, the SI for reflex afferents was usually subthreshold for exciting motor fibers. Similarly, for nerve roots, reflexes were evoked by drs at SIs generally less than that for M responses evoked by vr stimulation (vrs). In contrast, M responses evoked by drs required SIs that were on average 20 times greater. Finally, costimulation of contralateral vr after ipsilateral vrs occurred at SIs shown to evoke M responses after drs. We conclude that: (1) reflex and M responses evoked by drs are distinguishable on the basis of the required SI; and (2) drs employing SIs greater than required for vrs evokes M rather than reflex responses due to costimulation of ipsilateral and contralateral vr.
Cerebral Palsy↗
How would you treat this patient? Dysembryoplastic neuroepithelial tumor.
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Brain Neoplasms↗
Giant cell ependymoma of the filum terminale. A report of two cases.
We describe two histologically unusual cases of ependymoma of the filum terminale. Both tumors occurred in 14-year-old boys. An intradural encapsulated mass attached to the filum terminale was demonstrated radiologically in both cases and totally resected at surgery. In case 1 the neoplasm was uniformly composed of pleomorphic giant cells and was without perivascular pseudorosettes or myxopapillary changes. Case 2 was a myxopapillary ependymoma with multiple foci of pleomorphic giant cells. Neither tumor had prominent mitotic activity, necrosis, or endothelial proliferation. Both tumors were immunopositive for cytokeratin and glial fibrillary acidic protein. Ultrastructural features included basal laminae, interdigitating cell processes, microvilli, cilia, intercellular junctions, and cytoplasmic intermediate filaments. Cytogenetic analysis in case 1 showed a hypodiploid karyotype with monosomy of chromosomes 1, 10, 14, 16, 20, and 22. We interpret both tumors as most consistent with a variant of ependymoma. Because of the unique gigantocellular light microscopic appearance of the entire tumor in case 1, we propose classifying this tumor as a new morphologic subtype: giant cell ependymoma of the filum terminale. The combination of gigantocellular and myxopapillary features in case 2 supports a histogenetic relationship between giant cell ependymoma and myxopapillary ependymoma.
Adolescent↗