OBJECTIVES: Congenital choanal atresia is a complete obliteration of the posterior nasal aperture leading to life-threatening airway emergencies. Several surgical options including sublabial, transpalatal, transseptal or external approaches have been developed for the repair of choanal atresia. So far, no gold standard has been established, but transnasal endoscopic approaches have been favored by many surgeons in recent years. METHODS: Since 2008 a standard procedure for bilateral choanal atresia repair in neonates using an endoscopic transnasal approach supported by balloon dilatation has been established at the Department of Otorhinolaryngology at Ulm University Medical Center. During the last five years, six cases of bilateral choanal atresia were diagnosed and treated, including two male and four female patients aged between three days and two months, at the date of surgery. All interventions were performed in transnasal endoscopic technique. In all patients the abnormally thick posterior vomer and the atretic bony plate were resected and the mucosa was perforated. A balloon dilator was used to dilate the neochoanae and prevent restenosis. All six patients were intraoperatively stented for at least six weeks. RESULTS: All six neonates with bilateral choanal atresia, who were operated in endoscopic transnasal technique had patent neo-choanae on both sides. No severe postoperative complications were found. The number of revisions depends on the age at primary surgery. CONCLUSIONS: Endonasal endoscopic approach and balloon dilatation is a safe, reproducible technique for surgical repair of choanal atresia. We recommend the use of bilateral stents, especially in very young patients, as a prerequisite to prevent early restenosis.

Over the past 15 years, Clostridium difficile infection (CDI) in patients with inflammatory bowel disease (IBD) has increased both in incidence and severity. Traditional risk factors for CDI are similar in IBD and non-IBD populations, but there is a significant proportion of IBD patients which have distinctive characteristics. Patients with ulcerative colitis (UC) are more susceptible to CDI and have more severe outcomes than those with Crohn's disease (CD). CDI may be difficult to distinguish from an IBD flare due to similar clinical presentation, and therefore screening for CDI is recommended at every flare in such patients. Several studies showed worse clinical outcomes in IBD patients with CDI, including longer hospital stay, higher colectomy and mortality rates than in those without CDI. Vancomycin and metronidazole appear to have similar efficacy in patients with moderate disease, but vancomycin is preferred in severe disease. Measures must be taken to prevent the spread of infection. Clinicians should have a high index of suspicion for CDI when evaluating a patient with IBD flare, as rapid detection and prompt treatment of infection improve outcomes. This review summarizes the available literature on epidemiology, risk factors, clinical aspects, diagnostic methods, treatment, outcome, and prevention of CDI in IBD patients.

OBJECT: There is increasing evidence that simulation provides high-quality, time-effective training in an era of resident duty-hour restrictions. Simulation may also permit trainees to acquire key skills in a safe environment, important in a specialty such as neurosurgery, where technical error can result in devastating consequences. The authors systematically reviewed the application of simulation within neurosurgical training and explored the state of the art in simulation within this specialty. To their knowledge this is the first systematic review published on this topic to date. METHODS: The authors searched the Ovid MEDLINE, Embase, and PsycINFO databases and identified 4101 articles; 195 abstracts were screened by 2 authors for inclusion. The authors reviewed data on study population, study design and setting, outcome measures, key findings, and limitations. RESULTS: Twenty-eight articles formed the basis of this systematic review. Several different simulators are at the neurosurgeon's disposal, including those for ventriculostomy, neuroendoscopic procedures, and spinal surgery, with evidence for improved performance in a range of procedures. Feedback from participants has generally been favorable. However, study quality was found to be poor overall, with many studies hampered by nonrandomized design, presenting normal rather than abnormal anatomy, lack of control groups and long-term follow-up, poor study reporting, lack of evidence of improved simulator performance translating into clinical benefit, and poor reliability and validity evidence. The mean Medical Education Research Study Quality Instrument score of included studies was 9.21 ± 1.95 (± SD) out of a possible score of 18. CONCLUSIONS: The authors demonstrate qualitative and quantitative benefits of a range of neurosurgical simulators but find significant shortfalls in methodology and design. Future studies should seek to improve study design and reporting, and provide long-term follow-up data on simulated and ideally patient outcomes.

Background: Pediatric Anti-Phospholipid Syndrome (APS) is the most commonly acquired state of hypercoagulability in children, and is defined by venous and/or arterial thrombosis in the presence of circulating anti-phospholipid antibodies (aPLs) (most commonly lupus anticoagulant (LA), anticardiolipin antibody (aCL) and anti-β2-glycoprotein antibody (aβ2GP)). Catastrophic APS (CAPS) is a rare manifestation of APS, with thrombosis of multiple vessels that can lead to multi-system organ failure. Purpose: To determine the thromboses outcomes and factors predisposing to the risk of thrombotic recurrence in pediatric patients with a diagnosis of APS. Methods: Retrospective review of patients between the ages of 0-21 years diagnosed and treated with APS from 1997-2013 at our institution. Clinical data and long-term outcomes of these patients were reviewed. Exclusion criteria included neonatal APS and thrombosis with fetal loss. Statistical tests were performed using JMP software (© 2014 SAS Institute Inc., North Carolina, US). Associations between categorical variables were tested using chi-square test or Fisher's exact test when required. Differences were considered statistically significant if p<0.05. Results: The study identified 23 patients (12 female; 11 male). Three patients with CAPS were identified and excluded from the analysis (3 female). Gender distribution for primary and secondary APS was similar (5 female/6 male primary APS; 4 female/5 male secondary APS). The median follow up period was 3.2 years (range; 0.01 - 16.87 years ). Median age at diagnosis of first episode of thrombosis was 16.16 years (range; 6.05 - 20.6 years). Nine patients (45%) with secondary APS had underlying autoimmune disease or malignancy (7 systemic lupus erythematosus, 1 ulcerative colitis, 1 Non-Hodgkin Lymphoma) and the remaining 11 patients were diagnosed as primary APS. Arterial and venous thrombosis occurred as the first thrombotic event in 7 (35%) and 13 (65%) patients, respectively. Lower extremity deep vein thrombosis (DVT) was the most frequent thrombotic event 11/20 (55%), followed by pulmonary embolism 7/20 (35%), ischemic stroke 4/20 (20%), lower extremity arterial thrombosis 3/20 (15%), upper extremity DVT 2/20 (10%), and IVC thrombosis 2/20 (10%). Recurrent or progressive thrombotic events occurred in 12/20 patients (60%). Of those, 7 were venous (58%), 5 were arterial (42%). At the time of recurrence, 3 patients were fully anticoagulated, (INR 2.5-3.5) versus 9 who were subtherapeutic (INR<2.0). Recurrent/progression thrombotic events occurred at the original site of thrombosis in 8 patients (3 arterial, 5 venous) while the remaining 4 recurrent thrombotic events occurred at other sites than the primary site. Overall venous and arterial thrombotic events were no different among primary or secondary APS (p=0.88). Thrombosis recurrence/progression risk at the original site or distant site was no different among primary or secondary APS (p=0.22 and p=0.30 respectively). Risk of recurrence/progression was not associated with subtherapeutic levels of anticoagulation (p=0.08), or presence of residual thrombosis at primary site at the time of recurrence (p=0.48). Conclusion: Recurrence and/or progression of thrombosis in APS occurred in 60% of the patients in our cohort, with 25% of the thrombosis recurrences occurring despite documented therapeutic anticoagulation in these patients. In contrast to other studies, our APS cohort demonstrated a higher rate of recurrence with other groups reporting thrombosis rates of recurrence such as 19-25%. Our cohort demonstrated similar rates of primary and secondary APS among female patients, in contrast to other studies suggesting higher rates of secondary APS in females. Potential risk factors such as primary versus secondary APS, subtherapeutic anticoagulation, and residual thrombosis from primary thrombotic event were not found as significant risk factors for thrombosis recurrence. Larger pediatric APS studies are needed in order to address other potential risk factors for thrombosis (ie obesity, inherited thrombophilia, intensity of anticoagulation, immune suppression approach for secondary APS), in order to establish treatment strategies for the prevention of recurrent thrombosis.

In recent years, the United States has experienced record-breaking summer heat. Climate change models forecast increasing US temperatures and more frequent heat wave events in the coming years. Exposure to environmental heat is a significant, but overlooked, workplace hazard that has not been well-characterized or studied. The working population is diverse; job function, age, fitness level, and risk factors to heat-related illnesses vary. Yet few studies have examined or characterized the incidence of occupational heat-related morbidity and mortality. There are no federal regulatory standards to protect workers from environmental heat exposure. With climate change as a driver for adaptation and prevention of heat disorders, crafting policy to characterize and prevent occupational heat stress for both indoor and outdoor workers is increasingly sensible, practical, and imperative.

Multiple myeloma (MM) is the second most common hematological malignancy, with an annual incidence in Europe and the USA of about 4-6 cases per 100,000. Several forms of renal disease are found in the course of MM, including: cast nephropathy, light chain (LC) deposition disease and primary amyloidosis. Less frequent forms include: acute and chronic tubulopathies, neoplastic plasma cell infiltration and interstitial nephritis. In this paper, we discuss a case of 53-year-old male patient with MM who presented with massive proteinuria (24 g/24 h), mild renal insufficiency (eGFR 43 mL/min), and Fanconi-like syndrome (as reflected by normoglycemic glycosuria). In kidney biopsy glomeruli were normal, whereas abundant AFOG-positive deposits were found in the cytoplasm of proximal tubular epithelial cells. These deposits were strongly positive for kappa light chains on immunofluorescence. Electron microscopy revealed electron-dense, intracytoplasmic crystalloid deposits of variable shape (needle-shaped, round and rectangular), and size in the proximal tubular cells. This unusual variant of microscopic renal lesions in the course of MM coupled with coincidence of Fanconi-like and nephrotic syndrome as a clinical manifestation has not been reported to date.

Rib fracture is the most common thoracic injury, present in 10% of all traumatic injuries and almost 40% of patients who sustain severe nonpenetrating trauma. Although rib fractures can produce significant morbidity, the diagnosis of associated complications (such as pneumothorax, hemothorax, pulmonary contusion, atelectasis, flail chest, cardiovascular injury, and injuries to solid and hollow abdominal organs) may have a more significant clinical impact. When isolated, rib fractures have a relatively low morbidity and mortality, and failure to detect isolated rib fractures does not necessarily alter patient management or outcome in uncomplicated cases. A standard posteroanterior chest radiograph should be the initial, and often the only, imaging test required in patients with suspected rib fracture after minor trauma. Detailed radiographs of the ribs rarely add additional information that would change treatment, and, although other imaging tests (eg, computed tomography, bone scan) have increased sensitivity for detection of rib fractures, there are little data to support their use. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every 3 years by a multidisciplinary expert panel. The guideline development and review process include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.

Imaging is paramount in the setting of blunt trauma and is now the standard of care at any trauma center. Although anteroposterior radiography has inherent limitations, the ability to acquire a radiograph in the trauma bay with little interruption in clinical survey, monitoring, and treatment, as well as radiography's accepted role in screening for traumatic aortic injury, supports the routine use of chest radiography. Chest CT or CT angiography is the gold-standard routine imaging modality for detecting thoracic injuries caused by blunt trauma. There is disagreement on whether routine chest CT is necessary in all patients with histories of blunt trauma. Ultimately, the frequency and timing of CT chest imaging should be site specific and should depend on the local resources of the trauma center as well as patient status. Ultrasound may be beneficial in the detection of pneumothorax, hemothorax, and pericardial hemorrhage; transesophageal echocardiography is a first-line imaging tool in the setting of suspected cardiac injury. In the blunt trauma setting, MRI and nuclear medicine likely play no role in the acute setting, although these modalities may be helpful as problem-solving tools after initial assessment. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every 2 years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.

Given the upsurge of research in posttraumatic stress disorder (PTSD) and traumatic brain injury (TBI), much of which has focused on military samples who served in Iraq and Afghanistan, the purpose of this article is to review the literature published after September 11th, 2001 that addresses the epidemiology, pathophysiology, evaluation, and treatment of PTSD in the context of TBI.

OBJECTIVES: Evidence-based traumatic brain injury guidelines support cerebral perfusion pressure thresholds for adults at a class 2 level, but evidence is lacking in younger patients. The purpose of this study is to identify the impact of age-specific cerebral perfusion pressure thresholds on short-term survival among patients with severe traumatic brain injury. DESIGN: Institutional review board-approved, prospective, observational cohort study. SETTING: Level I or II trauma centers in New York State. PATIENTS: Data on all patients with a postresuscitation Glasgow Coma Score less than 9 were added in the Brain Trauma Foundation prospective New York State TBI-trac database. MEASUREMENTS AND MAIN RESULTS: We calculated the survival rates and relative risks of mortality for patients with severe traumatic brain injury based on predefined age-specific cerebral perfusion pressure thresholds. A higher threshold and a lower threshold were defined for each age group: 60 and 50 mm Hg for 12 years old or older, 50 and 35 mm Hg for 6-11 years, and 40 and 30 mm Hg for 0-5 years. Patients were stratified into age groups of 0-11, 12-17, and 18 years old or older. Three exclusive groups of CPP-L (events below low cerebral perfusion pressure threshold), CPP-B (events between high and low cerebral perfusion pressure thresholds), and CPP-H (events above high cerebral perfusion pressure threshold) were defined. As an internal control, we evaluated the associations between cerebral perfusion pressure events and events of hypotension and elevated intracranial pressure. Survival was significantly higher in 0-11 and 18 years old or older age groups for patients with CPP-H events compared with those with CPP-L events. There was a significant decrease in survival with prolonged exposure to CPP-B events for the 0-11 and 18 years old and older age groups when compared with the patients with CPP-H events (p = 0.0001 and p = 0.042, respectively). There was also a significant decrease in survival with prolonged exposure to CPP-L events in all age groups compared with the patients with CPP-H events (p< 0.0001 for 0- to 11-yr olds, p = 0.0240 for 12- to 17-yr olds, and p < 0.0001 for 18-yr old and older age groups). The 12- to 17-year olds had a significantly higher likelihood of survival compared with adults with prolonged exposure to CPP-L events (< 50 mm Hg). CPP-L events were significantly related to systemic hypotension for the 12- to 17-year-old group (p = 0.004) and the 18-year-old and older group (p < 0.0001). CPP-B events were significantly related to systemic hypotension in the 0- to 11-year-old group (p = 0.014). CPP-B and CPP-L events were significantly related to elevated intracranial pressure in all age groups. CONCLUSIONS: Our data provide new evidence that cerebral perfusion pressure targets should be age specific. Furthermore, cerebral perfusion pressure goals above 50 or 60 mm Hg in adults, above 50 mm Hg in 6- to 17-year olds, and above 40 mm Hg in 0- to 5-year olds seem to be appropriate targets for treatment-based studies. Systemic hypotension had an inconsistent relationship to events of low cerebral perfusion pressure, whereas elevated intracranial pressure was significantly related to all low cerebral perfusion pressure events across all age groups. This may impart a clinically important difference in care, highlighting the necessity of controlling intracranial pressure at all times, while targeting systolic blood pressure in specific instances.