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OBJECTIVE: Standard MRI protocols lack a quantitative sequence that can be used to evaluate shunt-treated patients with a history of hydrocephalus. The objective of this study was to investigate the use of phase-contrast MRI (PC-MRI), a quantitative MR sequence, to measure CSF flow through the shunt and demonstrate PC-MRI as a useful adjunct in the clinical monitoring of shunt-treated patients. METHODS: The rapid (96 seconds) PC-MRI sequence was calibrated using a flow phantom with known flow rates ranging from 0 to 24 mL/hr. Following phantom calibration, 21 patients were scanned with the PC-MRI sequence. Multiple, successive proximal and distal measurements were gathered in 5 patients to test for measurement error in different portions of the shunt system and to determine intrapatient CSF flow variability. The study also includes the first in vivo validations of PC-MRI for CSF shunt flow by comparing phase-contrast-measured flow rate with CSF accumulation in a collection burette obtained in patients with externalized distal shunts. RESULTS: The PC-MRI sequence successfully measured CSF flow rates ranging from 6 to 54 mL/hr in 21 consecutive pediatric patients. Comparison of PC-MRI flow measurement and CSF volume collected in a bedside burette showed good agreement in a patient with an externalized distal shunt. Notably, the distal portion of the shunt demonstrated lower measurement error when compared with PC-MRI measurements acquired in the proximal catheter. CONCLUSIONS: The PC-MRI sequence provided accurate and reliable clinical measurements of CSF flow in shunt-treated patients. This work provides the necessary framework to include PC-MRI as an immediate addition to the clinical setting in the noninvasive evaluation of shunt function and in future clinical investigations of CSF physiology.

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OBJECTIVE: The aim of this study was to delineate the clinical and socioeconomic variables associated with shunt revision in pediatric patients presenting to the emergency department (ED) with concerns of ventricular shunt malfunction. METHODS: A retrospective analysis of pediatric ED consultations for shunt malfunction over a 1-year period was conducted, examining clinical symptoms, radiographic findings, and socioeconomic variables. Sensitivities, specificities, and positive and negative predictive values were calculated for each presenting symptom collected. Logistic regression models were used to estimate the odds ratios for shunt revision based on these variables, and multivariate analyses were used to adjust for potential confounders. RESULTS: Of the 271 ED visits from 137 patients, 19.2% resulted in shunt revision. Increased ventricle size on imaging (OR 11.38, p < 0.001), shunt site swelling (OR 9.04, p = 0.01), bradycardia (OR 7.08, p < 0.001), and lethargy (OR 5.77, p < 0.001) were significantly associated with shunt revision. Seizure-like activity was inversely related to revision needs (OR 0.24, p < 0.001). Patients with private or self-pay insurance were more likely to undergo revision compared with those with public insurance (p = 0.028). Multivariate analysis further confirmed the significant associations of increased ventricle size, lethargy, and bradycardia with shunt revision, while also revealing that seizure-like activity inversely affected the likelihood of revision. Patients with severe cognitive and language disabilities were more likely to be admitted to the hospital from the ED but were not more likely to undergo revision. CONCLUSIONS: Clinical signs such as increased ventricle size, shunt site swelling, bradycardia, and lethargy may be strong predictors of the need for shunt revision in pediatric patients presenting to the ED with concerns of shunt malfunction. Socioeconomic factors play a less clear role in predicting shunt revision and admission from the ED; however, the nature of their influence is unclear. These findings can help inform clinical decision-making and optimize resource utilization in the ED.

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Background: A ventriculoatrial shunt (VAS) proves to be an excellent alternative in the treatment of hydrocephalus. Its usage is a viable option when ventriculoperitoneal shunt (VPS) is contraindicated in any age of patients. Case Description: This report highlights a successful case involving a 6-month-old patient who underwent VAS catheter positioning. The child presented with hydrocephalus and biliary atresia, making him a candidate for a liver transplant. Notably, a VPS was considered a relative contraindication in this scenario. Conclusion: The VAS emerges as a viable option for patients in whom a VPS might be contraindicated. This case demonstrates the successful application of a VAS in a pediatric patient.

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PURPOSE: Posthemorrhagic hydrocephalus (PHH) and necrotizing enterocolitis (NEC) are two comorbidities associated with prematurity. The management of patients with both conditions is complex and it is necessary to intercept them to avoid meningitis and multilocular hydrocephalus. METHODS: In a single-center retrospective study, we analyzed 19 patients with NEC and PHH admitted from 2012 to 2022. We evaluated perinatal, imaging, and NEC-related data. We documented shunt obstruction and infection and deaths within 12 months of shunt insertion. RESULTS: We evaluated 19 patients with NEC and PHH. Six cases (31.58%) were male, the median birth weight was 880 g (650-3150), and the median gestational age was 26 weeks (23-38). Transfontanellar ultrasound was performed on 18 patients (94.74%) and Levine classification system was used: 3 cases (15.79%) had a mild Levine index, 11 cases (57.89%) had moderate, and 5 cases (26.32%) were graded as severe. Magnetic resonance showed intraventricular hemorrhage in 14 cases (73.68%) and ventricular dilatation in 15 cases (78.95%). The median age at shunt insertion was 24 days (9-122) and the median length of hospital stay was 120 days (11-316). Sepsis was present in 15 cases (78.95%). NEC-related infection involved the peritoneal shunt in 4 patients and 3 of them had subclinical NEC. At the last follow-up, 6 (31.58%) patients presented with psychomotor delay. No deaths were reported. CONCLUSIONS: Although recognition of subclinical NEC is challenging, the insertion of a ventriculoperitoneal shunt is not recommended in these cases and alternative treatments should be considered to reduce the risk of meningitis and shunt malfunction.

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OBJECTIVE: The authors designed a low-profile device for reliable ventricular access and prospectively studied its safety, efficacy, and accuracy at a large academic center. METHODS: A novel device for ventricular entry, the Device for Intraventricular Entry (DIVE) guide, was designed and created by the first and senior authors. Fifty patients undergoing external ventricular drainage (EVD) or shunt placement were prospectively enrolled for DIVE-assisted catheter placement at a single academic center. The primary outcome was the catheter tip location on postprocedural CT. Secondary outcomes included number of catheter passes, clinically significant hemorrhages, and procedure-related infections. RESULTS: Fifty patients were enrolled. Indications included subarachnoid hemorrhage, intraventricular hemorrhage, traumatic brain injury, hydrocephalus, pseudotumor, and postsurgical wound drainage. In total, 76% (38/50) of patients underwent right-sided placement and 24% (12/50) underwent left-sided placement. All 100% (50/50) of patients had successful cannulation with an average of 1.06 passes. Postprocedural head CT confirmed ipsilateral frontal horn or third ventricle placement (Kakarla grade 1) in 92% (46/50) of patients and placement in the contralateral lateral ventricle in 8% (4/50) (Kakarla grade 2). There were no clinically significant track hemorrhages or procedural infections. CONCLUSIONS: This single-center prospective study investigated the safety and efficacy of DIVE-assisted ventricular access. In total, 100% of procedures had successful ventricular cannulation, with 92% achieving Kakarla grade 1, with an average of 1.06 passes without any clinical complications.

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INTRODUCTION: Hydrocephalus treatment can be very challenging. While some hydrocephalic patients can be treated endoscopically, many will require ventricular shunting. Frequent shunt issues over a lifetime is not uncommon. Although most shunt malfunctions are of the ventricular catheter or valve, distal failures occur as well. A subset of patients will accumulate non-functioning distal drainage sites. CASE DESCRIPTION: We present a 27-year-old male with developmental delay who was shunted perinatally for hydrocephalus from intraventricular hemorrhage of prematurity. After failure of the peritoneum, pleura, superior vena cava (SVC), gallbladder, and endoscopy, an inferior vena cava (IVC) shunt was placed minimally-invasively via the common femoral vein. We believe this is only the eighth reported ventriculo-inferior-venacaval shunt. IVC occlusion years later was successfully treated with endovascular angioplasty and stenting followed by anticoagulation. To our knowledge, a ventriculo-inferior-venacaval shunt salvaged by endovascular surgery has not been previously described in the literature. CONCLUSION: After failure of the peritoneum, pleura, SVC, gallbladder, and endoscopy, IVC shunt placement is an option. Subsequent IVC occlusion can be rescued by endovascular angioplasty and stenting. Anticoagulation after stenting (and potentially after initial IVC placement) is advised.

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To retrospectively analyze the clinical data of a case of shunt nephritis as the manifestation of systemic lupus erythematosus (SLE) admitted to the Department of Nephrology, Wuhan Children′s Hospital in November 2019, and to analyze its clinical characteristics and diagnosis and treatment through literature review.A 11-year-old female child was diagnosed as SLE for fatigue after exercise, onset of gross hematuria, auxiliary detection of anemia, hematuria albuminuria, hypocomplementemia, positive test for the antinuclear antibody, positive test for anti-human globulin, and hyperplastic lesions detected by renal pathology.However, immunosuppressive therapy was not effective.Considering the previous history of congenital hydrocephalus and ventricular and atrial shunt and through literature review, the patient was finally diagnosed was shunt nephritis.After active anti-infection and ventriculoatrial shunt to ventriculoperitoneal shunt, the symptoms were relieved.Shunt nephritis is a rare complication caused by ventricle shunt tract infection.Early recognition and treatment can avoid misdiagnosis and improve prognosis.

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PURPOSE: Neonates with ventricular shunts inserted for hydrocephalus following germinal matrix haemorrhage (GMH) have high rates of shunt revision. The shunt valve plays a key role in regulating the function of the shunt. In this study, we aim to determine if the choice of flow-regulated or pressure-regulated valve used in the initial implantation of a shunt affects the rate of shunt revision. METHODS: A retrospective cohort comparison study was performed on 34 neonates with hydrocephalus following GMH who underwent placement of a ventricular shunt at the Queensland Children's Hospital from November 2014 to June 2020. The primary outcome examined was the need for revision or replacement of the ventricular shunt after successful initial placement within 2 years of implantation. The secondary outcome examined was the survival time of the shunt. RESULTS: 16 patients had placement of a flow-regulated valve, and 18 patients had placement of a pressure-regulated valve. 14 (87.5%) patients with flow-regulated valves required replacement during the follow-up period. 2 (18.18%) patients with a fixed pressure regulated underwent revision, while 2 (28.57%) programmable pressure-regulated shunts required revision. Patients that had a flow-regulated valve had a statistically significant higher rate of revision compared to those who had a pressure-regulated valve, (87.5% flow vs 22.22% pressure) with a P-value of < 0.001. Valve obstruction was also more common in patients with flow-regulated valves than pressure-regulated valves (4 vs 0) with a P-value of 0.010. Overall mean median survival time was 22.06 months, shunts with flow-regulated valves had a shorter median survival time of 3.19 months compared with over 24 months for pressure-regulated valves with a P-value of < 0.001. CONCLUSION: Our study suggests that the initial implantation of flow-regulated valves may carry an increased total rate of shunt revision and valve obstruction within the first 2 years following implantation compared to pressure-regulated valves in patients with hydrocephalus following GMH.

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OBJECTIVE: Advancements in MRI technology have provided improved ways to acquire imaging data and to more seamlessly incorporate MRI into modern pediatric surgical practice. One such situation is image-guided navigation for pediatric neurosurgical procedures, including intracranial catheter placement. Image-guided surgery (IGS) requires acquisition of CT or MR images, but the former carries the risk of ionizing radiation and the latter is associated with long scan times and often requires pediatric patients to be sedated. The objective of this project was to circumvent the use of CT and standard-sequence MRI in ventricular neuronavigation by investigating the use of fast MR sequences on the basis of 3 criteria: scan duration comparable to that of CT acquisition, visualization of ventricular morphology, and image registration with surface renderings comparable to standard of care. The aim of this work was to report image development, implementation, and results of registration accuracy testing in healthy subjects. METHODS: The authors formulated 11 candidate MR sequences on the basis of the standard IGS protocol, and various scan parameters were modified, such as k-space readout direction, partial k-space acquisition, sparse sampling of k-space (i.e., compressed sensing), in-plane spatial resolution, and slice thickness. To evaluate registration accuracy, the authors calculated target registration error (TRE). A candidate sequence was selected for further evaluation in 10 healthy subjects. RESULTS: The authors identified a candidate imaging protocol, termed presurgical imaging with compressed sensing for time optimization (PICO). Acquisition of the PICO protocol takes 25 seconds. The authors demonstrated noninferior TRE for PICO (3.00 ± 0.19 mm) in comparison with the default MRI neuronavigation protocol (3.35 ± 0.20 mm, p = 0.20). CONCLUSIONS: The developed and tested sequence of this work allowed accurate intraoperative image registration and provided sufficient parenchymal contrast for visualization of ventricular anatomy. Further investigations will evaluate use of the PICO protocol as a substitute for CT and conventional MRI protocols in ventricular neuronavigation.

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Background: Lumboperitoneal (LP) shunt placement is a good option for treating elderly patients with communicating normal pressure hydrocephalus (NPH) who are also on antiplatelet therapy following endovascular treatment of unruptured bilateral internal carotid artery aneurysms. Here, in an 80-year-old male with an LP shunt, the catheter was "pinched" between adjacent spinous processes, resulting in laceration of the catheter and intrathecal catheter migration. Case Description: An 80-year-old male was treated with a LP shunt for NPH 1 year after undergoing endovascular treatment of unruptured bilateral internal carotid artery aneurysms. The lumbar catheter was placed at the L2-3 level. Six months later, when he clinically deteriorated, the follow-up computed tomography showed recurrent ventricular enlargement. Further, studies additionally confirmed intrathecal migration of the lumbar catheter, warranting secondary ventriculoperitoneal shunt placement. Conclusion: Patients with LP shunts may develop lumbar catheter lacerations secondary to a "pinching" effect from adjacent spinous processes, resulting in intrathecal catheter migration.

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PURPOSE: Ventricular drainage remains a usual but challenging procedure for neurosurgical trainees. The objective of the study was to describe reliable skin landmarks for ideal entry points (IEPs) to catheterize brain ventricles via frontal and parieto-occipital approaches. METHODS: We included 30 subjects who underwent brain MRI and simulated the ideal catheterization trajectories of lateral ventricles using anterior and posterior approaches and localized skin surface IEPs. The optimal frontal target was the interventricular foramen and that for the parieto-occipital approach was the atrium. We measured the distances between these IEPs and easily identifiable skin landmarks. RESULTS: The frontal IEP was localized to 116.8 ± 9.3 mm behind the nasion on the sagittal plane and to 39.7 ± 4.9 mm lateral to the midline on the coronal plane. The ideal catheter length was estimated to be 68.4 ± 6.4 mm from the skin surface to the interventricular foramen. The parieto-occipital point was localized to 62.9 ± 7.4 mm above the ipsilateral tragus on the coronal plane and to 53.1 ± 9.1 mm behind the tragus on the axial plane. The ideal catheter length was estimated to be 48.3 ± 9.6 mm. CONCLUSION: The IEP for the frontal approach was localized to 11 cm above the nasion and 4 cm lateral to the midline. The IEP for the parieto-occipital approach was 5.5 cm behind and 6 cm above the tragus. These measurements lightly differ from the classical descriptions of Kocher's point and Keen's point and seem relevant to neurosurgical practice while using an orthogonal insertion.

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BACKGROUND: An entrapped temporal horn (ETH) is one of the critical complications after tumor removal in the lateral ventricle trigone that sometimes becomes life threatening. OBJECTIVE: We sought to develop a novel intraoperative method of prophylactic intraventricular piping (PIP) just after tumor removal to prevent ETH. METHODS: Three patients with meningiomas in the lateral ventricle trigone were treated by a novel intraoperative method of PIP just after tumor removal to prevent ETH. Silicone catheters normally used as ventricular drainage catheters were cut to 5- to 6-cm length and inserted into the tumor cavity to ensure communication between the temporal horn and the atrium or the body of the lateral ventricle through the piping straddling the trigone. RESULTS: None of our patients developed ETH during the follow-up period without complications caused by the tube placement. CONCLUSIONS: PIP might be beneficial to prevent ETH because constant osmotic pressure and constant cerebrospinal fluid pulse wave transmission are maintained between each compartment of the lateral ventricle.

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Intracranial aneurysms (IAs) are localized dilations of the cerebral vasculature, representing the leading cause for non-traumatic subarachnoid hemorrhage and an important source of morbidity and mortality. Despite it being a frequent pathology and most often diagnosed incidentally, IAs in infants are a very rare occurrence, and the ruptured variant is exceptional. A 4-month-old boy with a negative family history was brought to our department because of several episodes of incoercible vomiting and fever. Upon examination, the child was somnolent, without any noticeable deficit. Transfontanellar ultrasonography and CT angiography revealed a ruptured aneurysm of the anterior communicating artery (AComA), whereas the pre-clipping MRI showed thin, almost angiographically invisible anterior cerebral arteries (ACAs) on both sides due to vasospasm. We intervened surgically by placing an external ventricular shunt in an emergency setting, followed by clipping of the IA in a delayed manner. The child was discharged a month after admission with no deficit, despite the paradoxical aspect of the ACA. Ruptured IAs can be safely treated via microsurgery, even in infants. However, this requires a great amount of experience and surgical expertise. Furthermore, the lack of proper management would most likely result in a severe deficit in the long term. Lastly, the lack of visibility of the ACA on angiographic studies may not have neurological consequences if they occur in this age group.

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Hydrocephalus is the most common neurosurgical disorder in children, and cerebrospinal fluid (CSF) diversion with shunt placement is the most commonly performed pediatric neurosurgical procedure. CT is frequently used to evaluate children with suspected CSF shunt malfunction to assess change in ventricular size. Moreover, careful review of the CT images is important to confirm the integrity of the imaged portions of the shunt system. Subtle shunt disruptions can be missed on multiplanar two-dimensional (2-D) CT images, especially when the disruption lies in the plane of imaging. The use of volume-rendered CT images enables radiologists to view the extracranial shunt tubing within the field of view as a three-dimensional (3-D) object. This allows for a rapid and intuitive method of assessing the integrity of the extracranial shunt tubing. The purpose of this pictorial essay is to discuss how volume-rendered CT images can be generated to evaluate CSF shunts in the pediatric population and to provide several examples of their utility in diagnosing shunt disruption. We also address the potential pitfalls of this technique and ways to avoid them.

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PURPOSE: Ventriculoperitoneal shunt insertion is one of the most commonly performed procedures in neurosurgery but has a relatively high complication rate. One important source of complications is shunt malposition from erroneous placement of the parieto-occipital burr hole or poor shunt trajectory. There are significant variations in the freehand parieto-occipital approach amongst neurosurgeons that are derived from variations in technique or experience. The patient's skull shape or size is also often not taken into consideration if fixed measurements are used to define the burr hole entry point. The authors suggest a variation to the technique of ventricular catheter placement by relying on the patient's own craniometrics and skull landmarks. METHODS: The technique is illustrated and supported by analysis of a case series of 25 patients undergoing shunt placement. RESULTS: By this method, all shunts were positioned in the lateral ventricle. Using a 3-point scale, the catheter position was evaluated: grade 1, free floating in cerebrospinal fluid; grade 2, touching the choroid plexus or ventricular wall; and grade 3, tip within the parenchyma. The catheter position was grade 1 in sixteen (64%) cases and grade 2 in nine (36%) cases; none was grade 3. Only one shunt malfunction occurred from proximal shunt obstruction in the series. CONCLUSION: The use of this technique aims to reduce operator and patient variability as contributors to shunt malposition, to increase user reproducibility and decrease the learning curve for trainees. Further prospective study could be designed to validate the technique.

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OBJECTIVE: Cerebrospinal fluid diversion via ventricular shunting is a common surgical treatment for hydrocephalus in the pediatric population. No longitudinal follow-up data for a multistate population-based cohort of pediatric patients undergoing ventricular shunting in the United States have been published. In the current review of a nationwide population-based data set, the authors aimed to assess rates of shunt failure and hospital readmission in pediatric patients undergoing new ventricular shunt placement. They also review patient- and hospital-level factors associated with shunt failure and readmission. METHODS: Included in this study was a population-based sample of pediatric patients with hydrocephalus who, in 2010-2014, had undergone new ventricular shunt placement and had sufficient follow-up, as recorded in the Nationwide Readmissions Database. The authors analyzed the rate of revision within 6 months, readmission rates at 30 and 90 days, and potential factors associated with shunt failure including patient- and hospital-level variables and type of hydrocephalus. RESULTS: A total of 3520 pediatric patients had undergone initial ventriculoperitoneal shunt placement for hydrocephalus at an index admission. Twenty percent of these patients underwent shunt revision within 6 months. The median time to revision was 44.5 days. Eighteen percent of the patients were readmitted within 30 days and 31% were readmitted within 90 days. Different-hospital readmissions were rare, occurring in ≤ 6% of readmissions. Increased hospital volume was not protective against readmission or shunt revision. Patients with grade 3 or 4 intraventricular hemorrhage were more likely to have shunt malfunctions. Patients who had private insurance and who were treated at a large hospital were less likely to be readmitted. CONCLUSIONS: In a nationwide, population-based database with longitudinal follow-up, shunt failure and readmission were common. Although patient and hospital factors were associated with readmission and shunt failure, system-wide phenomena such as insufficient centralization of care and fragmentation of care were not observed. Efforts to reduce readmissions in pediatric patients undergoing ventricular shunt procedures should focus on coordinating care in patients with complex neurological diseases and on reducing healthcare disparities associated with readmission.

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BACKGROUND: Coronavirus Disease 2019 (COVID-19) pandemic raised global attention especially due to the severe acute respiratory symptoms associated to it. However, almost one third of patients also develop neurological symptoms. The aim of the present study is to describe the case of a previously health adult that evolved cerebral ventricular empyema in the IV ventricle during COVID-19 infection treatment. CASE DESCRIPTION: A 49-year-old man with COVID-19 developed pneumonia caused by multidrug-resistant Acinetobacter baumannii. After treating adequate treatment, sedation was switched off without showing appropriate awakening. Brain CT was performed with evidence of communicating hydrocephalus. External ventricular shunt (EVD) was implant with intraoperative cerebrospinal fluid suggestive of meningitis with a positive culture for oxacillin-sensitive Staphylococcus hominis. Twenty days after EVD, meningitis treatment was finished and with 2 negative cultures, conversion to ventriculoperitoneal shunt was performed. In the following week, during the evaluation of the patient in intensive care, quadriplegia and absence of spontaneous respiratory movement were evidenced, just maintaining head movement. Brain MRI was performed with a diagnosis of ventriculitis associated with pus collections on the IV ventricle. The patient underwent microsurgical drainage removal of the shunt, with a positive intraventricular collection culture for Klebsiella pneumoniae carbapenemase and multidrug-resistant Pseudomonas aeruginosa, without improvement in the neurological condition. After 14 weeks of hospitalization, the patient died. CONCLUSION: It is well known that COVID-19 has potential to directly attack and cause severe damage to the central nervous system; however, ventricular empyema is an extremely rare life-threatening complication.

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OBJECTIVE: Studies on surgical site infection (SSI) in adult neurosurgery have presented all subtypes of SSIs as the general 'SSI'. Given that SSIs constitute a broad range of infections, we hypothesized that clinical outcomes and management vary based on SSI subtype. METHODS: A retrospective analysis of all neurosurgical SSI from 2012-2019 was conducted at a tertiary care institution. SSI subtypes were categorized as deep and superficial incisional SSI, brain, dural or spinal abscesses, meningitis or ventriculitis, and osteomyelitis. RESULTS: 9620 craniotomy, shunt, and fusion procedures were studied. 147 procedures (1.5%) resulted in postoperative SSI. 87 (59.2%) of these were associated with craniotomy, 36 (24.5%) with spinal fusion, and 24 (16.3%) with ventricular shunting. Compared with superficial incisional primary SSI, rates of reoperation to treat SSI were highest for deep incisional primary SSI (91.2% vs 38.9% for superficial, p < 0.001) and second-highest for intracranial SSI (90.9% vs 38.9%, p = 0.0001). Postoperative meningitis was associated with the highest mortality rate (14.9%). Compared with superficial incisional SSI, the rate of readmission for intracranial SSI was highest (57.6% vs 16.7%, p = 0.022). CONCLUSION: Deep incisional and organ space SSI demonstrate a greater association with morbidity relative to superficial incisional SSI. Future studies should assess subtypes of SSI given these differences.

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A 34-year-old Caucasian male who underwent a ventricular shunt at age 21 presented with bilateral enophthalmos, poor eyelid-globe apposition and exposure keratopathy characteristic of silent brain syndrome. Progressive enophthalmos and corneal decompensation were documented in serial photographs and radiographic studies over 36 years. Over this period, no sequelae of shunt over-drainage were observed. A lumbar puncture at the last follow-up measured CSF opening pressure to be within the normal range. Additional systemic findings included pneumosinus dilatans, loss of adipose tissue in the temporalis fossa and atrophy of the dorsal interossei of the hand. Surgical interventions to preserve ocular function included insertion of orbital floor wedge and sheet implants, sheet orbital roof implants, and retroplacement of canthal tendons. This report chronicles the long-term clinical course of a patient with silent brain syndrome. The systemic changes suggest factors beyond low intracranial pressure may contribute to the pathogenesis of the condition in our patient.

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OBJECTIVE: There is no consensus on the optimal clinical management of ventriculomegaly and hydrocephalus in patients with diffuse intrinsic pontine glioma (DIPG). To date, the impact on survival in patients with ventriculomegaly and CSF diversion for hydrocephalus in this population remains to be elucidated. Herein, the authors describe their institutional experience. METHODS: Patients diagnosed with DIPG and treated with up-front radiation therapy (RT) at The Hospital for Sick Children between 2000 and 2019 were identified. Images at diagnosis and progression were used to determine the frontal/occipital horn ratio (FOR) as a method to measure ventricular size. Patients with ventriculomegaly (FOR ≥ 0.36) were stratified according to the presence of symptoms and categorized as follows: 1) asymptomatic ventriculomegaly and 2) symptomatic hydrocephalus. For patients with ventriculomegaly who did not require CSF diversion, post-RT imaging was also evaluated to assess changes in the FOR after RT. Proportional hazards analyses were used to identify clinical and treatment factors correlated with survival. The Kaplan-Meier method was used to perform survival estimates, and the log-rank method was used to identify survival differences between groups. RESULTS: Eighty-two patients met the inclusion criteria. At diagnosis, 28% (n = 23) of patients presented with ventriculomegaly, including 8 patients who had symptomatic hydrocephalus and underwent CSF diversion. A ventriculoperitoneal shunt was placed in the majority of patients (6/8). Fifteen asymptomatic patients were managed without CSF diversion. Six patients had resolution of ventriculomegaly after RT. Of 66 patients with imaging at the time of progression, 36 (55%) had ventriculomegaly, and 9 of them required CSF diversion. The presence of ventriculomegaly at diagnosis did not correlate with survival on univariate analysis. However, patients with symptomatic hydrocephalus at the time of progression who underwent CSF diversion had a survival advantage (p = 0.0340) when compared to patients with ventriculomegaly managed with conservative approaches. CONCLUSIONS: Although ventriculomegaly can be present in up to 55% of patients with DIPG, the majority of patients present with asymptomatic ventriculomegaly and do not require surgical interventions. In some cases ventriculomegaly improved after medical management with steroids and RT. CSF diversion for hydrocephalus at the time of diagnosis does not impact survival. In contrast, our results suggest a survival advantage in patients who undergo CSF diversion for hydrocephalus at the time of progression, albeit that advantage is likely to be confounded by biological and individual patient factors. Further research in this area is needed to understand the best timing and type of interventions in this population.