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1.
Childs Nerv Syst ; 40(6): 1783-1790, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38578480

RESUMO

PURPOSE: Patients and caregivers impacted by myelomeningocele (MMC) use online discussion board forums to create community and share information and concerns about this complex medical condition. We aim to identify the primary concerns expressed on these forums with the goal of understanding gaps in care that may merit investment of resources to improve care received by this population. METHODS: Anonymous posts from online MMC discussion boards were compiled using internet search engines. Posts were then analyzed using an adaptation of the Grounded Theory Method, a three-step system involving open, axial, and selective coding of the data by two independent researchers to identify common themes. RESULTS: Analysis of 400 posts written primarily by parents (n = 342, 85.5%) and patients (n = 45, 11.25%) yielded three overarching themes: questions surrounding quality of life, a lack of support for mothers of children with MMC, and confusion with a complex healthcare system. Many posts revealed concerns about management and well-being with MMC, including posts discussing symptoms and related conditions (n = 299, 75.75%), treatments (n = 259, 65.75%), and emotional aspects of MMC (n = 146, 36.5%). Additionally, families, especially mothers, felt a lack of support in their roles as caregivers. Finally, in 118 posts (29.5%), patients and families expressed frustration with navigating a complex healthcare system and finding specialists whose opinions they trusted. CONCLUSIONS: MMC is a complex medical condition that impacts patients and families in unique ways. Analysis of online discussion board posts identified key themes to be addressed in order to improve the healthcare experiences of those impacted by MMC.


Assuntos
Cuidadores , Meningomielocele , Humanos , Meningomielocele/psicologia , Cuidadores/psicologia , Feminino , Masculino , Qualidade de Vida/psicologia , Pesquisa Qualitativa , Pais/psicologia , Criança , Adulto , Internet
2.
Neurocrit Care ; 40(2): 587-602, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37470933

RESUMO

BACKGROUND: Surgical revascularization decreases the long-term risk of stroke in children with moyamoya arteriopathy but can be associated with an increased risk of stroke during the perioperative period. Evidence-based approaches to optimize perioperative management are limited and practice varies widely. Using a modified Delphi process, we sought to establish expert consensus on key components of the perioperative care of children with moyamoya undergoing indirect revascularization surgery and identify areas of equipoise to define future research priorities. METHODS: Thirty neurologists, neurosurgeons, and intensivists practicing in North America with expertise in the management of pediatric moyamoya were invited to participate in a three-round, modified Delphi process consisting of a 138-item practice patterns survey, anonymous electronic evaluation of 88 consensus statements on a 5-point Likert scale, and a virtual group meeting during which statements were discussed, revised, and reassessed. Consensus was defined as ≥ 80% agreement or disagreement. RESULTS: Thirty-nine statements regarding perioperative pediatric moyamoya care for indirect revascularization surgery reached consensus. Salient areas of consensus included the following: (1) children at a high risk for stroke and those with sickle cell disease should be preadmitted prior to indirect revascularization; (2) intravenous isotonic fluids should be administered in all patients for at least 4 h before and 24 h after surgery; (3) aspirin should not be discontinued in the immediate preoperative and postoperative periods; (4) arterial lines for blood pressure monitoring should be continued for at least 24 h after surgery and until active interventions to achieve blood pressure goals are not needed; (5) postoperative care should include hourly vital signs for at least 24 h, hourly neurologic assessments for at least 12 h, adequate pain control, maintaining normoxia and normothermia, and avoiding hypotension; and (6) intravenous fluid bolus administration should be considered the first-line intervention for new focal neurologic deficits following indirect revascularization surgery. CONCLUSIONS: In the absence of data supporting specific care practices before and after indirect revascularization surgery in children with moyamoya, this Delphi process defined areas of consensus among neurosurgeons, neurologists, and intensivists with moyamoya expertise. Research priorities identified include determining the role of continuous electroencephalography in postoperative moyamoya care, optimal perioperative blood pressure and hemoglobin targets, and the role of supplemental oxygen for treatment of suspected postoperative ischemia.


Assuntos
Revascularização Cerebral , Doença de Moyamoya , Acidente Vascular Cerebral , Criança , Humanos , Técnica Delphi , Doença de Moyamoya/cirurgia , Acidente Vascular Cerebral/etiologia , Assistência Perioperatória , Cuidados Pós-Operatórios , Revascularização Cerebral/efeitos adversos , Resultado do Tratamento , Estudos Retrospectivos
3.
Cleft Palate Craniofac J ; : 10556656231214125, 2024 Jan 09.
Artigo em Inglês | MEDLINE | ID: mdl-38193166

RESUMO

OBJECTIVE: To evaluate the safety and efficacy of helmet therapy for deformational plagiocephaly in patients with shunted hydrocephalus. DESIGN: Retrospective chart review. SETTING: Institutional, tertiary-care hospital. PATIENTS: All patients at St. Louis Children's Hospital between 2014 and 2021 with shunted hydrocephalus who underwent helmet therapy for deformational plagiocephaly. INTERVENTIONS: Helmet therapy. MAIN OUTCOME MEASURES: Cranial vault asymmetry (CVA), cranial vault asymmetry index (CVAI), and cephalic index (CI) were measured before and after completion of helmet therapy. RESULTS: There were 37 patients with shunted hydrocephalus and documented deformational plagiocephaly. Twelve were managed with helmet therapy. Average age at helmeting initiation and time between shunt placement and helmeting initiation was 5.8 and 4.6 months, respectively. Average CVA, CVAI, and CI at helmeting initiation and termination was 11.6, 7.98, and 85.2, and 6.95, 4.49, and 83.7, respectively. Average duration of helmeting was 3.7 months. CVA and CVAI were significantly lower after helmeting (P = .0028 and .0021) and 11/12 patients had overall improvement in plagiocephaly. CONCLUSIONS: Helmet therapy appears to be a safe and efficacious management strategy for deformational plagiocephaly in patients with shunted hydrocephalus. Despite the occasional need for additional fittings and surveillance beyond the normal schedule, in all cases appropriately fitting helmets were achieved and no major adverse events occurred. This cohort represents a proof of principle for the safety and efficacy of helmet therapy in patients with shunted hydrocephalus. Further work in larger prospective cohorts is needed to confirm these initial findings.

4.
Cleft Palate Craniofac J ; : 10556656231190043, 2023 Jul 24.
Artigo em Inglês | MEDLINE | ID: mdl-37488963

RESUMO

OBJECTIVE: Apply thematic analysis of online discussion boards to characterize families' experiences and concerns regarding craniosynostosis diagnoses to aid physicians in tailoring care to families. DESIGN: Grounded theory-based qualitative analysis. SETTING: Discussion boards related to craniosynostosis identified via Google and Yahoo. PATIENTS/PARTICIPANTS: Posts about craniosynostosis between 2017-2022. INTERVENTIONS: Thematic analysis was performed using three rounds of coding. Post features including author type and use of technical language were examined. MAIN OUTCOME MEASURE: Overarching themes emerging from analysis of posts, with forums analyzed until sufficient thematic repetition was observed. RESULTS: 366 posts from 4 websites by 290 unique users were included. Parents of patients with craniosynostosis wrote 59% of posts while patients wrote 4%. Five selective codes were identified: 1) Building Community, 2) Diagnosis/Evaluation, 3) Treatment, 4) Outcomes, and 5) Emotional Concerns. Building Community was the most assigned code (85% of posts). 71% of parents' posts expressing emotional concerns expressed negative emotions, commonly regarding anxiety about diagnosis (71%), frustration about doctors' responses (21%), or negative reactions to online search results (17%). 88% of patients' posts expressed positive emotions, discussing positive long-term outcomes. Concerns that may guide physicians included anxiety about delayed diagnosis, difficulty distinguishing postpartum head shape changes from craniosynostosis, and difficulty finding a care team. CONCLUSIONS: Online discussion boards allow families of patients with craniosynostosis to share experiences and find community. Improving communication between surgeons, pediatricians, and families about timing of evaluation and revising online information about this condition may ameliorate some anxiety associated with this diagnosis.

5.
Ann Neurol ; 90(2): 217-226, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-34080727

RESUMO

OBJECTIVE: Iron has been implicated in the pathogenesis of brain injury and hydrocephalus after preterm germinal matrix hemorrhage-intraventricular hemorrhage, however, it is unknown how external or endogenous intraventricular clearance of iron pathway proteins affect the outcome in this group. METHODS: This prospective multicenter cohort included patients with posthemorrhagic hydrocephalus (PHH) who underwent (1) temporary and permanent cerebrospinal fluid (CSF) diversion and (2) Bayley Scales of Infant Development-III testing around 2 years of age. CSF proteins in the iron handling pathway were analyzed longitudinally and compared to ventricle size and neurodevelopmental outcomes. RESULTS: Thirty-seven patients met inclusion criteria with a median estimated gestational age at birth of 25 weeks; 65% were boys. Ventricular CSF levels of hemoglobin, iron, total bilirubin, and ferritin decreased between temporary and permanent CSF diversion with no change in CSF levels of ceruloplasmin, transferrin, haptoglobin, and hepcidin. There was an increase in CSF hemopexin during this interval. Larger ventricle size at permanent CSF diversion was associated with elevated CSF ferritin (p = 0.015) and decreased CSF hemopexin (p = 0.007). CSF levels of proteins at temporary CSF diversion were not associated with outcome, however, higher CSF transferrin at permanent CSF diversion was associated with improved cognitive outcome (p = 0.015). Importantly, longitudinal change in CSF iron pathway proteins, ferritin (decrease), and transferrin (increase) were associated with improved cognitive (p = 0.04) and motor (p = 0.03) scores and improved cognitive (p = 0.04), language (p = 0.035), and motor (p = 0.008) scores, respectively. INTERPRETATION: Longitudinal changes in CSF transferrin (increase) and ferritin (decrease) are associated with improved neurodevelopmental outcomes in neonatal PHH, with implications for understanding the pathogenesis of poor outcomes in PHH. ANN NEUROL 2021;90:217-226.


Assuntos
Hemorragia Cerebral/líquido cefalorraquidiano , Ventrículos Cerebrais , Ferritinas/líquido cefalorraquidiano , Hidrocefalia/líquido cefalorraquidiano , Recém-Nascido Prematuro/líquido cefalorraquidiano , Transferrina/líquido cefalorraquidiano , Hemorragia Cerebral/diagnóstico por imagem , Hemorragia Cerebral/cirurgia , Ventrículos Cerebrais/diagnóstico por imagem , Ventrículos Cerebrais/cirurgia , Proteínas do Líquido Cefalorraquidiano/líquido cefalorraquidiano , Derivações do Líquido Cefalorraquidiano/tendências , Desenvolvimento Infantil/fisiologia , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Hidrocefalia/diagnóstico por imagem , Hidrocefalia/cirurgia , Lactente , Recém-Nascido , Recém-Nascido Prematuro/crescimento & desenvolvimento , Ferro/líquido cefalorraquidiano , Estudos Longitudinais , Masculino , Tamanho do Órgão/fisiologia , Nascimento Prematuro/líquido cefalorraquidiano , Nascimento Prematuro/diagnóstico por imagem , Nascimento Prematuro/cirurgia , Estudos Prospectivos
6.
Am J Med Genet A ; 188(5): 1538-1544, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35006644

RESUMO

Subdural hematoma (SDH) in infants raises the concern for nonaccidental trauma (NAT), especially when presenting with associated injuries. However, isolated SDH could be caused by multiple etiologies. NFIA (MIM# 600727) encodes nuclear factor I A protein (NFI-A), a transcription factor which plays important roles in gliogenesis. Loss-of-function variants in NFIA are associated with autosomal dominant brain malformations with or without urinary tract defects (MIM# 613735). Intracranial hemorrhage of various types besides SDH has been reported in patients with this condition. Here, we report a patient with a heterozygous novel NFIA pathogenic variant affecting splicing who initially presented with SDH concerning for NAT. We also review previous NFIA-related disorder cases with intracranial hemorrhage. This report emphasizes the importance of genetic evaluation in infants presenting with isolated SDH.


Assuntos
Hematoma Subdural , Fatores de Transcrição NFI , Diagnóstico Diferencial , Hematoma Subdural/diagnóstico , Hematoma Subdural/genética , Humanos , Lactente
7.
Cleft Palate Craniofac J ; : 10556656221129978, 2022 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-36177519

RESUMO

OBJECTIVE: Lambdoid craniosynostosis affects approximately 1 in 33 000 live births per year, and surgical correction is often sought in order to achieve normocephaly and allow for adequate brain growth. However, the effects of lambdoid synostosis and its treatment on cognitive development are unknown. DESIGN: Systematic review and meta-analysis. PATIENTS, PARTICIPANTS: A systematic review of Ovid Medline, Embase, Web of Science, Scopus, Cochrane Central Register of Controlled Trials, and Clinicaltrials.gov was conducted in January 2022. Included studies assessed cognitive development of patients with nonsyndromic unilateral lambdoid craniosynostosis. MAIN OUTCOME MEASURES: Results of developmental tests were compared to normative data or controls to generate Hedges' g for meta-analysis. RESULTS: Nine studies met the inclusion criteria. Meta-analysis of 3 studies describing general cognition showed that cases scored significantly lower than their peers, but within 1 standard deviation (g = 0.37, 95% CI [-0.64, -0.10], P = .01). Meta-analysis of verbal and psychomotor development showed no significant differences in children with lambdoid synostosis. Studies were of fair quality and had moderate-to-high heterogeneity. CONCLUSIONS: Patients with lambdoid craniosynostosis may score slightly below average on tests of general cognition in comparison to normal controls, but results in other domains are variable. Analyses were limited by small sample sizes. Multidisciplinary care and involvement of a child psychologist may be helpful in identifying areas of concern and providing adequate scholastic support. Further research recruiting larger cohorts will be necessary to confirm these findings and extend them to other developmental domains such as attention and executive function.

8.
Childs Nerv Syst ; 37(8): 2719-2722, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33388923

RESUMO

BACKGROUND: Ventriculoperitoneal (VP) shunts are the most common treatment for hydrocephalus in both pediatric and adult patients. Complications resulting from the abdominal portion of shunts include tube disconnection, obstruction of the shunt tip, catheter migration, infection, abdominal pseudocysts, and bowel perforation. However, other less common complications can occur. The authors present a unique case of a patient with a longstanding VP shunt presenting with an acute abdomen secondary to knotting of the peritoneal portion of the catheter tubing. CASE DESCRIPTION: A 13-year-old male with past medical history significant for myelomeningocele, requiring ventriculoperitoneal shunt placement at 18 months of age, presented to an outside hospital with chief complaint of abdominal pain. Cross-sectional imaging revealed spontaneous knot formation within the shunt tubing around the base of the small bowel mesentery. He was then transferred to our facility for general and neurosurgical evaluation. His abdominal exam was notable for diffuse distension in addition to tenderness to palpation with guarding and rebound. Given his tenuous clinical status and peritonitis, he was emergently booked for abdominal exploration. He underwent bowel resection, externalization of his shunt, with later re-anastomosis and shunt internalization. He eventually made a full recovery. DISCUSSION: Given the potential for significant bowel loss with this and other shunt-related complications, this case serves as a reminder that even longstanding VP shunts should be considered in the differential diagnosis of abdominal pain in any patient with a shunt.


Assuntos
Hidrocefalia , Derivação Ventriculoperitoneal , Adolescente , Catéteres , Humanos , Hidrocefalia/etiologia , Hidrocefalia/cirurgia , Masculino , Mesentério , Peritônio , Derivação Ventriculoperitoneal/efeitos adversos
9.
Stroke ; 51(6): 1712-1719, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32397930

RESUMO

Background and Purpose- Preterm neonates with intraventricular hemorrhage (IVH) are at risk for posthemorrhagic hydrocephalus and poor neurological outcomes. Iron has been implicated in ventriculomegaly, hippocampal injury, and poor outcomes following IVH. We hypothesized that levels of cerebrospinal fluid blood breakdown products and endogenous iron clearance proteins in neonates with IVH differ from those of neonates with IVH who subsequently develop posthemorrhagic hydrocephalus. Methods- Premature neonates with an estimated gestational age at birth <30 weeks who underwent lumbar puncture for clinical evaluation an average of 2 weeks after birth were evaluated. Groups consisted of controls (n=16), low-grade IVH (grades I-II; n=4), high-grade IVH (grades III-IV; n=6), and posthemorrhagic hydrocephalus (n=9). Control subjects were preterm neonates born at <30 weeks' gestation without brain abnormality or hemorrhage on cranial ultrasound, who underwent lumbar puncture for clinical purposes. Cerebrospinal fluid hemoglobin, total bilirubin, total iron, ferritin, ceruloplasmin, transferrin, haptoglobin, and hemopexin were quantified. Results- Cerebrospinal fluid hemoglobin levels were increased in posthemorrhagic hydrocephalus compared with high-grade IVH (9.45 versus 6.06 µg/mL, P<0.05) and cerebrospinal fluid ferritin levels were increased in posthemorrhagic hydrocephalus compared with controls (511.33 versus 67.08, P<0.01). No significant group differences existed for the other cerebrospinal fluid blood breakdown and iron-handling proteins tested. We observed positive correlations between ventricular enlargement (frontal occipital horn ratio) and ferritin (Pearson r=0.67), hemoglobin (Pearson r=0.68), and total bilirubin (Pearson r=0.69). Conclusions- Neonates with posthemorrhagic hydrocephalus had significantly higher levels of hemoglobin than those with high-grade IVH. Levels of blood breakdown products, hemoglobin, ferritin, and bilirubin correlated with ventricular size. There was no elevation of several iron-scavenging proteins in cerebrospinal fluid in neonates with posthemorrhagic hydrocpehalus, indicative of posthemorrhagic hydrocephalus as a disease state occurring when endogenous iron clearance mechanisms are overwhelmed.


Assuntos
Hemorragia Cerebral , Hidrocefalia , Recém-Nascido Prematuro , Hemorragia Cerebral/sangue , Hemorragia Cerebral/líquido cefalorraquidiano , Hemorragia Cerebral/diagnóstico por imagem , Estudos Transversais , Feminino , Humanos , Hidrocefalia/sangue , Hidrocefalia/líquido cefalorraquidiano , Hidrocefalia/diagnóstico por imagem , Recém-Nascido , Recém-Nascido Prematuro/sangue , Recém-Nascido Prematuro/líquido cefalorraquidiano , Masculino , Ultrassonografia
10.
Childs Nerv Syst ; 34(12): 2485-2490, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-29961083

RESUMO

BACKGROUND: Dermoid cysts are rare congenital teratomas that can occasionally rupture and cause chemical meningitis, neurological deficit, or hydrocephalus. Rarely, dermoid cysts in the pediatric population can rupture spontaneously and even more rarely rupture due to trauma. To date, there are only five documented cases of traumatic rupture of a dermoid cyst. A 2-year-old male presented with 5 days of left eye ptosis and ophthalmoplegia after suffering a fall and was found to have a ruptured left anterior clinoid dermoid cyst that was surgically resected. The patient had significant improvement postoperatively. SIGNIFICANCE: To the authors' knowledge, this is the first reported case in the literature of a ruptured dermoid cyst after trauma in a pediatric patient and the first case of a traumatically ruptured dermoid cyst presenting with neurological deficit.


Assuntos
Acidentes por Quedas , Neoplasias Encefálicas/patologia , Cisto Dermoide/patologia , Pré-Escolar , Humanos , Masculino , Ruptura
11.
Childs Nerv Syst ; 34(11): 2203-2213, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-29987373

RESUMO

PURPOSE: The goal of this longitudinal, population-level study was to examine factors affecting mortality in preterm infants with intraventricular hemorrhage (IVH). METHODS: The study examined patients who were born at 36 weeks estimated gestational age (EGA) or less with a diagnosis of IVH between the years 2005 and 2014 using data from the New York and Nebraska State Inpatient Databases. Potential predictors for mortality were investigated with multivariable survival analysis. RESULTS: The cohort included 7437 preterm infants with IVH. All-cause inpatient mortality occurred in 746 (10.0%). The majority of deaths were in infants born at less than 25 weeks EGA (378 or 50.7%) and with birthweight less than 750 g (459 or 61.5%). Mortality was highest for children with grade IV IVH (306/848 or 36.1%), followed by grades III (203/955 or 21.3%), II (103/1328 or 7.8%), and I (134/4306 or 3.1%). Hydrocephalus was diagnosed within 6 months in 627 (8.4%) patients, with cerebrospinal fluid shunts required in 237 (3.2%). Shunts were eventually revised in 122 (51.5% of shunts), and 43 (18.1%) had infections. Multivariable Cox survival analyses found male sex (HR 1.3 [95% CI 1.1-1.5]), Asian race (HR 1.5 [1.1-2.2]), lower EGA (HR 9.9 [6.3-15.5] for < 25 weeks), higher IVH grade (HR 6.1 [4.9-7.6] for grade IV), gastrostomy (HR 4.0 [2.0-7.7]), tracheostomy (HR 3.5 [1.7-7.1]), and shunt infection (HR 3.2 [1.0-9.9]) to be independently associated with increased mortality risk. CONCLUSIONS: This database is the first of its kind assembled for population-based investigations of long-term neurosurgical outcomes in preterm infants with IVH.


Assuntos
Hemorragia Cerebral Intraventricular/mortalidade , Bases de Dados Factuais , Doenças do Prematuro/mortalidade , Recém-Nascido Prematuro , Feminino , Humanos , Recém-Nascido , Masculino , Fatores de Risco
13.
Childs Nerv Syst ; 33(11): 1917-1926, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28884229

RESUMO

PURPOSE: The purpose of this study is to report time points relevant to the neurosurgical management of posthemorrhagic hydrocephalus (PHH). METHODS: Data were collected retrospectively on 104 preterm infants with intraventricular hemorrhage (IVH) who received neurosurgical intervention for PHH at St. Louis Children's Hospital from 1994 to 2016. Kaplan-Meier curves were constructed for various endpoints. RESULTS: IVH grade on head ultrasound obtained through routine clinical care was II, III, and IV in 5 (4.8%), 33 (31.7%), and 66 (63.5%) of the patients, respectively. Neither IVH size nor location appeared to affect development of PHH. Days from birth to IVH, ventriculomegaly, temporizing neurosurgical procedure (TNP), and permanent neurosurgical intervention were 2.0 (95% CI 1.7-2.3), 3.0 (2.5-3.5), 24.0 (22.2-25.8), and 101.0 (90.4-111.6), respectively. Grades III and IV IVH did not differ in age at IVH diagnosis (Χ 2 (1 d.f.) = 1.32, p = 0.25), ventriculomegaly (Χ 2 = 0.73, p = 0.40), TNP (Χ 2 = 0.61, p = 0.43), or permanent intervention (Χ 2 = 2.48, p = 0.17). Ventricular reservoirs and ventriculosubgaleal shunts were used in 71 (68.3%) and 30 (28.8%), respectively. Eighty (76.9%) of the patients ultimately received a VPS. Five (4.8%) underwent a primary endoscopic third ventriculostomy (ETV), and two (1.9%) had ETV for a revision procedure. Four of the seven ETVs had choroid plexus cauterization. CONCLUSIONS: Although most infants who develop IVH and ventriculomegaly will do so within a few days of birth, at-risk infants should be observed for at least 4 weeks with serial head ultrasounds to monitor for PHH requiring surgery.


Assuntos
Hemorragia Cerebral/complicações , Hidrocefalia/complicações , Hidrocefalia/epidemiologia , Hidrocefalia/cirurgia , Feminino , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Estimativa de Kaplan-Meier , Masculino , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco
14.
J Craniofac Surg ; 25(1): 82-6, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24240766

RESUMO

BACKGROUND: Cranial vault remodeling (CVR) for craniosynostosis is a procedure with the potential for significant blood loss. Aminocaproic acid (ACA) has been used at our institution during CVR for its antifibrinolytic effects. The purpose of this study was to investigate the effect of ACA on blood loss and transfusion rates during primary CVR. METHODS: Three hundred eighty-three patients with craniosynostosis underwent primary CVR at a single institution by a single surgeon over 15 years. Patients were included if they received either ACA or no antifibrinolytic. The estimated blood loss (EBL) and volume of blood transfused was recorded. Thrombotic-related complications were identified. Comparisons were made between subgroups using independent Student t test and Fisher exact test. RESULTS: Among the study population, 148 patients met inclusion criteria. ACA was given to 30 patients, while 118 patients received no antifibrinolytic. There was no difference in the average intraoperative EBL between the ACA (322 mL) and control groups (327 mL, P > 0.05). Additionally, the incidence of transfusion was not significantly different between subgroups (97% vs. 86%, respectively, P > 0.05). Patients treated with ACA, however, received lower average perioperative transfusion volumes (25.5 mL/kg) compared to control patients (53.3 mL/kg, P < 0.0001). Furthermore, patients in the ACA subgroup were less likely to require a second unit of blood (21% vs. 43%, P < 0.0001) and therefore had fewer exposures to donor blood antigens (ARR = 22%, NNT = 4.6). CONCLUSIONS: The use of intraoperative ACA minimizes blood transfusion volumes and donor exposures in children who undergo primary CVR for craniosynostosis. Antifibrinolytics should be considered for routine use in pediatric craniofacial surgery.


Assuntos
Ácido Aminocaproico/uso terapêutico , Antifibrinolíticos/uso terapêutico , Perda Sanguínea Cirúrgica/prevenção & controle , Transfusão de Sangue , Craniossinostoses/cirurgia , Pressão Arterial/fisiologia , Estudos de Coortes , Transfusão de Eritrócitos , Hematócrito , Humanos , Procedimentos de Cirurgia Plástica/métodos , Estudos Retrospectivos , Crânio/cirurgia , Trombose/etiologia
15.
J Neurosurg Pediatr ; 33(6): 554-563, 2024 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-38457805

RESUMO

OBJECTIVE: The need for permanent CSF diversion is lower in patients who have undergone prenatal surgery for myelomeningocele (MMC) than in those who have undergone postnatal closure. Differences in brain development and head growth between treatment groups are not known, particularly for those who do not require surgical treatment for hydrocephalus. The objective of this study was to determine differences in head growth and to generate MMC-specific head circumference (HC) growth curves for patients who underwent either prenatal or postnatal surgery. METHODS: The authors retrospectively identified patients from St. Louis Children's Hospital who were treated for MMC between 2016 and 2021. HC data were obtained from birth until the most recent follow-up or hydrocephalus treatment. Nonlinear least-squares regression analysis was performed to fit the data into four models: two-term power, Gompertz, West ontogenetic, and Weibull. Subsequently, the curves were assessed for their utility in predicting hydrocephalus treatment. RESULTS: Sixty-one patients (29 females [47.5%], 25 [41%] underwent prenatal surgery, mean gestational age at birth 36.6 weeks) were included in the study. The Weibull model best fit the HC data (prenatal adjusted R2 = 0.95, postnatal adjusted R2 = 0.95), while the Gompertz model had the worst fit (prenatal adjusted R2 = 0.56, postnatal adjusted R2 = 0.39) across both cohorts. Prenatal MMC repair patients had significantly larger HC measurements than their postnatal repair counterparts. The 50th percentile of the Weibull curve was determined as a useful threshold for hydrocephalus treatment: children with HC measurements that crossed and remained above this threshold were significantly more likely to have hydrocephalus treatment regardless of time of MMC repair (prenatal relative risk [RR] 10.0 [95% CI 1.424-70.220], sensitivity 85.7% [95% CI 0.499-0.984], and specificity 82.4% [95% CI 0.600-0.948]; postnatal RR 4.750 [95% CI 1.341-16.822], sensitivity 90.5% [95% CI 0.728-0.980], and specificity 75.0% [95% CI 0.471-0.924]). The HC growth curves of the MMC patients treated prenatally were significantly larger than the WHO HC curves (p < 0.001). CONCLUSIONS: The Weibull model was identified as the HC growth curve with the best fit for MMC patients and serves as a useful predictor of hydrocephalus treatment. For MMC patients with hydrocephalus, prenatal repair patients fit the model well but postnatal repair patients did not, potentially indicating different mechanisms of hydrocephalus development. Those treated prenatally had significantly larger HC measurements compared with both the general population and those treated postnatally. Further study is needed to understand the long-term cognitive outcomes and optimal management of clinically asymptomatic patients with large HC measurements who were treated prenatally for MMC.


Assuntos
Cabeça , Hidrocefalia , Meningomielocele , Humanos , Meningomielocele/cirurgia , Meningomielocele/complicações , Feminino , Masculino , Estudos Retrospectivos , Cabeça/cirurgia , Recém-Nascido , Hidrocefalia/cirurgia , Gravidez , Lactente , Idade Gestacional , Cefalometria
16.
J Neurosurg Pediatr ; 33(4): 382-389, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38306636

RESUMO

OBJECTIVE: Patients and their caregivers utilize online discussion board forums as a means to seek and exchange information about their or a loved one's condition. It is important for providers to be aware of such concerns and experiences. The goal of this study was to identify the primary concerns expressed on these discussion boards regarding Chiari malformation type I (CM) and to help guide clinicians in understanding patient challenges in the treatment of CM. METHODS: The authors performed thematic analysis of anonymous online discussion board posts as identified through internet search engines. They then adopted a previously developed grounded theory method that utilizes a three-tiered coding and grouping process of posts based on commonly discovered content themes. RESULTS: Analysis of 400 discussion board posts identified four distinct themes raised by CM patients and their caregivers: the path to diagnosis, symptoms experienced, surgical intervention, and high emotional burden. Although each individual experience was unique, the path toward a CM diagnosis was expressed as a journey involving multiple physicians, alternative diagnoses, and feelings of dismissal from providers. The most common reported symptoms included dizziness, headaches, neck and back pain, sensory issues, weakness and paresthesias of the extremities, speech issues, and general fatigue. Additionally, there was an overall sense of uncertainty from patients seeking advice regarding surgical intervention, with users expressing diverse sentiments that included both positive and negative outcomes regarding surgical treatment. Lastly, a wide range of emotions was expressed related to a CM diagnosis, including concern, worry, anxiety, depression, stress, fear, and frustration. CONCLUSIONS: CM is a frequent imaging diagnosis identified in patients presenting with a wide range of symptoms, and as a result this leads to a diverse set of patient experiences. Analysis of CM patient and caregiver discussion boards revealed key themes that clinicians may address when counseling for CM.


Assuntos
Malformação de Arnold-Chiari , Cuidadores , Humanos , Malformação de Arnold-Chiari/cirurgia , Cefaleia
17.
World Neurosurg ; 182: 193-199.e4, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38040329

RESUMO

BACKGROUND: The social determinants of health, which influence healthcare access, patient outcomes, and population-level burden of disease, contribute to health disparities experienced by marginalized patient populations. In the present study, we sought to evaluate the landscape of health disparities research within neurosurgery. METHODS: Embase, Ovid-MEDLINE, Scopus, Web of Science, Cochrane Library, and ProQuest Dissertations databases were queried for original research on health disparities regarding access to, outcomes of, and/or postoperative management after neurosurgical procedures in the United States. RESULTS: Of 883 studies screened, 196 were included, of which 144 had a neurosurgery-affiliated author. We found a significant increase in the number of neurosurgical disparities reports beginning in 2010, with only 10 studies reported before 2010. Of the included studies, 3.1% used prospective methods and 63.8% used data from national registries. The disparities analyzed were racial/ethnic (79.6%), economic/socioeconomic (53.6%), gender (18.9%), and disabled populations (0.5%), with 40.1% analyzing multiple or intersecting disparities. Of the included reports, 96.9% were in phase 1 (detecting phase of disparities research), with a few studies in phase 2 (understanding phase), and none in phase 3 (reducing phase). The spine was the most prevalent subspecialty evaluated (34.2%), followed by neuro-oncology (19.9%), cerebrovascular (16.3%), pediatrics (10.7%), functional (9.2%), general neurosurgery (5.1%), and trauma (4.1%). Senior authors with a neurosurgical affiliation accounted for 79.2% of the reports, 93% of whom were academically affiliated. CONCLUSIONS: Although a recent increase has occurred in neurosurgical disparities research within the past decade, most studies were limited to the detection of disparities without understanding or evaluating any interventions for a reduction in disparities. Future research in neurosurgical disparities should incorporate the latter 2 factors to reduce disparities and improve outcomes for all patients.


Assuntos
Disparidades em Assistência à Saúde , Neurocirurgia , Humanos , Criança , Estados Unidos , Grupos Raciais , Procedimentos Neurocirúrgicos , Bibliometria
18.
Fluids Barriers CNS ; 21(1): 4, 2024 Jan 08.
Artigo em Inglês | MEDLINE | ID: mdl-38191402

RESUMO

BACKGROUND: CSF has long been accepted to circulate throughout the subarachnoid space, which lies between the arachnoid and pia maters of the meninges. How the CSF interacts with the cellular components of the developing postnatal meninges including the dura, arachnoid, and pia of both the meninges at the surface of the brain and the intracranial meninges, prior to its eventual efflux from the cranium and spine, is less understood. Here, we characterize small and large CSF solute distribution patterns along the intracranial and surface meninges in neonatal rodents and compare our findings to meningeal CSF solute distribution in a rodent model of intraventricular hemorrhage-posthemorrhagic hydrocephalus. We also examine CSF solute interactions with the tela choroidea and its pial invaginations into the choroid plexuses of the lateral, third, and fourth ventricles. METHODS: 1.9-nm gold nanoparticles, 15-nm gold nanoparticles, or 3 kDa Red Dextran Tetramethylrhodamine constituted in aCSF were infused into the right lateral ventricle of P7 rats to track CSF circulation. 10 min post-1.9-nm gold nanoparticle and Red Dextran Tetramethylrhodamine injection and 4 h post-15-nm gold nanoparticle injection, animals were sacrificed and brains harvested for histologic analysis to identify CSF tracer localization in the cranial and spine meninges and choroid plexus. Spinal dura and leptomeninges (arachnoid and pia) wholemounts were also evaluated. RESULTS: There was significantly less CSF tracer distribution in the dura compared to the arachnoid and pia maters in neonatal rodents. Both small and large CSF tracers were transported intracranially to the arachnoid and pia mater of the perimesencephalic cisterns and tela choroidea, but not the falx cerebri. CSF tracers followed a similar distribution pattern in the spinal meninges. In the choroid plexus, there was large CSF tracer distribution in the apical surface of epithelial cells, and small CSF tracer along the basolateral surface. There were no significant differences in tracer intensity in the intracranial meninges of control vs. intraventricular hemorrhage-posthemorrhagic hydrocephalus (PHH) rodents, indicating preserved meningeal transport in the setting of PHH. CONCLUSIONS: Differential CSF tracer handling by the meninges suggests that there are distinct roles for CSF handling between the arachnoid-pia and dura maters in the developing brain. Similarly, differences in apical vs. luminal choroid plexus CSF handling may provide insight into particle-size dependent CSF transport at the CSF-choroid plexus border.


Assuntos
Hidrocefalia , Nanopartículas Metálicas , Animais , Ratos , Pia-Máter , Ouro , Meninges , Aracnoide-Máter , Hemorragia Cerebral
19.
Nat Rev Dis Primers ; 10(1): 35, 2024 May 16.
Artigo em Inglês | MEDLINE | ID: mdl-38755194

RESUMO

Hydrocephalus is classically considered as a failure of cerebrospinal fluid (CSF) homeostasis that results in the active expansion of the cerebral ventricles. Infants with hydrocephalus can present with progressive increases in head circumference whereas older children often present with signs and symptoms of elevated intracranial pressure. Congenital hydrocephalus is present at or near birth and some cases have been linked to gene mutations that disrupt brain morphogenesis and alter the biomechanics of the CSF-brain interface. Acquired hydrocephalus can develop at any time after birth, is often caused by central nervous system infection or haemorrhage and has been associated with blockage of CSF pathways and inflammation-dependent dysregulation of CSF secretion and clearance. Treatments for hydrocephalus mainly include surgical CSF shunting or endoscopic third ventriculostomy with or without choroid plexus cauterization. In utero treatment of fetal hydrocephalus is possible via surgical closure of associated neural tube defects. Long-term outcomes for children with hydrocephalus vary widely and depend on intrinsic (genetic) and extrinsic factors. Advances in genomics, brain imaging and other technologies are beginning to refine the definition of hydrocephalus, increase precision of prognostication and identify nonsurgical treatment strategies.


Assuntos
Hidrocefalia , Humanos , Hidrocefalia/fisiopatologia , Hidrocefalia/diagnóstico , Hidrocefalia/terapia , Hidrocefalia/etiologia , Hidrocefalia/complicações , Criança , Lactente , Ventriculostomia/métodos , Derivações do Líquido Cefalorraquidiano/métodos , Recém-Nascido
20.
Fluids Barriers CNS ; 21(1): 17, 2024 Feb 21.
Artigo em Inglês | MEDLINE | ID: mdl-38383424

RESUMO

BACKGROUND: Interpretation of cerebrospinal fluid (CSF) studies can be challenging in preterm infants. We hypothesized that intraventricular hemorrhage (IVH), post-hemorrhagic hydrocephalus (PHH), and infection (meningitis) promote pro-inflammatory CSF conditions reflected in CSF parameters. METHODS: Biochemical and cytological profiles of lumbar CSF and peripheral blood samples were analyzed for 81 control, 29 IVH grade 1/2 (IVH1/2), 13 IVH grade 3/4 (IVH3/4), 15 PHH, 20 culture-confirmed bacterial meningitis (BM), and 27 viral meningitis (VM) infants at 36.5 ± 4 weeks estimated gestational age. RESULTS: PHH infants had higher (p < 0.02) CSF total cell and red blood cell (RBC) counts compared to control, IVH1/2, BM, and VM infants. No differences in white blood cell (WBC) count were found between IVH3/4, PHH, BM, and VM infants. CSF neutrophil counts increased (p ≤ 0.03) for all groups compared to controls except IVH1/2. CSF protein levels were higher (p ≤ 0.02) and CSF glucose levels were lower (p ≤ 0.003) for PHH infants compared to all other groups. In peripheral blood, PHH infants had higher (p ≤ 0.001) WBC counts and lower (p ≤ 0.03) hemoglobin and hematocrit than all groups except for IVH3/4. CONCLUSIONS: Similarities in CSF parameters may reflect common pathological processes in the inflammatory response and show the complexity associated with interpreting CSF profiles, especially in PHH and meningitis/ventriculitis.


Assuntos
Infecções do Sistema Nervoso Central , Hidrocefalia , Meningite , Lactente , Recém-Nascido , Humanos , Recém-Nascido Prematuro , Relevância Clínica , Hemorragia Cerebral/complicações , Hidrocefalia/líquido cefalorraquidiano , Infecções do Sistema Nervoso Central/complicações , Meningite/complicações , Líquido Cefalorraquidiano
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