[Potential Impact of Public Communication on Emergency Presentations due to Headache during the Covid-19 Pandemic]. / Epidemiologische Untersuchung zu möglichen Auswirkungen öffentlicher Kommunikation im Rahmen der COVID-19-Pandemie auf Notfallvorstellungen von Kopfschmerzpatient*innen.

Headache is a frequent presenting symptom in the emergency department. While most cases are of benign aetiology, it is crucially important to identify potentially dangerous underlying disorders. We hypothesized an increase in headache-related emergency presentations after venous thrombosis of cerebral sinuses had been identified as a rare side effect of vaccination with adenovirus vector-based Coronavirus-disease 2019 (COVID-19) vaccines and that information had been publicly communicated by the Paul Ehrlich Institute. Data from patients with the diagnosis of primary headache disorders or unspecified headache presenting to the Interdisciplinary Emergency Department of the University Medicine Mannheim were retrospectively analysed. Based on vaccination dashboard data published by the Federal Ministry of Health, calendar weeks 14-30 and 47-48, on the one hand, and 1-13 and 31-46, on the othe, were categorized into a variable "vaccination epoch" (14-30, 47-48: high vaccination activity if≥3 million weekly vaccinations in Germany; 1-13, 31-46: low vaccination activity if<3 million weekly vaccinations). The number of patients with headache was the dependent variable. A Poisson regression was performed to analyze whether the frequency of events, i. e., patient presentations, was a function of year (2019, 2021), epoch (high, low) and an interaction of year and epoch - the latter reflecting an impact of vaccination activity during the pandemic and expressed as incidence rate ratio. Compared to 2019, there was a more than 70% increase in presentations due to headache during periods of high vaccination activity in 2021 (p<0.001; 95% confidence interval 1.272-2.316), in 25% of presentations in 2021, patients considered their headache as vaccination-associated. Public communication and resulting nocebo effects may, among other factors, have contributed to our observation of increased numbers of emergency headache presentations, illustrating the impact of public distribution of medical information on practical aspects of emergency care during crises.

PSEN1/SLC20A2 double mutation causes early-onset Alzheimer's disease and primary familial brain calcification co-morbidity.

Primary familial brain calcification (PFBC; formerly Fahr's disease) and early-onset Alzheimer's disease (EOAD) may share partially overlapping pathogenic principles. Although the heterozygous loss-of-function mutation c.1523 + 1G > T in the PFBC-linked gene SLC20A2 was detected in a patient with asymmetric tremor, early-onset dementia, and brain calcifications, CSF ß-amyloid parameters and FBB-PET suggested cortical ß-amyloid pathology. Genetic re-analysis of exome sequences revealed the probably pathogenic missense mutation c.235G > A/p.A79T in PSEN1. The SLC20A2 mutation segregated with mild calcifications in two children younger than 30 years. We thus describe the stochastically extremely unlikely co-morbidity of genetic PFBC and genetic EOAD. The clinical syndromes pointed to additive rather than synergistic effects of the two mutations. MRI data revealed the formation of PFBC calcifications decades before the probable onset of the disease. Our report furthermore exemplifies the value of neuropsychology and amyloid PET for differential diagnosis.

Use of Prognostication Instruments in Prognostication Procedures of Postanoxic Coma Patients over Time: A Retrospective Study.

BACKGROUND: Many survivors of cardiovascular arrest remain in a postanoxic coma. The neurologist's task is to provide the most accurate assessment of the patient's neurologic prognosis through a multimodal approach of clinical and technical tests. The aim of this study is to analyze differences and developments in the concept of neurological prognosis assessment and in-hospital outcome of patients over a five year-period. METHODS: This retrospective observational study included 227 patients with postanoxic coma treated in the medical intensive care unit of the University Hospital, Mannheim from January 2016 to May 2021. We retrospectively analyzed patient characteristics, post-cardiac arrest care, and the use of clinical and technical tests for neurological prognosis assessment and patient outcome. RESULTS: Over the observation period, 215 patients received a completed neurological prognosis assessment. Regarding the multimodal prognostic assessment, patients with poor prognosis (54%) received significantly fewer diagnostic modalities than patients with very likely poor (20.5%), indeterminate (24.2%), or good prognosis (1.4%; p = 0.001). The update of the DGN guidelines in 2017 had no effect on the number of performed prognostic parameters per patient. The finding of bilaterally absent pupillary light reflexes or severe anoxic injury on CT contributed most to a poor prognosis category (OR 8.38, 95%CI 4.01-7.51 and 12.93, 95%CI 5.55-30.13, respectively), whereas a malignant EEG pattern and NSE > 90 µg/L at 72 h resulted in the lowest OR (5.11, 95%CI 2.32-11.25, and 5.89, 95%CI 3.14-11.06, respectively) for a poor prognosis category. Assessment of baseline NSE significantly increased over the years (OR 1.76, 95%CI 1.4-2.22, p < 0.001), and assessment of follow-up NSE at 72 h trended to increase (OR 1.19, 95%CI 0.99-1.43, p = 0.06). In-hospital mortality was high (82.8%), remained unchanged over the observation period, and corresponded to the number of patients in whom life-sustaining measures were discontinued. CONCLUSIONS: Among comatose survivors of cardiac arrest, the prognosis remains poor. Prognostication of a poor outcome led nearly exclusively to withdrawal of care. Prognostic modalities varied considerably with regard to their contribution to a poor prognosis category. Increasing enforcement of a standardized prognosis assessment and standardized evaluation of diagnostic modalities are needed to avoid false-positive prognostication of poor outcomes.

A genome-wide association study with tissue transcriptomics identifies genetic drivers for classic bladder exstrophy.

Classic bladder exstrophy represents the most severe end of all human congenital anomalies of the kidney and urinary tract and is associated with bladder cancer susceptibility. Previous genetic studies identified one locus to be involved in classic bladder exstrophy, but were limited to a restrict number of cohort. Here we show the largest classic bladder exstrophy genome-wide association analysis to date where we identify eight genome-wide significant loci, seven of which are novel. In these regions reside ten coding and four non-coding genes. Among the coding genes is EFNA1, strongly expressed in mouse embryonic genital tubercle, urethra, and primitive bladder. Re-sequence of EFNA1 in the investigated classic bladder exstrophy cohort of our study displays an enrichment of rare protein altering variants. We show that all coding genes are expressed and/or significantly regulated in both mouse and human embryonic developmental bladder stages. Furthermore, nine of the coding genes residing in the regions of genome-wide significance are differentially expressed in bladder cancers. Our data suggest genetic drivers for classic bladder exstrophy, as well as a possible role for these drivers to relevant bladder cancer susceptibility.

Human primary muscle stem cells regenerate injured urethral sphincter in athymic rats.

BACKGROUND: The aim of the study was to demonstrate the efficacy of human muscle stem cells (MuSCs) isolated using innovative technology in restoring internal urinary sphincter function in a preclinical animal model. METHODS: Colonies of pure human MuSCs were obtained from muscle biopsy specimens. Athymic rats were subjected to internal urethral sphincter damage by electrocauterization. Five days after injury, 2 × 105 muscle stem cells or medium as control were injected into the area of sphincter damage (n = 5 in each group). Peak bladder pressure and rise in pressure were chosen as outcome measures. To repeatedly obtain the necessary pressure values, telemetry sensors had been implanted into the rat bladders 10 days prior to injury. RESULTS: There was a highly significant improvement in the ability to build up peak pressure as well as a pressure rise in animals that had received muscle stem cells as compared to control (p = 0.007) 3 weeks after the cells had been injected. Only minimal histologic evidence of scarring was observed in treated rats. CONCLUSION: Primary human muscle stem cells obtained using innovative technology functionally restore internal urethral sphincter function after injury. Translation into use in clinical settings is foreseeable.

[Health care reality of selected pediatric urologic surgeries in Germany from 2006 to 2019]. / Versorgungswirklichkeit ausgewählter kinderurologischer Eingriffe in Deutschland von 2006 bis 2019.

BACKGROUND: The conservative and surgical treatment of children is a fundamental pillar of the urological specialist training program and represents one of the core competencies within urological healthcare. The loss of this expertise has been a reoccurring topic within urologic occupational policy. The aim of this study is to analyse actual case numbers and to compare the distribution and dynamics of pediatric urologic surgeries between the specialist departments of urology and pediatric surgery in Germany. MATERIALS AND METHODS: We defined the surgical treatments of maldecensus testis, hypospadias, and vesicoureteral reflux (VUR) as index interventions. Using the tool reimbursement.INFO (RI Innovation GmbH, Hürth, Germany) we analysed publicly available quality report data of German hospitals between 2006 and 2019. RESULTS: While orchidopexy was more commonly performed in the field of urology, the correction of hypospadias and the surgical treatment of VUR showed higher case numbers in the field of pediatric surgery. Proportionally, there was no relevant shift between urologic and pediatric surgical clinics for orchidopexy and surgical VUR therapy during the study period. For hypospadias corrections, the proportion of surgeries performed in pediatric surgical units is increasing (p < 0.0001). In pediatric surgery 84-93% of the analyzed procedures are performed in high-volume units, while this proportion is 56-73% in urology. In particular, a high proportion of VUR therapy in urology is performed as an occasional procedure (30% very low volume). CONCLUSIONS: The quality report data enable the compilation of case numbers and the analysis of the distribution between urology and pediatric surgery in Germany. Merely the correction of hypospadias has shown a relevant shift towards pediatric surgery. The causes and possible consequences for professional policy of this preliminary investigation are complex and require further analysis.

SLC20A1 Is Involved in Urinary Tract and Urorectal Development.

Previous studies in developing Xenopus and zebrafish reported that the phosphate transporter slc20a1a is expressed in pronephric kidneys. The recent identification of SLC20A1 as a monoallelic candidate gene for cloacal exstrophy further suggests its involvement in the urinary tract and urorectal development. However, little is known of the functional role of SLC20A1 in urinary tract development. Here, we investigated this using morpholino oligonucleotide knockdown of the zebrafish ortholog slc20a1a. This caused kidney cysts and malformations of the cloaca. Moreover, in morphants we demonstrated dysfunctional voiding and hindgut opening defects mimicking imperforate anus in human cloacal exstrophy. Furthermore, we performed immunohistochemistry of an unaffected 6-week-old human embryo and detected SLC20A1 in the urinary tract and the abdominal midline, structures implicated in the pathogenesis of cloacal exstrophy. Additionally, we resequenced SLC20A1 in 690 individuals with bladder exstrophy-epispadias complex (BEEC) including 84 individuals with cloacal exstrophy. We identified two additional monoallelic de novo variants. One was identified in a case-parent trio with classic bladder exstrophy, and one additional novel de novo variant was detected in an affected mother who transmitted this variant to her affected son. To study the potential cellular impact of SLC20A1 variants, we expressed them in HEK293 cells. Here, phosphate transport was not compromised, suggesting that it is not a disease mechanism. However, there was a tendency for lower levels of cleaved caspase-3, perhaps implicating apoptosis pathways in the disease. Our results suggest SLC20A1 is involved in urinary tract and urorectal development and implicate SLC20A1 as a disease-gene for BEEC.

Treatment Strategies and Outcome of the Exstrophy-Epispadias Complex in Germany: Data From the German CURE-Net.

Introduction: To evaluate the impact of reconstructive strategies and post-operative management on short- and long-term surgical outcome and complications of classical bladder exstrophy (CBE) patients' comprehensive data of the multicenter German-wide Network for Congenital Uro-Rectal malformations (CURE-Net) were analyzed. Methods: Descriptive analyses were performed between 34 prospectively collected CBE patients born since 2009, median 3 months old [interquartile range (IQR), 2-4 months], and 113 cross-sectional patients, median 12 years old (IQR, 6-21 years). Results: The majority of included individuals were males (67%). Sixty-eight percent of the prospectively observed and 53% of the cross-sectional patients were reconstructed using a staged approach (p = 0.17). Although prospectively observed patients were operated on at a younger age, the post-operative management did not significantly change in the years before and after 2009. Solely, in prospectively observed patients, peridural catheters were used significantly more often (p = 0.017). Blood transfusions were significantly more frequent in males (p = 0.002). Only half of all CBE individuals underwent inguinal hernia repair. Cross-sectional patients after single-stage reconstructions showed more direct post-operative complications such as upper urinary tract dilatations (p = 0.0021) or urinary tract infections (p = 0.023), but not more frequent renal function impairment compared to patients after the staged approach (p = 0.42). Continence outcomes were not significantly different between the concepts (p = 0.51). Self-reported continence data showed that the majority of the included CBE patients was intermittent or continuous incontinent. Furthermore, subsequent consecutive augmentations and catheterizable stomata did not significantly differ between the two operative approaches. Urinary diversions were only reported after the staged concept. Conclusions: In this German multicenter study, a trend toward the staged concept was observed. While single-stage approaches tended to have initially more complications such as renal dilatation or urinary tract infections, additional surgery such as augmentations and stomata appeared to be similar after staged and single-stage reconstructions in the long term.

[Bilateral reflux: contra "intravesical techniques"]. / Bilateraler Reflux ­ Kontra intravesikale Techniken.

In reflux surgery, decisions on surgical procedures can be made on an individual basis today. Almost all open surgical implantation techniques provide very good, sustainable results, and parents prefer less invasive techniques. Therefore, if intravesical techniques are used to correct bilateral reflux, it should be considered and discussed with the parents that these procedures are associated with increased invasiveness in terms of longer operation times, longer catheter dwell times, a longer inpatient stay and more complications such as macrohaematuria and bladder spams compared with extravesical techniques.

Classic bladder exstrophy and adenocarcinoma of the bladder: Methylome analysis provide no evidence for underlying disease-mechanisms of this association.

The bladder exstrophy-epispadias complex (BEEC) represents the severe end of uro-rectal malformation spectrum involving aberrant embryonic morphogenesis of the cloacal membrane and the urorectal septum. The most common form of BEEC is isolated classic bladder exstrophy (CBE). Long-term complications in CBE are malignancies of the bladder with 95% of them being adenocarcinomas. Since CBE and adenocarcinoma of the bladder are rare entities, their frequent co-occurrence suggests a common etiology. Recent studies suggest that promoter methylation of various genes play a crucial role during the phenotypical morphogenesis of adenocarcinomas of urinary bladder. To examine, whether epigenetic processes such as DNA methylation patterns are potentially associated with CBE, we performed Illumina 450 K methylation arrays in blood (n = 10) and tissue samples (n = 2) of CBE patients and healthy matched controls (n = 12). In our analysis, we found total lack of methylation in the blood and methylation differences were restricted to 10 CpG sites in the tissue samples. In comparison to other bladder anomalies, CBE tissue methylation profiles differ from those of adenocarcinoma, adenocarcinoma with CBE, urothelial carcinoma and urachal carcinoma. In this preliminary study, we did not provide any strong evidence of major DNA methylation alterations which would be suggestive for strong underlying epigenetic mechanism. However, larger studies are required to provide more robust statistical evidence to exclude smaller effects in the tissues.

Off-label use of IV t-PA in patients with intracranial neoplasm and cavernoma.

BACKGROUND: The safety of systemic thrombolysis in patients with intracranial tumor and cavernoma are unknown. So far evidence is limited to a number of case reports and few case series or unspecified data based on population-based analysis. Our aim was to comprehend the risk of systemic thrombolysis in these patients. METHODS: Patients with additional evidence of intracranial tumor or cavernoma who received IV tissue plasminogen activator (t-PA) treatment at our comprehensive stroke center over a period of 7 years were identified in our stroke database and compared to the same number of matched control subjects without any evidence of intracranial tumor and cavernoma. Clinical history and imaging patterns before and after t-PA therapy were individually reviewed for each patient. RESULTS: Thirty-four patients with additional evidence of meningioma (19/34), cavernoma (13/34) or malignant intracranial neoplasm (2/34) were identified. The incidence of secondary intracranial hemorrhage observed showed no difference between control subjects (9/34, 26%) and patients (6/34, 18%; p = 0.56). Symptomatic hemorrhage in patients with meningioma or cavernoma could not be observed. Likewise, the prevalence of stroke mimics showed no difference between patients (8/34, 24%) and control subjects (5/34, 15%; p = 0.54). However, both patients with malignant intracranial neoplasm presented with a stroke mimic and intracranial hemorrhage was observed in one of them. CONCLUSIONS: In compliance with existing evidence, treatment in patients with meningioma and cavernoma appears to be safe and reasonable, while the therapy should be avoided in patients with malignant intracranial neoplasm with blood-brain barrier disruption.

[Acute Flank Pain in Children and Adolescents]. / Akute Flankenschmerzen im Kindes- und Jugendalter.

Acute flank pain in children and adolescents is a clinically demanding symptom requiring profound diagnostic work-up. Thorough history taking, careful clinical examination as well as urine and laboratory tests in addition to ultrasound are required to guide towards the diagnosis. This article presents possible underlying conditions with specific diagnostic and therapeutic considerations.

Phimosis in a 10-yr-old Boy Without Urinary Infection-How to Inform Parents: Against Circumcision.

In a 10-yr-old boy with no abnormalities or symptoms other than nonretractability of the foreskin, the foreskin should be preserved. If treatment is needed, local corticoid application should be used as first-line therapy, as it gives excellent results in up to 90% of cases, before removing this sensitive part of the body.

The effect of adipose tissue-derived stem cells in a middle cerebral artery occlusion stroke model depends on their engraftment rate.

BACKGROUND: In the field of experimental stem cell therapy, intra-arterial (IA) delivery yields the best results concerning, for example, migrated cell number at the targeted site. However, IA application also appears to be associated with increased mortality rates and infarction. Since many rodent studies systemically apply 1 × 106 cells, this could also be a consequence of engrafted cell number. The aim of this study was therefore to investigate the effect of different doses of adipose tissue-derived stem cells (ASCs) on engraftment rates and stroke outcome measured in vivo using 9.4-T high-field magnetic resonance imaging (MRI). METHODS: Male Wistar rats (n = 43) underwent a middle cerebral artery occlusion (MCAo) for 45 or 90 min, followed by IA delivery of either saline or 1 × 106, 3 × 105, or 5 × 104 ASCs pre-labelled with very small superparamagnetic iron oxide particles (VSOPs). MRI (9.4-T) analysis was performed 48 h and 9 days post-MCAo. Lesion volumes were assessed by analysis of T2-weighted images and cell signal tracking showing cell engraftment and active cell migration by an improved T2*-analysis. RESULTS: The ASC-derived signal intensity increased in the affected hemisphere 48 h post MCAo with injected cell number (p < 0.05). The analysis of stroke volumes revealed an increased infarction after injection of 1 × 106 ASCs compared to controls or application of 5 × 104 ASCs (p < 0.05). At 9 days post-MCAo, injection of 3 × 105 ASCs resulted in reduced infarct volumes (p < 0.05). Correspondingly, MRI analysis revealed no changes in cell numbers between both MRI examinations but showed active ASC migration to the site of infarction. CONCLUSION: Our results confirm that IA injection is an efficient way of targeting damaged brain tissue but its usefulness strongly depends on the right dose of delivered stem cells since this factor has a strong influence on migration rate and infarct volume, with better results for doses below 1 × 106 cells. Future challenges will include the determination of therapeutic doses for best cellular engraftment and stroke outcome.

Evaluation of sexual function in females with exstrophy-epispadias-complex: A survey of the multicenter German CURE-Net.

OBJECTIVE: Standardized knowledge about genital function in adult female individuals with exstrophy-epispadias complex (EEC) is scarce. The aim of this study was to investigate sexual function using the standardized Female Sexual Function Index (FSFI), and to assess the influence of bladder and vaginal reconstruction and the presence of incontinence on FSFI results. METHOD: Sixty-one females (aged ≥18 years) recruited by the German multicenter network for congenital uro-rectal malformations (CURE-Net) were asked to complete the FSFI and a self-designed semi-structured questionnaire assessing comprehensive medical data, gynecological, and psychosocial items. Twenty-one eligible females (34%) returned both questionnaires (mean ± standard deviation [SD] age of 26 ± 5.1 years). RESULTS: In 43% of participants, a staged or single-staged approach had been used for reconstruction, and these had their bladder in use. A primary or secondary urinary diversion (UD) after cystectomy had been performed in 38% of participants. Of the participants, 57% lived in a committed partnership, and 62% had sexual intercourse on a regular basis, with a further 19% experiencing pain or discomfort thereby. Introitus plasty was done in 43%. Mean total FSFI for all participants was 21.3 (SD 1.9). Most domain scores of patients after introitus plasty were similar compared with those without an operative vaginal approach, except for satisfaction (p = 0.057) and pain (p = 0.024). Comparing incontinent with continent patients, significant differences were found for desire (mean 4.6 vs. 3.5, p = 0.021), lubrication (mean 3.1 vs. 4.2, p = 0.049), and satisfaction (mean 1.6 vs. 3.6, p = 0.0065). In contrast pain was not significant between groups. CONCLUSIONS: Sexual activity rate in the present study was similar to that reported in the literature (81% vs. 89%), whereas dyspareunia rate was lower in our cohort (19% vs. 24%). The risk for sexual dysfunction seems to be lower in patients reconstructed with primary or secondary UD than patients with bladder in use. It is surprising that lubrification was better after UD than after bladder neck surgery. Incontinence and in some parts the history of an introitus plasty may play an additional role in development of sexual dysfunction in EEC. Although most of the female EEC patients lived in a committed partnership and had sexual intercourse, total FSFI values <26.55 clearly indicate a risk of sexual dysfunction. Although continence itself played a major role, females reconstructed with UD seem to have better sexual function. Further evaluation of sexual outcome and improvement of care for these patients is mandatory.

Cowper's syringocele: A rare differential diagnosis of infravesical obstruction in boys and young adults.

OBJECTIVE AND INTRODUCTION: Cowper's syringocele is a cystic dilation of the bulbourethral gland duct, initially defined by Maizels et al. in 1983. Although obstructive and non-obstructive types of this rare anomaly are described, clinical symptoms are highly unspecific. Therefore, we report 12 cases of children and young adults diagnosed with Cowper's syringocele to further clarify clinical course, comorbidity and treatment strategies. STUDY DESIGN: We retrospectively collected clinical data of 12 children and young adults from birth to 18.5 years (median 7.2 years) who had been treated in four different institutes during a period of 16 years. The primary specific diagnostic work-up consisted of ultrasound, cystourethrography, and cystoscopy. RESULTS: 3Older patients with a median age of 11.8 years clinically presented with obstructive voiding pattern or gross hematuria; infants with a median age of 0.6 years presented with febrile urinary tract infections (UTIs). After cystoscopic confirmation in all patients, endoscopic treatment was possible in nine; open surgical resection was necessary in three patients. Because of intrauterine megacystis and chronic renal failure, one boy underwent suprapubic diversion with a cystostomy soon after birth. Owing to urological comorbidity or later complications, additional procedures were necessary, for example, resection of minor urethral valves, prophylactic circumcision for UTI, endoscopic or open antireflux procedures, and occasionally complex bladder reconstructions in the long term. DISCUSSION: According to our data, the initial clinical symptoms of Cowper's syringocele were related to presenting age rather than the previously described type of syringocele according to Maizels et al. Infants presented with febrile UTIs; however, older boys and young adults had mainly voiding problems or nocturnal enuresis. Therefore, the clinical significance of the described syringocele types must be questioned. Eighty-three percent of our patients showed additional urological pathology such as vesicoureterorenal reflux, ureteropelvic junction obstruction, megaureter, or minor urethral valves. Thus, Cowper's syringocele hardly seems to be an isolated pathology. CONCLUSION: Although rare, Cowper's syringoceles should be considered in differential diagnosis of infravesical obstruction in boys and young adults. Diagnostics are usually justified by presenting symptoms such as UTI or urinary flow impairment, which seem to be age dependent. Despite modern diagnostic tools, diagnosis is usually made by cystourethrography and sometimes accidentally by cystoscopy. Considerable urological comorbidities and consecutive bladder dysfunction need long-term follow-up.

Decompressive Hemicraniectomy in Malignant Middle Cerebral Artery Infarction: The 'Real World' Beyond Studies.

BACKGROUND: Decompressive hemicraniectomy (DHC) is life-saving in patients with malignant middle cerebral artery infarction (MMI), but outcome, perspectives and complications after DHC in daily practice are largely unknown. METHODS: From 2008 until 2014, we extracted patient's characteristics as well as complications from our database for patients with MMI who underwent DHC. Additionally, we analysed medical records from the different rehabilitation steps. RESULTS: We identified 48 consecutive patients (mean 57 years, 21 male, 41.7% >60 years) with MMI who underwent DHC. The decision for DHC was made on an individual basis, including patients without impaired consciousness or stroke onset >48 h. In-hospital patients achieved only marginal clinical improvement. Seventy-five percent attended an early-rehabilitation, 44% achieved post-stroke rehabilitation and 6% carried on late-stage rehabilitation. In all, 45.5% returned home after rehabilitation. In-hospital mortality was 14.6%, overall mortality was 16.7%. Surviving patients (78.9%) had a modified Rankin Scale of 4-5. Frequent neurologic complications were symptomatic epilepsy and delirium. Following DHC/bone-flap-reimplantation, wound-healing disorders, epidural hematoma and wound infections were major surgery-related complications. Pulmonary infections were frequent in the acute-phase and urinary tract infections were predominant in the late-phase. CONCLUSIONS: DHC is a life-saving technique in patients with MMI, but complications are frequent, were underestimated in randomized clinical trials and may worsen the functional outcome.

Morphological and Functional Hip Long-Term Results after Exstrophy Repair.

Introduction Abnormalities of the bony pelvis in exstrophy-epispadias complex (EEC) and their possible relation to hip disease are well described. However, there is a lack of information about long-term orthopedic consequences and hip function in patients with EEC. Therefore, we investigated clinical and radiological results in an EEC patient cohort after long-term follow-up. Patients and Methods We conducted a cross-sectional study using standardized radiography, clinical investigation, and the Harris hip score. Seventeen postpuberty consecutive unselected EEC patients (3 female, 14 male; mean age 18.2 years) that presented to our clinic due to urological procedures or routine check-up from 2010 to 2011 were included. All had undergone symphysis approximation with a traction bandage without osteotomy in early childhood. Radiological analysis was conducted offline by two independent investigators. Results Radiological analysis showed a mean pubic diastasis of 5.1 cm (range 2.8-8.5 cm). Borderline hip dysplasia was present in four patients, one of them having had co-occurring developmental hip dysplasia in previous history. No severe dysplasia, subluxation, or luxation of the hip was found; however, one patient showed early hip arthrosis. Clinical examination revealed no relevant restriction of range of motion, although rotation and abduction were slightly altered in five patients. None of the EEC patients complained about pain or restriction in sports or daily activities. Harris hip score was perfect for all but one study participants. Conclusion Despite EEC-specific hip morphology, long-term hip function is not impaired in patients after symphyseal approximation without osteotomy in the newborn period. The symphysis diastasis after this procedure is comparable to available postosteotomy data. The large majority of EEC patients did not show dysplastic or degenerative hip disease. Functional hip score results confirmed reasonable age-related hip function in nearly all examined patients. However, postnatal ultrasound hip screening is recommended to prevent and adequately treat potential co-occurring developmental hip dysplasia.

Body weight gain in patients with bilateral deep brain stimulation for dystonia.

In patients with Parkinson's disease, significant weight gain following chronic deep brain stimulation (DBS) has been reported. Recently, relevant weight gain could be demonstrated also following subthalamic nucleus DBS in patients with primary cervical dystonia. Prospective analyses of body weight changes following DBS in patients with dystonia, however, have not been published so far. We aimed to analyse the changes of body weight following DBS in patients with dystonia. The body mass index (BMI) of 17 consecutive patients with segmental or generalised dystonia (mean age 54.6 ± 16.1 years) treated with bilateral DBS of the globus pallidus internus (GPi) (n = 14) or the thalamic ventral intermediate nucleus (n = 3) was measured preoperatively (pre-OP) and at three follow-up (FU) time points post-DBS surgery (FU1 = 7 months, FU2 = 17 months, FU3 = 72 months). All patients benefited from marked improvement in their dystonia. The mean BMI pre-OP (SD) was 22.5 (±3.7) kg/m(2) and increased stepwise to 24.0 (±3.3) kg/m(2) at FU1, 24.4 (±3.7) kg/m(2) at FU2 and 24.9 (±3.7) kg/m(2) at FU3 (p < 0.05 at all three FUs compared to pre-OP). Relative BMI increase and improvement of dystonia were correlated (p = 0.025). Chronic bilateral GPi DBS in patients with dystonia is associated with significant body weight gain, in particular during the first 6 months post-OP. This probably is a result of improvement of dystonic motor symptoms and recovery of eating dysfunction rather than a target-specific phenomenon.

DWI Lesion Patterns in Cancer-Related Stroke--Specifying the Phenotype.

BACKGROUND: Due to the lack of specific diagnostic markers, the diagnosis of cancer-related stroke strongly depends on its phenotype. Distinct DWI lesion patterns with involvement of multiple vascular territories have been reported repeatedly in cancer-related stroke but have not been addressed in detail in a selected cohort of prospectively recruited cancer patients with emphasis on hypercoagulable conditions. PATIENTS AND METHODS: Ischemic stroke patients with known malignant cancer activity, laboratory evidence of strong plasmatic hypercoagulation (D-dimer levels > 3 µg/ml) and without competing stroke etiologies according to the recently introduced ASCOD (A - atherosclerosis, S - small vessel disease, C - cardiac pathology, O - other cause, and D - dissection) classification of evidence-rated etiology of stroke subtypes were included in the analysis. Cerebral MRI on admission was reviewed with respect to ischemic lesion patterns. RESULTS: Thirty-two patients met the inclusion criteria. The mean D-dimer levels were 15.39 µg/ml (± 10.84). Acute infarction in ≥ 2 vascular territories was present in 27/32 (84%) patients. (Micro-) embolic scattering of infarction was present in 25/32 (78%) patients. Evidence for previous, potentially oligosymptomatic infarction was found in 16 (50%) patients, demonstrated by the additional presence of subacute or chronic ischemic lesions. CONCLUSION: When excluding competing embolic and nonembolic stroke etiologies, the pattern of scattered DWI lesions in multiple vascular supply territories strongly dominates the phenotype of cancer-related stroke. Additionally, evidence of recurrent infarction is frequent in this cohort of patients. This is not only important for the diagnosis of cancer-related stroke itself but may prove helpful for the identification of cancer-related stroke patients with unknown malignancy at the time of stroke manifestation and evaluation of strategies for secondary prevention.