[Extremely preterm infants with severe intraventricular hemorrhage: neurological evolution and long term and educational status]. / Niños muy prematuros con hemorragia intraventricular extensa: evolución neurológica y escolaridad a largo plazo.

Extensive intraventricular hemorrhage (IVH) in very preterm newborns (VPNB) is associated with mortality and severe long-term neurological sequelae. OBJECTIVES: To know the most frequent neurological pathologies associated with extensive IVH, to determine the functional outcomes of mobility in the motor area and intellectual capacity in the cognitive area, to analyze the association between both areas and to know the schooling achieved. PATIENTS AND METHOD: Descriptive and longitudinal study in VPNB with extensive IVH born between 2001 and 2014. They underwent protocolized neurological follow-up until school age. The functional outcomes in mobility and intellectual capacity were categorized into 4 levels: level 1 corresponds to good functionality and autonomy; level 2, functionality that allows independence, with support in some tasks; level 3 requires constant external support; and level 4 where there is total dependence. The association was analyzed using Chi-square and Cramer's V coefficient. RESULTS: 74 children completed the follow-up; the most frequent associated neurological pathologies were neurodevelopmental disorders, hypertensive hydrocephalus, and epilepsy. Independent mobility (normal or with limitations) reached 74.4% while 24.3% used wheelchairs. 51.3% was categorized as normal to borderline intellectual range, 12.2% as mild intellectual disability (ID), 17.6% as moderate ID, and 19.9% as severe to profound ID. There was a strong statistical association between functional levels of mobility and intellectual capacity (p < 0.000 and V = 0.62). Schooling was proportional to intellectual capacity: 56.8% attended regular schools, 27.0% attended special schools, and 16.2% had no schooling. CONCLUSIONS: 2/3 VPNB with extensive IVH showed positive functional outcomes, from normal to mild limitations that allow an almost autonomous life; in 1/3 the outcomes were unfavorable in mobility and cognitive performance, and there was a strong statistical correlation between both areas studied. Schooling was consistent with the intellectual level.

Optimal intraventricular hemorrhage volume cutoff for predicting poor outcome in patients with intracerebral hemorrhage.

BACKGROUND AND OBJECTIVES: The prognosis of patients with spontaneous intracerebral hemorrhage (ICH) is often influenced by hematoma volume, a well-established predictor of poor outcome. However, the optimal intraventricular hemorrhage (IVH) volume cutoff for predicting poor outcome remains unknown. METHODS: We analyzed 313 patients with spontaneous ICH not undergoing evacuation, including 7 cases with external ventricular drainage (EVD). These patients underwent a baseline CT scan, followed by a 24-hour CT scan for measurement of both hematoma and IVH volume. We defined hematoma growth as hematoma growth > 33 % or 6 mL at follow-up CT, and poor outcome as modified Rankin Scale score≥3 at three months. Cutoffs with optimal sensitivity and specificity for predicting poor outcome were identified using receiver operating curves. RESULTS: The receiver operating characteristic analysis identified 6 mL as the optimal cutoff for predicting poor outcome. IVH volume> 6 mL was observed in 53 (16.9 %) of 313 patients. Patients with IVH volume>6 mL were more likely to be older and had higher NIHSS score and lower GCS score than those without. IVH volume>6 mL (adjusted OR 2.43, 95 % CI 1.13-5.30; P = 0.026) was found to be an independent predictor of poor clinical outcome at three months in multivariable regression analysis. CONCLUSIONS: Optimal IVH volume cutoff represents a powerful tool for improving the prediction of poor outcome in patients with ICH, particularly in the absence of clot evacuation or common use of EVD. Small amounts of intraventricular blood are not independently associated with poor outcome in patients with intracerebral hemorrhage. The utilization of optimal IVH volume cutoffs may improve the clinical trial design by targeting ICH patients that will obtain maximal benefit from therapies.

Development and validation of a diagnostic prediction model for severe periventricular-intraventricular hemorrhage in newborns: insights from a retrospective analysis utilizing the MIMIC-III database.

OBJECTIVE: Periventricular-intraventricular hemorrhage is the most common type of intracranial bleeding in newborns, especially in the first 3 days after birth. Severe periventricular-intraventricular hemorrhage is considered a progression from mild periventricular-intraventricular hemorrhage and is often closely associated with severe neurological sequelae. However, no specific indicators are available to predict the progression from mild to severe periventricular-intraventricular in early admission. This study aims to establish an early diagnostic prediction model for severe PIVH. METHOD: This study was a retrospective cohort study with data collected from the MIMIC-III (v1.4) database. Laboratory and clinical data collected within the first 24 h of NICU admission have been used as variables for both univariate and multivariate logistic regression analyses to construct a nomogram-based early prediction model for severe periventricular-intraventricular hemorrhage and subsequently validated. RESULTS: A predictive model was established and represented by a nomogram, it comprised three variables: output, lowest platelet count and use of vasoactive drugs within 24 h of NICU admission. The model's predictive performance showed by the calculated area under the curve was 0.792, indicating good discriminatory power. The calibration plot demonstrated good calibration between observed and predicted outcomes, and the Hosmer-Lemeshow test showed high consistency (p = 0.990). Internal validation showed the calculated area under a curve of 0.788. CONCLUSIONS: This severe PIVH predictive model, established by three easily obtainable indicators within the NICU, demonstrated good predictive ability. It offered a more user-friendly and convenient option for neonatologists.

The role of the brain-sparing effect of growth-restricted fetuses in newborn germinal matrix/intraventricular hemorrhage.

OBJECTIVES: The aim of the study was to evaluate the effect of the brain-sparing effect (BSE) of fetal growth restriction (FGR) in newborn germinal matrix/intraventricular hemorrhage (GM/IVH). METHODS: A total of 320 patients who delivered prior to the 34th gestational week were analyzed from data records. 201 patients were divided into two groups according to cerebro-placental ratio (CPR): early fetal growth restriction (FGR) with abnormal CPR group (n=104) and appropriate for gestational age with normal Doppler group (control) (n=97). Using the normal middle cerebral artery (MCA) Doppler as a reference, multivariate logistic regression analysis was used to assess the association between the BSE and the primary outcome. RESULTS: The rate of Grade I-II germinal matrix/intraventricular hemorrhage (GM/IVH) was 31(29.8%) in the group possessing early FGR with abnormal CPR and 7(7.2%) in the control group, showing a statistically significant difference. The rate of grade III-IV GM/IVH was 7(6.7%) in the group possessing early FGR with abnormal CPR and 2 (2.1%) in the control group, showing no statistically significant difference. We found that gestational age at delivery <32 weeks was an independent risk factor for GM/IVH. In addition, we found that other variables such as the presence of preeclampsia, fetal weight percentile <10, emergency CS delivery, 48-h completion after the first steroid administration and 24-h completion rate after MgSO4 administration were not independently associated with the primary outcome. CONCLUSIONS: Our results indicate that the rate of GM-IVH was increased in the group possessing early FGR with abnormal CPR; however, multivariate logistic regression analysis showed that BSE was not an independent risk factor for GM/IVH.

Efficiency of a deep learning-based artificial intelligence diagnostic system in spontaneous intracerebral hemorrhage volume measurement.

BACKGROUND: Accurate measurement of hemorrhage volume is critical for both the prediction of prognosis and the selection of appropriate clinical treatment after spontaneous intracerebral hemorrhage (ICH). This study aimed to evaluate the performance and accuracy of a deep learning-based automated segmentation algorithm in segmenting spontaneous intracerebral hemorrhage (ICH) volume either with or without intraventricular hemorrhage (IVH) extension. We compared this automated pipeline with two manual segmentation techniques. METHODS: We retrospectively reviewed 105 patients with acute spontaneous ICH. Depending on the presence of IVH extension, patients were divided into two groups: ICH without (n = 56) and with IVH (n = 49). ICH volume of the two groups were segmented and measured using a deep learning-based artificial intelligence (AI) diagnostic system and computed tomography-based planimetry (CTP), and the ABC/2 score were used to measure hemorrhage volume in the ICH without IVH group. Correlations and agreement analyses were used to analyze the differences in volume and length of processing time among the three segmentation approaches. RESULTS: In the ICH without IVH group, the ICH volumes measured using AI and the ABC/2 score were comparable to CTP segmentation. Strong correlations were observed among the three segmentation methods (r = 0.994, 0.976, 0.974; P < 0.001; concordance correlation coefficient [CCC] = 0.993, 0.968, 0.967). But the absolute error of the ICH volume measured by the ABC/2 score was greater than that of the algorithm (P < 0.05). In the ICH with IVH group, there is no significant differences were found between algorithm and CTP(P = 0.614). The correlation and agreement between CTP and AI were strong (r = 0.996, P < 0.001; CCC = 0.996). The AI segmentation took a significantly shorter amount of time than CTP (P < 0.001), but was slightly longer than ABC/2 score technique (P = 0.002). CONCLUSIONS: The deep learning-based AI diagnostic system accurately quantified volumes of acute spontaneous ICH with high fidelity and greater efficiency compared to the CTP measurement and more accurately than the ABC/2 scores. We believe this is a promising tool to help physicians achieve precise ICH quantification in practice.

Complicaciones en la hemorragia intraventricular del recién nacido, a propósito de un caso / Complications in the intraventricular hemorrhage of the newborn, a propos of a case

RESUMEN La hemorragia interventricular es una complicación frecuente en el recién nacido prematuro. Se presentó el caso con el objetivo de describir las complicaciones en la hemorragia intraventricular en el recién nacido. Se trató de una recién nacida, producto de un embarazo de 30 semanas de gestación, con peso al nacer de 1 600 g. Desarrolló una hemorragia intraventricular e hidrocefalia que requirió diferentes intervenciones neuroquirúrgicas y desarrolló complicaciones sépticas graves. La ventriculitis y los abscesos cerebrales fueron las complicaciones más peligrosas. Se realizaron lavados ventriculares. Se utilizó antibióticos intraventriculares y también antibioticoterapia sistémica, cambios frecuentes de catéter de derivación al exterior y permanencia de una derivación ventricular externa por 102 días. Después de seis meses de evolución, de más de veinte intervenciones quirúrgicas, y de haber sufrido severas complicaciones sépticas, se logró realizar la derivación ventrículo-peritoneal definitiva, lográndose su egreso. Al año de vida, la paciente mantiene un desarrollo psicomotor adecuado (AU).

ABSTRACT The interventricular hemorrhage is a frequent complication in the premature newborn baby. The case was presente with the aim of describing the complications of intraventricular hemorrhage in the newborn baby. It dealed with a female newborn baby, product of a 30 weeks pregnancy, with weight at birth of 1 600 g. She developed intraventricular hemorrhage and hydrocephalus that required different neurosurgical interventions and developed serious septic complications. Ventriculitis and brain abscesses were the most dangerous complications. Ventricular lavages were performed. Intraventricular antibiotics were used as well as systemic antibiotic therapy, frequent changes of bypass catheter to the exterior and permanence of an external ventricular bypass for 102 days. And permanence of an external ventricular shunt for 102 days. After six months of evolution, more than twenty surgical interventions, and having suffered severe septic complications, it was possible to perform the definitive ventricular-peritoneal bypass, achieving her discharge. At a year of life, the patient maintains adequate psychomotor development (AU).

Predicting intraventricular hemorrhage growth with a machine learning-based, radiomics-clinical model.

We constructed a radiomics-clinical model to predict intraventricular hemorrhage (IVH) growth after spontaneous intracerebral hematoma. The model was developed using a training cohort (N=626) and validated with an independent testing cohort (N=270). Radiomics features and clinical predictors were selected using the least absolute shrinkage and selection operator (LASSO) method and multivariate analysis. The radiomics score (Rad-score) was calculated through linear combination of selected features multiplied by their respective LASSO coefficients. The support vector machine (SVM) method was used to construct the model. IVH growth was experienced by 13.4% and 13.7% of patients in the training and testing cohorts, respectively. The Rad-score was associated with severe IVH and poor outcome. Independent predictors of IVH growth included hypercholesterolemia (odds ratio [OR], 0.12 [95%CI, 0.02-0.90]; p=0.039), baseline Graeb score (OR, 1.26 [95%CI, 1.16-1.36]; p<0.001), time to initial CT (OR, 0.70 [95%CI, 0.58-0.86]; p<0.001), international normalized ratio (OR, 4.27 [95%CI, 1.40, 13.0]; p=0.011), and Rad-score (OR, 2.3 [95%CI, 1.6-3.3]; p<0.001). In the training cohort, the model achieved an AUC of 0.78, sensitivity of 0.83, and specificity of 0.66. In the testing cohort, AUC, sensitivity, and specificity were 0.71, 0.81, and 0.64, respectively. This radiomics-clinical model thus has the potential to predict IVH growth.

Treatment of a self-inflicted intracranial nail gun injury.

A 30-year-old man walked into the emergency department after a suicide attempt by firing a nail from a pneumatic nail gun directed at his left temple. He was haemodynamically stable and neurologically intact, able to recall all events and moving all extremities with a Glascow Coma Scale of 15. CT of the brain showed a 6.3 cm nail in the right frontal region without major intracerebral vessel disruption. He was taken to the operating room for left temporal wound washout, debridement of gross contamination and closure with titanium cranial fixation plate. The foreign body was not accessible on initial surgical intervention and was left in place to define anatomy and plan for subsequent removal. Thin slice CT images were used to create 3D reconstructions to facilitate stereotactic navigation and foreign body removal via right craniotomy the following day. The patient tolerated the procedures well and recovered with full neurological function.

Antenatal diagnosis of fetal intraventricular hemorrhage: systematic review and meta-analysis.

AIM: To determine how the severity of antenatally diagnosed germinal matrix-intraventricular hemorrhage (GMH-IVH) relates to morbidity and mortality, and to explore potential risk factors. METHOD: We conducted a systematic review and individual patient data meta-analysis of antenatally diagnosed fetal GMH-IVH. The primary outcomes were mortality and morbidity. Potential associations with clinical factors during pregnancy were explored. Analysis employed Fisher's exact test and logistic regression. RESULTS: We included 240 cases from 80 studies. Presence of venous infarction was associated with mortality (odds ratio [OR] 4.3, 95% confidence interval [CI] 1.4-13.25), motor impairment (OR 103.2, 95% CI 8.6-1238), epilepsy (OR 6.46, 95% CI 2.64-16.06), and developmental delay (OR 8.55, 95% CI 2.12-48.79). Shunt placement was associated with gestational age at GMH-IVH diagnosis and in utero progression. Many cases had uncomplicated pregnancies but possible co-occurring conditions included twin gestation, small for gestational age, and congenital anomalies. INTERPRETATION: Severity of fetal GMH-IVH, specifically venous infarction, is associated with overall mortality and morbidity. Risk factors for fetal GMH-IVH are poorly understood and controlled studies are required. WHAT THIS PAPER ADDS: Preterm germinal matrix-intraventricular hemorrhage (GMH-IVH) grading can be applied to fetuses. Many fetal germinal matrix hemorrhages occur in otherwise typical pregnancies. Half of fetuses with post-hemorrhagic ventricular dilatation receive a shunt after delivery. Fetuses with grade I or II GMH-IVH have few sequelae. Fetuses with periventricular hemorrhagic infarction have a high burden of motor impairment.

Predictive Value of Early Amplitude Integrated EEG in Extremely Premature Infants.

Amplitude integrated EEG (aEEG) is increasingly utilized in preterm infants. The aim of the study was to evaluate whether semiquantitative visual assessment of aEEG background during the first 72 hours of life is associated with long-term outcome in a group of premature infants born less than 28 weeks' gestation. Infants were prospectively enrolled and monitored in the first 72 hours after birth. aEEG was classified daily according to background activity, appearance of cyclical activity and presence of seizures activity. Log-rank and multivariable cox analysis were used to explore associations of background aEEG activity with short and long-term outcome. Overall, 51 infants were enrolled into the study. Depressed aEEG background on the third day of life was associated with poor outcome (P = .028). Similarly, absence of cycling on the third day of life was associated with death or poor outcome (P = .004 and .012, respectively). In different multivariable models adjusted for gestational age, severe intraventricular hemorrhage or use of sedative medication, neither background nor cycling activities were associated with outcome. Depressed aEEG background and absence of aEEG cycling on the third day of life are associated with poor outcome in univariable analysis. Although continuous aEEG monitoring of premature infants can provide real-time assessment of cerebral function, its use as a predictive tool for long-term outcome using visual analysis requires caution as its predictive power is not greater than that of gestational age or intraventricular hemorrhage.

Assessing key clinical parameters before and after intraventricular hemorrhage in very preterm infants.

Intraventricular cerebral hemorrhage (IVH) is one of the most severe complications of premature birth, potentially leading to lifelong disability. The purpose of this paper is the assessment of the evolution of three of the most relevant parameters, before and after IVH: mean arterial pressure (MAP), arterial carbon dioxide pressure (pCO2), and cerebral blood flow (CBF). Clinical records of 254 preterm infants with a gestational age of 23-30 weeks, with and without a diagnosis of IVH, were reviewed for MAP and arterial pCO2 in the period up to 7 days before and 3 days after IVH or during the first 10 days of life in cases without IVH.Conclusion: A statistically significant increase in pCO2 and decrease in MAP in patients with IVH compared with those without were detected. Both the mean values and the mean absolute deviations of CBF were computed in this study, and the latter was significantly higher than in control group. High deviations of CBF, as well as hypercapnia and hypotension, are likely to contribute to the rupture of cerebral blood vessels in preterm infants, and consequently, to the development of IVH.What is Known:• The origin of IVH is multifactorial, but mean arterial pressure, carbon dioxide partial pressure, and cerebral blood flow are recognized as the most important parameters.• In premature infants, the autoregulation mechanisms are still underdeveloped and cannot compensate for cerebral blood flow fluctuations.What is New:• The numerical simulation of CBF is shown to be a promising approach that may be useful in the care of preterm infants.• The mean values of CBF before and after IVH in the affected group were similar to those in the control group, but the mean absolute deviations of CBF in the affected group before and after IVH were significantly higher than that in the control group.

Outcomes at 18 to 22 Months of Corrected Age for Infants Born at 22 to 25 Weeks of Gestation in a Center Practicing Active Management.

OBJECTIVE: To assess the outcomes in actively managed extremely preterm infants after admission to a neonatal intensive care unit. STUDY DESIGN: Retrospective cohort of 255 infants born at 22-25 weeks of gestation between 2006 and 2015 at a single study institution. Infants were excluded for congenital anomaly, death in delivery room, or parental request for palliation (n = 7). Neurodevelopmental outcomes were analyzed for 169 of 214 survivors (78.9%) at 18-22 months of corrected age. Outcomes were evaluated using the Mann-Whitney U, χ2, or Fisher exact test, where appropriate. In addition, cognitive scores of the Bayley Scales of Infant-Toddler Development (3rd edition) were assessed using generalized estimating equations. RESULTS: Seventy infants born at 22-23 weeks of gestation (22 weeks, n = 20; 23 weeks, n = 50) and 178 infants born at 24-25 weeks of gestation (24 weeks, n = 79; 25 weeks, n = 99 infants) were included. Survival to hospital discharge of those surviving to NICU admission was 78% (55/70; 95% CI, 69%-88%) at 22-23 weeks and 89% (159/178; 95% CI, 84%-93% at 24-25 weeks; P = .02). No or mild neurodevelopmental impairment in surviving infants was 64% (29/45; 95% CI, 50%-77%) at 22-23 weeks and 76% (94/124; 95% CI, 68%-83%; P = .16) at 24-25 weeks. CONCLUSIONS: Although survival was lower in infants born at 22-23 weeks than at 24-25 weeks of gestation, the majority of survivors in both groups had positive outcomes with no or mild neurodevelopmental impairments. Further evaluation of school performance is warranted.

Modified behavioural tests to detect white matter injury- induced motor deficits after intracerebral haemorrhage in mice.

Motor function deficit induced by white matter injury (WMI) is one of the most severe complications of intracerebral haemorrhage (ICH). The degree of WMI is closely related to the prognosis of patients after ICH. However, the current behavioural assessment of motor function used in the ICH mouse model is mainly based on that for ischaemic stroke and lacks the behavioural methods that accurately respond to WMI. Here, a series of easy-to-implement behavioural tests were performed to detect motor deficits in mice after ICH. The results showed that the grip strength test and the modified pole test not only can better distinguish the degree of motor dysfunction between different volumes of blood ICH models than the Basso Mouse Scale and the beam walking test but can also accurately reflect the severity of WMI characterized by demyelination, axonal swelling and the latency of motor-evoked potential delay induced by ICH. In addition, after ICH, the results of grip tests and modified pole tests, rather than the Basso Mouse Scale and the beam walking test, were worse than those observed after intraventricular haemorrhage (IVH), which was used as a model of brain haemorrhage in non-white matter areas. These results indicate that the grip strength test and the modified pole test have advantages in detecting the degree of motor deficit induced by white matter injury after ICH in mice.

Early caloric deprivation in preterm infants affects Bayley-III scales performance at 18-24 months of corrected age.

BACKGROUND: Adequate nutrition is essential for optimal neurodevelopment to preterm infants. Our aim was to evaluate the impact of caloric deprivation on Bayley-III scales performance at 18-24 months of corrected age, in a cohort of preterm infants. METHODS: We prospectively enrolled infants with gestational age <30 weeks and birth weight <1500 g. Apart from a whole cohort analysis, we performed a subgroup analysis between infants received inadequate calories (<85 Kcal/kg/day) during the first two weeks of age, compared to a standard nutrition group. All infants underwent a Bayley-III assessment at 18-24 months of corrected age. RESULTS: From the 63 preterm infants analysed, 25% had caloric deprivation compared to 75% with adequate nutrition. Caloric deprived infants were of lower gestational age and birth weight, and received a lower amount of enteral feeding during the first 14 days of age. There were no differences between the two groups regarding the common neonatal co-morbidities. Caloric deprived infants had significantly lower composite index scores at 18-24 months of corrected age. Caloric deprivation, late onset sepsis, necrotizing enterocolitis, and bronchopulmonary dysplasia were significant risk factors of neurodevelopmental impairment. CONCLUSIONS: Several neonatal factors affect the neurodevelopmental outcome of preterm infants, and nutrition may pose an important role.

Influence of the Extent of Intraventricular Hemorrhage on Functional Outcome and Mortality in Intracerebral Hemorrhage.

BACKGROUND AND OBJECTIVE: Intraventricular hemorrhage (IVH) is a verified independent prognostic parameter in patients with intracerebral hemorrhage (ICH). However, the impact of the extent of IVH on clinical outcomes is unestablished. METHODS: We analyzed 1,112 consecutive primary ICH patients of the UKER-ICH cohort (NCT03183167) and hypothesized that there is no difference in outcome between patients without IVH and patients with minor IVH not leading to obstructive hydrocephalus. Propensity score matching and multivariable analyses were performed to account for imbalances in baseline characteristics. Primary outcome was defined as functional outcome 3 months after ICH -assessed using the modified Rankin Scale (mRS) dichotomized into favorable (mRS = 0-3) and unfavorable outcome (mRS = 4-6). Secondary outcomes included mortality at 3  months and a Graeb score-based threshold analysis for association of the extent of IVH with unfavorable clinical outcome. RESULTS: Among the 461 out of 1,112 (41.5%) ICH patients with IVH, 191 out of 461 (41.4%) showed IVH without obstructive hydrocephalus and no requirement of external ventricular drain (EVD) placement. After adjusting for baseline imbalances we found no difference in functional outcome at 3 months between patients without IVH (No-IVH) and patients with IVH not requiring EVD (IVH-w/o-EVD): mRS 0-3: No-IVH 64/161 (39.8%) vs. IVH-w/o-EVD 53/170 (31.2%); p = 0.103. However, there was a trend toward a higher mortality in IVH-w/o-EVD patients (mRS 6: No IVH 40/161 [24.8%] vs. IVH-w/o-EVD 57/170 [33.5%]; p = 0.083). Multivariable analysis revealed that a Graeb score >2 was independently associated with unfavorable outcome (mRS 4-6: OR 3.16 [1.54-6.48]; p = 0.002), and higher mortality (mRS 6: OR 2.57 [1.40-4.74]; p = 0.002) in IVH patients. CONCLUSIONS: Small amounts of intraventricular blood (Graeb score ≤2) not leading to obstructive hydrocephalus are not associated with unfavorable outcome or death after ICH. Thus, IVH per se should not be considered a binary variable in outcome prediction for ICH patients.

Intraventricular hemorrhage and posthemorrhagic hydrocephalus in preterm infants: diagnosis, classification, and treatment options.

PURPOSE: Intraventricular hemorrhage is the most important adverse neurologic event for preterm and very low weight birth infants in the neonatal period. This pathology can lead to various delays in motor, language, and cognition development. The aim of this article is to give an overview of the knowledge in diagnosis, classification, and treatment options of this pathology. METHOD: A systematic review has been made. RESULTS: The cranial ultrasound can be used to identify the hemorrhage and grade it according to the modified Papile grading system. There is no standardized protocol of intervention as there are controversial results on which of the temporizing neurosurgical procedures is best and about the appropriate parameters to consider a conversion to ventriculoperitoneal shunt. However, it has been established that the most important prognosis factor is the involvement and damage of the white matter. CONCLUSION: More evidence is required to create a standardized protocol that can ensure the best possible outcome for these patients.

Genotype-Phenotype Correlation in Long-Term Cohort of Japanese Patients with Moyamoya Disease.

BACKGROUND: Homozygosity of this p.R4810K founder variant of RNF213moyamoya disease (MMD) susceptibility gene is known to influence the severity of the clinical disease phenotype at disease onset. However, the association between this genotype and long-term clinical manifestations has remained unclear. OBJECTIVES: The principal goal of this study was to investigate whether and how the p.R4810K variant of RNF213influences the long-term phenotype in Japanese patients with MMD. METHOD: This retrospective cohort study included 94 Japanese patients with MMD who underwent direct or combined bypass for revascularization with the p.R4810K genotype determined in our hospital. The following phenotypic parameters were analyzed at disease onset and over a long-term period: age and initial presentation at onset, recurrent stroke after initial revascularization, and final modified Rankin Scale. RESULTS: The p.R4810K genotype was significantly associated with the phenotype at onset, especially in younger patients. Over a median follow-up period of 100 months, recurrent stroke occurred in 6 out of 94 patients: none out of 5 patients with the homozygous variant, 5 out of 64 with the heterozygous variant, and 1 out of 25 in the wild-type group. There were no significant differences among the genotypes. In particular, recurrent cerebral hemorrhage occurred in 5 patients, all possessing the heterozygous variant. The log-rank test showed no difference between the genotypes in the stroke-free survival rate. Furthermore, the p.R4810K genotype was not associated with a poor functional condition. CONCLUSIONS: The p.R4810K founder variant of RNF213 affects the phenotype at disease onset. However, the optimal revascularization may be effective, regardless of the genotype, even for the homozygous variant, which has been thought to be the most pathogenic. This genotype may not strongly influence the long-term clinical manifestations or poor prognosis in MMD.

Clinical Value of Neutrophil-to-Lymphocyte Ratio in Primary Intraventricular Hemorrhage.

OBJECTIVES: The neutrophil-to-lymphocyte ratio (NLR) reflects the balance between innate and adaptive inflammatory responses. This study intended to evaluate parameters associated with admission high NLR and its impact on clinical outcome in patients with primary intraventricular hemorrhage (PIVH). METHODS: This study retrospectively analyzed consecutive patients with PIVH without a history of head trauma or parenchymal/subarachnoid hemorrhage on computed tomography scan between 2010 and 2016 in a single center. Clinical outcomes at discharge and 90 days were assessed with the modified Rankin Score (mRS) and dichotomized as good (mRS 0-2) and poor (mRS 3-6) outcomes. Associations were estimated using multivariable logistic regression. RESULTS: We had 171 patients with PIVH included in the present study. There were 94 male (55.0%) and 77 female (45.0%) patients, with an average age of 46.1 ± 17.2 years. Multivariable logistic analyses revealed independent associations of high NLR (≥8.25) with higher Graeb score, Moyamoya disease, higher admission blood glucose level, and lower platelet count. The patients with high NLR had poorer outcome at discharge and 90 days. After adjustment, the patients with high NLR tended to be associated with poor outcome both at discharge and 90 days. In addition, NLR exhibited a superior predictive power of pneumonia in PIVH than absolute neutrophil count and white blood cell count. CONCLUSIONS: NLR tended to be associated with 90-day clinical outcomes of patients with PIVH and exhibited independent predictive power for pneumonia in PIVH.

Neonatal Outcomes of Very Low Birth Weight Infants in Korean Neonatal Network from 2013 to 2016.

BACKGROUND: This study was performed to determine survival and morbidity rates in very low birth weight infants (VLBWIs) in the Korean Neonatal Network (KNN), and to compare neonatal outcomes with those in other countries. METHODS: Data were collected for 8,269 VLBWIs with gestational age (GA) ≥ 22 weeks who were born between January 1, 2013 and December 31, 2016, and admitted to the neonatal intensive care units of the KNN. RESULTS: The survival rate of all VLBWIs and of infants with GA 22-23, 24-25, 26-27, 28-29, 30-32, and > 32 weeks were 86% (total), 33%, 65%, 84%, 94%, 97%, and 98%, respectively. The bronchopulmonary dysplasia (BPD) rates of all VLBWIs and of infants with GA 22-23, 24-25, 26-27, 28-29, 30-32, and > 32 weeks were 30% (total), 88%, 64%, 47%, 26%, 14%, and 5%, respectively. The intraventricular hemorrhage rates (≥ grade III) of all VLBWIs and of infants with GA 22-23, 24-25, 26-27, 28-29, 30-32, and > 32 weeks were 10% (total), 45%, 27%, 12%, 5%, 2%, and 1%, respectively. In an international comparison, the survival rate of VLBWIs with GA 24-27 weeks in KNN was lower, and the BPD rate of VLBWIs in the KNN was higher than that of the neonatal networks of other countries. CONCLUSION: Despite overall improvements in neonatal outcomes, the survival and morbidity rates of more immature infants with GA 22-27 weeks need further improvement. Therefore, it would be necessary to develop more optimal treatment strategies and perform more active quality improvement to further improve neonatal outcomes of VLBWIs in Korea.