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Objective: When the peritoneal cavity cannot serve as the distal shunt terminus, nonperitoneal shunts, typically terminating in the atrium or pleural space, are used. The comparative effectiveness of these two terminus options has not been evaluated. The authors directly compared shunt survival and complication rates for ventriculoatrial (VA) and ventriculopleural (VPl) shunts in a pediatric cohort., Methods: The Hydrocephalus Clinical Research Network Core Data Project was used to identify children ≤ 18 years of age who underwent either VA or VPl shunt insertion. The primary outcome was time to shunt failure. Secondary outcomes included distal site complications and frequency of shunt failure at 6, 12, and 24 months., Results: The search criteria yielded 416 children from 14 centers with either a VA (n = 318) or VPl (n = 98) shunt, including those converted from ventriculoperitoneal shunts. Children with VA shunts had a lower median age at insertion (6.1 years vs 12.4 years, p < 0.001). Among those children with VA shunts, a hydrocephalus etiology of intraventricular hemorrhage (IVH) secondary to prematurity comprised a higher proportion (47.0% vs 31.2%) and myelomeningocele comprised a lower proportion (17.8% vs 27.3%) (p = 0.024) compared with those with VPl shunts. At 24 months, there was a higher cumulative number of revisions for VA shunts (48.6% vs 38.9%, p = 0.038). When stratified by patient age at shunt insertion, VA shunts in children < 6 years had the lowest shunt survival rate (p < 0.001, log-rank test). After controlling for age and etiology, multivariable analysis did not find that shunt type (VA vs VPl) was predictive of time to shunt failure. No differences were found in the cumulative frequency of complications (VA 6.0% vs VPl 9.2%, p = 0.257), but there was a higher rate of pneumothorax in the VPl cohort (3.1% vs 0%, p = 0.013)., Conclusions: Shunt survival was similar between VA and VPl shunts, although VA shunts are used more often, particularly in younger patients. Children < 6 years with VA shunts appeared to have the shortest shunt survival, which may be a result of the VA group having more cases of IVH secondary to prematurity; however, when age and etiology were included in a multivariable model, shunt location (atrium vs pleural space) was not associated with time to failure. The baseline differences between children treated with a VA versus a VPl shunt likely explain current practice patterns.

Objective: Persistent hydrocephalus following posterior fossa brain tumor (PFBT) resection is a common cause of morbidity in pediatric brain tumor patients, for which the optimal treatment is debated. The purpose of this study was to compare treatment outcomes between VPS and ETV in patients with persistent hydrocephalus following surgical resection of a PFBT., Methods: A post-hoc analysis was performed of the Hydrocephalus Clinical Research Network (HCRN) prospective observational study evaluating VPS and ETV for pediatric patients. Children who experienced hydrocephalus secondary to PFBT from 2008 to 2021 were included. Primary outcomes were VPS/ETV treatment failure and time-to-failure (TTF)., Results: Among 241 patients, the VPS (183) and ETV (58) groups were similar in age, extent of tumor resection, and preoperative ETV Success Score. There was no difference in overall treatment failure between VPS and ETV (33.9% vs 31.0%, p = 0.751). However, mean TTF was shorter for ETV than VPS (0.45 years vs 1.30 years, p = 0.001). While major complication profiles were similar, compared to VPS, ETV patients had relatively higher incidence of minor CSF leak (10.3% vs. 1.1%, p = 0.003) and pseudomeningocele (12.1% vs 3.3%, p = 0.02). No ETV failures were identified beyond 3 years, while shunt failures occurred beyond 5 years. Shunt infections occurred in 5.5% of the VPS cohort., Conclusions: ETV and VPS offer similar overall success rates for PFBT-related postoperative hydrocephalus. ETV failure occurs earlier, while susceptibility to VPS failure persists beyond 5 years. Tumor histology and grade may be considered when selecting the optimal means of CSF diversion., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: Myelomeningocele (MMC) is frequently complicated by symptomatic hydrocephalus, necessitating early permanent CSF diversion and revision surgeries. Shunt infections are a common cause of shunt malfunction. This study aims to characterize long-term shunt-related outcomes of patients undergoing MMC closure., Methods: A total of 170 patients undergoing MMC closure between the years of 1995 and 2017 were identified from a retrospective review of a prospectively populated surgical database at the Children's Hospital of Pittsburgh. Patients who underwent MMC closure and required ventriculoperitoneal (VP) shunt insertion met criteria and were included in the primary study analysis. Analysis with a Fisher exact test was performed for categorical variables, and Mann-Whitney U-tests were utilized for numerical data., Results: Of the 158 total patients undergoing MMC closure and meeting inclusion criteria, 137 (87%) required VP shunt insertion. These 137 patients demonstrated a shunt revision rate of 21.1% per person-year and a shunt infection rate of 2.1% per person-year over a mean follow-up of 10.8 years. Patients had a mean of 3.4 ± 0.6 shunt surgeries prior to their first infection. Patients undergoing immediate shunt removal, external ventricular drain placement, or shunt replacement after clearing the infection had lower rates of subsequent infections than patients who initially were managed with shunt externalization (p < 0.001). Placement of a shunt at the time of MMC closure was not found to be a risk factor for infection. Of patients with initial shunt placement after the implementation of the Hydrocephalus Clinical Research Network protocol in 2011, the authors' institution has had a shunt infection rate of 4.2% per person-year and a revision rate of 35.7% per person-year., Conclusions: This study describes long-term outcomes of shunted MMC patients and factors associated with shunt infections. Most patients underwent multiple revisions prior to the first shunt infection. Shunt externalization may be ineffective at clearing the infection and should be avoided in favor of early shunt removal and external ventricular drainage, followed by shunt replacement once infection is demonstrated to have cleared.

Objective: We sought to compare our large single-institution cohort of postnatal myelomeningocele closure to the 2 arms of the Management of Myelomeningocele Study (MOMS) trial at the designated trial time points, as well as assess outcomes at long-term follow-up among our postnatal cohort., Methods: A single-institutional retrospective review of myelomeningocele cases presenting from 1995 to 2015 at Children's Hospital of Pittsburgh was performed. We compared outcomes at 12 and 30 months to both arms of the MOMS trial and compared our cohort's outcomes at those designated time points to our long-term outcomes. Univariate statistical analysis was performed as appropriate., Results: One-hundred sixty-three patients were included in this study. All patients had at least 2-year follow-up, with a mean follow-up of 10 years (range 2-20 years). There was no difference in the overall distribution of anatomic level of defect. Compared to our cohort, the prenatal cohort had a higher rate of tethering at 12 months of age, 8 versus 1.8%. Conversely, the Chiari II decompression rate was higher in our cohort (10.4 vs. 1.0%). At 30 months, the prenatal cohort had a higher rate of independent ambulation, but our cohort demonstrated the highest rate of ambulation with or without assistive devices among the 3 groups. When comparing our cohort at these early time points to our long-term follow-up data, our cohort's ambulatory function decreased from 84 to 66%, and the rate of detethering surgery increased almost 10-fold., Conclusions: This study demonstrated that overall ambulation and anatomic-functional level were significantly better among our large postnatal cohort, as well as having significantly fewer complications to both fetus and mother, when compared to the postnatal cohort of the MOMS trial. Our finding that ambulatory ability declined significantly with age in this patient population is worrisome for the long-term outcomes of the MOMS cohorts, especially given the high rates of cord tethering at early ages within the prenatal cohort. These findings suggest that the perceived benefits of prenatal closure over postnatal closure may not be as substantial as presented in the original trial, with the durability of results still remaining a concern., (© 2021 S. Karger AG, Basel.)

Background: Ventriculoatrial shunts are common alternatives for patients who cannot tolerate ventriculoperitoneal shunts. The majority of ventriculoatrial shunt malfunctions are related to mechanical problems. We report an interesting case of ventriculoatrial shunt malfunction due to elevated central venous pressure from new-onset atrial fibrillation., Methods: After the patient was confirmed to have ventriculomegaly, he was taken to the operating room for exploration of his ventriculoatrial shunt; there were no obstructions. Subsequently, the patient was cardioverted to normal sinus rhythm for his new onset atrial fibrillation., Results: The clinical syndrome and ventriculomegaly both resolved after the patient's atrial fibrillation was corrected with chemical cardioversion., Conclusions: The cause of this patient's VA shunt malfunction was likely associated with his new onset atrial fibrillation., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction: Myelomeningocele (MMC) is an open neural tube defect routinely surgically closed within 48 h of birth to prevent secondary infection. Up to 18% of patients experience wound complications, and 85% require shunting for hydrocephalus. We hypothesized that wound complications could be reduced by cerebrospinal fluid (CSF) diversion at the time of closure., Methods: Institutional review board approval was obtained to review records of the 88 patients who underwent MMC closure between January 2005 and June 2016 at the Children's Hospital of Pittsburgh. Twenty-three patients (26%) had an external ventricular drain (EVD) placed at the time of MMC closure and underwent 7-11 days of CSF drainage. Fourteen patients (16%) had a shunt placed at the time of MMC closure, and 51 (58%) had no form of CSF diversion at the time of MMC closure., Results: Patients with an EVD or shunt placed at the time of closure had no wound complications. In contrast, 8 patients (16%) without CSF diversion at closure developed wound complications (p = 0.048). Seven of the 8 wound complications occurred in the 71 patients with evidence of hydrocephalus at birth (p = 0.98). Of patients with evidence of hydrocephalus at the time of MMC closure, wound complications had a higher rate of occurrence among patients who did not receive a shunt or EVD at closure (p = 0.01). When comparing only patients with evidence of hydrocephalus at birth, the EVD group alone had a lower rate of wound complications than patients who did not receive CSF diversion at the time of closure (p = 0.031)., Conclusions: These results suggest that addressing hydrocephalus at the time of MMC closure significantly reduces the likelihood of wound complications and may justify temporary CSF diversion at birth, at least in those patients manifesting hydrocephalus., (© 2018 S. Karger AG, Basel.)