Retroperitoneal fibrosis is still underdiagnosed entity with poor prognosis

Background: Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encircles abdominal organs including aorta and ureters. Data on the incidence of this disease are limited. Summary: The disease may be idiopathic or secondary to infections, malignancies, drugs or radiotherapy. Idiopathic form is an immune-mediated entity and a part of the broader spectrum of idiopathic diseases termed chronic periaortitis, characterized by a morphologically similar fibroinflammatory changes in aorta and surrounding tissues. Taking into account the dominant symptoms and clinical charac-teristics of patients with periaortitis, two subtypes of disease could be distinguished. Vascular subtype include patients with non-dilated aorta or with inflammatory abdominal aortic aneu-rysm, both with and without involvement of adjacent structures and with numerous risk factors for atherosclerosis. In renoureteral subtype obstructive uropathy manifesting with hydronephro-sis and acute kidney injury is predominant finding. Due to the variety of symptoms, diagnosis of RPF remains challenging, difficult and often delayed. A series of diagnostic tests should be performed, in order to confirm the diagnosis idiopathic RPF. Laboratory work-up include eval-uation of inflammatory indices and immunological studies. A biopsy and histopathological evaluation may be necessary to confirm diagnosis and differentiate the disease. Computed to-mography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET) are the modalities of choice for the diagnosis and follow-up of this disease. Management of ureteral obstruction, hydronephrosis, and aortic aneurysms often requires surgical evaluation and treatment. The pharmacological treatment of RPF has been evaluated in a few randomized trials and is mainly based on observational studies. Steroid therapy remains the gold standard of treatment. Key messages: Nowadays multidisciplinary team approach with clinical and diagnos-tic experience in both primary and secondary RPF as well as two major subtypes should be offered. Centers specialized in rare diseases with collaboration with other units and referral sys-tem yield the best possible outcomes.

The role of Ultrasound in Diagnosis of Acute Appendicitis: A Review Article

Acute appendicitis (AA) is a frequent abdominal infection that affects 7% of people at some time in their lives. As a result, any delay in diagnosis might lead to complications such as perforation and abscesses in the incision. Ultrasound (U/S) plays an essential role in the diagnosis and management of acute appendicitis, which improves patient outcomes. Recent technological advances have brought profound changes to diagnostic ultrasound imaging. Acute appendicitis is a frequent abdominal condition that affects many people at their lives acute appendicitis is a difficult diagnosis based on clinical and laboratory data. Abnormal US findings during appendiceal scan indicate a greater chance of having recurrent appendicitis, US scan play an important role in the pre-surgical evaluation and planning of patients with appendicitis. In this review, different US techniques and their applications in diagnosis of appendicitis and early and accurate diagnosis are discussed.

Imaging the Extent and Location of Spatiotemporally Distributed Epileptiform Sources from MEG Measurements

Non-invasive MEG/EEG source imaging provides valuable information about the epileptogenic brain areas which can be used to aid presurgical planning in focal epilepsy patients suffering from drug-resistant seizures. However, the source extent estimation for electrophysiological source imaging remains to be a challenge and is usually largely dependent on subjective choice. Our recently developed algorithm, fast spatiotemporal iteratively reweighted edge sparsity minimization (FAST-IRES) strategy, has been shown to objectively estimate extended sources from EEG recording, while it has not been applied to MEG recordings. In this work, through extensive numerical experiments and real data analysis in a group of focal drug-resistant epilepsy patients interictal spikes, we demonstrated the ability of FAST-IRES algorithm to image the location and extent of underlying epilepsy sources from MEG measurements. Our results indicate the merits of FAST-IRES in imaging the location and extent of epilepsy sources for pre-surgical evaluation from MEG measurements.

Understanding the Aortic Root Using Computed Tomographic Assessment: A Potential Pathway to Improved Customized Surgical Repair

There is continued interest in surgical repair of both the congenitally malformed aortic valve, and the valve with acquired dysfunction. Aortic valvar repair based on a geometric approach has demonstrated improved durability and outcomes. Such an approach requires a thorough comprehension of the complex 3-dimensional anatomy of both the normal and congenitally malformed aortic root. In this review, we provide an understanding of this anatomy based on the features that can accurately be revealed by contrast-enhanced computed tomographic imaging. We highlight the complimentary role that such imaging, with multiplanar reformatting and 3-dimensional reconstructions, can play in selection of patients, and subsequent presurgical planning for valvar repair. The technique compliments other established techniques for perioperative imaging, with echocardiography maintaining its central role in assessment, and enhances direct surgical evaluation. This additive morphological and functional information holds the potential for improving selection of patients, surgical planning, subsequent surgical repair, and hopefully the subsequent outcomes.

P.094 Referral Practices for Epilepsy Surgery in Pediatric Patients: A North American Study

Background: The International League Against Epilepsy recommends patients with drug resistant epilepsy (DRE) be referred for surgical evaluation, however prior literature suggests this is an underutilized intervention. This study captures practices of North American pediatric neurologists regarding the management of DRE and factors which may promote or limit referrals for epilepsy surgical evaluation. Methods: A REDCap survey distributed via the Child Neurology Society mailing list to pediatric neurologists practicing in North America. “R” was used to conduct data analyses. Ethics approval from the CHEO REB was granted prior to the start of data collection. Results: 102 pediatric neurologists responded, 77% of whom currently practice in the United States. 73% of respondents reported they would refer a patient for surgical consultation after two failed medications. Of all potential predictors tested in a logistic regression model, low referral volume was the only predictor of whether participants refer patients after more than three failed medications. Conclusions: Pediatric neurologists demonstrate fair knowledge of formal recommendations to refer patients for surgical evaluation after two failed medication trials. Other modifiable factors reported, especially family perceptions of epilepsy surgery, should be prioritized when developing tools to enhance effective referrals and increase utilization of epilepsy surgery in the management of pediatric DRE.