Search

Objectives: The transphenoidal bi-nostril endoscopic resection of pituitary adenomas is regarded as a minimally invasive treatment nowadays. However, sino-nasal outcome and health-related quality of life (HRQoL) might still be impaired after the adenomectomy, depending on patients’ prior medical history and health status. A systematic postoperative comparison is required to assess differences in perceived sino-nasal outcome and HRQoL. Methods: In this single-center observational study, we collected data from 81 patients, operated between August 2016 and August 2021, at a 3–6-month follow-up after adenomectomy. We employed the sino-nasal outcome test for neurosurgery (SNOT-NC) and the HRQoL inventory Short Form (SF)-36 to compare sino-nasal and HRQoL outcome in patients with or without allergies, previous nose surgeries, presence of pain, snoring, sleep apnea, usage of continuous positive airway pressure (cpap), and nose drop usage. Results: At the 3–6-month follow-up, patients with previous nasal surgery showed overall reduced subjective sino-nasal health, increased nasal and ear/head discomfort, increased visual impairment, and decreased psychological HRQoL (all p ≤ 0.026) after pituitary adenomectomy. Patients with pain before surgery showed a trend-level aggravated physical HRQoL (p = 0.084). Conclusion: Our data show that patients with previous nasal surgery have an increased risk of an aggravated sino-nasal and HRQoL outcome after pituitary adenomectomy. These patients should be thoroughly informed about potential consequences to induce realistic patient expectations. Moreover, the study shows that patients with moderately severe allergies, snoring, and sleep apnea (± cpap) usually do not have to expect a worsened sino-nasal health and HRQoL outcome.

Purpose Spinal cavernous malformations (SCM) present a risk for intramedullary hemorrhage (IMH), which can cause severe neurologic deficits. Patient selection and time of surgery have not been clearly defined. Methods This observational study included SCM patients who underwent surgery in our department between 2003 and 2021. Inclusion required baseline clinical factors, magnetic resonance imaging studies, and follow-up examination. Functional outcome was assessed using the Modified McCormick scale score. Results Thirty-five patients met the inclusion criteria. The mean age was 44.7 ± 14.5 years, and 60% of the patients were male. In univariate analysis, the unfavorable outcome was significantly associated with multiple bleeding events (p = .031), ventral location of the SCM (p = .046), and incomplete resection (p = .028). The time between IMH and surgery correlated with postoperative outcomes (p = .004), and early surgery within 3 months from IMH was associated with favorable outcomes (p = .033). This association remained significant in multivariate logistic regression analysis (p = .041). Conclusions Removal of symptomatic SCM should be performed within 3 months after IMH when gross total resection is feasible. Patients with ventrally located lesions might be at increased risk for postoperative deficits.

OBJECTIVE The aim of this study was to investigate the functional outcome in spinal cavernous malformation (SCM) patients with single or multiple intramedullary hemorrhagic events. METHODS SCM patients who were conservatively treated between 2003 and 2021 and had complete clinical baseline characteristics, an MRI data set, at least one SCM-related intramedullary hemorrhage (IMH), and at least one follow-up examination were included in this study. Functional status was assessed using the modified McCormick Scale score at diagnosis, before and after each bleeding event, and at the last follow-up. RESULTS A total of 45 patients were analyzed. Univariate analysis identified multiple bleeding events as the only statistically significant predictor for an unfavorable functional outcome at the last follow-up (OR 15.28, 95% CI 3.22–72.47; p < 0.001). Patients significantly deteriorated after the first hemorrhage (29.0%, p = 0.006) and even more so after the second hemorrhage (84.6%, p = 0.002). Multiple bleeding events were significantly associated with functional deterioration at the last follow-up (76.9%, p = 0.003). The time between the last IMH and the last follow-up did not influence this outcome. CONCLUSIONS IMH due to SCM is linked to functional worsening. Such outcomes tend to improve after each hemorrhage, but the probability of full recovery declines with each bleeding event.

Introduction Brachial plexus lipomas are extremely rare benign tumors that may cause slow progression of neurological deficits leading to thoracic outlet syndrome. Up to now, surgery remains challenging. The aim of this study is to present our surgical treatment regime and long-term neurological outcome in three cases of giant brachial plexus lipomas and to show results of systematic review.Patients and Methods Retrospective analysis of our database “peripheral nerve lesion” to identify patients suffering from brachial plexus lipomas between January 1, 2012, and December 31, 2019. Systematic review was performed for literature published until March 31, 2020, analyzing PubMed, Google Scholar, Scopus, and the Cochrane Collaboration Library independently by two authors.Results Over the past years, three patients suffering from giant brachial plexus lipomas attended to our neurosurgical department. All patients underwent preoperative magnetic resonance imaging (MRI), ultrasound examinations, and electrophysiological testing. Tumors were removed microsurgically via anterior/posterior, supraclavicular/infraclavicular, and combined approaches. The patients were accessed postoperatively by MRI and clinical follow-up. Systematic review of the literature revealed 22 cases, which were analyzed in regard to demographics, surgical treatment, and neurological outcome.Conclusion Brachial plexus lipomas are an extremely rare cause for brachial plexus compression. Total microsurgical removal with intraoperative electrophysiological monitoring is the treatment of choice with excellent long-term MRI and clinical outcome.

Objectives Isocitrate dehydrogenase (IDH1/2) mutations and O6-alkylguanine DNA methyltransferase (MGMT) promoter methylations are acknowledged survival predictors in patients with glioblastoma (GB). Moreover, tumor growth patterns like multifocality and subventricular zone (SVZ) involvement seem to be associated with poorer outcomes. Here, we wanted to evaluate the influence of the SVZ involvement and the multifocal tumor growth on the extent of surgical resection and its correlation with overall survival (OS) and molecular characteristics of patients with GB. Methods Adult patients with primary GB who underwent surgery at our department between 2012 and 2014 were included. Preoperative magnetic resonance imaging findings were analyzed with regard to tumor location, presence of multifocality and SVZ involvement. The extent of surgical resection as well as clinical and molecular parameters was collected from electronic patient records. Univariate and multivariate analyses were performed. Results Two hundred eight patients were retrospectively analyzed, comprising 90 (43.3%) female individuals with a mean age of 62.9 (±12.26) years and OS of 10.2 months (±8.9). Unifocal tumor location was a predictor for better OS with a mean of 11.4 (±9.4) months (vs. 8.0 [±7.4] months, p=0.008). Affection of the SVZ was also associated with lower surgical resection rates (p. All six IDH1/2 wildtype tumors showed an unifocal location (p=0.066). MGMT promoter methylation was not associated with multifocal tumor growth (p=0.649) or SVZ involvement (p=0.348). Multivariate analysis confirmed independent association between the SVZ involvement and OS (p=0.001). Conclusion The involvement of the SVZ appears to have an influence on a lower resection rate of GB. This negative impact of SVZ on GB outcome might be related to lesser extent of resection, higher rates of multifocality and greater surgical morbidity but not inevitably to IDH1/2 mutation and MGMT promoter methylation status.

Background: Anterior transarticular screw fixation (ATSF) of C1/C2 can be used for the treatment of unstable dens fractures. Here, we evaluated the feasibility of an anterior C1/C2 fixation in elderly patients with unstable dens fractures. Furthermore, we tried to analyze safe entry zones for ATSF surgery. Methods: A consecutive cohort of 13 patients with unstable dens fractures were treated with ATSF of C1/C2 between January 2015 and October 2016. If necessary, an additional screw was placed into the odontoid process. The placement was radiographically analyzed using the 3D Arcadis Orbic (Siemens, Erlangen, Germany). Additionally, computed tomography scans of the cervical spine from 50 trauma patients were analyzed to evaluate safe entry zones for anterior odontoid screw fixation and for ATSF. Results: ATSF was performed in 13 cases (7 female, 6 male; mean age 81.80 years). One screw had to be corrected intraoperatively due to initial malplacement. Neurological deficits or an injury of the vertebral artery were not observed. All patients suffered from swallowing difficulties during the postoperative course, without lesions of the esophagus or the trachea. In 4 patients (30.8%), an additional posterior fixation was offered to the patients due to progressive loosening of the screws. The anatomical-radiographic analyzes revealed a significantly shallower angle of trajectory for anterior odontoid screw fixation (24.9° ± 5.85°) than for ATSF (39.1° ± 6.44° (left); 40.5° ± 6.79° (right) P = 0.02). Conclusions: The ATSF of C1/C2 might be a valuable option in the treatment of instable C1/C2 fractures, especially in the elderly or in patients with short necks and/or high body mass index due to the steeper trajectory compared with odontoid screw placement. Yet available screws seem to be of inferior resistance compared with the biomechanical properties of a dorsal fixation. Further studies should focus on screws with better mechanical properties and probably additional cement augmentation. Level of Evidence: 4.

Preoperative traction with the Gardner-Wells tongs (PTGWT) is a valuable option for cervical spine injuries with malalignment. The aim of this study was to analyze the factors related to the treatment success of PTGWT.All consecutive cases with PTGWT due to cervical spine injury with malalignment treated between 01/2010 and 09/2020 were included. Patients' records were reviewed for demographic and clinical characteristics. Treatment success was evaluated upon the angle correction in the sagittal plane using the computed tomography scans before and after the treatment.Of 20 patients in the final analysis (median age: 77.5 years; 12 females [60%]), 14 individuals were treated for the type-II odontoid fracture, and six cases presented with subluxation fractures between C3 and C7. After PTGWT and subsequent intraoperative reposition, there was an improvement of the median deviation angle from initial 32° to 5.5°. PTGWT resulted in a significant improvement of the median deviation angle for the odontoid (17°, p0.0001), but not for the subluxation (4°, p=0.10) fractures. The time interval between trauma and PTGWT was associated with the treatment success of subluxation (p=0.051) but not of odontoid (p=0.87) fractures. Older individuals aged 51≥ years showed better reposition results with PTGWT (17° vs. 7.5°, p=0.02). There were no PTGWT-related complications in the cohort.PTGWT is an effective and safe treatment for cervical spine injuries with malalignment. The patients with odontoid fractures might particularly profit from the PTGWT. Treatment delay seems more relevant for PTGWT success in subluxation than in odontoid fractures.

Spinal epidural lipomatosis (SEL) is a rare condition caused by hypertrophic growth of epidural fat. The prevalence of SEL in the Western world is approximately 1 in 40 patients and is likely to increase due to current medical and socio-economic developments. Rarely, SEL can lead to rapid severe neurological deterioration. The pathophysiology, optimal treatment, and outcome of these patients remain unclear. This study aims to widen current knowledge about this “SEL subform” and to improve its clinical management. A systematic literature review according to the PRISMA guidelines using PubMed, Scopus, Web of Science, and Cochrane Library was used to identify publications before 7 November 2021 reporting on acute/rapidly progressing, severe SEL. The final analysis comprised 12 patients with acute, severe SEL. The majority of the patients were male (9/12) and multimorbid (10/12). SEL mainly affected the thoracic part of the spinal cord (11/12), extending a median number of 7 spinal levels (range: 4–19). Surgery was the only chosen therapy (11/12), except for one critically ill patient. Regarding the outcome, half of the patients regained independence (6/11; = modified McCormick Scale ≤ II). Acute, severe SEL is a rare condition, mainly affecting multimorbid patients. The prognosis is poor in nearly 50% of the patients, even with maximum therapy. Further research is needed to stratify patients for conservative or surgical treatment.

OA Förderung 2022 Background and Objectives: Spinal schwannomas are benign tumours that can present with various symptoms such as pain, radiculopathy and neurological deficit. Gross total resection (GTR) is of key importance for local recurrence. The aim of this study is to describe the clinical characteristics, resection rate, clinical outcome, as well as tumour recurrence, in patients with non-syndromic spinal schwannomas and to clarify which factors affect the resection rate. Materials and Methods: Patients with non-syndromic spinal schwannomas that underwent surgical resection between January 2009 and December 2018 at a single institution were included. Demographic parameters, clinical symptoms, tumour localisation and size, surgical approach and complications were noted. Factors influencing the extent of resection, the surgeon’s decision regarding the approach and the occurrence of new postoperative deficits were evaluated. Results: Fifty patients (18 females) were included. The most common presenting symptom was radiculopathy (88%). The lumbar spine was the most commonly affected site (58%). Laminotomy (72%) was the preferred surgical approach overall and specifically for exclusively intraspinal schwannomas (p = 0.02). GTR was achieved in 76.0% (n = 38). In multivariate analysis, only tumour localisation within the spinal canal (p = 0.014) independently predicted GTR, whereas the type of approach (p = 0.50) and tumour volume (p = 0.072) did not. New postoperative persisting deficits could not be predicted by any factor, including the use and alteration of intraoperative neuromonitoring. Recurrence was observed in four cases (8%) and was significantly higher in cases with STR (p = 0.04). Conclusions: In this retrospective study, GTR was solely predicted by tumour localisation within the spinal canal. The decision regarding the utilisation of different surgical approaches was solely influenced by the same factor. No factor could predict new persisting deficits. Tumour recurrence was higher in STR.

Purpose Degenerative cervical myelopathy (DCM) is the most common non-traumatic cause of spinal cord dysfunction. Prediction of the neurological outcome after surgery is important. The aim of this study was to analyze the relationship between first symptoms of DCM and the neurological outcome after surgery. Methods A retrospective analysis over a period of 10 years was performed. First symptoms such as cervicobrachial neuralgia, sensory and motor deficits and gait disturbances were evaluated regarding the postoperative neurological outcome. The modified Japanese Orthopedic Association Score (mJOA Score) was used to evaluate neurological outcome. Results In total, 411 patients (263 males, 64%) with a median age of 62.6 ± 12.1 years were included. Cervicobrachial neuralgia was described in 40.2%, gait disturbance in 31.6%, sensory deficits in 19% and motor deficits in 9.2% as first symptom. Patients with cervicobrachial neuralgia were significantly younger (median age of 58 years, p = 0.0005) than patients with gait disturbances (median age of 68 years, p = 0.0005). Patients with gait disturbances and motor deficits as first symptom showed significantly lower mJOA Scores than other patients (p = 0.0005). Additionally, motor deficits and gait disturbance were negative predictors for postoperative outcome according to the mJOA Score. Conclusion Motor deficits and gait disturbances as the first symptom of DCM are negative predictors for postoperative neurological outcome. Nevertheless, patients with motor deficits and gait disturbance significantly profit from the surgical treatment despite poor preoperative mJOA Score.

Objective: Spinal cord ependymomas account for 3–6% of all central nervous system tumors and around 60% of all intramedullary tumors. The aim of this study was to analyze the neurological outcome after surgery and to determine prognostic factors for functional outcome. Patients and Methods: Patients treated surgically due to a spinal cord ependymoma between 1990 and 2018 were retrospectively included. Demographics, neurological symptoms, radiological parameters, histopathology, and neurological outcome (using McCormick Score [MCS]) were analyzed. Possible prognostic factors for neurological outcome were evaluated. Results: In total, 148 patients were included (76 males, 51.4%). The mean age was 46.7 ± 15.3 years. The median follow-up period was 6.8 ± 5.4 years. The prevalence was mostly in the lumbar spine (45.9%), followed by the thoracic spine (28.4%) and cervical spine (25.7%). Gross-total resection was achieved in 129 patients (87.2%). The recurrence rate was 8.1% and depended on the extent of tumor resection (p = 0.001). Postoperative temporary neurological deterioration was observed in 63.2% of patients with ependymomas of the cervical spine, 50.0% of patients with ependymomas of the thoracic spine, and 7.4% of patients with ependymomas of the lumbosacral region. MCS 1–2 was detected in nearly two-thirds of patients with cervical and thoracic spinal cord ependymoma 36 months after surgery. Neurological recovery was superior in thoracic spine ependymomas compared with cervical spine ependymomas. Poor preoperative functional condition (MCS >2), cervical and thoracic spine location, and tumor extension >2 vertebrae were independent predictors of poor neurological outcome. Conclusion: Neurological deterioration was seen in the majority of cervical and thoracic spine ependymomas. Postoperative improvement was less in thoracic cervical spine ependymomas compared with thoracic spine ependymomas. Poor preoperative status and especially tumor extension >2 vertebrae are predictors of poor neurological outcome (MCS >2). OA Förderung 2021 in press

ObjectiveThe objective of this study is to investigate the relationship between the thrombus signal intensity and aneurysm wall thickness in partially thrombosed intracranial aneurysms in vivo with magnetization-prepared rapid acquisition gradient echo (MPRAGE) taken using 7T magnetic resonance imaging (MRI) and correlate the findings to wall instability.MethodsSixteen partially thrombosed intracranial aneurysms were evaluated using a 7T whole-body MR system with nonenhanced MPRAGE. To normalize the thrombus signal intensity, its highest signal intensity was compared to that of the anterior corpus callosum of the same subject, and the signal intensity ratio was calculated. The correlation between the thrombus signal intensity ratio and the thickness of the aneurysm wall was analyzed. Furthermore, aneurysmal histopathological specimens from six tissue samples were compared with radiological findings to detect any correlation.ResultsThe mean thrombus signal intensity ratio was 0.57 (standard error of the mean [SEM] 0.06, range 0.25–1.01). The mean thickness of the aneurysm wall was 1.25 (SEM 0.08, range 0.84–1.55) mm. The thrombus signal intensity ratio significantly correlated with the aneurysm wall thickness (p < 0.01). The aneurysm walls with the high thrombus signal intensity ratio were significantly thicker. In histopathological examinations, three patients with a hypointense thrombus had fewer macrophages infiltrating the thrombus and a thin degenerated aneurysmal wall. In contrast, three patients with a hyperintense thrombus had abundant macrophages infiltrating the thrombus.ConclusionThe thrombus signal intensity ratio in partially thrombosed intracranial aneurysms correlated with aneurysm wall thickness and histologic features, indicating wall instability.

Objective Demographic changes have led to a higher incidence of C-2 fractures, especially in elderly patients. For patients with type II fractures, treatment remains controversial, as discussed by Anderson and D'Alonzo, due to the rising morbidity and mortality rates for any treatment. The aim of this study was to compare conservative and surgical management in patients with type II C-2 fractures regarding outcomes, complications, and the mortality rate. Methods A retrospective analysis was performed of the medical records, X-rays, and/or computed tomography scans of patients ≥80 years of age with type II fractures who were admitted to our Department of Neurosurgery between January 1990 and December 2017. The success of treatment was evaluated 3 months after surgery. Results In total, 125 patients were included, of whom 98 were treated surgically and 27 were treated conservatively. Surgical treatment was successful in 90.8% of cases, while conservative treatment was successful in 70.0%. The in-hospital mortality was 14.29% and the 3-month mortality was 27.8% in the surgical group, compared to 3.7% and 20% in the conservatively treated group. The in-hospital complication rate was 22.4% in the surgically treated patients and 7.4% in the conservatively treated patients. Conclusion Surgical treatment of type II fractures seemed to be associated with higher success and complication rates than conservative treatment. Nevertheless, 3-month mortality was comparable in both groups. Therefore, we conclude that surgical treatment for type II fractures in elderly patients is superior to conservative management, although conservative treatment remains a valuable option in elderly patients with severe comorbidities.

Objective Extent of resection (EOR) and Karnofsky Performance Status (KPS) are at odds in glioblastoma (GBM) surgery, that is, the anticipated postoperative disability limits the EOR. This study analyzes the correlation of different surgical modalities with the resulting physical status and survival of patients with GBM. Methods A total of 565 patients with primary GBM were operated on in a single institution between 2006 and 2014. Possible surgical modalities comprised supratotal resection (SLR), gross total resection (GTR; ≥ 95% by volume), tumor debulking (TDB; ≤ 95% by volume), and stereotactic biopsy (SB). Pre- and postoperative KPS before and up to 4 weeks after surgery as well as overall survival (OS) rate were determined retrospectively. Hazard ratio (HR) and 95% confidence intervals were calculated using a Cox proportional hazards model. Results Median postoperative KPS was ≥ 70, irrespective of surgical modality. Mean OS was 12.5 months. Multivariate analysis revealed age ≥ 70 years (HR: 1.93), preoperative KPS Conclusion Higher EOR in patients with GBM does not seem inevitably correlated with increasing functional impairment, but better survival, provided there is a balanced preoperative indication. Nevertheless, SLR does not seem to be superior to GTR. Whenever possible, maximal safe resection should be considered in patients with GBM, even if an EOR ≥ 95% is not possible.

Background and Objectives: Spinal meningiomas, which are well characterized and are most frequently intradural extramedullary tumors, represent 25% of all intradural spinal tumors. The goal of this study was to compare the outcomes of surgically treated patients with spinal meningiomas in two time intervals with special emphasis on postoperative functional outcomes. Methods: Patients with spinal meningiomas admitted to our department between 1990 and 2020 were enrolled and divided into a historic cohort (HC; treated 1990–2007) and a current cohort (CC; treated 2008–2020). Patients’ clinical data and surgical and radiological reports were retrospectively analyzed up to 5 years. Preoperative and postoperative neurological function were assessed using the modified McCormick Scale (mMCS). The Charlson Comorbidity Index (CCI) was used to evaluate the effect of comorbidities on the preoperative status and postoperative outcome. Results: We included 300 patients. Participants in the CC (n = 144) were significantly younger compared to those in the HC (n = 156), with twice as many patients

STUDY DESIGN Retrospective study (data analysis). OBJECTIVE The purpose of this study was to assess the role of different factors on postoperative outcome of patients with degenerative cervical myelopathy (DCM). SUMMARY OF BACKGROUND DATA Ongoing degenerative changes of DCM lead to progressive neurological deficits. The optimal timing of surgical treatment is still unclear, especially in patients with mild DCM. METHODS Patients with DCM treated in our clinic between 2007 and 2016 were retrospectively analyzed. Pre- and postoperative neurological function was assessed by the modified Japanese Orthopaedic Association Score (mJOA Score) at different stages. The minimum clinically important difference (MCID) was used to evaluate the improvement after surgery. The comorbidities were recorded using the Charlson Comorbidity Index (CCI). Possible associations between age, sex, CCI, preoperative symptoms duration, high signal intensity (SI) on T2-weighted magnetic resonance imaging (MRI) with mJOA Score and MCID were analyzed using univariate analysis and multivariate regression models. Additionally, subgroup analysis was performed according to the severity of DCM (mild: mJOA Score ≥15 points; moderate: mJOA Score of 12-14 points; and severe: mJOA Score

Traumatic acute subdural hematoma (aSDH) is associated with a high mortality rate caused by post-operative cerebral infarction. Recently, brain natriuretic peptide (BNP) was considered a reliable biomarker in the acute phase of traumatic brain injuries. We therefore aimed in this study to analyze BNP levels on admission, identify the predictors of their elevation, and assess the relationship between BNP and the risk of post-operative cerebral infarction. Patients with isolated, unilateral, traumatic aSDH who were admitted to our department between July 2017 and May 2020 were enrolled in this study. On admission, cranial computer tomography (CCT) and BNP sampling were simultaneously performed. Additionally, the time between head trauma and BNP sampling (TTS) was assessed. Admission radiographic variables included hematoma volumes, midline shift, and degree of brain edema. Cerebral infarction was detected on postoperative CCT. In total, 130 patients were included in this study. Surgical treatment was performed in 82.3% (

Objective Microsurgical resection of spinal ependymomas is associated with a considerable risk of postoperative neurological deterioration. We aimed to develop a risk score for outcome prediction after surgery for spinal ependymoma. Materials and methods All patients who underwent microsurgical resection of spinal ependymoma between 1980 and 2015 were included. Different perioperative parameters were collected for the score construction. Poor outcome was defined as the modified McCormick Scale (MMCS) >2 at 6 months after surgery. Results Of 131 patients (mean age: 45.6 ± 16.7 years; 63 females), 38 cases (29%) showed poor outcome. Based on the univariate analysis, preoperative MMCS, subtotal tumor resection, proximal tumor level on the spinal cord, tumor extension, intramedullary location, and WHO grading were included in the multivariate analysis. The final risk score consisted of the following independent predictors: preoperative MMCS > 1 (1 point), proximal tumor level at Th 10 and higher (1 point), and tumor extension ≥ 3 vertebrae (1 point). The constructed score (0–3 points; Score for OUtcome after Resection of Spinal Ependymoma [SOURSE]) showed high diagnostic accuracy (area under the curve [AUC] = 0.883), which was superior to preoperative MMCS (AUC = 0.798) and Karnofsky Performance Status (AUC = 0.794). Patients scoring 0, 1, 2, and 3 points showed poor outcome in 0%, 12.9%, 54.6%, and 76.2% of the cases respectively. Conclusion The presented SOURSE score based on preoperative neurologic condition, tumor location, and tumor extension could accurately predict the postoperative outcome in patients undergoing microsurgery of spinal ependymoma. Our data should be validated in a prospective trial.

Objective The demographic change in the population leads to a rising number of patients presenting with fractures of the cervical spine, especially C-2, due to falls. With an increase of co-morbidities in these elder patients, the risk for intra- and postoperative complications is increased likewise. Thus, an alternative strategy instead of operative management of these fractures should be taken into account whenever possible. Conservative management of dens fractures in the elderly is still a subject of great controversy despite numerous studies on this topic. The aim of this study was to analyze the rate of successful osseous consolidation of C-2 fractures with conservative treatment by wearing a semi-rigid collar or halo thoracic vest without further surgical intervention. Patients and methods We analyzed the medical records and CT-scan of the cervical spine of 254 patients with C-2 fractures retrospectively, who were admitted to our department between January 1990 and September 2015. Fractures were diagnosed by CT-scan of the cervical spine and classified according to the Anderson - D`Alonzo classification. 183 patients were submitted to surgery as treatment of choice. In 71 patients a conservative management with external immobilization was chosen. The latter group was subjected to study analysis. Results 71 patients (mean age 74.08 years ± 16.06 years) were diagnosed with C-2 fractures (Typ I: 4; Typ II 36; Typ III: 31) and treated conservatively using a Philadelphia collar (n = 57), or a halo-thoracic vest (n = 14), respectively. 12 patients were lost to follow up and excluded from further analysis. Conservative treatment of the fractures was deemed successful when a bony consolidation of the fracture in follow-up CT scans was seen (45 of 59 patients; 76.3%). 20 patients with a Type II fracture (20/28, 71.4%) showed a successful ossification. In 14 patients (overall 23.7%; Typ I: 1 (7.14%), Typ II: 8 (57.14%), Typ III: 5 (35.71%)) external immobilization failed to achieve primary stability. These patients were submitted to consecutive surgery. Conclusion From our data it can be concluded that elderly patients, presenting with non-dislocated Type II fractures of the axis without accompaining neurological deficits, will have a more than 70% chance for a bony consolidation by conservative management. Consolidation rates may be estimated even higher in Type I and III fractures. Therefore, we suggest that external immobilization might be a valuable option to treat elderly patients with these fractures under certain circumstances.

Aneurysm rupture might be accompanied by intraventricular hemorrhage (IVH), which is associated with poor outcome of subarachnoid hemorrhage (SAH). The aim of this study was to analyze risk factors and clinical impact of IVH severity.A total of 995 consecutive patients with SAH treated at our institution between January 2003 and June 2016 were eligible for this study. Clinical and radiologic findings were correlated with the presence and severity of IVH assessed with the original Graeb score.A total of 487 patients with SAH (48.9%) presented with IVH (mean IVH severity, 5.48 points [±3.5]). IVH severity correlated with poorer initial clinical condition (World Federation of Neurosurgical Societies grade3; P 0.001), acute hydrocephalus (P = 0.001), and poor outcome at 6 months (modified Rankin Scale score2; P 0.001). The location of the ruptured aneurysm in the anterior cerebral artery independently predicted the occurrence (P = 0.007) and severity of IVH (P0.001). In turn, aneurysm size affected only the severity of IVH (P = 0.001) but not its occurrence (P = 0.153). Early complications of SAH occurring within 72 hours after the bleeding event (cerebral infarction [P = 0.043], early mortality [P = 0.001], and primary craniectomy [P = 0.043]) were independently associated with the severity of IVH.Severity of aneurysmal IVH is a strong contributor to initial severity and early complications of SAH. Patients with larger aneurysms, especially located in the anterior cerebral artery, are at particular risk of severe IVH in cases of aneurysm rupture.

Several parameters are known to predict the survival of glioblastoma (GB), including extent of resection and MGMT promotor methylation. Staining for glial fibrillary acidic protein (GFAP) is a common component of routine histological work-up, but its clinical utility in GB is unclear. The aim of the present study was to analyze the predictive value of quantitative GFAP measurements for survival of patients with GB.All subjects in our institutional database of patients with primary GB who underwent surgery between 2011 and 2014 with examination of immunohistochemical staining of GFAP were included. Percentage GFAP staining was measured in 5% increments (5-100%). Univariate and multivariate analyses were performed between GFAP values and survival data. Clinically relevant cut-offs for GFAP staining were identified by receiver operating characteristic (ROC) curves.The final cohort consisted of 272GB patients with available quantitative GFAP measurements (mean age, 62 (±11.1) years, 117 females [43%]). Overall survival was 11.4 months (±8.6). Median GFAP value was 70% (range, 5-100%). The ROC curve showed the clinically relevant cut-off for GFAP at 75% (area under the curve: 0.691). Accordingly, GB patients with GFAP≥75% presented poorer survival on Kaplan-Meier survival estimation (P=0.021). Multivariate analysis adjusted for age, extent of resection, preoperative Karnofsky performance status scale, IDH1 mutation and MGMT methylation status confirmed the independent predictive value of GFAP≥75% for overall survival (P=0.032). Finally, patients with GFAP≥75% showed significantly poorer long-term survival than those with GFAP75%: 5.8% vs. 15.2% (P=0.0183) and 0.8% vs. 8% (P=0.0076) for 2- and 3-year survival, respectively.Quantitative immunohistochemical assessment of GFAP staining could provide a novel biomarker for overall and especially long-term survival of patients with GB. Prospective multi-center validation of the prognostic value of GFAP for GB survival is needed.

Objective To evaluate the incidence of catheter-associated meningitis (CAM) in a pediatric population receiving brain tumor surgery, and to identify the major risk factors involved. Methods We retrospectively analyzed the medical and radiological records of 205 pediatric patients who received 251 external ventricular drains (EVDs) between January 2008 and December 2017. All patients less than 18 years old who underwent cerebrospinal fluid (CSF) diversion in the course of brain tumor surgery were included. Patients with central nervous system infection (CNS) at the time of EVD insertion were excluded. Results A total of 99 patients receiving 107 EVDs met the study selection criteria. Among this population, the incidence of CAM was 19.2%. Median time-to-infection was 5 days. CAM prolonged the period of drainage in 57.9% of the cases. An extended ICU stay (>3 days) was statistically significantly associated with the occurrence of CAM. In the multivariate analysis, the presence of a high-grade CNS tumor was a predictor of an extended intensive care unit (ICU) stay. Furthermore, CSF leakage along the catheter tunnel was an independent predictor of CAM. Conclusion Our data confirms CAM as a significant complication in the acute treatment of hydrocephalus associated with pediatric brain tumors. To limit the incidence of CAM, measures must be taken to prevent CSF leakage, particularly among patients with high-grade CNS tumors that are likely to stay longer in the ICU and need prompt postoperative radiotherapy and oncological treatment.

Background Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an increasingly recognized neuroinflammatory syndrome that predominantly affects the pontine and cerebellar brain structures. Characteristically, patients will develop glucocorticoid-responsive brainstem disorders, demonstrate pontocerebellar contrast enhancement on magnetic resonance imaging (MRI), and exhibit an angiocentric, lymphocytic infiltrate in brain biopsies. We have presented and discussed 2 novel and challenging cases of CLIPPERS syndrome to highlight the clinical and radiological diversity of the syndrome. Case Description The first case was of a 66-year-old male patient who had presented with dizziness, headaches, gait disturbances, mild cognitive impairment, and visual field loss to the left side. MRI revealed 1 cerebellar and 2 occipital contrast-enhancing lesions that were suspicious for intracerebral metastases. The second case was of a 53-year-old male patient who had presented with temporal lobe epilepsy, anomic aphasia, and mild cognitive impairment. MRI demonstrated 4 contrast-enhancing lesions in the pons, temporal lobe, and thalamus that were suspicious for intracerebral lymphoma. Because of the radiological presentation, neoplastic disease was the most plausible diagnosis for both patients. However, repeated biopsies ruled out tumor manifestation, and the findings were finally consistent with CLIPPERS syndrome. The significant and long-lasting response to immunosuppressive treatment confirmed the diagnosis. Conclusions In both cases, the characteristics of CLIPPERS syndrome imitated malignant tumor growth. This scenario can be challenging to clinicians and necessitates inclusion of this neuroinflammatory syndrome in the differential diagnosis of neuro-oncological disease.

Background Progressive multifocal leukoencephalopathy (PML) is a rare complication of patients treated with fingolimod. Case presentation Routine MRI eventually led to diagnosis of asymptomatic early PML that remained stable after discontinuation of fingolimod. As blood lymphocyte counts normalized, signs of immune reconstitution inflammatory syndrome (IRIS) and renewed MS activity developed. Both, advanced laboratory and ultrahigh field MRI findings elucidated differences between PML and MS. Conclusions In our case, early discontinuation of fingolimod yielded a good outcome, lymphocyte counts reflected immune system activity, and paraclinical findings helped to differentiate between PML-IRIS and MS.

Glioblastoma multiforme (GBM) has a peak incidence in patients older than 65 years. The aim of the present study is to evaluate the impact of surgical strategy on short- and long-term outcomes of older GBM patients.A total of 273 older patients (65-84 years) from 2006 to 2014 were operated in our neurosurgical center. The study endpoints were postoperative change of the Karnofsky performance status scale (KPS) and overall survival (OS). The extent of resection (EOR) was categorized into gross total resection (GTR,95% by volume), subtotal resection (STR, ≤95%) and stereotactic biopsy (SB). The subgroup analyses were performed in two age groups dichotomized at 75 years.EOR was associated with the risk of postoperative decline of KPS only in patients aged ≥75 years (p = 0.0002/p = 0.0014 for SB vs. GTR and STR respectively), but not in the age group 65-74 years (p = 0.1511/p = 0.2701). The mean OS in the whole cohort was 8.4 months (±9.6). GTR was superior to SB with regards to OS (p 0.0001/p = 0.0017). STR revealed a more favorable OS than SB only in patients aged 65-74 years (p = 0.0077). Multivariate analysis confirmed that only GTR was independently associated with OS (p 0.0001/p = 0.013).GTR may provide a better OS even in GBM patients with advanced age. STR still shows a benefit over SB for OS in older patients75 years. For older individuals, SB presents a safer option with regards to the risk of postoperative morbidity and should be favored in cases, when the surgical alternative is limited to STR.

BACKGROUND AND PURPOSE: The pathophysiology of wall contrast enhancement in thrombosed intracranial aneurysms is incompletely understood. This in vivo study aimed to investigate wall microstructures with gadolinium-enhanced 7T MR imaging. MATERIALS AND METHODS: Thirteen patients with 14 thrombosed intracranial aneurysms were evaluated using a 7T whole-body MR imaging system with nonenhanced and gadolinium-enhanced high-resolution MPRAGE. Tissue samples were available in 5 cases, and histopathologic findings were correlated with 7T MR imaging to identify the gadolinium-enhancing microstructures. RESULTS: Partial or complete inner wall enhancement correlated with neovascularization of the inner wall layer and the adjacent thrombus. Additional partial or complete outer wall enhancement can be explained by formation of vasa vasorum in the outer aneurysm wall layer. The double-rim enhancement correlated with perifocal edema and wall histologic findings suggestive of instability. CONCLUSIONS: Two distinct aneurysm wall microstructures responsible for gadolinium enhancement not depictable at lower spatial resolutions can be visualized in vivo using high-resolution gadolinium-enhanced 7T MR imaging.

Objective To investigate the role of brain natriuretic peptide (BNP) in predicting long-term functional outcome and develop a new predictive score of poor functional outcome after surgery for chronic subdural hematoma (cSDH). Methods Patients with cSDH that were surgically treated in our department between November 2016 and December 2019 were included in the study. Pre- and postoperative plasma BNP and clinical condition were prospectively recorded. At follow-up (5–6 months), a simplified modified Rankin Scale (mRS) questionnaire was conducted through a standardized telephone interview. A poor outcome was defined by a mRS > 3. Based on predictors of the long-term functional outcome, a score was calculated. Its accuracy was tested using the area under the curve (AUC) of the receiver operating characteristic analysis. Results In total, 119 patients were analyzed (median age: 76 years, range: 44–94 years). Preoperative plasma BNP (BNP-1) was elevated in 77 patients (64.7%). For the follow-up phone interview, 101 patients (84.8%) were available. In the multivariate analysis, poor outcome at follow-up could be predicted using BNP-1 (p = 0.034), age (p = 0.036), motor deficit (p = 0.013) and Glasgow coma scale score on admission (p = 0.008). The “Functional Long-term Outcome Predictive score” (FLOP-score) was therefore computed, and revealed an excellent discriminative capacity compared to other scores, with an AUC of 0.86 (0.77–0.95). Conclusions Preoperative plasma BNP is an independent predictor of functional outcome at follow-up. Using the FLOP-score, the risk of experiencing a poor outcome could accurately be predicted.

BACKGROUND Delayed cerebral ischemia (DCI) has a strong impact on outcome of patients with aneurysmal subarachnoid hemorrhage (SAH). Positive effect of antiplatelet therapy on DCI rates has been supposed upon smaller SAH series. OBJECTIVE To analyze the benefit/risk profile of antiplatelet use in SAH patients. METHODS This retrospective case-control study was based on institutional observational cohort with 994 SAH patients treated between January 2003 and June 2016. The individuals with postcoiling antiplatelet therapy (aspirin with/without clopidogrel) were compared to a control group without antiplatelet therapy. Occurrence of DCI, major/minor bleeding events in the follow-up computed tomography scans, and favorable outcome at 6 mo after SAH (modified Rankin scale

OBJECTIVETuberous sclerosis complex (TSC) is a rare multisystem genetic disease. Arterial wall developmental disorders, such as aneurysms, in association with TSC have been well described for extracranial vasculature. The characteristics of intracranial aneurysms (IAs) in TSC have not previously been addressed in the literature. This systematic review was performed to identify and assess the distinct characteristics of IAs in patients with TSC.METHODSThe authors searched PubMed, Scopus, and Web of Science for publications describing cases of TSC and IA reported before August 7, 2018. They also report 2 cases of IAs in TSC patients treated at their own institution.RESULTSThirty-three TSC patients with a total of 42 IAs were included in this review. Three individuals presented with subarachnoid hemorrhage. The IAs were large or giant in 57.1% and fusiform in 45.2% of the cases. Most of the IAs (61.9%, 26 of 42) originated from the internal carotid artery. There was a higher prevalence of pediatric cases (66.7%) and male patients (63.6%, 21 of 32 individuals with known sex) among the collected series.CONCLUSIONSTSC patients with IAs are characterized with a higher proportion of large/giant and fusiform IAs and young age, suggesting rapid aneurysmal growth. Furthermore, there is a distinct location pattern of IAs and an inverse sex ratio than in the healthy population. Large population-based patient registers are required to improve the understanding of epidemiology and pathophysiology of IA formation in TSC.

Objectives: Intracerebral hematoma (ICH) complicates the course of aneurysmal subarachnoid hemorrhage (SAH). To date, there are no unique guidelines for management of aneurysmal ICH. The aim of this study was to identify risk factors for and impact of aneurysmal ICH with special attention on treatment decisions derived from ICH volume. Patients and Methods: All patients admitted with aneurysmal SAH between 2003 and 2016 were eligible for this study. Various demographic, clinical and radiographic characteristics of patients were correlated with the occurrence and volume of ICH in univariate and multivariate manner. The associations between ICH volume and the need for surgical procedures and functional outcome were also analyzed. Results: 991 patients were included into final analysis. ICH occurred in 301 (30.4%) cases. Location in the middle cerebral artery (MCA, p 3). Conclusion: Of over 30 tested variables, the location of the ruptured aneurysm in the MCA remains the major risk factor for occurrence and volume of ICH. Given the presence of brain swelling and other bleeding components of SAH, surgical intervention on aneurysmal ICH is indicated at lower volume values, than it is generally accepted for spontaneous ICH.

Background: Cerebellar liponeurocytoma is a rare tumor of the central nervous system occurring mainly in the posterior fossa, which shows neuronal and variable astrocytic differentiation with foci of lipomatous differentiation. Liponeurocytoma develops in adult patients and is defined in the World Health Organization classification of 2016 as a rare benign grade II tumor. Case presentation: A 39-year-old Italian man presented to our department suffering from headache and nausea. Magnetic resonance imaging revealed a right-sided cerebellar lesion showing poor contrast enhancement without an obstructive hydrocephalus. Surgery was indicated and total tumor resection was achieved. He was discharged without any neurological deficits. Histopathological examinations revealed a cerebellar liponeurocytoma. A neurological follow-up examination revealed no neurological deficit directly after surgery and 1 year later. Radiotherapy was recommended at the neurooncological board despite the total removal of the tumor, but our patient refused adjuvant radiotherapy. Magnetic resonance imaging of his neurocranium with and without contrast enhancement 48 hours after surgery and 15 months after surgery showed no residual tumor. Conclusions: Liponeurocytomas are rare benign tumors occurring in the majority of cases in the cerebellum. The therapy of choice is surgery. Postoperative radiotherapy has to be discussed individually, but seems to be sufficient if complete tumor resection is not achieved or in cases of a tumor recurrence. OA Förderung 2018 - CA Gembruch

Outcome of aneurysmal subarachnoid hemorrhage (SAH) depends strongly on occurrence of symptomatic vasospasm (SV) leading to delayed cerebral ischemia (DCI). Various demographic, radiographic, and clinical predictors of SV have been reported so far, partially with conflicting results. The aim of this study was to analyze the role of patients’ age and sex on SV/DCI risk, especially to identify age and sex-specific risk groups. All patients admitted with acute SAH during a 14-year-period ending in 2016 were eligible for this study. The study endpoints were the following: SV requiring spasmolysis, occurrence of DCI in follow-up computed tomography scans and unfavorable outcome at 6 months (modified Rankin scale > 2). Nine hundred ninety-four patients were included in this study. The majority was female (666; 67%). SV, DCI, and unfavorable outcomes were observed in 21.5, 21.8, and 43.6% of the patients, respectively. Younger age (p

Objective Liponeurocytoma is a rare benign tumor of the central nervous system, which develops mainly in adult patients within the posterior fossa. The World Health Organization has categorized this entity in its last classification of 2016 as a benign grade II tumor. Histopathologic characteristics contain neuronal and variable astrocytic differentiation with foci of lipomatous distinction. Only a few case reports and case series have been reported and the knowledge of this tumor is limited. General treatment guidelines do not exist. The aim of this study was to analyze the literature to create treatment guidelines. Methods PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines were followed to search existing online databases between January 1, 1978 and May 15, 2018. Results A systematic review of the literature found 73 patients (40 female, 33 male) with liponeurocytoma, in 59 of whom (80.8%) the disease occurred in the posterior fossa. The Ki-67/MIB-1 proliferation index was described in 58 patients, showing a mean value of 3.73% ± 4.01%. Follow-up was performed in 49 patients, with a median length of 52.02 ± 50.52 months, showing tumor recurrence in 14 patients (28.57%). Tumor recurrence was observed in only 1 patient (1/12, 8.33%) after adjuvant radiotherapy, whereas tumor recurrence was seen in 13/29 patients (44.83%) who did not receive adjuvant radiotherapy. Conclusions Liponeurocytomas are rare benign tumors, occurring mainly in the cerebellum. The therapy of choice is surgery. Postoperative radiotherapy seems to decrease the risk of tumor recurrence and should be offered to the patient.

Background and Objective We present a treatment approach for a rare condition of patients with a ventral C1 fracture and a congenital cleft in the posterior arch (half-ring Jefferson fracture) with an intact transverse atlantal ligament. Our technique aims to achieve stability of the atlanto-occipital and atlantoaxial joints while preserving mobility of the upper cervical spine. Patients and Methods Two male patients, 43 years and 29 years of age, respectively, were admitted to our hospital due to a fracture of the ventral arch of the atlas with no damage of the transverse atlantal ligament. Both men also presented a congenital cleft of the posterior arch. Initial conservative management with a halo-thoracic vest was performed in one case and failed. As a result, surgical treatment was performed in both cases using bilateral C1 mass screws and a transverse connector. Results The patients showed no neurologic deficits on follow-up examination 4 weeks after surgery with a full range of head and neck motion. Computed tomography (CT) showed no dislocation of the implanted material with good dorsal alignment and a stable ventral fracture distance. Follow-up CT showed osseous stability in both cases with the beginning of bony ossification of the bone graft. Conclusion Isolated instable fractures of the ventral arch of the atlas with a congenital cleft of the posterior arch with no damage of the transverse atlantal ligament can be stabilized using bilateral C1 mass screws and a transverse connector preserving upper cervical spine mobility.

A novel vacuum stable proton sponge, 4-maleicanhydridoproton sponge (MAPS), was prepared and applied as the matrix in Matrix-Assisted Laser Desorption/Ionization Mass Spectrometry Imaging (MALDI-MSI) of an aggressive brain tumor tissue (glioblastoma multiforme). Ionic maps of lactate, 2-hydroxyglutarate and chloride anions (m/z 89, 147, 35, respectively) were obtained using a routine MALDI ToF mass spectrometer.

Background: Uterine lipoleiomyomas are rare benign tumors, which preferentially occur in postmenopausal women between the ages of 45 and 70 years. Histologically, these tumors consist of fully differentiated smooth muscle cells and adipocytes. Case: The unusual clinical presentation of a uterine lipoleiomyoma, which was first suspected to be a tumor of adnexal origin (e.g., a dermoid) is presented. However, subsequent magnetic resonance imaging (MRI) investigation revealed a uterine tumor with predominantly fatty appearance. Histologic examination after laparoscopically assisted total hysterectomy showed a benign lipoleiomyoma of the uterine corpus. Conclusions: Because of their fatty components, lipoleiomyomas can be sonographically misdiagnosed as cystic lesions. MRI can reveal the characteristic features of a fatty tumor, and complete surgical removal is required to exclude other malignant tumors with fatty components, such as sarcomas. (J GYNECOL SURG 27:163)

Spinal dural arteriovenous fistulas (SDAVFs) are rare pathologies with a yearly incidence of 5–10 new cases/million, constituting 60–80 % of spinal arteriovenous malformations. Clinical symptoms include progressive paraparesis, paresthesias, bladder, and bowel disturbances. The pathophysiology of SDAVFs is not well elucidated. Microneurosurgery and endovascular techniques are established treatment modalities for permanent fistula occlusion, which are oftentimes accompanied by an amelioration of neurological deficits in the long run. Here, we report our interdisciplinary neurosurgical/neuroradiological management strategy of SDAVFs in 32 patients who were evaluated retrospectively. We focused on clinical presentation, microneurosurgical and interventional technique, early, and late neurological results. Quality of life (QoL) was additionally assessed in 12 patients at last follow-up. We discuss the results against the background of the current literature. Our series and the literature indicate that clinical outcome after treatment of SDAVF is favorable in general. Both neurosurgical and neurointerventional therapies appear to be safe and effective, but short-term neurological deterioration after the intervention constitutes an as-of-yet unsolved problem. Beyond age and preoperative neurological state, presence of comorbidities had a significant influence on neurological outcome in our study sample. Self-assessed physical and mental QoL at long-term follow-up was reduced in quite a number of patients and was associated with a poorer neurological result as well as presence of comorbidities. The patients’ perspective in terms of QoL was first investigated in this study, but further research on QoL and psychosocial impairment of SDAVF patients is needed to enable individualized counseling and rehabilitation strategies.

Introduction Spinal vascular malformations can be classified in arteriovenous malformations, cavernomas, and capillary telangiectasias. Arteriovenous malformations are the most common spinal vascular anomaly and may be located intra- and/or perimedullary. According to their nidus type and hemodynamic flow patterns, they can be differentiated into fistulous, glomerular and juvenile categories. In our case, a hyperintense extradural mass was misinterpreted as a neurinoma. The histological analysis revealed typical signs of an arteriovenous malformation. Case presentation A 57-year-old Caucasian woman presented with back pain and hypesthesia in digiti two to four of her left foot. Magnetic resonance imaging showed a long-segment intraspinal extradural soft-tissue mass in the left L4 - S1 paravertebral region with homogeneous enhancement of contrast medium. Due to another similar lesion in the lower ankle and additional cutaneous manifestations, the suspected diagnosis was a systemic disease with neurinomas (e.g. Recklinghausen’s disease). To clear up the origin and type of this lesion exploratory surgery with a hemilaminectomy of L5 was performed. This showed abnormally arterialized, dilated, and tortuous vessels. After complete resection, the intra-operative impression of an arteriovenous malformation was confirmed by a neuropathologist. Conclusions Completely extradural intraspinal arteriovenous malformations in the lumbar spine are extremely rare. In magnetic resonance imaging they are often misinterpreted as a tumor. Arteriovenous malformations can cause compression and venous congestion, or mask symptoms like a spinal disk herniation. In cases presenting with these symptoms and magnetic resonance imaging findings, an extradural intraspinal arteriovenous malformation should be considered as a possible diagnosis. Pre-operative angiography or magnetic resonance imaging angiography can be used to verify the diagnosis.

Objectives To assess changes in the Doppler flow profiles of the middle cerebral artery in fetuses with cardiac defects theoretically associated with impaired cerebral oxygen delivery in utero. Methods Z-scores were calculated for pulsatility and resistance indices (PI and RI, respectively) of the middle cerebral artery (MCA) and the cerebroplacental ratio (CPR) between 19 and 41 weeks' gestation, and for head circumference at birth (HC), in 113 fetuses with the following isolated cardiac defects: transposition of the great arteries (TGA; n = 18), hypoplastic left heart (HLH; n = 46), severe aortic stenosis (n = 17), pulmonary atresia (n = 18) and tetralogy of Fallot (TOF; n = 14). Pregnancies with uteroplacental dysfunction (indicated by increased uterine and/or umbilical Doppler indices), growth restriction, extracardiac malformations, chromosomal anomalies as well as multiple pregnancies were excluded to avoid any additional hypoxemic effect as strictly as possible. The results were compared with 1378 normal controls. Results Fetuses with pulmonary atresia, severe aortic stenosis and TOF had no significant alterations of Doppler parameters or HC at birth. In fetuses with TGA, mean Z-scores of HC at birth were significantly smaller compared with controls (mean ± SD, −0.73 ± 1.25; P < 0.05), but there was no significant difference in the Doppler parameters. Fetuses with HLH had significantly lower MCA-PI (−0.57 ± 0.74; P < 0.05), MCA-RI (−0.73 ± 0.85; P < 0.05), CPR (−1.44 ± 1.05; P < 0.05) and HC (−0.50 ± 1.24; P < 0.05) Z-scores compared with controls. Conclusions Fetuses with cardiac defects theoretically associated with markedly impaired cerebral oxygen delivery in utero (TGA and HLH) have smaller HCs at birth. However, only fetuses with HLH have cerebrovascular alterations that are detectable by evaluation of the Doppler indices MCA-PI, MCA-RI and CPR. Copyright © 2009 ISUOG. Published by John Wiley & Sons, Ltd.