[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.

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.

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.

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.

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.

MRI of the lung (2/3). Why when how?

BACKGROUND: Among the modalities for lung imaging, proton magnetic resonance imaging (MRI) has been the latest to be introduced into clinical practice. Its value to replace X-ray and computed tomography (CT) when radiation exposure or iodinated contrast material is contra-indicated is well acknowledged: i.e. for paediatric patients and pregnant women or for scientific use. One of the reasons why MRI of the lung is still rarely used, except in a few centres, is the lack of consistent protocols customised to clinical needs. METHODS: This article makes non-vendor-specific protocol suggestions for general use with state-of-the-art MRI scanners, based on the available literature and a consensus discussion within a panel of experts experienced in lung MRI. RESULTS: Various sequences have been successfully tested within scientific or clinical environments. MRI of the lung with appropriate combinations of these sequences comprises morphological and functional imaging aspects in a single examination. It serves in difficult clinical problems encountered in daily routine, such as assessment of the mediastinum and chest wall, and even might challenge molecular imaging techniques in the near future. CONCLUSION: This article helps new users to implement appropriate protocols on their own MRI platforms. Main Messages • MRI of the lung can be readily performed on state-of-the-art 1.5-T MRI scanners. • Protocol suggestions based on the available literature facilitate its use for routine • MRI offers solutions for complicated thoracic masses with atelectasis and chest wall invasion. • MRI is an option for paediatrics and science when CT is contra-indicated.

Evaluating childhood obesity: magnetic resonance-based quantification of abdominal adipose tissue and liver fat in children.

PURPOSE: The purpose of this study is to establish and validate a magnetic resonance (MR)-based fat quantification package that provides an accurate assessment of abdominal adipose tissue and liver fat in children. MATERIALS AND METHODS: Ex vivo trials with a torso model and water-oil mixtures are conducted. Abdominal adipose tissue (AAT) is covered by magnetic resonance imaging (MRI) using a fat-selective sequence and is analyzed by a plug-in based on the open source software ImageJ. Liver fat (LF) is measured with localized 1H Magnetic Resonance Spectroscopy (1H MRS) and the jMRUI (java-based Magnetic Resonance User Interface) software package. Evaluation of the clinical methodology involved a study of 10 children in this feasibility study (mean age and body mass index: 13.3 yr; 33.3 kg/m²). To evaluate the method's validity, reference measurements were performed. RESULTS: Ex vivo trials with the torso model showed that adipose tissue was measured appropriately with a systematic underestimation by 9.3 ± 0.2 % (0.32 ± 0.064 kg). Coefficients of variation for both intra- and inter-observer measurements ranged between 0 - 2.7 % and repeated analyses showed significant equivalent results (p < 0.01). The lipid content obtained by 1H MRS ex vivo revealed significant equivalence with the predefined fat content in water-oil mixtures (p < 0.01). In vivo, the homemade plug-in significantly overestimated the AAT, with the visceral adipose tissue being most affected (+ 15.7 ± 8.4 %). CONCLUSION: Although an overestimation of the AAT by the presented plug-in should be taken into consideration, this children-friendly package enables the quantification of both LF and AAT within 30 min on a freeware-based platform.

[Cystic abdominal mass as a complication due to posterior urethral valves]. / Zystische abdominale Raumforderung als Komplikation von posterioren Harnröhrenklappen.

A case of urinoma with posterior urethral valves and its management is presented. Diagnostic investigations and therapy strategies are discussed. Though rare, this possibility should be considered in the differential diagnosis of neonates presenting with rapidly expanding cystic masses in the abdomen. Early diagnosis and management are the most important prognostic factors that ensure a good outcome in such cases.

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.