scholarly journals Impact of Brain Malformations on Neurodevelopmental Outcome in Children with a History of Prenatal Surgery for Open Spina Bifida

2021 ◽  
pp. 1-8
Author(s):  
David-Alexander Wille ◽  
Beth Padden ◽  
Ueli Moehrlen ◽  
Beatrice Latal ◽  
Sonja Schauer ◽  
...  

<b><i>Introduction:</i></b> This retrospective study investigates brain malformations and their impact on neurodevelopmental outcome in children after prenatal surgery for spina bifida (SB). <b><i>Methods:</i></b> Sixty-one patients were included. On neonatal MRI, SB-associated brain malformations were assessed. Ventricular size, ventriculo-peritoneal shunt (VPS), and endoscopic third ventriculostomy (ETV) were also documented. Neurodevelopment was assessed with the Bayley-III and correlated with brain malformations, ventricular size, and VPS/ETV placement. <b><i>Results:</i></b> Chiari II malformation was detected in all patients. Corpus callosum (CC) abnormality was noted in 40%, heterotopies in 35%, and cerebellar parenchymal defects in 11%. 96% had ventriculomegaly; in 46%, VPS/ETV was performed. Cognitive and language testing yielded results in the low-average range (Bayley-III: Cognitive Composite Score 93.6, Language Composite Score 89.7), motor testing was below average (Motor Composite Score 77.4). CC abnormalities, heterotopies, and cerebellar defects were not associated with poorer Bayley-III scores, whereas patients with severe ventriculomegaly performed poorer in all subtests, significantly so for the language composite score. Patients requiring intervention for hydrocephalus had significantly lower scores in motor testing. <b><i>Discussion/Conclusion:</i></b> Additional brain malformations in open SB do not seem to have an impact on cognitive function at 2 years of age. Severe ventriculomegaly is a risk factor for poorer cognitive outcome; hydrocephalus surgery adds an additional risk for delayed motor function.

2021 ◽  
pp. 1-11
Author(s):  
Mounica Paturu ◽  
Regina L. Triplett ◽  
Siddhant Thukral ◽  
Dimitrios Alexopoulos ◽  
Christopher D. Smyser ◽  
...  

OBJECTIVE Posthemorrhagic hydrocephalus (PHH) is associated with significant morbidity, smaller hippocampal volumes, and impaired neurodevelopment in preterm infants. The timing of temporary CSF (tCSF) diversion has been studied; however, the optimal time for permanent CSF (pCSF) diversion is unknown. The objective of this study was to determine whether cumulative ventricle size or timing of pCSF diversion is associated with neurodevelopmental outcome and hippocampal size in preterm infants with PHH. METHODS Twenty-five very preterm neonates (born at ≤ 32 weeks’ gestational age) with high-grade intraventricular hemorrhage (IVH), subsequent PHH, and pCSF diversion with a ventriculoperitoneal shunt (n = 20) or endoscopic third ventriculostomy (n = 5) were followed until 2 years of age. Infants underwent serial cranial ultrasounds from birth until 1 year after pCSF diversion, brain MRI at term-equivalent age, and assessment based on the Bayley Scales of Infant and Toddler Development, Third Edition, at 2 years of age. Frontooccipital horn ratio (FOHR) measurements were derived from cranial ultrasounds and term-equivalent brain MRI. Hippocampal volumes were segmented and calculated from term-equivalent brain MRI. Cumulative ventricle size until the time of pCSF diversion was estimated using FOHR measurements from each cranial ultrasound performed prior to permanent intervention. RESULTS The average gestational ages at tCSF and pCSF diversion were 28.9 and 39.0 weeks, respectively. An earlier chronological age at the time of pCSF diversion was associated with larger right hippocampal volumes on term-equivalent MRI (Pearson’s r = −0.403, p = 0.046) and improved cognitive (r = −0.554, p = 0.047), motor (r = −0.487, p = 0.048), and language (r = −0.414, p = 0.021) outcomes at 2 years of age. Additionally, a smaller cumulative ventricle size from birth to pCSF diversion was associated with larger right hippocampal volumes (r = −0.483, p = 0.014) and improved cognitive (r = −0.711, p = 0.001), motor (r = −0.675, p = 0.003), and language (r = −0.618, p = 0.011) outcomes. There was no relationship between time to tCSF diversion or cumulative ventricle size prior to tCSF diversion and neurodevelopmental outcome or hippocampal size. Finally, a smaller cumulative ventricular size prior to either tCSF diversion or pCSF diversion was associated with a smaller ventricular size 1 year after pCSF diversion (r = 0.422, p = 0.040, R2 = 0.178 and r = 0.519, p = 0.009, R2 = 0.269, respectively). CONCLUSIONS In infants with PHH, a smaller cumulative ventricular size and shorter time to pCSF diversion were associated with larger right hippocampal volumes, improved neurocognitive outcomes, and reduced long-term ventriculomegaly. Future prospective randomized studies are needed to confirm these findings.


Author(s):  
Polly Kellner ◽  
Jenny Kwon ◽  
Joan Smith ◽  
Roberta G Pineda

Objective: To 1) define the prevalence of motor, cognitive, and language delays in preterm infants born < 32 weeks estimated gestational age (EGA) and 2) identify the relationship between the timing of discharge from the NICU and neurodevelopmental outcome in early childhood. Study Design: This retrospective study of 176 preterm infants born < 32 weeks EGA and hospitalized in a level IV NICU captured medical factors, including timing of discharge, from the NICU stay. Standardized developmental testing at 1-2 years corrected age was conducted in the newborn follow-up clinic. Results: At 1-2 years corrected age, the sample had an average cognitive composite score of 91.5 ± 17.4, language composite score of 84.5 ± 17.3, and motor composite score of 88.9 ± 18.4. Lower EGA at birth, necrotizing enterocolitis, patent ductus arteriosus, and oxygen requirement for > 28 days were independently associated with higher postmenstrual age (PMA) at NICU discharge. After controlling for known risk factors, higher PMA at discharge was associated with poorer cognitive outcome [p < 0.001, (-0.16, -0.07), β = -0.35], poorer language outcome [p = 0.049 (-0.10, 0.00), β = -0.15] and poorer motor outcome [p < 0.001, (-0.14, -0.05), β = -0.30]. Conclusion: Poorer cognitive, language, and motor outcomes were associated with longer hospitalization, even after controlling for medical risk factors known to be associated with poorer outcome. This provides further evidence for the potential role of the environment that may be impacting the developmental outcomes of infants hospitalized in the NICU.


2021 ◽  
pp. 109352662110405
Author(s):  
Fabienne Paschereit ◽  
Kim Hannah Schindelmann ◽  
Michael Hummel ◽  
Joanna Schneider ◽  
Gisela Stoltenburg-Didinger ◽  
...  

Introduction Spina bifida (SB) is the most common neural tube defect in humans. Here, we analyzed systematically the neuropathological findings of the brain in SB cases. Methods 79 cases with SB aperta (SBA) and 6 cases with SB occulta (SBO) autopsied at the Charité Neuropathology from 1974 to 2000 were re-evaluated retrospectively. For this, case files and spinal cord as well as brain sections were studied. Results While no brain malformations were detected in SBO cases, 95% of SBA cases had brain malformations. Main brain anomalies identified were hydrocephalus (71%), Chiari II malformation (36%), heterotopia (34%), other cerebellar anomalies (36%), gyrification defects (33%), and ependymal denudation (29%). Hydrocephalus was observed as early as gestational week 17 and was highly associated to Chiari II and ependymal denudation. In 55% SBA was accompanied by further anomalies not primarily affecting the CNS. Conclusion We confirm using neuropathologic methods brain malformations in most SBA but none in SBO cases. In addition to our previous radiologic study, we now demonstrate the high prevalence of cerebellar malformations and cerebral heterotopias in SBA. The early detection of hydrocephalus and Chiari II malformation in fetuses raises the question whether these arise parallel rather than in strict temporal sequence.


2020 ◽  
Vol 56 (S1) ◽  
pp. 145-145
Author(s):  
P.I. Cavoretto ◽  
M. Canini ◽  
M. Candiani ◽  
M. Caglioni ◽  
A. Falini ◽  
...  

Author(s):  
Zehra S Hepp ◽  
Verena M Haas ◽  
Beatrice Latal ◽  
Martin Meuli ◽  
Ueli Möhrlen ◽  
...  

2000 ◽  
Vol 92 (6) ◽  
pp. 915-919 ◽  
Author(s):  
Abhaya V. Kulkarni ◽  
James M. Drake ◽  
Derek C. Armstrong ◽  
Peter B. Dirks

Object. The goal of this study was to determine and compare imaging correlates in pediatric patients who underwent successful or failed endoscopic third ventriculostomies (ETVs). To this end, the authors measured ventricular size changes and the presence of cerebrospinal fluid (CSF) flow void in both groups of children following ETV.Methods. Images obtained in children with hydrocephalus immediately before and at least 30 days after having undergone ETV were reviewed by four independent observers (two blinded and two nonblinded). Each observer independently measured the frontal and occipital horn ratio ([FOR], a reliable and valid measure of ventricular size) and provided a subjective assessment of the presence of a flow void at the ETV site, the degree of periventricular edema, and the amount of CSF over the cerebral hemispheres.There were 29 children whose mean age was 6.6 years at the time of ETV and who had a mean postoperative follow-up period lasting 1.6 years. Postoperatively, the mean reduction in ventricular size (as measured using the FOR) was 7% (95% confidence interval [CI] 3–11%) in cases that were deemed failures (eight patients) and 16% (95% CI 12–20%) in clinically successful cases (21 patients). This reduction was significantly greater in cases of clinical success compared with those that were deemed failures (p = 0.03, t-test). There were no substantial differences between blinded and nonblinded assessments. Flow void was present in 94% of successes and absent in 75% of failures (p = 0.01, Fisher's exact test). The other subjective assessments were not significantly different between the groups of successes and failures.Conclusions. Ventricular size appears to be somewhat reduced in both groups of patients who underwent clinically successful and failed ETV; however, the reduction is significantly greater among clinically successful cases. The presence of a flow void also appears to correlate with clinical success and its absence with clinical failure.


2015 ◽  
Vol 50 (6) ◽  
pp. 1037-1041 ◽  
Author(s):  
Beatrice Dionigi ◽  
Joseph A. Brazzo ◽  
Azra Ahmed ◽  
Christina Feng ◽  
Yaotang Wu ◽  
...  

Author(s):  
Travis J. Atchley ◽  
Blake Sowers ◽  
Anastasia A. Arynchyna ◽  
Curtis J. Rozzelle ◽  
Brandon G. Rocque

OBJECTIVE The advent of neuroendoscopy revolutionized the management of complex hydrocephalus. Fenestration of the septum pellucidum (septostomy) is often a therapeutic and/or necessary intervention in neuroendoscopy. However, these procedures are not without risk. The authors sought to record the incidence and types of complications. They attempted to discern if there was decreased likelihood of septostomy complications in patients who underwent endoscopic third ventriculostomy (ETV)/choroid plexus cauterization (CPC) as compared with those who underwent other procedures and those with larger ventricles preoperatively. The authors investigated different operative techniques and their possible relationships to septostomy complications. METHODS The authors retrospectively reviewed all neuroendoscopic procedures with Current Procedural Terminology code 62161 performed from January 2003 until June 2019 at their institution. Septostomy, either alone or in conjunction with other procedures, was performed in 118 cases. Basic demographic characteristics, clinical histories, operative details/findings, and adverse events (intraoperative and postoperative) were collected. Pearson chi-square and univariate logistic regression analyses were performed. Patients with incomplete records were excluded. RESULTS Of 118 procedures, 29 (24.5%) septostomies had either intraoperative or postoperative complications. The most common intraoperative complication was bleeding, as noted in 12 (10.2%) septostomies. Neuroendocrine dysfunction, including apnea, bradycardia, neurological deficit, seizure, etc., was the most common postoperative complication and seen after 15 (12.7%) procedures. No significant differences in complications were noted between ventricular size or morphology or between different operative techniques or ventricular approaches. There was no significant difference between the complication rate of patients who underwent ETV/CPC and that of patients who underwent septostomy as a part of other procedures. Greater length of surgery (OR 1.013) was associated with septostomy complications. CONCLUSIONS Neuroendoscopy for hydrocephalus due to varying etiologies provides significant utility but is not without risk. The authors did not find associations between larger ventricular size or posterior endoscope approach and lower complication rates, as hypothesized. No significant difference in complication rates was noted between septostomy performed during ETV/CPC and other endoscopic procedures requiring septostomy.


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