Palliative endoscopic third ventriculostomy for pediatric primary brain tumors: a single-institution case series

OBJECTIVE Children with nonoperative brain tumors, such as diffuse intrinsic pontine gliomas (DIPGs), often have life-threatening hydrocephalus. Palliative shunting is common in such cases but can be complicated by hardware infection and mechanical failure. Endoscopic third ventriculostomy (ETV) is a minimally invasive alternative to treat hydrocephalus without implanted hardware. Herein, the authors report their institutional experience with palliative ETV for primary pediatric brain tumors. METHODS The authors conducted a retrospective review of consecutive patients who had undergone palliative ETV for hydrocephalus secondary to nonresectable primary brain tumors over a 10-year period at Rady Children’s Hospital. Collected variables included age, sex, tumor type, tumor location, presence of leptomeningeal spread, use of a robot for ETV, complications, ETV Success Score (ETVSS), functional status, length of survival, and follow-up time. A successful outcome was defined as an ETV performed without clinically significant perioperative complications or secondary requirement for a new shunt. RESULTS Fifteen patients met the study inclusion criteria (11 males, 4 females; average age 7.9 years, range 0.8–21 years). Thirteen patients underwent manual ETV, and 2 patients underwent robotic ETV. Preoperative symptoms included gaze palsy, nausea/vomiting, headache, lethargy, hemiparesis, and seizures. Tumor types included DIPG (3), intraventricular/thalamic glioblastoma (2), and leptomeningeal spread of medulloblastoma (2), anaplastic oligo-/astrocytoma (2), rhabdoid tumor (2), primitive neuroectodermal tumor (1), ganglioglioma (1), pineoblastoma (1), and embryonal carcinoma (1). The mean preoperative ETVSS was 79 ± 8.8. There was 1 perioperative complication, a wound breakdown consistent with refractory hydrocephalus. The mean follow-up was 4.9 ± 5.5 months overall, and mean survival for the patients who died was 3.2 ± 3.6 months. Two patients remained alive at a mean follow-up of 15.7 months. Palliative ETV was successful in 7 patients (47%) and unsuccessful in 8 (53%). While patients with successful ETV were significantly older (11.9 ± 5.6 vs 4.4 ± 4.1 years, p = 0.010), there were no significant differences in preoperative ETVSS (p = 0.796) or postoperative survival (p = 0.476) between the successful and unsuccessful groups. Overall, functional outcomes were similar between the two groups; there was no significant difference in posttreatment Karnofsky Performance Status scores (68.6 ± 19.5 vs 61.3 ± 16.3, p = 0.454), suggesting that including ETV in the treatment algorithm did not worsen outcomes. CONCLUSIONS Palliative ETV is a safe and potentially efficacious treatment option in selected pediatric patients with hydrocephalus from nonoperative brain tumors. Close follow-up, especially in younger children, is required to ensure that patients with refractory symptoms receive appropriate secondary CSF diversion.

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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 ◽

10.1093/neuros/nyz310_347 ◽

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.

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Quality of life after endoscopic third ventriculostomy and cerebrospinal fluid shunting: an adjusted multivariable analysis in a large cohort

Journal of Neurosurgery Pediatrics ◽

10.3171/2010.3.peds09358 ◽

2010 ◽

Vol 6 (1) ◽

pp. 11-16 ◽

Cited By ~ 32

Author(s):

Abhaya V. Kulkarni ◽

Iffat Shams ◽

D. Douglas Cochrane ◽

P. Daniel McNeely

Keyword(s):

Quality Of Life ◽

Initial Treatment ◽

Endoscopic Third Ventriculostomy ◽

Large Cohort ◽

Independent Effect ◽

Third Ventriculostomy ◽

Csf Shunting ◽

Significant Difference

Object Quality of life (QOL) studies comparing treatment with endoscopic third ventriculostomy (ETV) and CSF shunting are very limited. The authors compared QOL outcomes following these 2 treatments in a large cohort of children with hydrocephalus by using multivariable statistical techniques to adjust for possible confounder variables. Methods The families of children between 5 and 18 years of age with previously treated hydrocephalus at 3 Canadian pediatric neurosurgery centers completed measures of QOL: the Hydrocephalus Outcome Questionnaire (HOQ) and the Health Utilities Index Mark 3 (HUI3). Medical records and recent brain imaging studies were reviewed. A linear regression analysis was performed with the QOL measures as the dependent variable. In multivariable analyses, the authors assessed the independent effect of initial hydrocephalus treatment (ETV vs shunting) while adjusting for the treatment center, current patient age, age at initial treatment, etiology of hydrocephalus, total number of days spent in the hospital for initial treatment, total number of days spent in the hospital for subsequent hydrocephalus complications, functioning ETV at follow-up assessment, frequency of seizures, and current ventricle size. Results Data from 603 patients were available for analysis. Fifty-eight patients had undergone ETV as their primary treatment and 545 had undergone CSF shunting. Endoscopic third ventriculostomy patients were slightly younger at the follow-up assessment, were older at the first surgery, and spent fewer days in the hospital for hydrocephalus complications. Without adjustment for any confounders, treatment with ETV was associated with significantly higher HOQ physical scores and HUI3 scores. After multivariable adjustment, however, there was no significant difference in any outcome measure. A functioning ETV at the time of the follow-up assessment was not significant in any model. Conclusions Treatment with either ETV or CSF shunting does not appear to be associated with any substantial difference in QOL outcome after adjusting for prognostic factors. Further study is needed to definitively determine the relative QOL benefit of either procedure, if any.

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Quality of life in children with hydrocephalus treated with endoscopic third ventriculostomy

Journal of Neurosurgery Pediatrics ◽

10.3171/2020.8.peds20384 ◽

2021 ◽

pp. 1-8

Author(s):

Ángela Ros-Sanjuán ◽

Sara Iglesias-Moroño ◽

Bienvenido Ros-López ◽

Francisca Rius-Díaz ◽

Andrea Delgado-Babiano ◽

...

Keyword(s):

Quality Of Life ◽

Endoscopic Third Ventriculostomy ◽

Epileptic Seizures ◽

Third Ventriculostomy ◽

Neurosurgical Procedures ◽

Physical Domain ◽

History Of ◽

The Mean

OBJECTIVEThe objectives of this study were to determine the quality of life of a pediatric cohort with hydrocephalus treated by endoscopic third ventriculostomy (ETV), using the Hydrocephalus Outcome Questionnaire–Spanish version (HOQ-Sv), and study the clinical and radiological factors associated with a better or worse functional status.METHODSThis cross-sectional study was undertaken between September 2018 and December 2019. It comprised a series of 40 patients ranging from 5 to 18 years old with hydrocephalus treated by ETV. ETV was considered to be successful if there was no need for surgery for the treatment of hydrocephalus after a minimum follow-up of 6 months. The clinical variables included gender, age at hydrocephalus diagnosis, age at the time of ETV, age at completion of the questionnaire, etiology and type of hydrocephalus (communicating or not), prior shunt, repeat ETV, number of neurosurgical procedures, number of epileptic seizures, presenting signs, and follow-up duration until last office revision. The radiological variables were the Evans Index and the pre- and posttreatment frontooccipital horn ratio. An analysis was conducted of the association between all these variables and the various dimensions on the HOQ-Sv, completed by the parents of the patients via telephone or in the outpatient offices.RESULTSThe mean age of the children at ETV was 7 years (range 7–194 months), and on completing the questionnaire was 12 years (range 60–216 months). The mean HOQ scores were as follows: overall 0.82, physical domain 0.86, social-emotional (SE) domain 0.84, cognitive domain 0.75, and utility score 0.90. A history of epileptic crises was a predictive factor for a worse score overall and in the SE and cognitive domains. Factors related to a worse score in the physical domain were a previous shunt, the number of procedures, and the etiology and type of hydrocephalus. The mean follow-up duration from ETV to the last office visit was 5 years (64.5 months). No association was found between the degree of ventricular reduction and the quality of life.CONCLUSIONSThe factors related to a worse score in the different dimensions of the HOQ were a history of epileptic seizures, the number of procedures, communicating hydrocephalus, and having had a previous valve. No association was found between the reduction in ventricular size and the quality of life as measured on the HOQ-Sv.

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Anterior third ventricular height and infundibulochiasmatic angle: two novel measurements to predict clinical success of endoscopic third ventriculostomy in the early postoperative period

Journal of Neurosurgery ◽

10.3171/2019.1.jns181330 ◽

2020 ◽

Vol 132 (6) ◽

pp. 1764-1772

Author(s):

Mehmet Sabri Gürbüz ◽

Adnan Dağçınar ◽

Yaşar Bayri ◽

Aşkın Şeker ◽

Hasan Güçlü

Keyword(s):

Endoscopic Third Ventriculostomy ◽

Clinical Success ◽

Obstructive Hydrocephalus ◽

Early Postoperative Period ◽

Third Ventriculostomy ◽

Mr Images ◽

Postoperative Changes ◽

Before And After ◽

The Mean

OBJECTIVEThe authors sought to develop a set of parameters that reliably predict the clinical success of endoscopic third ventriculostomy (ETV) when assessed before and after the operation, and to establish a plan for MRI follow-up after this procedure.METHODSThis retrospective study involved 77 patients who had undergone 78 ETV procedures for obstructive hydrocephalus between 2010 and 2015. Constructive interference in steady-state (CISS) MRI evaluations before and after ETV were reviewed, and 4 parameters were measured. Two well-known standard parameters, fronto-occipital horn ratio (FOHR) and third ventricular index (TVI), and 2 newly defined parameters, infundibulochiasmatic (IC) angle and anterior third ventricular height (TVH), were measured in this study. Associations between preoperative measurements of and postoperative changes in the 4 variables and the clinical success of ETV were analyzed.RESULTSOf the 78 ETV procedures, 70 (89.7%) were successful and 8 (10.3%) failed. On the preoperative MR images, the mean IC angle and anterior TVH were significantly larger in the successful procedures. On the 24-hour postoperative MR images of the successful procedures, the mean IC angle declined significantly from 114.2° to 94.6° (p < 0.05) and the mean anterior TVH declined significantly from 15 to 11.2 mm (p < 0.05). The mean percentage reduction of the IC angle was 17.1%, and that of the anterior TVH was 25.5% (both p < 0.05). On the 1-month MR images of the successful procedures, the mean IC angle declined significantly from 94.6° to 84.2° (p < 0.05) and the mean anterior TVH declined significantly from 11.2 to 9.3 mm (p < 0.05). The mean percentage reductions in IC angle (11%) and anterior TVH (16.9%) remained significant at this time point but were smaller than those observed at 24 hours. The 6-month and 1-year postoperative MR images of the successful group showed no significant changes in mean IC angle or mean anterior TVH. Regarding the unsuccessful procedures, there were no significant changes observed in IC angle or anterior TVH at any of the time points studied. Reduction of IC angle and reduction of anterior TVH on 24-hour postoperative MR images were significantly associated with successful ETV. However, no clinically significant association was found between FOHR, TVI, and ETV success.CONCLUSIONSAssessing the IC angle and anterior TVH on preoperative and 24-hour postoperative MR images is useful for predicting the clinical success of ETV. These 2 measurements could also be valuable as radiological follow-up parameters.

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Resolution of papilledema after endoscopic third ventriculostomy versus cerebrospinal fluid shunting in hydrocephalus: a comparative study

Journal of Neurosurgery ◽

10.3171/2014.2.jns132002 ◽

2014 ◽

Vol 120 (6) ◽

pp. 1465-1470 ◽

Cited By ~ 5

Author(s):

Ender Koktekir ◽

Bengu Ekinci Koktekir ◽

Hakan Karabagli ◽

Sansal Gedik ◽

Gokhan Akdemir

Keyword(s):

Endoscopic Third Ventriculostomy ◽

Csf Shunt ◽

Third Ventriculostomy ◽

Ophthalmological Examination ◽

Rnfl Thickness ◽

Csf Shunting ◽

Significant Difference ◽

The Mean ◽

Group 2 ◽

Group 1

Object In this study the authors compare the efficacy of endoscopic third ventriculostomy (ETV) versus CSF shunting for resolution of papilledema in hydrocephalus. Methods This comparative case series study recruited 12 patients (24 eyes) with hydrocephalus who underwent either an ETV (Group 1, 6 patients [12 eyes]) or CSF shunt treatment (Group 2, 6 patients [12 eyes]). A complete ophthalmological examination including retinal nerve fiber layer (RNFL) evaluation by optical coherence tomography was provided for all patients before surgery and in the 1st week, 1st month, and 3rd month postoperatively. The 2 groups were compared for quantitative changes in RNFL thickness and, thereby, resolution of papilledema. Statistical evaluation was performed using the Mann-Whitney U-test with the aid of SPSS version 16.0. Results The mean preoperative RNFL thickness was 259.7 ± 35.8 μm in Group 1 and 244.5 ± 53.4 μm in Group 2 (p = 0.798). The mean decrease in RNFL thickness was 101.3 ± 38.8 μm, 141.2 ± 34.6 μm, and 162.0 ± 35.9 μm in Group 1 versus 97.0 ± 44.6 μm, 143 ± 45.6 μm, and 130.0 ± 59.8 μm in Group 2 for the postoperative 1st week, 1st month, and 3rd month, respectively. There was no significant difference between the two groups with respect to decrease in RNFL thickness during the 1st week, 1st month, and 3rd month (p = 0.563, p = 0.753, and p = 0.528, respectively). Conclusions This is the first study to quantitatively evaluate papilledema in assessing the success of ETV and CSF shunting. The authors' results indicated that ETV is as effective as CSF shunting with respect to decreasing intracranial pressure and resolution of papilledema.

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Long-term change in ventricular size following endoscopic third ventriculostomy for hydrocephalus due to tectal plate gliomas

Journal of Neurosurgery Pediatrics ◽

10.3171/2012.9.peds12243 ◽

2013 ◽

Vol 11 (1) ◽

pp. 20-25 ◽

Cited By ~ 12

Author(s):

Andrew Romeo ◽

Robert P. Naftel ◽

Christoph J. Griessenauer ◽

Gavin T. Reed ◽

Richard Martin ◽

...

Keyword(s):

Endoscopic Third Ventriculostomy ◽

Third Ventriculostomy ◽

Ventricular Size ◽

Size Change ◽

Term Change ◽

Tectal Plate ◽

Additional Procedure ◽

Csf Diversion ◽

The Mean

Object Endoscopic third ventriculostomy (ETV) is an alternative to shunt placement in children with hydrocephalus due to tectal plate gliomas (TPGs). However, controversy remains regarding the amount of ventricular size reduction that should be expected after ETV. This study investigates ventricular size change after ETV for TPGs. Methods Twenty-two children were identified from a 15-year retrospective database of neuroendoscopic procedures performed at the authors' institution, Children's Hospital of Alabama, in patients with a minimum of 1 year of follow-up. Clinical outcomes, including the need for further CSF diversion and symptom resolution, were recorded. The frontal and occipital horn ratio (FOR) was measured on pre- and postoperative, 1-year, and last follow-up imaging studies. Results In 17 (77%) of 22 children no additional procedure for CSF diversion was required. Of those in whom CSF diversion failed, 4 underwent successful repeat ETV and 1 required shunt replacement. Therefore, in 21 (96%) of 22 patients, CSF diversion was accomplished with ETV. Preoperative and postoperative imaging was available for 18 (82%) of 22 patients. The FOR decreased in 89% of children who underwent ETV. The FOR progressively decreased 1.7%, 11.2%, and 12.7% on the initial postoperative, 1-year, and last follow-up images, respectively. The mean radiological follow-up duration for 18 patients was 5.4 years. When ETV failed, the FOR increased at the time of failure in all patients. Failure occurred 1.6 years after initial ETV on average. The mean clinical follow-up period for all 22 patients was 5.3 years. In all cases clinical improvement was demonstrated at the last follow-up. Conclusions Endoscopic third ventriculostomy successfully treated hydrocephalus in the extended follow-up period of patients with TPGs. The most significant reduction in ventricular size was observed at the the 1-year followup, with only modest reduction thereafter.

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Long-term Reliability of Endoscopic Third Ventriculostomy

Neurosurgery ◽

10.1227/01.neu.0000159712.48093.ad ◽

2005 ◽

Vol 56 (6) ◽

pp. 1271-1278 ◽

Cited By ~ 93

Author(s):

David Kadrian ◽

James van Gelder ◽

Danielle Florida ◽

Robert Jones ◽

Marianne Vonau ◽

...

Keyword(s):

Endoscopic Third Ventriculostomy ◽

Third Ventriculostomy ◽

Aqueduct Stenosis ◽

Previous Infection ◽

Long Term Follow Up ◽

The Mean ◽

The Individual ◽

Operating Surgeon

Abstract OBJECTIVE: To describe the short-term operative success and the long-term reliability of endoscopic third ventriculostomy (ETV) for treatment of hydrocephalus and to examine the influence of diagnosis, age, and previous shunt history on these outcomes. METHODS: We retrospectively analyzed 203 consecutive patients from a single institution who had ETV as long as 22.6 years earlier. Patients with hydrocephalus from aqueduct stenosis, myelomeningocele, tumors, arachnoid cysts, previous infection, or hemorrhage were included. RESULTS: The overall probability of successfully performing an ETV was 89% (84–93%). There was support for an association between the surgical success and the individual operating surgeon (odds ratios for success, 0.44–1.47 relative to the mean of 1.0, P = 0.08). We observed infections in 4.9%, transient major complications in 7.2%, and major and permanent complications in 1.1% of 203 procedures. Age was strongly associated with long-term reliability. The longest observed reliability for the 13 patients 0 to 1 month old was 3.5 years. The statistical model predicted the following reliability at 1 year after insertion: at 0 to 1 month of age, 31% (14–53%); at 1 to 6 months of age, 50% (32–68%); at 6 to 24 months of age, 71% (55–85%); and more than 24 months of age, 84% (79–89%). There was no support for an association between reliability and the diagnostic group (n = 181, P = 0.168) or a previous shunt. Sixteen patients had ETV repeated, but only 9 were repeated after at least 6 months. Of these, 4 procedures failed within a few weeks, and 2 patients were available for long-term follow-up. CONCLUSION: Age was the only factor statistically associated with the long-term reliability of ETV. Patients less than 6 months old had poor reliability.

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Endoscopic third ventriculostomy in patients younger than 2 years: outcome analysis of 41 hydrocephalus cases

Journal of Neurosurgery Pediatrics ◽

10.3171/2009.11.peds09197 ◽

2010 ◽

Vol 5 (4) ◽

pp. 392-401 ◽

Cited By ~ 52

Author(s):

Albert A. Sufianov ◽

Galina Z. Sufianova ◽

Iurii A. Iakimov

Keyword(s):

Endoscopic Third Ventriculostomy ◽

Third Ventricle ◽

Negative Relationship ◽

Third Ventriculostomy ◽

Patient Age ◽

The Third ◽

Patient Âgé ◽

Significant Difference ◽

Effective Procedures

Object The object of this study was to analyze the outcome of endoscopic third ventriculostomy (ETV) in patients under 2 years of age and investigate factors related to ETV success or failure in this patient population. Methods The authors reviewed their experience in using endoscopic third ventriculostomy (ETV) in the treatment of 41 hydrocephalus patients younger than 2 years. The mean duration of follow-up was 45 months. The relationship between ETV efficacy and the following variables was analyzed: cause of hydrocephalus, level of CSF occlusion, primary versus secondary ETV, type of surgical procedure, head circumference, patient age at ETV, patient age at first manifestation of hydrocephalus, and anatomical features of the ventricle. Success of ETV was assessed based on the results of neurological examination and postoperative imaging during the follow-up period. Results The authors performed 32 primary ETVs and 10 secondary ETVs (ETV after hydrocephalus surgery) in 41 patients (a second ETV was performed in 1 patient). The success rates of primary and secondary ETV were 75.8 and 55.6%, respectively (no significant difference, p = 0.15). The ETV was clinically and radiologically successful in 30 (71.4%) of 42 procedures during a mean (± SD) follow-up period of 45.0 ± 4.8 months (range 12–127 months). A negative relationship was found between success of ETV and the thickness of the floor of the third ventricle (the most effective procedures were those in which the floor of the ventricle was thinnest [p < 0.05]). There was a highly significant correlation between ETV success and prolapse of the ventricle floor (p < 0.001). Also, there was an inverse relationship between ventricle floor thickness and the width of the third ventricle (p < 0.005). In our group of patients there was significant correlation between ETV success and patient age at onset of hydrocephalus (the most effective procedures were in patients in whom signs of hydrocephalus first occurred after 1 month of age [p = 0.02]). Conclusions Endoscopic third ventriculostomy was successful in 71.4% of procedures in children younger than 2 years and in 75.0% of procedures in infants. Success of ETV in children younger than 2 years depends not on the age of the patient or cause of hydrocephalus but on the thickness of the floor of the third ventricle and the patient's age at first manifestation of hydrocephalus.

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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

Journal of Neurosurgery Pediatrics ◽

10.3171/2017.10.peds17421 ◽

2018 ◽

Vol 21 (4) ◽

pp. 339-345 ◽

Cited By ~ 6

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.

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Fourth Ventricle Outlet Obstruction and Diverticular Enlargement of Luschka Foramina in a Child with Down Syndrome

Pediatric Neurosurgery ◽

10.1159/000511088 ◽

2020 ◽

pp. 1-4

Author(s):

Valentina Orlando ◽

Pietro Spennato ◽

Maria De Liso ◽

Vincenzo Trischitta ◽

Alessia Imperato ◽

...

Keyword(s):

Down Syndrome ◽

Fourth Ventricle ◽

Endoscopic Third Ventriculostomy ◽

Obstructive Hydrocephalus ◽

Radiological Finding ◽

Outlet Obstruction ◽

Third Ventriculostomy ◽

Viable Option ◽

Case Presentation

Introduction: Hydrocephalus is not usually part of Down syndrome (DS). Fourth ventricle outlet obstruction is a rare cause of obstructive hydrocephalus, difficult to diagnose, because tetraventricular dilatation may suggest a communicant/nonobstructive hydrocephalus. Case Presentation: We describe the case of a 6-year-old boy with obstructive tetraventricular hydrocephalus, caused by Luschka and Magendie foramina obstruction and diverticular enlargement of Luschka foramina (the so-called fourth ventricle outlet obstruction) associated with DS. He was treated with endoscopic third ventriculostomy (ETV) without complications, and a follow-up MRI revealed reduction of the ventricles, disappearance of the diverticula, and patency of the ventriculostomy. Conclusion: Diverticular enlargement of Luschka foramina is an important radiological finding for obstructive tetraventricular hydrocephalus. ETV is a viable option in tetraventricular obstructive hydrocephalus in DS.

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