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Rosette-forming glioneuronal tumor (RGNT) is a rare tumor entity which has been reported mainly occurring in the fourth ventricle. It has been described as a benign lesion with limited extension into surrounding structures, including the cerebellar vermis, midbrain, and cerebral aqueduct. More recently, few cases involving also other midline structures have been documented as well. Here, we report about diagnosis and treatment of RGNT in the septum pellucidum in a pediatric patient which has not been described previously. A 7-year-old boy had a 3-week history of headache. Magnetic resonance imaging showed a solid mass in the septum pellucidum accompanied by hydrocephalus. The tumor was resected via a transcortical approach. Histological examination revealed the typical findings of a RGNT. At 2-year follow-up, there was no tumor recurrence, and clinical outcome was unremarkable. RGNT has to be considered in the differential diagnosis of pediatric midline tumors also outside of the fourth ventricle. Surgical resection is the first-line therapy which may result in beneficial outcome in the long term. The role of adjuvant therapy needs further definition since due to the rarity of this tumor entity, available data is very limited.

Objective Despite contemporary diagnostic and therapeutic techniques intracranial emergencies in the obstetric setting pose still a major challenge for the clinicians. There are limited guidelines and differing ethical views. Multidisciplinary teams are needed to support the pregnant woman in a way that she can deliver a viable and healthy child. The aim of the present study was to scrutinize the management of intracranial emergencies during pregnancy which needed urgent neurosurgical treatment. Patients and methods Data of all pregnant women who presented with newly diagnosed intracranial pathologies and neurological symptoms caused by these pathologies in an emergency setting were collected over a 10-year period (2008–2018). Patient characteristics including maternal age, gestational age, and preoperative work-up of both mother and fetus were recorded. Furthermore, the surgical treatment, mode of delivery, and neonatal and maternal outcomes were analysed. Results The mean maternal age was 32.7 years and most patients were in their third trimester. There was one twin pregnancy (total of 12 fetuses). Five out of eleven pregnant women suffered from intracerebral haemorrhage (epidural haematoma (1), arteriovenous malformation (1), subarachnoid haemorrhage (2) and intracerebral haemorrhage (1)) and the other six patients had intracranial neoplasms (primary meningeal sarcoma (1), trigeminal schwannoma (1), anaplastic astrocytoma (2), glioblastoma (1) and sphenoid wing meningioma (1)).Neurosurgical procedures were performed via craniotomies in eight patients. A stereotactic biopsy via a frontal burr hole was achieved one patient. The two other patients with subarachnoid haemorrhage due to rupture of PICA aneurysms were treated with coil embolization. Depending on the gestational age and the clinical condition of the pregnant women it was decided to perform an emergency Caesarean section prior to further therapeutic measures in seven patients. Two out of 12 fetuses were unviable. Six women survived, while five women succumbed to the intracranial pathology. Conclusion The individualized treatment approach in this peculiar obstetric scenario needs to consider various issues such as the clinical condition of the pregnant woman, prognosis of the disease, gestational age and the status of the pregnancy. The primary concern in this context must be the mother`s health and safety. Caesarean section is the primary mode of delivery in most cases. While contemporary care can insure survival for the majority of infants, maternal mortality still poses an extraordinary challenge. Interdisciplinary consulting of the patient and/or her family is necessary to develop a treatment strategy for both the expectant woman and her offspring.

Background Low back pain is a common complaint during pregnancy. However, spinal pathologies, which manifest with severe pain, radiculopathy, and acute neurologic deficits because of disk herniation or mass lesions require special attention. Here, we present our interdisciplinary experience in the surgical management of spinal emergencies during pregnancy. Methods The data of pregnant women who underwent surgery for spinal pathologies over a 10-year period were collected. Patient-related characteristics such as maternal age, gestational age, preoperative workup, signs and symptoms of mothers, and diagnostic procedures were evaluated. After an interdisciplinary conference, individualized treatment plans regarding available options were developed. Fetal Doppler and cardiotocography were obtained before and after surgery. Results Nine pregnant women presented with spinal disorders and underwent spinal emergency surgery within the study period. The mean maternal age was 32.2 years. Six women presented with lumbar disk herniations manifesting as severe sciatica or foot drop and 3 patients had thoracic mass lesions resulting in cauda equine syndrome and/or ataxia. The mean gestational age at the time of presentation was 26.5 weeks. Caesarean sections were performed in 3 women prior to the neurosurgical procedure, whereas the pregnancies were maintained in the 6 other patients. Eight infants who were healthy at birth had an unremarkable development. Conclusions Surgery for spinal emergencies in pregnancy can be performed safely according to individual treatment plans developed by an interdisciplinary team taking into account the expectant mother's decision. Maintenance of pregnancy is possible and feasible in most patients.

Navigated intracranial endoscopy with conventional technique usually requires sharp head fixation. In children, especially in those younger than 1 year of age and in older children with thin skulls due to chronic hydrocephalus, sharp head fixation is not possible. Here, we studied the feasibility, safety, and accuracy of electromagnetic (EM)-navigated endoscopy in a series of children, obviating the need of sharp head fixation. Seventeen children (ten boys, seven girls) between 12 days and 16.8 years (mean age 4.3 years; median 14 months) underwent EM-navigated intracranial endoscopic surgery based on 3D MR imaging of the head. Inclusion criteria for the study were intraventricular cysts, arachnoid cysts, aqueduct stenosis for endoscopic third ventriculostomy (ETV) with distorted ventricular anatomy, the need of biopsy in intraventricular tumors, and multiloculated hydrocephalus. A total of 22 endoscopic procedures were performed. Patients were registered for navigation by surface rendering in the supine position. After confirming accuracy, they were repositioned for endoscopic surgery with the head fixed slightly on a horseshoe headholder. EM navigation was performed using a flexible stylet introduced into the working channel of a rigid endoscope. Neuronavigation accuracy was checked for deviations measured in millimeters on screenshots after the referencing procedure and during surgery in the coronal (z = vertical), axial (x = mediolateral), and sagittal (y = anteroposterior) planes. EM-navigated endoscopy was feasible and safe. In all 17 patients, the aim of endoscopic surgery was achieved, except in one case in which a hemorrhage occurred, blurring visibility, and we proceeded with open surgery without complications for the patient. Navigation accuracy for extracranial markers such as the tragus, bregma, and nasion ranged between 1 and 2.5 mm. Accuracy for fixed anatomical structures like the optic nerve or the carotid artery varied between 2 and 4 mm, while there was a broader variance of accuracy at the target point of the cyst itself ranging between 2 and 9 mm. EM-navigated endoscopy in children is a safe and useful technique enhancing endoscopic intracranial surgery and obviating the need of sharp head fixation. It is a good alternative to the common opto-electric navigation system in this age group.

Summary Background Brain metastases (BM) from the gastrointestinal tract (GIT) cancers are relatively rare. Despite those advances in diagnostic and treatment options, life expectancy and quality of life in these patients are still poor. In this review, we present an overview of the studies which have been previously performed as well as a comprehensive strategy for the assessment and treatment of BM from the GIT cancers. Method To obtain information on brain metastases from GIT, we performed a systematic review of Medline, EMBASE and the Cochrane Central Register of Controlled Trials (CENTRAL). The collected data included patient characteristics, primary tumor data and brain metastases data. Result In our search of the literature, we found 74 studies between 1980 and 2011, which included 2538 patients with brain metastases originated from gastrointestinal cancer. Analysis of available data showed that among 2538 patients who had brain metastases from GIT, a total of 116 patients (4.57%) had esophageal cancer, 148 patients (5.83%) had gastric cancer, 233 patients (9.18%) had liver cancer, 13 patients had pancreas cancer (0.52%) and 2028 patients (79.90%) had colorectal cancer. The total median age of the patients was 58.9 years. Conclusion Brain metastases have been considered the most common structural neurological complication of systemic cancer. Due to poor prognosis they influence the survival rate as well as the quality of life of the patients. The treatment of cerebral metastasis depends on the patients' situation and the decisions of the treating physicians. The early awareness of a probable metastasis from GI to the brain will have a great influence on treatment outcomes as well as the survival rate and the quality-of-life of the patients.

Without adequate prophylaxis, liver transplantation (LTx) is frequently followed by hepatitis B virus (HBV) reinfection, which results in rapidly progressing liver disease and significantly decreased overall survival. In the last two decades, significant progress has been made in the prophylaxis and treatment of HBV.We present an overview of different protocols and regimens used for prophylaxis of HBV reinfection after LTx and describe the protocol implemented at our center. Following LTx, HBV reinfection can be effectively prevented by administration of anti-hepatitis B immunoglobulin (HBIg) alone or more recently in combination with antiviral nucleoside/nucleotide analogs (NUCs). Several studies reported good results with the use of HBIg alone, but combination treatment with HBIg and NUCs has proven to be a superior prophylactic regimen for HBV recurrence. At present, combination therapy (HBIg and a nucleoside or nucleotide analog) is the gold standard used in many transplantation centers. This preventive regimen reduces the risk of a recurrence of HBV infection and thereby the need for re-transplantation. Future and ongoing studies will show how long HBIg must be given after transplantation, especially when used in combination with potent antivirals, such as entecavir or tenofovir.

During the last decades, the disparity between the organ supply and the demand for kidney transplantation in Europe has led to consider living donors as a more acceptable option. In the last 7 years, we have established an interdisciplinary supporting transplant team to increase the rate of living donation. After 2001, the new interdisciplinary transplant team consisted of a transplant surgeon, a nephrologist, a pediatrician, a radiologist, a psychologist, a transplant coordinator, and a transplant nurse. We performed a prospective analysis to examine the effect of implementing this team on our living donation program. Demographic data, the annual number of procedures, the duration of waiting, and the cold ischemia time were evaluated among brain-dead and living donors. From January 2002 until December 2008, the number of patients who were annually on the waiting list increased 42% (from 377 to 536 patients). Consequently, the number of the total kidney transplants increased from 81 to 120 with an annual median of 98 cases. By implementing the interdisciplinary transplant team, a significant increase of living kidney donors was observed: from 18 to 42 cases; median = 27). In the last 7 years, a total number of 796 kidney transplants have been performed: 567 from brain-dead and 229 from living donors. In 2001, the waiting list times for recipients who received grafts from brain-dead versus living donors were 1356 versus 615 days respectively. Compared with 2008, the duration on the waiting list decreased significantly for patients receiving a living donor graft, whereas there was a slight increase for the patients in the brain-dead group: brain death versus living donors: 1407 versus 305 days. The interdisciplinary approach has also reduced the cold ischemia time for the living donor recipients: 3 hours and 42 minutes in 2001 versus 2 hours and 50 minutes in 2008. During the last years, by implementing an interdisciplinary transplant team, supporting living donor procedures has produce a gradual increase in the number of kidney transplants from living donors with a remarkable decrease in waiting and cold ischemia times, the latter presumably influencing graft quality.

Caring for a brain dead potential organ donor requires a shift in critical care from the extensive treatment of increased intracranial pressure towards strategies to maintain donor organ function. Suboptimal, unstandardized critical care management of organ donors, however, is one of the main reasons for insufficient organ procurement. The pathophysiological changes following brain death entail a high incidence of complications including hemodynamic instability, endocrine and metabolic disturbances, and disruption of internal homeostasis that jeopardize potentially transplantable organs. Strategies for the management of organ donors exist and consist of the normalization of donor physiology. This has resulted in standardized efforts to improve the critical care delivered to potential organ donors, increasing not only the number, but also the quality of suitable organs and aiming at an optimal outcome for the recipients. In this review, we discuss the pathophysiological changes associated with brain death and present the current guidelines at our department, which are optimized based on available literature.

Infection associated with ventriculoperitoneal (VP) shunt implantation can be a significant problem. VP shunt infection with Serratia marcescens, a gram-negative anaerobic rod, usually is related to underlying abdominal disease. This article describes treatment of two patients suffering from a VP shunt infection with S. marcescens without underlying abdominal disease.

Desmoplastic infantile gliomas (DIG) are rare tumors that occur in infants aged between 1 and 24 months. The tumor in general has a favorable prognosis after surgical resection. There are no treatment algorithms, however, for patients with multiple intracranial and intraspinal presentations. In an 11-month-old girl with a history of nystagmus, magnetic resonance imaging (MRI) demonstrated contrast-enhancing lesions in the suprasellar region, the cerebellar vermis, and the spinal axis. The tumor in the cerebellar vermis was removed via a suboccipital midline approach. The histological examination revealed a desmoplastic infantile astrocytoma (DIA) WHO grade I. Postoperatively, it was decided to adopt a wait-and-see strategy. Further development, up to 16 months after surgery, was unremarkable. Follow-up MRI showed no recurrence of the posterior fossa tumor, mild progress of the suprasellar tumor, and significant regression of the spinal tumors. DIA is a rare mostly benign brain tumor found in infants. The final diagnosis always relies on histology. Surgical resection is the recommended therapy for symptomatic tumors; however, more experience is needed to develop treatment recommendations for multiple-site tumors.

Background Laparoscopic distal pancreatectomy is regarded as a feasible and safe surgical alternative to open distal pancreatectomy for lesions of the pancreatic tail and body. The aim of the present systematic review was to provide recommendations for clinical practice and research on the basis of surgical morbidity, such as pancreas fistula, delayed gastric empting, safety, and clinical significance of laparoscopic versus open distal pancreatectomy for malignant and nonmalignant diseases of the pancreas. Methods A systematic literature search (MEDLINE) was performed to identify all types of studies comparing laparoscopic distal pancreatectomy and open distal pancreatectomy. Random effects meta-analyses were calculated after critical appraisal of the included studies and presented as odds ratios or mean differences each with corresponding 95% confidence intervals. Results A total of 4,148 citations were retrieved initially; available data of 29 observational studies (3,701 patients overall) were included in the meta-analyses. Five systematic reviews on the same topic were found and critically appraised. Meta-analyses showed superiority of laparoscopic distal pancreatectomy in terms of blood loss, time to first oral intake, and hospital stay. All other parameters of operative morbidity and safety showed no difference. Data on oncologic radicality and effectiveness are limited. Conclusion Laparoscopic distal pancreatectomy seems to be a safe and effective alternative to open distal pancreatectomy. No more nonrandomized trials are needed within this context. A large, randomized trial is warranted and should focus on oncologic effectiveness, defined end points, and cost-effectiveness.

Choledocholithiasis exists in approximately 15% of patients with gallstones and is present in 3%-10% of those undergoing cholecystectomy.In this study, we retrospectively analyzed the outcome patients with choledocholithiasis that were managed by open common bile duct (CBD) exploration according to our center's protocol. Endoscopic retrograde cholangiopancreatography (ERCP) was performed for CBD stone clearance. If ERCP and sphincterotomy were not successful, open surgical exploration of CBD was performed with T-tube insertion without routine intraoperative cholangiography (IOC).We studied 1462 patients with choledocholithiasis. ERCP was successful in in 1276 (87.2%) patients. A total of 186 (12.8%) underwent surgery. Of these, 82 (45.2%) had CBD exploration and T-tube insertion without IOC. Choledochoduodenostomy was performed in 82 (44.1%) patients and choledochojejunostomy was performed in 20 (10.8%). Retained stones were found only in 4 cases which were treated by ERCP.ERCP is successful in most cases with choledocholithiasis. If ERCP fails, open exploration of CBD and T-tube insertion, or biliary-enteric anastomosis are acceptable ways for CBD drainage. The rate of retained stone is not more than expected, thus elective IOC is more acceptable than routine IOC. Routine IOC is time-consuming and particularly difficult in elderly patients and emergency conditions.

OBJECTIVES Owing to an imbalance between demand and supply, which is more prominent in pediatric transplant, every year more patients lose their lives on waiting lists. In addition to the use of deceased-donor split and living-donor organs, xenotransplant could provide a solution if associated problems, such as immunologic and physiologic ones, are solved. This study sought to analyze the surgical aspects for liver xenotransplant in a porcine model. MATERIALS AND METHODS Landrace pigs (n=22, 23 to 37 kg) underwent a laparotomy under general anesthesia. The hepatic hilum was prepared and the common bile ducts, common hepatic artery, portal vein, supra- and infrahepatic inferior vena cava were identified. The length and diameter of each vessel and bile duct and the weight of the liver were measured. RESULTS Pearson tests showed a clear correlation between the increase of the pigs' weight and the livers' weight, and the length of the vessels and the bile ducts. We did not find a clear correlation between the increase of the pigs' liver weight and the diameters of the vessels and the bile duct. CONCLUSIONS As the first reporting, this study on xenotransplants from the surgical point of view, we postulate that it could be possible to estimate the size of the liver and the proper length of its vessels and bile duct by weighing only the pigs. It was not feasible to match the diameter of mentioned structures by the livers' weight. However, the weight of pig's liver as well as vascular anatomy of pigs appeared to be suitable alternative for the human liver.

Adrenal insufficiency is a life-threatening disorder which must be treated with glucocorticoid replacement and needs permanent dose adjustment during patient's different somatic situations. Insufficient glucocorticoid doses result in adrenal crisis and must be treated with intravenous hydrocortisone. The patient was known with Adrenal insufficiency and was treated optimally with fludrocortisone and prednisolone since seven years with no history of adrenal crisis. The patient was admitted with abdominal pain, weakness, fatigue and nausea developed 3-4 days after taking psyllium, a bulking agent, prescribed by a surgeon to diagnose anal fissure. Detailed medical history, physical examinations, laboratory and imaging examinations did not approve any other cause of adrenal crisis. Psyllium may interfere with gastrointestinal absorption of prednisolone and/or fludrocortisone and trigger acute adrenal crisis in patients with adrenal insufficiency.

Background An accident or a catastrophic disease may occasionally lead to brain death (BD) during pregnancy. Management of brain-dead pregnant patients needs to follow special strategies to support the mother in a way that she can deliver a viable and healthy child and, whenever possible, also be an organ donor. This review discusses the management of brain-dead mothers and gives an overview of recommendations concerning the organ supporting therapy. Methods To obtain information on brain-dead pregnant women, we performed a systematic review of Medline, EMBASE and the Cochrane Central Register of Controlled Trials (CENTRAL). The collected data included the age of the mother, the cause of brain death, maternal medical complications, gestational age at BD, duration of extended life support, gestational age at delivery, indication of delivery, neonatal outcome, organ donation of the mothers and patient and graft outcome. Results In our search of the literature, we found 30 cases reported between1982 and 2010. A nontraumatic brain injury was the cause of BD in 26 of 30 mothers. The maternal mean age at the time of BD was 26.5 years. The mean gestational age at the time of BD and the mean gestational age at delivery were 22 and 29.5 weeks, respectively. Twelve viable infants were born and survived the neonatal period. Conclusion The management of a brain-dead pregnant woman requires a multidisciplinary team which should follow available standards, guidelines and recommendations both for a nontraumatic therapy of the fetus and for an organ-preserving treatment of the potential donor.

Changes in liver microcirculation are considered essential in assessing ischemia-reperfusion injury, which in turn has an impact on liver graft function and outcome following liver transplantation (LTx). The aim of this study was to introduce dynamic magnetic resonance imaging (dMRI) as a new technique for overall quantification of hepatic microcirculation and compare it to perfusion measured by laser Doppler flowmetry (LDF; hepatic artery/portal vein) and thermal diffusion (TD). The study included 3 groups, measuring hepatic blood flow and microcirculation with the help of TD, LDF, and dMRI. In group I (9 landrace pigs; 26 +/- 5 kg), the native liver before and after partial portal occlusion was studied; in group II (6 landrace pigs; 25.5 +/- 4.4 kg), the liver 24 hours after LTx was studied; and in group III (14 patients), the liver on days 4 to 7 following LTx was studied. A close correlation was found between dMRI measurements and TD (r = 0.7-0.9, P0.01) in 4 defined regions of interest. Portal blood flow and partial occlusion of the portal vein were accurately detected by LDF flowmetry and correlated well with dMRI (r = 0.95, P0.01). In the clinical setting, representative TD measurements in segment 4b of the transplanted liver correlated well with dMRI analysis in other segments. Quantification of the portal blood flow and imaging of the whole liver could be performed simultaneously by dMRI. In conclusion, dMRI has been proved to be a sensitive modality for the quantification of liver microcirculation and hepatic blood flow in experimental and clinical LTx. It allows for a synchronous, noninvasive assessment of macrocirculation and microcirculation of the liver and could become a valuable diagnostic tool in advanced liver surgery and transplantation.

Background Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder of the central nervous system; it has only recently been defined and to date has received only limited attention. Its cause is as yet unknown. The pathologic characteristics are infiltration of T lymphocytes into the perivascular spaces of the pons, responsiveness to immunotherapy, and gadolinium-enhancing punctiform lesions in the brainstem seen on magnetic resonance imaging (MRI). Case Description We report here on the clinical, MRI, and brain biopsy findings in a 68-year-old man who presented with dysphagia, numbness and paresthesia on the right side of his face, as well as progressive gait ataxia. Brain and spinal MRI showed lesions in the pons and in the cervical spinal cord. The pontine lesion became progressively larger extending to the middle cerebellar peduncle and a tumor was suspected. After repeated biopsy, the histopathologic diagnosis confirmed CLIPPERS. Conclusions CLIPPERS syndrome may become manifest with a progressive tumor-like pontine lesion. This report adds clinical and radiologic aspects to the limited number of CLIPPERS cases reported to date, and underlines the importance of considering CLIPPERS in the differential diagnosis of tumor-like pontine processes.