scholarly journals A Successful Treatment of Endoscopic Third Ventriculostomy with Choroid Plexus Cauterization for Hydrocephalus in Walker-Warburg Syndrome

2016 ◽  
Vol 2016 ◽  
pp. 1-5
Author(s):  
Tomoko Tanaka ◽  
Catharine J. Harris ◽  
Sarah S. Barnett ◽  
N. Scott Litofsky
Keyword(s):  
Muscular Dystrophy ◽  
Choroid Plexus ◽  
Successful Treatment ◽  
Endoscopic Third Ventriculostomy ◽  
Third Ventricle ◽  
Congenital Muscular Dystrophy ◽  
Third Ventriculostomy ◽  
Csf Flow ◽  
Phenotypic Spectrum ◽  
Choroid Plexus Cauterization

Walker-Warburg syndrome (WWS) is a rare autosomal recessive congenital muscular dystrophy with brain malformations and ocular abnormalities that falls under the wider phenotypic spectrum of the dystroglycanopathies. Mutations in a number of genes including POMT1, POMT2, POMGNT1, POMGNT2, FKTN, FKRP, LARGE, and ISPD are known to cause alpha dystroglycan-related muscular dystrophy. Mutations in these genes result in a broad phenotypic spectrum ranging from the severe WWS to a mild congenital muscular dystrophy with no brain involvement. WWS is fatal to most patients early in life with mean survival of 9 months. The most common brain finding is cobblestone lissencephaly with the vast majority of patients (97%) also having ventricular dilation with or without hydrocephalus. Surgical treatment has not been frequently detailed. This report describes our successful treatment of a patient with WWS and hydrocephalus with Endoscopic Third Ventriculostomy (ETV) with choroid plexus cauterization (CPC). Fourteen months following treatment, a follow-up MRI CSF flow study demonstrated robust CSF flow through floor of third ventricle from interpeduncular cistern to lateral ventricle.

Predictors of endoscopic third ventriculostomy ostomy status in patients who experience failure of endoscopic third ventriculostomy with choroid plexus cauterization

2019 ◽  
Vol 24 (1) ◽  
pp. 41-46
Author(s):  
Andrew T. Hale ◽  
Amanda N. Stanton ◽  
Shilin Zhao ◽  
Faizal Haji ◽  
Stephen R. Gannon ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Endoscopic Third Ventriculostomy ◽  
Third Ventricle ◽  
Aqueductal Stenosis ◽  
Third Ventriculostomy ◽  
Time To Failure ◽  
Chi Square ◽  
Significant Difference ◽  
Chi Square Test ◽  
Choroid Plexus Cauterization

OBJECTIVEAt failure of endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC), the ETV ostomy may be found to be closed or open. Failure with a closed ostomy may indicate a population that could benefit from evolving techniques to keep the ostomy open and may be candidates for repeat ETV, whereas failure with an open ostomy may be due to persistently abnormal CSF dynamics. This study seeks to identify clinical and radiographic predictors of ostomy status at the time of ETV/CPC failure.METHODSThe authors conducted a multicenter, retrospective cohort study on all pediatric patients with hydrocephalus who failed initial ETV/CPC treatment between January 2013 and October 2016. Failure was defined as the need for repeat ETV or ventriculoperitoneal (VP) shunt placement. Clinical and radiographic data were collected, and ETV ostomy status was determined endoscopically at the subsequent hydrocephalus procedure. Statistical analysis included the Mann-Whitney U-test, Wilcoxon rank-sum test, t-test, and Pearson chi-square test where appropriate, as well as multivariate logistic regression.RESULTSOf 72 ETV/CPC failures, 28 patients (39%) had open-ostomy failure and 44 (61%) had closed-ostomy failure. Patients with open-ostomy failure were older (median 5.1 weeks corrected age for gestation [interquartile range (IQR) 0.9–15.9 weeks]) than patients with closed-ostomy failure (median 0.2 weeks [IQR −1.3 to 4.5 weeks]), a significant difference by univariate and multivariate regression. Etiologies of hydrocephalus included intraventricular hemorrhage of prematurity (32%), myelomeningocele (29%), congenital communicating (11%), aqueductal stenosis (11%), cyst/tumor (4%), and other causes (12%). A wider baseline third ventricle was associated with open-ostomy failure (median 15.0 mm [IQR 10.3–18.5 mm]) compared to closed-ostomy failure (median 11.7 mm [IQR 8.9–16.5 mm], p = 0.048). Finally, at the time of failure, patients with closed-ostomy failure had enlargement of their ventricles (frontal and occipital horn ratio [FOHR], failure vs baseline, median 0.06 [IQR 0.00–0.11]), while patients with open-ostomy failure had no change in ventricle size (median 0.01 [IQR −0.04 to 0.05], p = 0.018). Previous CSF temporizing procedures, intraoperative bleeding, and time to failure were not associated with ostomy status at ETV/CPC failure.CONCLUSIONSOlder corrected age for gestation, larger baseline third ventricle width, and no change in FOHR were associated with open-ostomy ETV/CPC failure. Future studies are warranted to further define and confirm features that may be predictive of ostomy status at the time of ETV/CPC failure.

scholarly journals Comparison of hydrocephalus metrics between infants successfully treated with endoscopic third ventriculostomy with choroid plexus cauterization and those treated with a ventriculoperitoneal shunt: a multicenter matched-cohort analysis

2018 ◽  
Vol 21 (4) ◽  
pp. 339-345 ◽  
Author(s):  
Michael C. Dewan ◽  
Jaims Lim ◽  
Stephen R. Gannon ◽  
David Heaner ◽  
Matthew C. Davis ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Successful Treatment ◽  
Ventriculoperitoneal Shunt ◽  
Endoscopic Third Ventriculostomy ◽  
Third Ventriculostomy ◽  
Z Score ◽  
Significant Difference ◽  
Ventricle Size ◽  
Choroid Plexus Cauterization

OBJECTIVEIt has been suggested that the treatment of infant hydrocephalus results in different craniometric changes depending upon whether ventriculoperitoneal shunt (VPS) placement or endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) is performed. Without an objective and quantitative description of expected changes to the infant cranium and ventricles following ETV/CPC, asserting successful treatment of hydrocephalus is difficult. By comparing infants successfully treated via ETV/CPC or VPS surgery, the authors of this study aimed to define the expected postoperative cranial and ventricular alterations at the time of clinical follow-up.METHODSPatients who underwent successful treatment of hydrocephalus at 4 institutions with either VPS placement or ETV/CPC were matched in a 3:1 ratio on the basis of age and etiology. Commonly used cranial parameters (including head circumference [HC], HC z-score, fontanelle status, and frontooccipital horn ratio [FOHR]) were compared pre- and postoperatively between treatment cohorts. First, baseline preoperative values were compared to ensure cohort equivalence. Next, postoperative metrics, including the relative change in metrics, were compared between treatment groups using multivariate linear regression.RESULTSAcross 4 institutions, 18 ETV/CPC-treated and 54 VPS-treated infants with hydrocephalus were matched and compared at 6 months postoperatively. The most common etiologies of hydrocephalus were myelomeningocele (61%), followed by congenital communicating hydrocephalus (17%), aqueductal stenosis (11%), and intraventricular hemorrhage (6%). The mean age at the time of CSF diversion was similar between ETV/CPC- and VPS-treated patients (3.4 vs 2.9 months; p = 0.69), as were all preoperative cranial hydrocephalus metrics (p > 0.05). Postoperatively, the ventricle size FOHR decreased significantly more following VPS surgery (−0.15) than following ETV/CPC (−0.02) (p < 0.001), yielding a lower postoperative FOHR in the VPS arm (0.42 vs 0.51; p = 0.01). The HC percentile was greater in the ETV/CPC cohort than in the VPS-treated patients (76th vs 54th percentile; p = 0.046). A significant difference in the postoperative z-score was not observed. With both treatment modalities, a bulging fontanelle reliably normalized at last follow-up.CONCLUSIONSClinical and radiographic parameters following successful treatment of hydrocephalus in infants differed between ETV/CPC and VPS treatment. At 6 months post-ETV/CPC, ventricle size remained unchanged, whereas VPS-treated ventricles decreased to a near-normal FOHR. The HC growth control between the procedures was similar, although the final HC percentile may be lower after VPS. The fontanelle remained a reliable indicator of success for both treatments. This study establishes expected cranial and ventricular parameters following ETV/CPC, which may be used to guide preoperative counseling and postoperative decision making.

scholarly journals Early Outcome of Endoscopic Third Ventriculostomy With or Without Choroid Plexus Cauterization and Ventriculoperitoneal Shunt as Primary Treatment of Hydrocephalus in Infants With Myelomeningocele

Neurosurgery ◽  
2019 ◽  
Vol 66 (Supplement_1) ◽  
Author(s):  
Bamidele O Adebayo ◽  
Okezie Kanu ◽  
Olufemi B Bankole ◽  
Omotayo Ojo
Keyword(s):  
Success Rate ◽  
Choroid Plexus ◽  
Ventriculoperitoneal Shunt ◽  
Endoscopic Third Ventriculostomy ◽  
Successful Outcome ◽  
Csf Leak ◽  
Third Ventriculostomy ◽  
Prospective Longitudinal ◽  
Choroid Plexus Cauterization

Abstract INTRODUCTION Myelomeningocele is associated with hydrocephalus in 35% to 90% of cases. Hydrocephalus is usually treated with insertion of ventriculoperitoneal shunt. However, endoscopic third ventriculostomy with or without choroid plexus cauterization is a viable alternative. METHODS A prospective longitudinal study from January 2016 to December 2018 on patients with myelomeningocele who presented to Lagos University Teaching Hospital, Nigeria and who had hydrocephalus.Informed consent for research and surgery were obtained. ETV with or without CPC or VPS was done with general anesthesia using standard operative techniques. Patients were followed up monthly for at least 6 mo. The surgery was adjudged failed if there was progressive head enlargement as well as other signs of hydrocephalus necessitating another surgery for hydrocephalus. RESULTS A total of 48 patients completed the study. A total of Six patients were lost to follow-up. Age range of the patients was 1 to 44 wk. A total of Five patients had ETV, 21 had ETV/CPC, and 22 patients had VPS. The mean OFC at the time of surgery were 45.3 cm and 44.9 cm for the endoscopic and VPS arms respectively. There were 2 (7.6%) cases of CSF leak in the endoscopic arm and 3 (13.6%) cases of wound dehiscence, with 1 (4.5%) case of CSF leak in the VPS arm. Mean age at surgery for ETV/CPC arm was 12.3 wand 11.5 wk for the VPS arm. Mean time to failure was 9.9 and 6.3 wk for endoscopic and VPS arms respectively. Four (80%) of the patients who had ETV had a successful outcome, 11 (52.3%) of those who had ETV/CPC had a successful outcome and 13 (59%) of those who had VPS had a successful outcome. At 6 mo follow up, overall success rate for the endoscopic arm was 57.6% and 59% for the VPS. CONCLUSION Endoscopic third ventriculostomy with or without choroid plexus cauterization had similar success rate with VPS at 6 mos.

Combined endoscopic third ventriculostomy and choroid plexus cauterization as primary treatment of hydrocephalus for infants with myelomeningocele: long-term results of a prospective intent-to-treat study in 115 East African infants

2008 ◽  
Vol 2 (5) ◽  
pp. 310-316 ◽  
Author(s):  
Benjamin C. Warf ◽  
Jeffrey W. Campbell
Keyword(s):  
Choroid Plexus ◽  
Endoscopic Third Ventriculostomy ◽  
Primary Treatment ◽  
Third Ventriculostomy ◽  
Life Table Analysis ◽  
Multivariate Logistic Regression ◽  
East African ◽  
Table Analysis ◽  
Choroid Plexus Cauterization

Object Shunt dependence is more dangerous for children in less developed countries. Combining endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) was previously shown to treat hydrocephalus more effectively than ETV alone in infants < 1 year of age. The goal of this prospective study was to evaluate the effectiveness of ETV-CPC as primary treatment of hydrocephalus in infants with myelomeningocele. Methods One hundred fifteen consecutive East African infants with myelomeningocele requiring treatment for hydrocephalus were intended for primary management using ETV-CPC. Patient information was prospectively entered into a database. Outcomes were evaluated by life table analysis. Potential predictors for treatment failure were evaluated using multivariate logistic regression. Results Ninety-three patients had a completed ETV-CPC with > 1 month of follow-up. The ETV-CPC procedure was successful in 71 patients (76%), with a mean and median follow-up of 19.0 months. Treatment failures occurred before 6 months in 86% of the patients, and none occurred after 10 months. The operative mortality rate was 1.1%, and there were no infections. Life table analysis suggested that 72% of the patients would be successfully treated using a single ETV-CPC and 78% would remain shunt-independent with reopening of a closed ETV stoma. Multivariate logistic regression showed scarring of the cistern (p = 0.021) or choroid plexus (p = 0.026) as predictors of failure, but age at the time of surgery was not a significant predictor. Conclusions Using ETV-CPC appears to successfully provide a more durable primary treatment of hydrocephalus for infants with spina bifida than does shunt placement. These results support ETV-CPC as the better treatment option for these children in developing countries.

scholarly journals Predictors of success for combined endoscopic third ventriculostomy and choroid plexus cauterization in a North American setting: a Hydrocephalus Clinical Research Network study

2019 ◽  
Vol 24 (2) ◽  
pp. 128-138
Author(s):  
Jay Riva-Cambrin ◽  
John R. W. Kestle ◽  
Curtis J. Rozzelle ◽  
Robert P. Naftel ◽  
Jessica S. Alvey ◽  
...  
Keyword(s):  
Cohort Study ◽  
Clinical Research ◽  
Choroid Plexus ◽  
Patient Selection ◽  
Operative Technique ◽  
Intraventricular Hemorrhage ◽  
Endoscopic Third Ventriculostomy ◽  
Third Ventriculostomy ◽  
Success Rates ◽  
Choroid Plexus Cauterization

OBJECTIVEEndoscopic third ventriculostomy combined with choroid plexus cauterization (ETV+CPC) has been adopted by many pediatric neurosurgeons as an alternative to placing shunts in infants with hydrocephalus. However, reported success rates have been highly variable, which may be secondary to patient selection, operative technique, and/or surgeon training. The objective of this prospective multicenter cohort study was to identify independent patient selection, operative technique, or surgical training predictors of ETV+CPC success in infants.METHODSThis was a prospective cohort study nested within the Hydrocephalus Clinical Research Network’s (HCRN) Core Data Project (registry). All infants under the age of 2 years who underwent a first ETV+CPC between June 2006 and March 2015 from 8 HCRN centers were included. Each patient had a minimum of 6 months of follow-up unless censored by an ETV+CPC failure. Patient and operative risk factors of failure were examined, as well as formal ETV+CPC training, which was defined as traveling to and working with the experienced surgeons at CURE Children’s Hospital of Uganda. ETV+CPC failure was defined as the need for repeat ETV, shunting, or death.RESULTSThe study contained 191 patients with a primary ETV+CPC conducted by 17 pediatric neurosurgeons within the HCRN. Infants under 6 months corrected age at the time of ETV+CPC represented 79% of the cohort. Myelomeningocele (26%), intraventricular hemorrhage associated with prematurity (24%), and aqueductal stenosis (17%) were the most common etiologies. A total of 115 (60%) of the ETV+CPCs were conducted by surgeons after formal training. Overall, ETV+CPC was successful in 48%, 46%, and 45% of infants at 6 months, 1 year, and 18 months, respectively. Young age (< 1 month) (adjusted hazard ratio [aHR] 1.9, 95% CI 1.0–3.6) and an etiology of post–intraventricular hemorrhage secondary to prematurity (aHR 2.0, 95% CI 1.1–3.6) were the only two independent predictors of ETV+CPC failure. Specific subgroups of ages within etiology categories were identified as having higher ETV+CPC success rates. Although training led to more frequent use of the flexible scope (p < 0.001) and higher rates of complete (> 90%) CPC (p < 0.001), training itself was not independently associated (aHR 1.1, 95% CI 0.7–1.8; p = 0.63) with ETV+CPC success.CONCLUSIONSThis is the largest prospective multicenter North American study to date examining ETV+CPC. Formal ETV+CPC training was not found to be associated with improved procedure outcomes. Specific subgroups of ages within specific hydrocephalus etiologies were identified that may preferentially benefit from ETV+CPC.

Postoperative seizures following endoscopic third ventriculostomy and choroid plexus cauterization: a case series

2021 ◽  
pp. 1-7
Author(s):  
Randaline R. Barnett ◽  
Allie L. Harbert ◽  
Hengameh B. Pajer ◽  
Angela Wabulya ◽  
Valerie L. Jewells ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Endoscopic Third Ventriculostomy ◽  
Medical Center ◽  
Academic Medical Center ◽  
Case Series ◽  
Third Ventriculostomy ◽  
Mri Findings ◽  
Entry Site ◽  
Postoperative Seizure ◽  
Choroid Plexus Cauterization

OBJECTIVE In this study, the authors sought to investigate variables associated with postoperative seizures following endoscopic third ventriculostomy and choroid plexus cauterization (ETV/CPC) for treatment of pediatric hydrocephalus. METHODS A retrospective analysis of 37 patients who underwent ETV/CPC for treatment of hydrocephalus at an academic medical center from September 2016 to March 2021 was conducted. Demographics, etiology of hydrocephalus, operative details, electroencephalography (EEG) data, MRI findings, need for subsequent procedures, perioperative laboratory tests, medical history, and presence of clinical postoperative seizures were collected. Postoperative seizures were defined as clinical seizures within 24 hours of surgery. Eighteen patients received levetiracetam intraoperatively as well as over the next 7 days postoperatively for seizure prophylaxis. RESULTS Of 37 included patients, 9 (24%) developed clinical seizures within 24 hours after surgery, 5 of whom subsequently had electroclinical seizures captured on video-EEG. The clinical seizures in 4 of those 5 patients (80%) may have been associated with the hemisphere of the brain through which the endoscope was introduced. The median corrected age of the cohort was 3.4 months. The median corrected age of patients who did not develop postoperative seizures was 2.3 months compared with 0.7 months for patients who did develop postoperative seizures (p > 0.99). Postoperative seizures occurred in 43% (3/7) of prenatally repaired myelomeningocele patients versus 29% (2/7) of postnatally repaired myelomeningocele patients. Of the 18 patients who received prophylactic levetiracetam, none (0%) developed postoperative seizures compared with 9 of the 19 patients (47%) who did not receive prophylactic levetiracetam (p = 0.014). CONCLUSIONS Postoperative seizures were recorded in 24% of the pediatric patients who underwent ETV/CPC for hydrocephalus, which is higher than previously reported rates in the literature of 5%. Since 80% of the postoperative electrographic seizures may have been associated with the hemisphere through which the endoscope was introduced, the surgical entry site may contribute to postoperative seizure development. In patients who received prophylactic perioperative levetiracetam, the postoperative seizure incidence dropped to 0% compared with 47% in those who did not receive prophylactic perioperative levetiracetam. This finding indicates that the use of prophylactic perioperative levetiracetam may be efficacious in the prevention of clinical seizures in this patient population.

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