[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.

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.

[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.

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.

[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.

[Developmental malformations of the cerebral cortex]. / Heterotopie, Polymikrogyrie, Lissenzephalie und Co - Malformationen der kortikalen Hirnentwicklung.

Migration disorders (MD) are increasingly recognized as an important cause of epilepsy and developmental delay. Up to 25 % of children with refractory epilepsy have a cortical malformation. MD encompass a wide spectrum with underlying genetic etiologies and clinical manifestations. Research regarding the delineation of the genetic and molecular basis of these disorders has provided greater insight into the pathogenesis of not only the malformation but also the process involved in normal cortical development. Diagnosis of MD is important since patients who fail three antiepileptic medications are less likely to have their seizures controlled with additional trials of medications and therefore epilepsy surgery should be considered. Recent improvements in neuroimaging have resulted in a significant increase in the recognition of MD. Findings can be subdivided in disorders due to abnormal neurogenesis, neuronal migration, neuronal migration arrest and neuronal organization resulting in different malformations like microcephaly, lissencephaly, schizencephaly and heterotopia. The examination protocol should include T 1-w and T 2-w sequences in adequate slice orientation. T 1-w turbo-inversion recovery sequences (TIR) can be helpful to diagnose heterotopia. Contrast agent is needed only to exclude other differential diagnoses.

Whole-body MRI for primary evaluation of malignant disease in children.

OBJECTIVE: The aim of this study was to compare diagnostic accuracy of whole-body (WB) MRI to a combined reference standard of conventional cross-sectional imaging methods and FDG-PET in the detection of malignant disease spread in children. MATERIALS AND METHODS: 24 children (age between 5 and 18 years) with malignant diseases (mainly Hodgkin's lymphoma and different types of sarcoma) initially examined with conventional cross-sectional imaging methods (ultrasound, computed tomography, or magnetic resonance imaging) were examined prospectively with whole-body MRI (1.5T) and FDG-PET. Studies were read by two nuclear medicine physicians (FDG-PET) and two radiologists (WB-MRI) independently in a blinded manner and each study type was evaluated in consensus. The reference standard was defined as pathological lesions detected in the same location both in FDG-PET and another conventional cross-sectional imaging method. RESULTS: Overall 190 lesions were detected by WB-MRI and 155 lesion were found by FDG-PET. 106 lesions fulfilled the criteria of the reference standard (42 osseous and 64 extraosseous lesions) from which 102 were detected by WB-MRI (sensitivity of 96%). All bone lesions were detected and extra-skeletal lesions were identified with a sensitivity of 93.8%. Overall 88 lesions detected by WB-MRI were not part of the reference standard from which 33 were lesions of the peripheral skeleton not imaged by conventional cross-sectional imaging studies. 4 lesions of the reference standard were not identified by WB-MRI which were all lymph nodes. CONCLUSION: WB-MRI is a radiation free imaging technique with high sensitivity for the detection of malignant disease spread in particular beneficial for children. In patients with suspected bone lesions it should be considered for initial disease evaluation prior to specific and regional imaging methods to reduce the overall number of imaging examinations and radiation exposure.

[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.

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.