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PURPOSE: Chiari I malformation (CM-I) in pediatric patients can impose substantial neurologic and functional impairment. Additionally, the presence of syrinx is often a harbinger of clinical compromise, but little attention has been devoted to identifying features associated with syrinx development and the clinical impact of syrinx resolution. Therefore, this study aims to identify clinical and radiographic variables associated with preoperative syrinx presence and postoperative syrinx reduction in pediatric patients with CM-I and determine the relationship between postoperative syrinx reduction and clinical symptom improvement. METHODS: The authors performed a retrospective analysis of 435 consecutive pediatric patients who underwent surgical treatment of CM-I from 2001 to 2021 at a single tertiary pediatric medical center. All patients underwent pre- and postoperative MRI, and clinical and radiographic variables were recorded and subject to inferential analysis. RESULTS: Syrinx at presentation was independently associated with symptoms of spinal cord dysfunction at presentation (OR 2.17 (95% CI 1.05-4.48); p = 0.036), scoliosis (OR 5.33 (2.34-10.86); p = 0.001), and greater pB-C2 (posterior basion to C2 distance) measurement length (OR 1.14 (95% CI 1.01-1.30); p = 0.040). Syrinx at presentation was inversely associated with tussive headaches at presentation (OR 0.27 (95% CI 0.16-0.47); p = 0.001) and cranial nerve deficits at presentation (OR 0.49 (95% CI 0.26-0.92); p = 0.025). Postoperatively, patients with radiographic evidence of syrinx improvement had greater rates of symptom improvement (93.1% vs 82.1%; p = 0.049), better CCOS scores (15.4 vs 14.2; p = 0.001), and decreased rates of readmission (6.0% vs 25.0%, p = 0.002) and reoperation (0.5% vs 35.7%; p = 0.001). The difference in syrinx resolution was similar but not statistically significant (10.3% vs 16.7%; p = 0.251). AO joint anomaly (OR 0.20, 95% CI 0.04-0.95; p = 0.026) and foramen magnum diameter (OR 1.12, 95% CI 1.00-1.25; p = 0.049) were the only independent predictors of syrinx improvement, and surgical technique was the only predictor for syrinx resolution (OR 2.44, 95% CI 1.08-5.50; p = 0.031). Patients that underwent tonsil reduction surgery whose syrinx improved had a wider foramen magnum diameter than those whose did not improve (34.3 vs 31.7; p = 0.028). CONCLUSIONS: Radiographic syrinx improvement is associated with greater rates of symptom improvement and less readmissions and reoperations for CM-I. AO joint anomalies and narrower foramen magnums were independent risk factors for the lack of syrinx improvement. These novel insights will help guide preoperative patient counseling, pre- and intraoperative surgical decision-making, and postoperative clinical prognostication in the treatment of pediatric CM-I.
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Malformación de Arnold-Chiari , Siringomielia , Humanos , Malformación de Arnold-Chiari/cirugía , Malformación de Arnold-Chiari/diagnóstico por imagen , Malformación de Arnold-Chiari/complicaciones , Masculino , Femenino , Siringomielia/cirugía , Siringomielia/diagnóstico por imagen , Niño , Estudios Retrospectivos , Adolescente , Preescolar , Resultado del Tratamiento , Imagen por Resonancia Magnética , LactanteRESUMEN
OBJECTIVE: Foramen magnum (FM) decompression with or without duraplasty is considered a common treatment strategy for Chiari malformation type I (CM-I). The authors' objective was to determine a predictive model of risk factors for clinical and radiological worsening after CM-I surgery. METHODS: A retrospective review of electronic health records was conducted at an academic tertiary care hospital from 2001 to 2019. A multivariable Cox proportional hazards regression model was used to determine the risk factors. The Kaplan-Meier estimate was plotted to delineate outcomes based on FM size. FM was measured as the preoperative distance between the basion and opisthion and dichotomized into < 34 mm and ≥ 34 mm. Syrinx was measured preoperatively and postoperatively in the craniocaudal and anteroposterior directions using a T2-weighted MRI sequence. RESULTS: A total of 454 patients (231 females [50.9%]) with a median (range) age of 8.0 (0-18) years were included in the study. The median duration of follow-up was 21.0 months (range 3.0-144.0 years). The model suggested that patients with symptoms consisting of occipital/tussive headache (HR 4.05, 95% CI 1.34-12.17, p = 0.01), cranial nerve symptoms (HR 3.46, 95% CI 1.16-10.2, p = 0.02), and brainstem/spinal cord symptoms (HR 3.25, 95% CI 1.01-11.49, p = 0.05) had higher risk, whereas those who underwent arachnoid dissection/adhesion lysis had 75% lower likelihood (HR 0.25, 95% CI 0.10-0.64, p = 0.004) of clinical worsening postoperatively. Similarly, patients with evidence of brainstem/spinal cord symptoms (HR 7.9, 95% CI 2.84-9.50, p = 0.03), scoliosis (HR 1.04, 95% CI 1.01-2.80, p = 0.04), and preoperative syrinx (HR 16.1, 95% CI 1.95-132.7, p = 0.03) had significantly higher likelihood of postoperative worsening of syrinx. Patients with symptoms consisting of occipital/tussive headache (HR 5.44, 95% CI 1.86-15.9, p = 0.002), cranial nerve symptoms (HR 2.80, 95% CI 1.02-7.68, p = 0.04), and nonspecific symptoms (HR 6.70, 95% CI 1.99-22.6, p = 0.002) had significantly higher likelihood, whereas patients with FM ≥ 34 mm and those who underwent arachnoid dissection/adhesion lysis had 73% (HR 0.27, 95% CI 0.08-0.89, p = 0.03) and 70% (HR 0.30, 95% CI 0.12-0.73, p = 0.008) lower likelihood of reoperation, respectively. The Kaplan-Meier curve showed that patients with FM size ≥ 34 mm had significantly better clinical (p = 0.02) and syrinx (p = 0.03) improvement postoperatively when the tonsils were resected. CONCLUSIONS: These results showed that preoperative and intraoperative factors may help to provide better clinical decision-making for CM-I surgery. Patients with FM size ≥ 34 mm may have better outcomes when the tonsils are resected.
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Malformación de Arnold-Chiari , Niño , Femenino , Humanos , Adolescente , Radiografía , Factores de Riesgo , Cefalea , Médula EspinalRESUMEN
Morbidity and mortality in prematurely born infants have significantly improved due to advancement in perinatal care, development of NeuroNICU collaborative multidisciplinary approaches, and evidence-based management protocols that have resulted from a better understanding of perinatal risk factors and neuroprotective treatments. In premature infants with intraventricular hemorrhage (IVH), the detrimental secondary effect of posthemorrhagic ventricular dilation (PHVD) on the neurodevelopmental outcome can be mitigated by surgical intervention, though management varies considerably across institutions. Any benefit derived from the use of neuromonitoring to optimize surgical timing and technique stands to improve neurodevelopmental outcome. In this review, we summarize (1) the approaches to surgical management of PHVD in preterm infants and outcome data; (2) neuromonitoring modalities and the effect of neurosurgical intervention on this data; (3) our resultant protocol for the monitoring and management of PHVD. In particular, our protocol incorporates cerebral near-infrared spectroscopy (NIRS) and transcranial doppler ultrasound (TCD) to better understand cerebral physiology and to enable the hypothesis-driven study of the management of PHVD. IMPACT: Review of the published literature concerning the use of near-infrared spectroscopy (NIRS) and a cerebral Doppler ultrasound to study the effect of cerebrospinal fluid drainage on infants with posthemorrhagic ventricular dilation. Presentation of our institution's evidence-based protocol for the use of NIRS and cerebral Doppler ultrasound to study the optimal neurosurgical treatment of posthemorrhagic ventricular dilation, an as yet inadequately studied area.
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Ventrículos Cerebrales/diagnóstico por imagen , Enfermedades del Prematuro/fisiopatología , Recien Nacido Prematuro , Unidades de Cuidado Intensivo Neonatal , Algoritmos , Ventrículos Cerebrales/fisiopatología , Diagnóstico por Imagen/métodos , Humanos , Enfermedades del Prematuro/cirugía , Monitoreo Fisiológico/métodosRESUMEN
INTRODUCTION: Extracranial carotid artery pseudoaneurysm is a rare complication of deep neck space infection, and no evidence-based treatment guidelines are available in the literature. METHOD: To clarify the existing experience of the different treatment strategies, the authors performed a systematic literature search using the PubMed, Ovid EMBASE, and Scopus databases in accordance with PRISMA guidelines to review all reported cases of pediatric patients with infectious carotid pseudoaneurysms larger than 1 cm. RESULTS: Twenty-six patients with a median age of 4 years (range 6 months-15 years) were identified. Eighteen patients (69.2%) were treated with endovascular methods, 6 patients (23.1%) with surgical methods, 1 patient (3.8%) with a hybrid endovascular/surgical approach, and 1 patient (3.8%) with conservative management. Recurrence of the pseudoaneurysm occurred in 2 cases (7.7%), both of which were successfully retreated. Of the 6 patients (23.1%) who presented with pre-procedure neurologic deficits, 3 patients had complete or near complete resolution of symptoms after intervention and 3 patients had persistent deficits at last follow-up. Four patients (15.4%) experienced new neurologic deficits post-procedure that resolved at last follow-up. CONCLUSION: The endovascular treatment tends to be the preferred option to treat a large or giant infectious pseudoaneurysm of the carotid artery in the pediatric patient. However, more evidence is necessary to elucidate comparative safety and efficacy profiles of endovascular and surgical management strategies.
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Aneurisma Falso , Traumatismos de las Arterias Carótidas , Procedimientos Endovasculares , Aneurisma Falso/diagnóstico por imagen , Aneurisma Falso/etiología , Aneurisma Falso/cirugía , Arterias Carótidas , Niño , Humanos , Lactante , Stents , Resultado del TratamientoRESUMEN
OBJECTIVE: Abdominal CSF pseudocysts are an uncommon but challenging complication of ventriculoperitoneal shunts. Pseudocysts consist of a loculated intraperitoneal compartment that inadequately absorbs CSF and may be infected or sterile at diagnosis. The treatment goal is to clear infection if present, reduce inflammation, and reestablish long-term function in an absorptive (intraperitoneal) space. This aim of this paper was to study the efficacy of primary laparoscopic repositioning of the distal shunt catheter for treatment of sterile abdominal CSF pseudocysts. METHODS: All patients treated for abdominal CSF pseudocysts at Dallas Children's Health from 1991 to 2021 were retrospectively reviewed. Patient history and pseudocyst characteristics were analyzed, with a primary outcome of pseudocyst recurrence at 1 year. RESULTS: Of 92 primary pseudocysts, 5 initial treatment strategies (groups) were used depending on culture status, clinical history, and surgeon preference: 1) shunt explant/external ventricular drain (EVD) placement (23/92), 2) distal tubing externalization (13/92), 3) laparoscopic repositioning (35/92), 4) open repositioning (4/92), and 5) other methods such as pseudocyst drainage or direct revision to another terminus (17/92). Seventy pseudocysts underwent shunt reimplantation in the peritoneal space. The 1-year peritoneal shunt survival for groups 1 and 2 combined was 90%, and 62% for group 3. In group 3, 1-year survival was better for those with normal systemic inflammatory markers (100%) than for those with high markers (47%) (p = 0.042). In a univariate Cox proportional hazards model, the risk of pseudocyst recurrence was increased if the most recent abdominal procedure was a nonshunt abdominal surgery (p = 0.012), and it approached statistical significance with male sex (p = 0.054) and elevated inflammatory markers (p = 0.056. Multivariate Cox analysis suggested increased recurrence risk with male sex (p = 0.05) and elevated inflammatory markers (p = 0.06), although the statistical significance threshold was not reached. The length of hospital stay was shorter for laparoscopic repositioning (6 days) than for explantation/EVD placement (21 days) (p < 0.0001). Ultimately, 62% of patients had a peritoneal terminus at the last follow-up, 33% (n = 30) had an extraperitoneal terminus (19 pleura, 8 right heart, and 3 gallbladder), and 5 patients were shunt free. CONCLUSIONS: Some sterile pseudocysts with normal systemic inflammatory markers can be effectively treated with laparoscopic repositioning, resulting in a significantly shorter hospitalization and modestly higher recurrence rate than shunt explantation.
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Laparoscopía , Niño , Humanos , Masculino , Estudios Retrospectivos , Catéteres , Salud Infantil , Remoción de DispositivosRESUMEN
BACKGROUND AND PURPOSE: The use of iodinated contrast-enhanced imaging studies is increasing in acute cerebrovascular diseases, especially in subarachnoid hemorrhage (SAH). In SAH, such studies are essential for both diagnosis and treatment of the cause and sequela of hemorrhage. These patients are often subjected to multiple contrast studies such as computed tomographic angiography, computed tomographic perfusion, and cerebral angiography. They are also predisposed to intravascular volume depletion as a part of the disease process from cerebral salt wasting (CSW) and as a result of multiple contrast exposure can develop contrast-induced nephropathy (CIN). Data regarding CIN in this population are scarce. We aimed to examine the incidence of CIN in SAH and identify potential associative risk factors. METHODS: We analyzed data from a prospectively collected patient database of patients with SAH admitted to the neurocritical intensive care unit in a single center over a period of 1 year. CIN was defined as an increase in serum creatinine by >1.5 times or >0.3 mg/dl greater than the admission value, or urine output <0.5 ml/kg/h during one 6-h block. RESULTS: In this cohort of 75 patients with SAH who had undergone at least one contrast study, the mean age was 57.3 ± 15.6 years and 70.7% were women. Four percent developed CIN which resolved within 72 h and none required renal replacement therapy or dialysis. Patients older than 75 years (20%, p < 0.05), those with borderline renal function (14.3%, p = 0.26), diabetics (11.1%, p = 0.32), and those with lower recommended "maximum contrast dose" volume (33.3%, p = 0.12) had a trend toward development of CIN, although most were not statistically significant. Twenty-seven patients (36 %) were on 3% hypertonic saline (HTS) for CSW during the contrasted study but none developed CIN. CONCLUSIONS: The incidence of CIN in SAH patients is comparable to previously published reports on non-neurological cohorts. No definite association was noted with any predisposing factors postulated to be responsible for CIN, except for advanced age. Concurrent use of 3% HTS was not associated with CIN in this population.
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Lesión Renal Aguda/inducido químicamente , Angiografía Cerebral/efectos adversos , Medios de Contraste/efectos adversos , Hemorragia Subaracnoidea/diagnóstico por imagen , Tomografía Computarizada por Rayos X/efectos adversos , Lesión Renal Aguda/epidemiología , Lesión Renal Aguda/prevención & control , Adulto , Anciano , Estudios de Cohortes , Creatinina/sangre , Cuidados Críticos , Bases de Datos Factuales , Femenino , Fluidoterapia/métodos , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Factores de Riesgo , Hemorragia Subaracnoidea/epidemiologíaRESUMEN
OBJECTIVE: The goal of this study was to review the efficacy and safety of different surgical techniques used for treatment of Chiari malformation type I (CM-I) in children. METHODS: The authors retrospectively reviewed 437 consecutive children surgically treated for CM-I. Procedures were classified into four groups: bone decompression (posterior fossa decompression [PFD]) and duraplasty (PFD with duraplasty [PFDD]), PFDD with arachnoid dissection (PFDD+AD), PFDD with tonsil coagulation of at least one cerebellar tonsil (PFDD+TC), and PFDD with subpial tonsil resection of at least one tonsil (PFDD+TR). Efficacy was measured as a greater than 50% reduction in the syrinx by length or anteroposterior width, patient-reported improvement in symptoms, and rate of reoperation. Safety was measured as the rate of postoperative complications. RESULTS: The mean patient age was 8.4 years (range 3 months to 18 years). In total, 221 (50.6%) patients had syringomyelia. The mean follow-up was 31.1 months (range 3-199 months), and there was no statistically significant difference between groups (p = 0.474). Preoperatively, univariate analysis showed that non-Chiari headache, hydrocephalus, tonsil length, and distance from the opisthion to brainstem were associated with the surgical technique used. Multivariate analysis demonstrated that hydrocephalus was independently associated with PFD+AD (p = 0.028), tonsil length was independently associated with PFD+TC (p = 0.001) and PFD+TR (p = 0.044), and non-Chiari headache was inversely associated with PFD+TR (p = 0.001). In the treatment groups postoperatively, symptoms improved in 57/69 (82.6%) PFDD patients, 20/21 (95.2%) PFDD+AD patients, 79/90 (87.8%) PFDD+TC patients, and 231/257 (89.9%) PFDD+TR patients, and differences between groups were not statistically significant. Similarly, there was no statistically significant difference in postoperative Chicago Chiari Outcome Scale scores between groups (p = 0.174). Syringomyelia improved in 79.8% of PFDD+TC/TR patients versus only 58.7% of PFDD+AD patients (p = 0.003). PFDD+TC/TR remained independently associated with improved syrinx outcomes (p = 0.005) after controlling for which surgeon performed the operation. For those patients whose syrinx did not resolve, no statistically significant differences between surgery groups were observed in the length of follow-up or time to reoperation. Overall, there was no statistically significant difference between groups in postoperative complication rates, including aseptic meningitis and CSF- and wound-related issues, or reoperation rates. CONCLUSIONS: In this single-center retrospective series, cerebellar tonsil reduction, by either coagulation or subpial resection, resulted in superior reduction of syringomyelia in pediatric CM-I patients, without increased complications.
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Here we report a case of a 14-week-old girl with a history of intrauterine drug exposure and hypoxic ischemic encephalopathy secondary to cardiac arrest requiring prolonged resuscitation at birth presented with irritability and a bulging anterior fontanelle. After neurosurgical resection, pathologic examination showed fungal hyphae, and Epicoccum nigrum was detected by fungal PCR and sequencing. To our knowledge, this is the first reported case of a central nervous system infection due to Epicoccum nigrum.
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OBJECTIVE Large myelomeningocele defects and poor surrounding tissue quality make some defects particularly difficult to close primarily. This paper describes the superior gluteal artery perforator (SGAP) flap technique for defect closure and long-term clinical outcomes. METHODS The technique for closing a myelomeningocele with an SGAP flap is described. A retrospective chart review was performed on a cohort of 11 patients who underwent closure in this manner. RESULTS Between 1999 and 2015, 271 myelomeningoceles were closed, 11 of which were SGAP flap closures. The mean defect size was 5.5 × 7.2 cm. All patients underwent ventriculoperitoneal shunting. There were no cases of CSF infection. Five patients had minor wound issues (small dehiscence or eschar formation) that healed satisfactorily. Two patients had soft-tissue wound infections and required multiple revisions; one patient had multiple severe developmental abnormalities, and the other patient's flap had healed with a thick underlying fat pad 4 months postoperatively. No patients had significant surgical site pain on long-term follow-up. CONCLUSIONS The SGAP flap technique achieves tension-free closure with vascularized, fat-bearing full-thickness skin. It is useful for closure of large, complex defects, is not associated with chronic pain, and carries a morbidity risk that is comparable to other complex myelomeningocele closure techniques.