Non-adjustable gravitational valves or adjustable valves in the treatment of hydrocephalus after aneurysmal subarachnoid hemorrhage patients?

PURPOSE: Hydrocephalus requiring permanent CSF shunting after aneurysmal subarachnoid hemorrhage (aSAH) is frequent. It is unknown which type of valve is optimal. This study evaluates if the revision rate of gravitational differential pressure valves (G-DPVs, GAV® system (B Braun)) (G-DPV) is comparable to adjustable pressure valves (Codman Medos Hakim) (APV) in the treatment of post-aSAH hydrocephalus. METHODS: The use of a gravitational differential pressure valve is placed in direct comparison with an adjustable pressure valve system. A retrospective chart review is performed to compare the revision rates for the two valve systems. RESULTS: Within the registry from Radboud University Medical Center, 641 patients with a SAH could be identified from 1 January 2013 until 1 January 2019, whereas at the Heinrich Heine University, 617 patients were identified, totaling 1258 patients who suffered from aSAH. At Radboud University Medical Center, a gravitational differential pressure valve is used, whereas at the Heinrich Heine University, an adjustable pressure valve system is used. One hundred sixty-six (13%) patients required permanent ventricular peritoneal or atrial shunting. Shunt dysfunction occurred in 36 patients: 13 patients of the 53 (25%) of the gravitational shunt cohort, and in 23 of the 113 (20%) patients with an adjustable shunt (p = 0.54). Revision was performed at a mean time of 3.2 months after implantation with the gravitational system and 8.2 months with the adjustable shunt system. Combined rates of over- and underdrainage leading to revision were 7.5% (4/53) for the gravitational and 3.5% (4/113) for the adjustable valve system (p = 0 .27). CONCLUSION: The current study does not show a benefit of a gravitational pressure valve (GAV® system) over an adjustable pressure valve (CODMAN ® HAKIM®) in the treatment of post-aSAH hydrocephalus. The overall need for revision is high and warrants further improvements in care.

Implementation of an Automated Cerebrospinal Fluid Drainage System for Early Mobilization in Neurosurgical Patients.

BACKGROUND: Automated cerebrospinal fluid (CSF) drainage systems allow for the mobilization of patients with an external CSF drain. The aim of this study is to describe the implementation of an automated CSF drainage system in neurosurgical patients with external CSF drains. METHODS: A feasibility study was performed using an automated CSF drainage system (LiquoGuard®7, Möller Medical GmbH, Fulda, Germany) in adult neurosurgical patients treated with external lumbar or external ventricular drains between December 2017 and June 2020. Limited mobilization was allowed-patients were allowed to adjust their inclined beds, sit in chairs and walk under the supervision of a nurse or physical therapist. The primary outcome was the number of prematurely terminated drainage sessions. RESULTS: Twenty-three patients were included. Drainage was terminated prematurely in eight (35%) patients. In three (13%) of these patients, drainage was terminated due to signs of hydrocephalus. Pressure-controlled drainage in patients with external lumbar drains (ELD) showed inaccurate pressure curves, which was solved by using volume-controlled drainage in ELD patients. CONCLUSION: The implementation of an automated CSF drainage system (LiquoGuard®7) for CSF drainage allows for early mobilization in a subset of patients with external CSF drains. External lumbar drains require volume-based drainage rather than differential pressure-dependent drainage.

Complications of external cerebrospinal fluid drainage in aneurysmal subarachnoid haemorrhage.

BACKGROUND: The need for external cerebrospinal fluid (CSF) drains in aneurysmal subarachnoid haemorrhage (aSAH) patients is common and might lead to additional complications. OBJECTIVE: A relation between the presence of an external CSF drain and complication risk is investigated. METHODS: A prospective complication registry was analysed retrospectively. We included all adult aSAH patients admitted to our academic hospital between January 2016 and January 2018, treated with an external CSF drain. Demographic data, type of external drain used, the severity of the aSAH and complications, up to 30 days after drain placement, were registered. Complications were divided into (1) complications with a direct relation to the external CSF drain and (2) complications that could not be directly related to the use of an external CSF drain referred to as medical complications RESULTS: One hundred and forty drains were implanted in 100 aSAH patients. In total, 112 complications occurred in 59 patients. Thirty-six complications were drain related and 76 were medical complications. The most common complication was infection (n = 34). Drain dislodgement occurred 16 times, followed by meningitis (n = 11) and occlusion (n = 9). A Poisson model showed that the mean number of complications raised by 2.9% for each additional day of drainage (95% CI: 0.6-5.3% p = 0.01). CONCLUSION: Complications are common in patients with aneurysmal subarachnoid haemorrhage of which 32% are drain-related. A correlation is present between drainage period and the number of complications. Therefore, reducing drainage period could be a target for further improvement of care.

Need for post-operative outpatient appointments after discharge following cervical spinal surgery - a narrative review.

BACKGROUND: In the Netherlands most patients are currently seen in an outpatient clinic after an anterior cervical discectomy, which is an effective neurosurgical procedure with relatively low rate of severe complications. In this back sight, the need for patients returning to the post-operative outpatient clinic could be questioned. The aim of the study is to evaluate whether a post-operative outpatient appointment after anterior cervical discectomy could be replaced by an alternative or be omitted without adversely impacting, or increasing, the value of healthcare and patient satisfaction for this procedure. METHODS: A narrative review was performed to evaluate the quality of care and patient satisfaction for patients with and without a post-operative outpatient appointment after spinal surgery. A literature search of the previous ten years was performed in Pubmed, CENTRAL and EMBASE. RESULTS: A total of 403 articles were identified. Four studies remained after title and abstract selection by 3 independent reviewers. No papers were selected for further analysis, due to the absence of interventional studies that compared the utility of a post-operative outpatient clinic appointment with an intervention after spinal surgery. CONCLUSIONS: Currently, there is a lack of evidence for the need of a post-operative follow-up after anterior cervical discectomy. Nor is there any literature in favor of omitting these appointments. No determinants which patients benefits from these outpatient appointments could be identified. Potential harmful and beneficial effects of omitting these post-operative follow-ups should be investigated to identify possible determinant for patients who might benefit from a post-operative appointment.

Combining deep learning with 3D stereophotogrammetry for craniosynostosis diagnosis.

Craniosynostosis is a condition in which cranial sutures fuse prematurely, causing problems in normal brain and skull growth in infants. To limit the extent of cosmetic and functional problems, swift diagnosis is needed. The goal of this study is to investigate if a deep learning algorithm is capable of correctly classifying the head shape of infants as either healthy controls, or as one of the following three craniosynostosis subtypes; scaphocephaly, trigonocephaly or anterior plagiocephaly. In order to acquire cranial shape data, 3D stereophotographs were made during routine pre-operative appointments of scaphocephaly (n = 76), trigonocephaly (n = 40) and anterior plagiocephaly (n = 27) patients. 3D Stereophotographs of healthy infants (n = 53) were made between the age of 3-6 months. The cranial shape data was sampled and a deep learning network was used to classify the cranial shape data as either: healthy control, scaphocephaly patient, trigonocephaly patient or anterior plagiocephaly patient. For the training and testing of the deep learning network, a stratified tenfold cross validation was used. During testing 195 out of 196 3D stereophotographs (99.5%) were correctly classified. This study shows that trained deep learning algorithms, based on 3D stereophotographs, can discriminate between craniosynostosis subtypes and healthy controls with high accuracy.

Bedside placement of ventricular access devices under local anaesthesia in neonates with posthaemorrhagic hydrocephalus: preliminary experience.

PURPOSE: Posthaemorrhagic ventricular dilatation in preterm infants is primarily treated using temporising measures, of which the placement of a ventricular access device (VAD) is one option. Permanent shunt dependency rates are high, though vary widely. In order to improve the treatment burden and lower shunt dependency rates, we implemented several changes over the years. One of these changes involves the setting of the surgery from general anaesthesia in the OR to local anaesthesia in bed at the neonatal intensive care unit (NICU), which may seem counterintuitive to many. In this article, we describe our surgical technique and present the results of this regimen and compare it to our previous techniques. METHODS: Retrospective study of a consecutive series of 37 neonates with posthaemorrhagic ventricular dilatation (PHVD) treated using a VAD, with a cohort I (n = 13) treated from 2004 to 2008 under general anaesthesia in the OR, cohort II (n = 11) treated from 2009 to 2013 under general anaesthesia in the NICU and cohort III (n = 13) treated from December 2013 to December 2017 under local anaesthesia on the NICU. RESULTS: The overall infection rate was 14%; the VAD revision rate was 22% and did not differ significantly between the cohorts. Procedures under local anaesthesia never required conversion to general anaesthesia and were well tolerated. After an average of 33 tapping days, 38% of the neonates received a permanent ventriculoperitoneal (VP) shunt. The permanent VP shunt rate was 9% with VAD placement under local anaesthesia and 52% when performed under general anaesthesia (p = 0.02). CONCLUSION: Bedside placement of VADs for PHVD under local anaesthesia in neonates is a low-risk, well-tolerated procedure that results in at least equal results to surgery performed under general anaesthesia and/or performed in an OR.

Route of antibiotic prophylaxis for prevention of cerebrospinal fluid-shunt infection.

BACKGROUND: The main complication of cerebrospinal fluid (CSF) shunt surgery is shunt infection. Prevention of these shunt infections consists of the perioperative use of antibiotics that can be administered in five different ways: orally; intravenously; intrathecally; topically; and via the implantation of antibiotic-impregnated shunt catheters. OBJECTIVES: To determine the effect of different routes of antibiotic prophylaxis (i.e. oral, intravenous, intrathecal, topical and via antibiotic-impregnated shunt catheters) on CSF-shunt infections in persons treated for hydrocephalus using internalised CSF shunts. SEARCH METHODS: We conducted a systematic electronic search without restrictions on language, date or publication type. We performed the search on the Cochrane Central Register of Controlled Trials (CENTRAL) in the Cochrane Library, MEDLINE and Embase, with the help of the Information Specialist of the Cochrane Multiple Sclerosis and Rare Diseases of the CNS Group. The search was performed in January 2018. SELECTION CRITERIA: All randomised and quasi-randomised controlled trials that studied the effect of antibiotic prophylaxis, in any dose or administration route, for the prevention of CSF-shunt infection in patients that were treated with an internal cerebrospinal fluid shunt. Patients with external shunts were not eligible. DATA COLLECTION AND ANALYSIS: Two review authors independently extracted data from included studies. We resolved disagreements by discussion or by referral to an independent researcher within our department when necessary. Analyses were also performed by at least two authors. MAIN RESULTS: We included a total of 11 small randomised controlled trials, containing 1109 participants, in this systematic review. Three of these studies included solely children, and the remaining eight included participants of all ages. Most studies were limited to the evaluation of ventriculoperitoneal shunts. However, five studies included participants with ventriculoatrial shunts, of which one study contained four participants with a subduroperitoneal shunt. We judged four out of 11 (36%) trials at unclear risk of bias, while the remaining seven trials (64%) scored high risk of bias in one or more of the components assessed.We analysed all included studies in order to estimate the effect of antibiotic prophylaxis on the proportion of shunt infections regardless of administration route. Although the quality of evidence in these studies was low, there may be a positive effect of antibiotic prophylaxis on the number of participants who had shunt infections (RR 0.55, 95% CI 0.36 to 0.84), meaning a 55% reduction in the number of participants who had shunt infection compared with standard care or placebo.Within the different administration routes, only within intravenous administration of antibiotic prophylaxis there may be evidence of an effect on the risk of shunt infections (RR 0.55, 95% CI 0.33 to 0.90). However, this was the only route that contained more than two studies (8 studies; 797 participants). Evidence was uncertain for both, intrathecal administration of antibiotics (RR 0.73, 95% CI 0.28 to 1.93, 2 studies; 797 participants; low quality evidence) and antibiotic impregnated catheters (RR 0.36, 95% CI 0.10 to 1.24, 1 study; 110 participants; very low quality evidence) AUTHORS' CONCLUSIONS: Antibiotic prophylaxis may have a positive effect on lowering the number of participants who had shunt infections. However, the quality of included studies was low and the effect is not consistent within the different routes of administration that have been analysed. It is therefore uncertain whether prevention of shunt infection varies by different antibiotic agents, different administration routes, timing and doses; or by characteristics of patients, e.g. children and adults. The results of the review should be seen as hypothesis-generating rather than definitive, and the results should be confirmed in adequately powered trials or large multicentre studies in order to obtain high-quality evidence in the field of ventricular shunt infection prevention.

Evaluation of anesthesia in endoscopic strip craniectomy: A review of 121 patients.

OBJECTIVE: The aim of this study was to evaluate pre-, intra-, and postoperative anesthetic parameters in endoscopic strip craniectomy in order to improve anesthesiological care. MATERIALS AND METHODS: This is a retrospective patient cohort study of our first 121 patients treated by endoscopic strip craniectomy. Preoperative as well as intra- and postoperative anesthesiological and neurological parameters were analyzed. Furthermore, the need for intensive care unit admission, blood loss, and blood transfusion rate were measured. RESULTS: The mean age of patients was 3.9 months (standard deviation = 1) at a mean weight of 6.3 kg (standard deviation = 1.3). Comorbidity was registered in 13 (11%) patients of which 5 had syndrome-related comorbidities. Mean duration of anesthesia was 131 minutes (standard deviation = 32) . One hundred and sixteen patients were induced by mask induction with sevoflurane and 5 patients were induced intravenously. In 10 patients, mild intraoperative hypothermia (between 35 and 36 degrees Celsius) occurred. The mean estimated blood loss was 35.4 mL (standard deviation = 28.9) and blood transfusion rate was 21.5%. Brief and small intraoperative oxygen saturation drops were common during this study. No indication for venous air embolism was found based on endtidal CO2 . Postoperative temperature above 38 degrees Celsius occurred 16 times and benign deviations in postoperative cardiopulmonary parameters occurred in 17 patients. Postoperative pain management was mainly established by paracetamol and low-dose morphine when necessary. No postoperative neurological symptoms were reported and no deaths occurred. CONCLUSION: These patients had a relatively short intraoperative course with stable vital parameters during surgery. We report a low incidence of significant venous air embolism, a blood transfusion rate of 21% and only minor perioperative disturbances in vital parameters.

Topical vancomycin reduces the cerebrospinal fluid shunt infection rate: A retrospective cohort study.

OBJECT: Despite many efforts at reduction, cerebrospinal fluid (CSF) shunt infections are a major cause of morbidity in shunt surgery, occurring in 5-15% of cases. To attempt to reduce the shunt infection rate at our institution, we added topical vancomycin (intrashunt and perishunt) to our existing shunt infection prevention protocol in 2012. METHODS: We performed a retrospective cohort study comparing all shunted patients in January 2010 to December 2011 without vancomycin (control group, 263 procedures) to all patients who underwent shunt surgery between April 2012 and December 2015 with vancomycin (intervention group, 499 procedures). RESULTS: The overall shunt infection rate significantly decreased from 6.8% (control group) to 3.0% (intervention group) (p = 0.023, absolute risk reduction 3.8%, relative risk reduction 56%). Multivariate logistic regression analysis confirmed that the addition of topical vancomycin showed that cases treated under a protocol of topical vancomycin were associated with a decreased shunt infection rate (odds ratio [OR] 0.49 95% CI 0.25-0.998; p = 0.049). Age < 1 year was associated with an increased risk of infection (OR) 4.41, 95% CI 2,10-9,26; p = 0.001). Time from surgery to infection was significantly prolonged in the intervention group (p = 0.001). CONCLUSION: Adding intraoperative vancomycin to a shunt infection prevention protocol significantly reduces CSF shunt infection rate.

Toward Shorter Hospitalization After Endoscopic Transsphenoidal Pituitary Surgery: Day-by-Day Analysis of Early Postoperative Complications and Interventions.

BACKGROUND: It is unclear which patients have the greatest risk of developing complications in the first days after endoscopic transsphenoidal pituitary surgery (ETS) and how long patients should stay hospitalized after surgery. The objective of this study is to identify which patients are at risk for early postoperative medical and surgical reinterventions to optimize the length of hospitalization. METHODS: The medical records of 146 patients who underwent ETS for a pituitary adenoma between January 2013 and July 2016 were reviewed retrospectively. Data were collected on baseline patient-related characteristics, characteristics of the pituitary adenoma, perioperative complications and interventions, and postoperative outcomes. Patients who underwent additional interventions on days 2, 3, and 4 after ETS were identified as cases, and patients who did not have any interventions after day 1 postoperatively were identified as controls. RESULTS: Diabetes mellitus (odds ratio [OR], 4.279; 95% confidence interval [CI], 1.149-15.933; P = 0.03), incomplete adenoma resection (OR, 2.840; 95% CI, 1.228-6.568; P = 0.02) and increased morning sodium concentration on day 2 after surgery (OR, 5.211; 95% CI, 2.158-12.579; P <0.001) were associated with reinterventions. Patients without interventions on day 1 or 2 had only an 18.6% chance of a reintervention (OR, 0.201; 95% CI, 0.095-0.424). CONCLUSIONS: Patients with diabetes mellitus, incomplete adenoma resection, and increased morning sodium concentration on day 2 after surgery have an increased chance on reinterventions. In addition, patients without any interventions on day 1 and 2 are at low risk for later reinterventions. These patients could be suitable candidates for early hospital discharge.

Intraoperative and postoperative complications in the surgical treatment of craniosynostosis: minimally invasive versus open surgical procedures.

OBJECTIVE To compare minimally invasive endoscopic and open surgical procedures, to improve informed consent of parents, and to establish a baseline for further targeted improvement of surgical care, this study evaluated the complication rate and blood transfusion rate of craniosynostosis surgery in our department. METHODS A prospective complication registration database that contains a consecutive cohort of all pediatric neurosurgical procedures in the authors' neurosurgical department was used. All pediatric patients who underwent neurosurgical treatment for craniosynostosis between February 2004 and December 2014 were included. In total, 187 procedures were performed, of which 121 were endoscopically assisted minimally invasive procedures (65%). Ninety-three patients were diagnosed with scaphocephaly, 50 with trigonocephaly, 26 with plagiocephaly, 3 with brachycephaly, 9 with a craniosynostosis syndrome, and 6 patients were suffering from nonsyndromic multisutural craniosynostosis. RESULTS A total of 18 complications occurred in 187 procedures (9.6%, 95% CI 6.2-15), of which 5.3% (n = 10, 95% CI 2.9-10) occurred intraoperatively and 4.2% (n = 8, 95% CI 2.2-8.2) occurred postoperatively. In the open surgical procedure group, 9 complications occurred: 6 intraoperatively and 3 postoperatively. In the endoscopically assisted procedure group, 9 complications occurred: 4 intraoperatively and 5 postoperatively. Blood transfusion was needed in 100% (n = 66) of the open surgical procedures but in only 21% (n = 26, 95% CI 15-30) of the endoscopic procedures. One patient suffered a transfusion reaction, and 6 patients suffered infections, only one of which was a surgical site infection. A dural tear was the most common intraoperative complication that occurred (n = 8), but it never led to postoperative sequelae. Intraoperative bleeding from a sagittal sinus occurred in one patient with only minimal blood loss. There were no deaths, permanent morbidity, or neurological sequelae. CONCLUSIONS Complications during craniosynostosis surgery were relatively few and minor and were without permanent sequelae in open and in minimally invasive procedures. The blood transfusion rate was significantly reduced in endoscopic procedures compared with open procedures.

Radiation-free 3D head shape and volume evaluation after endoscopically assisted strip craniectomy followed by helmet therapy for trigonocephaly.

INTRODUCTION: Radiation-free 3D post-operative sequential follow-up in craniosynostosis is hindered by the lack of consistent markers restricting evaluation to subjective comparison. However, using the computed cranial focal point (CCFP), it is possible to perform correct sequential image superposition and objective evaluation. We used this technique for mean volume and shape change evaluation of the head utilizing 3D photos after endoscopically assisted trigonocephaly surgery. METHODS: We performed a mean head shape and volume evaluation on age grouped 3D photos (n = 86) of children who underwent endoscopically assisted strip craniectomy with helmet therapy. We used CT-scans of healthy children as reference. We performed a mean shape evolution analysis and calculated the anterior fossa to total volume ratio (A/T-ratio). The volume- and A/T-ratio pattern were compared with the reference group. RESULTS: The mean anterior fossa volume evolved from 336 ml (33.4% A/T-ratio) pre-surgery to 664 ml (36.0% A/T-ratio) at 37-48 months post-surgery. Both groups have a near similar volume- and A/T-ratio pattern over time. The first 18 months show a predominant growth around the resected metopic suture. Between 18 and 24 months we observed mostly anterior orbital rim growth. From 24 months till 36-48 months the head grows predominantly at the temporal area. The least outward growth was observed at the temporal bones. CONCLUSION: Using a novel technique we were able to objectively evaluate head shape and volume using stereophotogrammetry after endoscopically assisted strip craniectomy. The A/T-ratio and volume growth pattern of endoscopically treated patients is near identical to that of the normal reference group.

Endoscopically assisted craniosynostosis surgery (EACS): The craniofacial team Nijmegen experience.

INTRODUCTION: An evaluation of our first 111 consecutive cases of non-syndromic endoscopically assisted craniosynostosis surgery (EACS) followed by helmet therapy. METHODS: Retrospective analysis of a prospective registration database was performed. Age, duration of surgery, length of hospital stay, blood loss, transfusion rate, cephalic index and duration of helmet therapy were evaluated. An online questionnaire was used to evaluate the burden of the helmet therapy for the child and parents. RESULTS: 111 EAC procedures were performed: 64 for scaphocephaly, 34 for trigonocephaly and13 for anterior plagiocephaly. The mean age at the time of surgery was 3.9 (±1) months, mean surgical time was 58 (±18) minutes, mean blood loss was 34 (±28) ml, transfusion rate was 22% (n = 26), mean duration of postoperative helmet therapy was 10 (±2.5) months, mean preoperative and postoperative CI were respectively 0.67(±0.057) and 0.72 (±0.062) in scaphocephalic patients and the mean length of hospital stay was 2.6 (±1) days. The burden of the helmet therapy for the child and his family was deemed very low. CONCLUSION: EACS for non-syndromic patients shows low morbidity rates, short surgical time, short length of hospital stay, little blood loss and low need for blood transfusion and is associated with satisfying cosmetic results.

Intraoperative complications in pediatric neurosurgery: review of 1807 cases.

OBJECTIVE Minimal literature exists on the intraoperative complication rate of pediatric neurosurgical procedures with respect to both surgical and anesthesiological complications. The aim of this study, therefore, was to establish intraoperative complication rates to provide patients and parents with information on which to base their informed consent and to establish a baseline for further targeted improvement of pediatric neurosurgical care. METHODS A clinical complication registration database comprising a consecutive cohort of all pediatric neurosurgical procedures carried out in a general neurosurgical department from January 1, 2004, until July 1, 2012, was analyzed. During the study period, 1807 procedures were performed on patients below the age of 17 years. RESULTS Sixty-four intraoperative complications occurred in 62 patients (3.5% of procedures). Intraoperative mortality was 0.17% (n = 3). Seventy-eight percent of the complications (n = 50) were related to the neurosurgical procedures, whereas 22% (n = 14) were due to anesthesiology. The highest intraoperative complication rates were for cerebrovascular surgery (7.7%) and tumor surgery (7.4%). The most frequently occurring complications were cerebrovascular complications (33%). CONCLUSIONS Intraoperative complications are not exceptional during pediatric neurosurgical procedures. Awareness of these complications is the first step in preventing them.

High resolution pituitary gland MRI at 7.0 tesla: a clinical evaluation in Cushing's disease.

OBJECTIVE: To evaluate the detection of pituitary lesions at 7.0 T compared to 1.5 T MRI in 16 patients with clinically and biochemically proven Cushing's disease. METHODS: In seven patients, no lesion was detected on the initial 1.5 T MRI, and in nine patients it was uncertain whether there was a lesion. Firstly, two readers assessed both 1.5 T and 7.0 T MRI examinations unpaired in a random order for the presence of lesions. Consensus reading with a third neuroradiologist was used to define final lesions in all MRIs. Secondly, surgical outcome was evaluated. A comparison was made between the lesions visualized with MRI and the lesions found during surgery in 9/16 patients. RESULTS: The interobserver agreement for lesion detection was good at 1.5 T MRI (κ = 0.69) and 7.0 T MRI (κ = 0.62). In five patients, both the 1.5 T and 7.0 T MRI enabled visualization of a lesion on the correct side of the pituitary gland. In three patients, 7.0 T MRI detected a lesion on the correct side of the pituitary gland, while no lesion was visible at 1.5 T MRI. CONCLUSION: The interobserver agreement of image assessment for 7.0 T MRI in patients with Cushing's disease was good, and lesions were detected more accurately with 7.0 T MRI. KEY POINTS: Interobserver agreement for lesion detection on 1.5 T MRI was good; Interobserver agreement for lesion detection on 7.0 T MRI was good; 7.0 T enabled confirmation of unclear lesions at 1.5 T; 7.0 T enabled visualization of lesions not visible at 1.5 T.

Intradural prepontine chordoma in an 11-year-old boy. A case report.

CASE REPORT: The authors report a case of an 11-year-old boy that presented with headache and vomiting that was present for several months. CT and MR imaging revealed a large prepontine mass and an obstructive hydrocephalus. A ventriculoperitoneal shunt was inserted, and in a second operation, a radiologically proven total resection was performed, using a left frontotemporal transsylvian approach. The tumour showed no involvement of the dura or clivus. Histological examination showed the characteristics of a chordoma. No further adjuvant treatment was given. The patient remained disease or tumour free after a 6-year follow-up. DISCUSSION: Intradural chordomas are extremely rare tumours that originate from notochordal remnants. Only three other cases have been reported in the paediatric population. Ecchordosis physaliphora (EP) is an ectopic notochordal remnant that has a similar biological behaviour and is difficult to distinguish from intradural chordomas. They might exist in a continuum from benign notochordal tumour to malignant chordoma. A surgical resection without adjuvant radiation therapy is suggested to be the treatment of choice in the paediatric population. CONCLUSION: The authors describe a rare case of an intradural prepontine chordoma in an 11-year-old boy that stayed disease free after a 6-year follow-up.

Acute toxicity profile of craniospinal irradiation with intensity-modulated radiation therapy in children with medulloblastoma: A prospective analysis.

BACKGROUND: To report on the acute toxicity in children with medulloblastoma undergoing intensity-modulated radiation therapy (IMRT) with daily intrafractionally modulated junctions. METHODS: Newly diagnosed patients, aged 3-21, with standard-risk (SR) or high-risk (HR) medulloblastoma were eligible. A dose of 23.4 or 36.0 Gy in daily fractions of 1.8 Gy was prescribed to the craniospinal axis, followed by a boost to the primary tumor bed (54 or 55.8 Gy) and metastases (39.6-55.8 Gy), when indicated. Weekly, an intravenous bolus of vincristine was combined for patients with SR medulloblastoma and patients participating in the COG-ACNS-0332 study. Common toxicity criteria (CTC, version 2.0) focusing on skin, alopecia, voice changes, conjunctivitis, anorexia, dysphagia, gastro-intestinal symptoms, headache, fatigue and hematological changes were scored weekly during radiotherapy. RESULTS: From 2010 to 2014, data from 15 consecutive patients (SR, n = 7; HR, n = 8) were collected. Within 72 h from onset of treatment, vomiting (66 %) and headache (46 %) occurred. During week 3 of treatment, a peak incidence in constipation (33 %) and abdominal pain/cramping (40 %) was observed, but only in the subgroup of patients (n = 9) receiving vincristine (constipation: 56 vs 0 %, P = .04; pain/cramping: 67 vs 0 %, P = .03). At week 6, 73 % of the patients developed faint erythema of the cranial skin with dry desquamation (40 %) or moist desquamation confined to the skin folds of the auricle (33 %). No reaction of the skin overlying the spinal target volume was observed. CONCLUSIONS: Headache at onset and gastro-intestinal toxicity, especially in patients receiving weekly vincristine, were the major complaints of patients with medulloblastoma undergoing craniospinal irradiation with IMRT.

[Idiopathic normal pressure hydrocephalus and the older patient]. / Idiopathische Normale Druk Hydrocephalie en de oudere patiënt.

Idiopathic Normal Pressure Hydrocephalus (iNPH) is characterized by the clinical triad of cognitive disorders, gait impairment and urinary incontinence. The treatment is the implantation of a ventriculoperitoneal shunt. At present there are still no high quality determinants to predict the long term outcome after shunt implantation, because studies are likely to be biased, use many different study methods and are difficult to interpret by potential concomitant neurodegenerative diseases, like Alzheimer and vascular dementia. Because this comorbidity also determines whether the expected positive outcome of a shunt outweighs the risk of complications, a critical multidisciplinary analysis of comorbidity, frailty and patient preferences is a precondition to realize added value.

Clinical outcome of cloacal exstrophy, current status, and a change in surgical management.

INTRODUCTION: Cloacal exstrophy is a complex and rare congenital malformation. Because of improvements in medicine, operative techniques, and perioperative management the survival rates are now approaching 100%. Currently, treatment is focused on improving quality of life. Since 1974, we encountered 20 patients with cloacal exstrophy in our hospital. The aim of this study is to evaluate our clinical experience and outcome during the last 39 years. PATIENTS AND METHODS: A retrospective study of the medical records was performed. We evaluated anatomical status, phenotype and genotype at birth, subsequent surgical treatment and current gastrointestinal, urinary, spinal, genital, and gender status and outcome. RESULTS: The records of all 20 patients who were treated in our center were included in this study. Of the 20 patients, 18 were primary patients, 2 referred. Six patients died within the first year of life. Median age of the surviving 14 patients was 25 years (range, 4-39 years) at time of evaluation. At the last follow-up, 11 patients had an endileostomy or endcolostomy. Three patients had a pull-through; two of them were continent for feces. Two patients were continent for urine; one with a sinus urogenitalis and one after urethra reconstruction. Eleven patients are incontinent; six of them had an incontinent urinary deviation (Bricker deviation). Of the other five incontinent patients, two had a urethral reconstruction, one vesicovaginal anastomosis, one perineotomy, and one epispadias bladder. Urinary continence was unknown in one patient. Of the surviving patients, nine have 46 XY karyotype and five 46 XX karyotype. Initially, six XY patients underwent external genital reconstruction with reassignment to female gender; however, one died postoperatively. One declared to feel like a man at age of 15 years. Remaining four XY patients underwent male external genital reconstruction. All XX patients underwent vagina or vulva reconstruction, except for one who still has aplasia vaginae. All patients were born with varying types of spinal dysraphism. DISCUSSION: Despite medical efforts and development in treatment and support, satisfaction in outcome of cloacal exstrophy remains a challenge. Although reconstruction may be very difficult, male genital reconstruction in 46 XY patients has been given preference for more than a decade. A specialized multidisciplinary team should provide lifelong colorectal, urological, sexual, and psychosocial support to these patients, taking into account their age and phase of life.