Is there a predictive value of EEG and MRI after a first afebrile seizure in children?

BACKGROUND: After a first afebrile seizure, EEG in addition to cMRI is recommended for pediatric patients. Once indications requiring immediate treatment are excluded, it is of interest to determine if the results provide a prognostic tool for seizure relapses. METHODS: Patients aged between 1 month and 18 years who had a first afebrile seizure between 2006 and 2008 were retrospectively studied and monitored for another 48 months. RESULTS: Out of 248 patients, 62.5% had generalized and 36.3% focal seizures. 34.7% of the EEG results were pathological. 176 patients had a cMRI that showed in 23.3% probable epileptogenic lesions. 3 patients with benign cerebral tumours needed surgical therapy. In the following 48 months 29.4% of the children showed seizure relapses. There was a correlation between epileptic patterns in the EEG and further seizures (p=0.0001). However, the sensitivity of the EEG based diagnoses was 0.6, the specificity 0.78 and the positive predictive value 0.52. There was no correlation between epileptogenic lesions and the probability of seizure relapses. The sensitivity of the cMRI to this effect was 0.36, the specificity 0.74 and the positive predictive value 0.34. DISCUSSION: The EEG is superior to cMRI for predicting seizure relapses. The percentage of noticeable cMRI findings is high but this has low therapeutic relevance and is assumed to largely represent "incidental findings". It is important to question the value of MRI investigations for sedated small children except in the case of emergencies. The key question is whether the cMRI should be deployed to diagnose epilepsy, the probability of seizure recurrences or to classify the entity of a most likely epilepsy.

[Congenital pulmonary malformations : Diagnosis and treatment]. / Kongenitale Lungenfehlbildungen : Diagnostik und Therapie.

PERFORMANCE: Congenital pulmonary malformations (CPM) are rare and can be associated with high morbidity. Clinical presentation, diagnostic procedures, imaging, and therapy of CPM are discussed. ACHIEVEMENTS: Today, most CPM can be diagnosed prenatally by ultrasound. Postnatally, respiratory symptoms up to respiratory failure and recurrent lower respiratory tract infection are typical findings. Due to low diagnostic accuracy of chest x­ray in CPM, all children with prenatal diagnosis of CPM or postnatally suspected CPM should undergo cross-sectional imaging. PRACTICAL RECOMMENDATIONS: Based on imaging alone, the various subtypes of CPM cannot be definitively differentiated, which is why histological confirmation remains the gold standard. Surgical resection is the standard of care with minimally invasive procedures increasingly being employed. In certain situations, a watch-and-wait approach is possible.

Updated physiology and pathophysiology of CSF circulation--the pulsatile vector theory.

INTRODUCTION: Hydrocephalus is still a not well-understood diagnostic and a therapeutic dilemma because of the lack of sufficient and comprehensive model of cerebrospinal fluid circulation and pathological alterations. CONCLUSIONS: Based on current studies, reviews, and knowledge of cerebrospinal fluid dynamics, brain water dynamics, intracranial pressure, and cerebral perfusion physiology, a new concept is deducted that can describe normal and pathological changes of cerebrospinal fluid circulation and pathophysiology of idiopathic intracranial hypertension.

Acute two-compartment low pressure hydrocephalus--a case report.

A case of an 8-year-old-boy with shunt-dependent occlusive hydrocephalus after resection of a cerebellar medulloblastoma is presented, who experienced repeated episodes of severe neurologic deterioration with signs and symptoms of raised intracranial pressure after spinal tapping. However, intracranial pressure was recorded within low ranges, only up to the opening pressure of the implanted adjustable shunt valve. Multiple shunt revisions were performed, until the condition was recognized as acute normal pressure hydrocephalus. Either enforced recumbency and downadjustment of the valve system to 0 cm H(2)O alone or external ventricular drainage seems to be successful to resolve the critical condition, depending on severity of the symptoms. The case illustrates that acute pathologic enlargement of the ventricular system is not always associated with increased intracranial pressure, even when typical signs and symptoms are present. The very rare entity of acute normal pressure hydrocephalus by two separated compartments is postulated based on the pulsatile vector force theory of brain water circulation.

Effectiveness of bevacizumab for radiation-induced cerebral necrosis in children.

INTRODUCTION: Bevacizumab has been reported to effectively reduce cerebral edema caused by radiation therapy. However, only limited data with a short follow-up in tumor patients are available so far. PATIENTS AND METHODS: Two children suffering from hemorrhage from arteriovenous malformation (AVM) have been treated with stereotactic radiotherapy and developed radiation-induced cerebral edema with deteriorating neurological status despite maximized steroid therapy. Bevacizumab administration at 5 mg/kg body weight was initiated every 2 weeks. RESULTS: Bevacizumab treatment rapidly ameliorated the neurological deficits, malignant edema and prevented catastrophic complications. Corticoid therapy could be reduced and discontinued. However, after 18 months, both patients showed identical or worse neurological status than before bevacizumab therapy. AVM radiation therapy had been successful to completely obliterate AVMs. DISCUSSION: In our limited experience, bevacizumab may be an effective and safe option for rescue therapy for malignant cerebral edema on the basis of radiation-induced necrosis especially in patients who experience rapid deterioration despite corticoid therapy and/or intolerable steroid side effects. Despite the fact that functional improvement could not be achieved in long-term outcome patients significantly stabilized and improved during periods of acute deterioration. In order to determine the long-term effectiveness of bevacizumab further investigation in placebo-controlled studies with a higher number of patients are required.

[Diverticulitis and indication for surgery--are there new data?]. / Operationsindikation bei Divertikulitis--Gibt es neue Daten?

The treatment in acute diverticulitis has undergone a considerable shift from an offensive to a more restrictive and individual indication for surgery. This review of the very recent literature with special regard to long-term observation of conservatively treated patients clearly shows that surgery is not required in any case of a first episode of severe diverticulitis, but should be recommended in high-risk patients under immunosuppression or chronic renal failure. In all other groups of patients the indication for surgery should be weighed on an individual basis after each episode, again aiming for the laparoscopic procedure. A therapeutic algorithm is proposed according to the Hansen-Stock classification.

Coincidence of immune thrombocytopenia, intracranial hemorrhage and graft-versus-host disease in an 11-year-old girl.

Primary immune thrombocytopenia (ITP) in children is usually self-limiting and harmless but can, rarely, result in life-threatening complications. The case of an 11-year-old girl with ITP is presented who developed recurrent intracranial hemorrhages followed by cerebral infarctions. The clinical course was complicated by a graft-versus-host disease involving several organs. Treatment was performed according to the current international consensus report of 2010 with glucocorticoids, immunoglobulin G, anti-D-immunoglobulin and additionally embolisation of the splenic artery. The girl survived. Reliable predictors, preventive measures for life-threatening complications in ITP and more information about the effectiveness and side-effects of the recommended treatment are urgently needed.

Efficacy of laparoscopic sleeve gastrectomy (LSG) as a stand-alone technique for children with morbid obesity.

BACKGROUND: Laparoscopic sleeve gastrectomy (LSG) is basically unknown as a stand-alone technique for bariatric surgery in children and adolescents. It may be advantageous for this age group though, since it requires neither foreign body placement nor life-long malabsorption. We present the first report about the efficacy of LSG in a small pediatric series. METHODS: All patients (n = 4, female) had been in a multi-modal weight loss program for several years without long-term success. At referral, the mean age was 14.5 years (range 8-17), mean body mass index (BMI in kg/m(2)) was 48.4 (range 40.6-56.3). All suffered from various features of a metabolic-vascular syndrome like diabetes, dislipidemia, cholecystolithiasis, arterial hypertension. The 8-year-old girl was diagnosed Prader-Willi Syndrome at the age of 2. The decision for bariatric surgery was taken unanimously by the parents, patient, and the obesity team. LSG was performed in a five-trocar technique. With a gastroscope (size 40-F) protecting the lesser curvature, the stomach was resected from the proximal antrum to the angle of His using an ENDO-GIA stapler. The stapler line was secured by a continuous suture 3-0 vicryl. RESULTS: There were no intra- or postoperative complications. Contrast studies confirmed a J-like gastric remnant (mean volume 76 ml) and ruled out leaks in all cases. After a mean follow-up time of 12 months (range 6-19 months), all the patients had reduced weight (mean BMI to 37.2). The girl with the longest postoperative period went from 121 to 83 kg (BMI from 40.6 to 28.4). Laboratory studies ruled out malnutrition or vitamin deficiency. Monitoring of metabolic parameters showed gradual improvement or even resolution for most features. CONCLUSION: At a 1-year follow-up, LSG proved a safe and effective option for bariatric surgery in children, achieving moderate weight loss and improvement of comorbidities. Thus, it may be considered as stand-alone technique. Long-term studies however must compare these results with time-tested procedures like gastric banding and Roux-en-Y gastric bypass.

Long gap esophageal atresia and arterial circle of Willis variant leading to ischemic stroke during gastric transposition.

In children, strokes occur with a frequency of 2 to 13 per 100,000. Risk factors include hematological, embolic and anatomical anomalies. But often the exact cause of strokes in pediatric patients remains unclear. We report here on a patient with long gap esophageal atresia, who suffered an ischemic stroke during gastric transposition. Postoperative assessment revealed a cerebral vascular variant of the circle of Willis. Follow-up clinical controls showed a favorable course. Today, at the age of 14 months, almost no neurological deficits are evident. To the best of our knowledge, no description of vascular cerebral complications combined with esophageal atresia can be found in the literature.

[Treatment strategies and results of orbital malignancies in childhood]. / Therapiekonzepte und Behandlungsergebnisse maligner Orbitatumoren im Kindesalter.

INTRODUCTION: Each year approximately 1,800 children are diagnosed with cancer in Germany. The orbit is a very rare manifestation site for malignancies in childhood. The most common primary malignant orbital tumour is the rhabdomyosarcoma. Secondary orbital tumours are generally metastases of neuroblastomas or haematological diseases. The five-year survival rate of children with rhabdomyosarcomas has increased to 90 % in the past 30 years due to innovations in radiation and chemotherapy. MATERIAL AND METHODS: A paediatric oncological interdisciplinary approach is taken in the diagnostics and therapy for these rare tumours and is based on therapy optimisation studies that include polychemotherapy, radiation therapy, and surgical treatment. RESULTS: We discuss the current multimodal therapy strategies and results, as well as the role of the ophthalmologist, in the medical care of young patients with malignant orbital tumours, with examples of individual courses of rhabdomyosarcoma, non-Hodgkin's lymphoma, and metastases. CONCLUSIONS: Orbital rhabdomyosarcoma is one of the few life-threatening diseases often first seen by an ophthalmologist. The ophthalmologist's prompt diagnosis and initiation of therapy therefore strongly influences the patient's chance of survival. The role of the ophthalmologist in the medical care of children with orbital malignancies includes a timely clinical diagnosis with histological confirmation, close monitoring of visual functions, the management of ocular complications, as well as long-term follow-up care to detect later therapeutic side effects and/or complications. The treatment of children with suspected tumours is best performed in paediatric oncological centres with access to all necessary specialties in order to ensure rapid diagnosis and therapy.

Emergency department utilisation for inflammatory bowel disease in the United States from 2006 to 2014.

BACKGROUND: Despite advances in treatment, patients with inflammatory bowel disease (IBD) frequently require emergency department (ED) visits and hospitalisations. AIMS: To analyse trends in ED visits and subsequent hospitalisations for IBD in the United States (US). METHODS: Data were analysed from the Nationwide Emergency Department Sample (NEDS) years 2006-2014. The NEDS is the largest all-payer ED database in the US, weighted to represent 135 million visits/year. IBD was identified using ICD-9 codes for Crohn's disease (CD) or ulcerative colitis (UC). Surgeries were identified using procedure codes. RESULTS: The frequency of IBD-ED visits increased 51.8%, from 90 846 visits in 2006 to 137 946 in 2014, which was statistically significant in linear regression. For comparison, all-case ED use between 2006 and 2014 increased 14.8%. In-patient hospitalisations from the ED decreased 12.1% for IBD (from 64.7% rate of hospitalisation from the ED in 2006 to 52.6% in 2014), with a UC:CD ratio of 1.2:1 in 2006 and 1.3:1 in 2014. Chi-square analysis revealed that this was a significant decrease. Surgery rates also showed a statistically significant decrease. The mean ED charge per patient rose 102.5% and the aggregate national cost of IBD-ED visits increased 207.5%. CD accounted for over twice as many visits as UC in both years. UC, age, male gender, highest income quartile, private insurance, Medicaid/Medicare, and tobacco use were associated with in-patient admissions. CONCLUSIONS: The number of ED visits due to IBD and associated charges have continued to rise, while the rates of in-patient hospitalisations referred from the ED and surgeries have decreased.

Administration of arginine plus growth hormone releasing hormone to evaluate growth hormone (GH) secretory status in children with GH deficiency.

BACKGROUND: Diagnosis of growth hormone deficiency (GHD) in childhood is usually based on growth hormone (GH) response to at least two provocative stimuli. The aim of this study was to determine whether sequential administration of arginine (Arg) plus GH releasing hormone (GHRH) could be a useful tool in evaluating GHD in children. METHODS: Thirty patients with short stature (mean age 9.0 years) with decreased growth rate were tested for GHD with Arg and the insulin tolerance test (ITT). Patients with confirmed GHD (peak GH <8 ng/ml) were subsequently tested with Arg + GHRH. RESULTS: Maximum GH stimulation for Arg and ITT was 6.3 (1.0-7.8) and 6.7 (0.5-7.7) ng/ml, respectively. Peak GH for the Arg + GHRH test was 36.3 (4.3-84.5) ng/ml and significantly different from the other provocative tests. Peak GH values for the three tests were not significantly correlated between tests or with clinical parameters. There were no significant differences in Arg + GHRH results between children with or without abnormal hypothalamic-pituitary MRI scans. CONCLUSION: Arg + GHRH gave higher GH levels than insulin or Arg alone. Because of the different causes of childhood GHD (hypothalamic and/or pituitary dysfunction), the Arg + GHRH test is unsuitable .for evaluating GHD and deciding whether GH replacement therapy is indicated.

Gender differences in chronic constipation on anorectal motility.

BACKGROUND: The epidemiology of chronic constipation (CC) skews toward female predominance, yet men make up an important component of those suffering from CC. We sought to determine whether there are sex-specific differences in symptoms and physiologic parameters on anorectal manometry (ARM). METHODS: We performed a case-control analysis of sequential men and age-matched women (2:1 ratio) presenting for ARM as part of the evaluation of CC. We collected physiologic parameters derived from 3D high-resolution ARM in addition to the ROME III constipation module and the Pelvic Floor Distress Inventory 20 (PFDI-20) questionnaires. We analyzed univariate, sex-specific differences in ARM physiologic parameters and PFDI-20 parameters and adjusted for putative confounders using multivariate logistic regression. KEY RESULTS: Our study enrolled 80 men and 165 age-matched women. Men had a higher median sphincter resting pressure (81.2 vs 75.2 mm Hg, P=.01) and mean squeeze pressure (257.0 vs 170.5 mm Hg, P<.0001) than women. Although men reported significantly less severe straining and incomplete evacuation, they had greater mean rectoanal pressure differential (-106.7 vs -71.1 mm Hg, P<.0001), smaller mean defecation index (0.17 vs 0.27, P=.03) and higher volume threshold for urgency (115.2 v. 103.4 mL, P=.03). However, women were more likely to have abnormal balloon expulsion time (BET) than men (52.7% vs 35.0%, P=.01). After multivariate analysis, male gender was the only independent predictor of a normal BET (OR: 0.48, 95% CI: 0.27-0.86, P=.01). CONCLUSIONS & INFERENCES: Men and women with CC differ with regard to symptom severity and physiologic parameters derived from ARM suggesting differences in their pathophysiology.

Congenital oesophageal atresia discordant for tracheo-oesophageal fistula occurring in a set of dizygotic twins.

The authors present a set of female diamnionic and dichorionic twins with different blood types and congenital oesophageal atresia (EA) in both. Surgical management was successful. It can be assumed that EA with tracheo-oesophageal fistula in twin B occurred during an early embryological stage whereas the isolated EA in twin A was the result of a later event. To our knowledge, this is the first published set of dizygotic twins with different types of EA.

Pediatric MR-guided interventions.

MRI guided interventions are a relatively new but steadily growing field within surgery in pediatric age. Besides the advantages of MRI, such as multiplanar capability and excellent soft tissue contrast and spatial resolution, particularly relevant for the pediatric population is the lack of ionizing radiation. There is meanwhile a group of well defined diagnostic or therapeutic indications for applying MR imaging during pediatric interventions. Aim of this review is to give an overview about indications of MR-guided procedures in children as well as the advantages and disadvantages of MR-guided interventions. We also briefly discuss interventional MR-systems and MR-compatible devices. It is our opinion that MR-guidance for pediatric interventions is a promising technique at the beginning of its development.

Correlative angiographic and pathologic findings in the diagnosis of ulcerated plaques in the carotid artery.

We determined the accuracy of angiography in the diagnosis of internal carotid ulcers by comparing the angiographic reports with the pathologic findings in 36 endarterectomy specimens. Eighteen of these specimens had microscopic ulcerations, and the observer with the highest accuracy rate read 12, of which ten were ulcerated. These results revealed a sensitivity of 56%, a specificity of 89%, and an overall accuracy of 61% for angiography. The ulcers were classified into types A, B, and C to assess the interobserver agreement rate among three readers. This resulted in a 4% interobserver agreement among a total of 75 ulcers. Because of the high interobserver disagreement and the poor correlation between angiographic and pathologic findings in the surgical specimens, we conclude that the diagnosis of carotid artery ulceration by angiography is not reliable.