Moyamoya disease, a known cause of pediatric stroke, is an unremitting cerebrovascular occlusive disorder of unknown etiology that can lead to devastating, permanent neurological disability if left untreated. It is characterized by progressive stenosis of the intracranial internal carotid arteries and their distal branches and the nearly simultaneous appearance of basal arterial collateral vessels that vascularize hypoperfused brain distal to the occluded vessels. Moyamoya disease may be idiopathic or may occur in association with other syndromes. Most children with moyamoya disease present with recurrent transient ischemic attacks or strokes. Although there is no definitive medical treatment, numerous direct and indirect revascularization procedures have been used to improve the compromised cerebral circulation, with outcomes varying according to procedure type. Such techniques improve the long-term outcome of patients with both idiopathic and syndrome-associated moyamoya disease. This review provides a comprehensive discussion of moyamoya disease in children, with an emphasis on the most effective surgical treatment options.
Search Results
菲利普S. Smucker and Jodi L. Smith
✓ The authors present the case of a 2.5-year-old African-American boy with desmoplastic medulloblastoma (MB) and nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, an autosomal dominant disorder resulting from mutations in thepatched (PTCH)gene that predisposes to neoplasias (including basal cell carcinomas [BCCs] and MB) and to widespread congenital malformations. The diagnosis of NBCCS was suspected based on the clinical examination, patient and family medical histories, and histopathological characteristics of the tumor. Radio-therapy was withheld. The diagnosis of NBCCS was confirmed by DNA testing, which revealed a novel mutation in thePTCHgene. This is the first report of an African-American child with MB diagnosed with NBCCS prior to radio-therapy.
Although only a small number of patients with MB have NBCCS, the diagnosis must be considered because radio-therapy in such patients can lead to the formation of BCCs and other intracranial neoplasms within the irradiated field. This case emphasizes the importance of obtaining thorough family and patient medical histories and of carefully examining the patient and close relatives for signs of NBCCS to avoid the potentially devastating consequences of missing this diagnosis.
Daniel H. Fulkerson and Jodi L. Smith
Daniel H. Fulkerson and Jodi L. Smith
Management of cervical spine injuries in young children: lessons learned
Report of 2 cases
Jodi L. Smith and Laurie L. Ackerman
以前的圣udies have shown that the correct use of car safety seats can protect infants and children from vehicular injury. Although child passenger devices are increasingly used in the US, motor vehicle crashes continue to be the leading cause of death and acquired disability in infants and children younger than 14 years of age. These events are likely related, at least in part, to the high percentage of children who are unrestrained or improperly restrained. The authors present 2 cases of severe cervical spine trauma in young children restrained in car safety seats during a motor vehicle crash: 1) a previously healthy 14-month-old girl who was improperly restrained in a forward-facing booster seat secured to the vehicle by a lap belt, and 2) a previously healthy 30-month-old girl who was a rear seat passenger restrained in a car safety seat. This study points out the unique challenges encountered in treating cervical spine injuries in infants and young children, as well as the lessons learned, and emphasizes the significance of continuing efforts to increase family and public awareness regarding the importance of appropriate child safety seat selection and use.
Andrea G. Scherer, Ian K. White, Kashif A. Shaikh, Jodi L. Smith, Laurie L. Ackerman, and Daniel H. Fulkerson
OBJECTIVE
The risk of venous thromboembolism (VTE) from deep venous thrombosis (DVT) is significant in neurosurgical patients. VTE is considered a leading cause of preventable hospital deaths and preventing DVT is a closely monitored quality metric, often tied to accreditation, hospital ratings, and reimbursement. Adult protocols include prophylaxis with anticoagulant medications. Children’s hospitals may adopt adult protocols, although the incidence of DVT and the risk or efficacy of treatment is not well defined. The incidence of DVT in children is likely less than in adults, although there is very little prospectively collected information. Most consider the risk of DVT to be extremely low in children 12 years of age or younger. However, this consideration is based on tradition and retrospective reviews of trauma databases. In this study, the authors prospectively evaluated pediatric patients undergoing a variety of elective neurosurgical procedures and performed Doppler ultrasound studies before and after surgery.
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A total of 100 patients were prospectively enrolled in this study. All of the patients were between the ages of 1 month and 12 years and were undergoing elective neurosurgical procedures. The 91 patients who completed the protocol received a bilateral lower-extremity Doppler ultrasound examination within 48 hours prior to surgery. Patients did not receive either medical or mechanical DVT prophylaxis during or after surgery. The ultrasound examination was repeated within 72 hours after surgery. An independent, board-certified radiologist evaluated all sonograms. We prospectively collected data, including potential risk factors, details of surgery, and details of the clinical course. All patients were followed clinically for at least 1 year.
RESULTS
There was no clinical or ultrasound evidence of DVT or VTE in any of the 91 patients. There was no clinical evidence of VTE in the 9 patients who did not complete the protocol.
CONCLUSIONS
In this prospective study, no DVTs were found in 91 patients evaluated by ultrasound and 9 patients followed clinically. While the study is underpowered to give a definitive incidence, the data suggest that the risk of DVT and VTE is very low in children undergoing elective neurosurgical procedures. Prophylactic protocols designed for adults may not apply to pediatric patients.
Clinical trial registration no.: NCT02037607 (clinicaltrials.gov)
Laurie L. Ackerman, Daniel H. Fulkerson, Andrew Jea, and Jodi L. Smith
OBJECTIVE
Patients with shunts often interact with providers distant from their primary hospital, making it important that the parent(s)/guardian(s) is well versed in the type of shunt implanted and symptoms of malfunction/infection. This is particularly important with magnetic-sensitive programmable valves, as the use of MRI becomes more prevalent.
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Over a 6-month period, primary caregivers of 148 consecutive patients who received shunts were prospectively administered questionnaires at clinic visits. Caregivers were asked to do the following: 1) identify shunt valve name, type, and setting if applicable; 2) list symptoms of shunt malfunction/infection; and 3) indicate whether they had access to references regarding shunt type/setting, booklets from the Hydrocephalus Association, and quick reference cards with symptoms of shunt malfunction/infection. One cohort of caregivers (n = 75) was asked to carry informational cards with shunt valve/setting information (group I); this cohort was compared with another subgroup of caregivers (n = 73) not carrying cards (group II).
RESULTS
(±SD)的平均年龄的病人在植入/ revision was 3.71 ± 4.91 years, and the age at follow-up was 6.12 ± 5.4 years. The average time from surgery to administration of the questionnaire was 2.38 ± 3.22 years. There were 86 new shunt insertions and 62 revisions. One hundred twenty-eight caregivers (87%) could identify the type of valve (programmable vs nonprogrammable). On the other hand, only 72 caregivers (49%) could identify the valve name. Fifty-four of 73 (74%) caregivers of patients who had shunts with programmable valves could correctly identify the valve setting. One hundred caregivers (68%) had a copy of the Hydrocephalus Association booklet, and 103 (70%) had quick reference cards. Eighty caregivers (54%) had references on shunt type/setting. Most caregivers (127 [86%]) could name ≥ 3 signs/symptoms of shunt malfunction, with vomiting (61%), headache (49%), and sleeps more/lethargic (35%) most frequently reported. Caregivers of patients in group I were more likely to have cards with symptoms of shunt infection or malfunction (p = 0.015); have information cards regarding shunt type/setting (p < 0.001); and correctly identify valve type (p = 0.001), name (p < 0.001), and setting if programmable (p = 0.0016). There were no differences in ability to list symptoms of shunt malfunction or infection (p = 0.8812) or in access to Hydrocephalus Association booklets (p = 0.1288). There were no significant demographic differences between the groups, except that group I patients had a shorter time from surgery to last follow-up (1.66 vs 3.17 years; p = 0.0001).
CONCLUSIONS
Education regarding the care of patients with shunts by providing written cards with shunt type/setting and access to reference materials seems to be effective. Developing plans for guided instruction with assessment in the clinic setting of a caregiver’s knowledge is important for patient safety.
Jodi L. Smith, Jon Hobbs, Aonan Tang, David Jackson, Wei Chen, Hema Patel, Anita Prieto, Alexander Sher, Alan Litke, and John M. Beggs
Introduction
Epileptogenicity of neuronal tissues requires both altered excitability and altered synchronization of neurons. However, the network-level mechanisms responsible for neuronal hyperexcitability and synchronization remain unknown, and there is much to learn regarding how even small networks of neurons interact. The present study examines local and network properties of cortical neurons from epileptogenic human and excited (“epileptic”) rat cortex.
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Epileptogenic cortex was harvested from pediatric patients with medically refractory seizures undergoing resective surgery. Local field potential signals (LFPs) were recorded continuously for up to several hours with a 60-channel microelectrode array. We also recorded LFPs from slices and organotypic and dissociated cultures of rat cortex bathed in high K+ and low Mg++. We then compared the human and rat data, applied a second-order maximum entropy model (MEM) to the data, and explored how well the MEM predicted sequences of correlated states over time.
Results
Both human and rat cortex produced LFP signals in the form of interictal spikes on almost all electrodes. However, only human cortex demonstrated spontaneous activity in normal cerebrospinal fluid, and the LFPs from human cortex showed greater synchrony across electrodes than the rat LFPs. Moreover, when a second-order MEM was applied to human and rat data, the model accounted for roughly 88% of network correlations. However, in 8/13 preparations the observed sequences of correlated states were significantly longer than predicted by independently concatenating states from the model, suggesting that temporal dependencies are a common feature of cortical network activity.
Conclusion
片大鼠皮层的兴奋无法捕获一些important features of network activity found in epileptogenic human cortex. Furthermore, a second-order MEM successfully predicts correlated states in cortical networks, but not their evolution over time. Thus, higher-order MEMs are necessary to account for temporal correlations observed between states.
Megan M. Jack, Brandon W. Smith, Stepan Capek, Tomas Marek, Jodi M. Carter, Stephen M. Broski, Kimberly K. Amrami, and Robert J. Spinner
OBJECTIVE
Perineural spread of breast cancer to the brachial plexus can lead to pain, sensory alterations, and upper-extremity weakness. Although rare, perineural spread is an often-misdiagnosed long-term complication following breast cancer diagnosis. The objective of this study was to critically review the clinical, radiological, and pathological findings of biopsy-proven perineural spread of breast cancer to the brachial plexus.
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This is a retrospective study from a single institution in which a total of 19 patients with brachial plexus involvement from perineural spread of breast cancer who underwent fascicular biopsy between 1999 and 2021 were identified. Clinical, radiographic, and pathological data were retrospectively collected. Descriptive statistics were calculated for the cohort.
RESULTS
The mean age of patients at the time of diagnosis of breast cancer perineural spread was 60.6 ± 11.5 years. The diagnosis of brachial plexopathy due to perineural spread was on average 12 years after the primary diagnosis of breast cancer. There was also a delay in diagnosis due to the rarity of this disease, with a mean time from initial symptom onset to diagnosis of perineural spread of 25 ± 30 months. All patients at the time of presentation had upper-extremity weakness and pain. Nearly all patients demonstrated T2 signal change and nodular so-called sugar-coating contrast enhancement on brachial plexus MRI. Similarly, all patients who underwent PET/MRI or PET/CT had increased FDG uptake in the involved brachial plexus. Breast cancer perineural spread has an overall poor prognosis, with 16 of 19 patients dying within 5.9 ± 3.0 years after diagnosis of perineural spread.
CONCLUSIONS
Perineural spread should be considered in patients with a history of breast cancer, even 10 years after primary diagnosis, especially in patients who present with arm pain, weakness, and/or sensory changes. Further diagnostic workup with electrodiagnostic studies; brachial plexus MRI, PET/CT, or PET/MRI; and possibly nerve biopsy is warranted to ensure accurate diagnosis.
R. Michael Scott, Jodi L. Smith, Richard L. Robertson, Joseph R. Madsen, Sulpicio G. Soriano, and Mark A. Rockoff
Object.Moyamoya syndrome, a narrowing of the basal intracranial vessels accompanied by the development of a cloud of collateral “moyamoya” vasculature, causes cerebral ischemia and stroke. This study was undertaken to determine if a standardized neurosurgical revascularization procedure, pial synangiosis, conferred long-term benefit in pediatric patients.
Methods.The authors reviewed the clinical and radiographic records obtained in a consecutive series of patients with moyamoya syndrome. Patients were 21 years of age or younger and underwent surgery performed by a single neurosurgeon during a 17-year period.
There were 143 patients (89 females and 54 males). Sixteen patients were Asian. Neurofibromatosis was present in 16 patients, 13 had undergone therapeutic cranial irradiation, and Down syndrome was present in 10. In 66 there was no known predisposing condition. Stroke had occurred in 67.8% of the population and transient ischemic attacks (TIAs) in 43.4% prior to surgery. Within the first 30 days following 271 craniotomies for pial synangiosis, there were 11 episodes of stroke (7.7% per patient; 4% per surgically treated hemisphere) and three severe TIAs.
Follow-up evaluation was performed in all but one patient (mean period 5.1 years). In 126 patients followed for more than 1 year, four suffered a late-onset stroke, one suffered a severe reversible TIA without magnetic resonance imaging—documented evidence of stroke, and two experienced persistent TIAs. In 46 patients followed for more than 5 years in whom the major initial presentation was stroke alone, only two late-onset strokes have occurred. Functional status at the time of surgery determined long-term functional status.
Conclusions.Following pial synangiosis, the majority of pediatric patients with moyamoya syndrome stop having strokes and TIAs, and they appear to experience an excellent long-term prognosis.
