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BACKGROUND Chin-on-chest kyphotic cervical deformity can be debilitating. Surgical deformity correction serves to decompress neural elements and restore lordosis. This can be achieved through multiple osteotomies with instrumentation and fusion, sometimes requiring a staged approach. Such procedures carry a high risk of neurological injury. Here we present examples of transient neurological dysfunction not previously reported in the common literature. CASE DESCRIPTION The authors present three patients who underwent extreme cervical deformity correction for chin-on-chest deformity. Deformity correction in all cases was obtained through multiple osteotomies with multi-level cervicothoracic posterior instrumentation and arthrodesis. selleck chemical On postoperative examination, all three patients developed transient ataxia, dysmetria, and decreased proprioception in all four extremities - exam findings consistent with dorsal column dysfunction. All symptoms resolved within two to three weeks post-operatively. CONCLUSION Incomplete spinal cord syndromes such as posterior cord syndrome can be caused by compression or stretching of the ascending dorsal spinal tracts. Considering the large degree of correction obtained, we hypothesize the resulting shortening of the dorsal columns as the pathomechanism. Providers should be aware and patients should be counseled pre-operatively that these symptoms may occur. If these symptoms are present postoperatively, appropriate diligence is warranted with the understanding that these deficits may be transient. INTRODUCTION Neurosurgical training usually requires long hours on hands-on procedures, making it difficult for inexperienced surgeons to quickly learn in an error-proof environment. OBJECTIVE To propose a puzzle-like new model for neurosurgical education, that simulates craniosynostosis correction (scaphocephaly type) using Renier's H technique. A model of a 3D anatomical simulator for craniosynostosis training will be presented and evaluated. METHODS The cranial model was created using 1-mm CT scan images from patients with scaphocephaly in the DICOM format. This information was processed using an algorithm to generate a three-dimensional (3D) bio model in resin. The puzzle model and its variable training models were assessed qualitatively by a team of expert neurosurgeons. Next, the model was applied in trainees and was evaluated using specific questionnaires. RESULTS Experts and trainees evaluated the model. The mean of attempts without errors was 2.3 (SD0.675), for one error was 2.2 (SD0.918) and for two errors 1.3 (SD 0.707). The mean of the score of the simulator was 9.2 (SD0.421). Twelve residents (second evaluation) answered the questionnaire with a positive assessment of diagnosis capabilities, appropriateness of the model, time commitment, adequate environment, reliable 3D reconstruction and teaching method. Three participants have ever used a 3D simulator previously and the simulator was evaluated obtaining 9.9 final average (0-10 graduation). CONCLUSION The puzzle may be a complementary tool for surgical training. It allows several degrees of immersion and realism, offering symbolic, geometric and dynamic information with 3D visualization. It provides additional data to support the practice of complex surgical procedures without exposing real patients to undue risk. BACKGROUND Cervical myelomeningocele (MMC) is a very rare type of neural type defect that is usually discovered and managed in childhood. It is best described as a closed type of spinal dysraphism, where the posterior portion of the cervical thecal sac forms a pouch that bulges out through a narrow posterior spina bifida and contains spinal neural tissue with or without cerebrospinal fluid (CSF). CASE DESCRIPTION We report a 47-year-old male patient who presented with neck pain and decreased ability to use his fingers that has progressed over 3 years prior to presentation. Cervical spine MRI revealed a posterior bulge between the spinous processes of C4 and C6, absence of the spinous process of C5, and presence of CSF and spinal cord tissue and nerve roots within the bulging sac, suggestive of MMC. Simple untethering of the cord tissue was sufficient to halt the progression and allow for improvement in neurological deficits. CONCLUSION Cervical MMC is extremely rare in adults, the symptomatic progression of which is most likely due to cord tethering by fibrotic tissue formation over years. Early surgical correction and release of the tethered cord is relatively safe and prevents the evolution of neurological symptoms. OBJECTIVE Simulation models enable trainees to master microsurgical skills before performing surgeries. Vascular bypass is a critical component of cerebrovascular and many non-neurological procedures. However, most available bypass training models lack important spatial, tactile, and physiologic aspects of real surgery. Animal and placental models provide true physiology, but are expensive. While some models adequately simulate superficial temporal artery-middle cerebral artery bypass, there is no model for side-to-side distal anterior cerebral artery bypass. The objective is to create a realistic and inexpensive training model for this important procedure. METHODS The depth of interhemispheric fissures in cadaver brains were compared to the grapefruit radii. Grapefruits were dissected to simulate the operative field within the deep and narrow interhemispheric fissure. Pericallosal arteries were mimicked with chicken wing vessels or synthetic tubing, with an aquarium pump providing closed circulation. Twelve board-certified neurosurgeons who were given bypass training using the grapefruit model were blindly surveyed on model realism and training suitability. RESULTS Grapefruit depths from pith to central column were comparable to interhemispheric cadaveric fissure depths. Approximate preparation time of grapefruit training models was 5-10 minutes. Surveyed neurosurgeons rated the model a better replicate for cerebral artery bypass (p less then 0.02), and more challenging than common training models (p less then 0.01). They also rated the grapefruit model as likely to be superior for improving surgical skills prior to surgery (p less then 0.05). CONCLUSION This grapefruit model provides a realistic simulation of side-to-side distal anterior cerebral artery bypass procedure that can be inexpensively and easily implemented in nearly any resource environment. BACKGROUND & AIMS A subset of patients with Crohn's disease (CD) do not respond to ustekinumab at the standard dose of 90 mg every 8 weeks. Little is known about the efficacy of shortening the interval between doses. METHODS We performed a retrospective study to determine the effectiveness of ustekinumab dose interval shortening, collecting data from 506 patients with CD who received subcutaneous ustekinumab 90 mg every 8 weeks at a single center. We obtained data from 110 patients who initially received subcutaneous ustekinumab 90 mg every 8 weeks and then had their interval shortened to every 4 weeks. Harvey Bradshaw Index (HBI) scores before and after the dose interval shortening was available for 78 patients in the cohort (71%), levels of C-reactive protein (CRP) for 60 patients (55%), and levels of fecal calprotectin for 8 patients (7%). RESULTS Following dose interval shortening, the patients' median HBI decreased from 4.5 to 3 (P=.002), the median level of CRP decreased from 8 mg/l to 3 mg/l (P=.031), and median level of fecal calprotectin decreased from 378 μg/g to 157 μg/g (P=.57). Among patients who had an HBI >4, a level of CRP ≥5mg/dl, a level of fecal calprotectin >250ug/g, or endoscopic evidence for disease activity before dose interval shortening, after the dose interval was shortened, 28% achieved clinical remission (an HBI score ≤4), 22% had a normal level of CRP ( less then 5 mg/dl), 50% had reduced levels of fecal calprotectin, and 36% achieved endoscopic remission. CONCLUSIONS Shortening the ustekinumab 90 mg dose interval to 4 weeks for patients with CD who did not respond to doses every 8 weeks improved clinical and biological indices of disease activity. Patients who lose response to the standard dose of ustekinumab might benefit from dose interval shortening, which was effective and safe. BACKGROUND & AIMS Individuals with hypermobility spectrum disorder or hypermobile Ehlers-Danlos Syndrome (HSD/hEDS) are increasingly encountered by gastroenterologists and pose complex clinical challenges. Uncontrolled studies have found functional gastrointestinal disorders (FGIDs) to be common in patients with HSD/hEDS. Some patients have somatic symptoms (medically unexplained symptoms) that might affect FGIDs. We performed a case-control study to determine the prevalence of and factors associated with Rome IV FGIDs in subjects with HSD/hEDS compared with age- and sex- matched population-based controls. METHODS An online general health survey was completed by 603 individuals with HSD/hEDS in October 2018 (cases) and 603 matched individuals from the population of the United Kingdom (controls) in 2015. The mean participant age was 39 yrs, and 96% were women. The survey included questions about Rome IV FGIDs, non-GI and non-musculoskeletal somatic symptoms (maximum number, 10), quality of life, medical histor including abdominal surgeries and medication use (for example, 84% used analgesics compared with 29% of controls). Almost 40% of subjects with HSD/hEDS reported a diagnosis of chronic fatigue syndrome and/or fibromyalgia. Following adjustments for somatic symptoms, the association for FGIDs in subjects with HSD/hEDS was reduced by as much as 4-fold and in some instances was eliminated. CONCLUSIONS In a large case-control study of persons with HSD/hEDS, almost all of the cases met criteria for Rome IV FGIDs, incurred considerable health impairment, and had high healthcare use. Patients with HSD/hEDS frequently have somatic symptoms that should be treated to reduce the high burden of gastrointestinal illness in this population. BACKGROUND & AIMS Portal vein thrombosis (PVT) is a common and serious complication in patients with cirrhosis. However, little is known about PVT in patients with cirrhosis and acute decompensation (AD). We investigated the prevalence and clinical significance of PVT in nonmalignant patients with cirrhosis and AD. METHODS We performed a retrospective study of 2 cohorts of patients with acute exacerbation of chronic liver disease who participated in the CATCH-LIFE study, established by the Chinese Chronic Liver Failure Consortium, from January 2015 through December 2016 (n=2600 patients) and July 2018 through January 2019 (n=1370 patients). We analyzed data on the prevalence, clinical manifestations, and risk factors of PVT from 2826 patients with cirrhosis, with and without AD. RESULTS The prevalence of PVT in patients with cirrhosis and AD was 9.36%, which was significantly higher than in patients with cirrhosis without AD (5.24%) (P=.04). Among patients with cirrhosis and AD, 63.37% developed PVT recently (the first detected PVT with no indication of chronic PVT). Compared to patients without PVT, a significantly higher proportion of patients with PVT had variceal bleeding (47.33% vs 19.63%; P less then .001) and patients with PVT had a significantly higher median serum level of D-dimer (2.07 vs 1.25; P less then .001). Splenectomy and endoscopic sclerotherapy were independent risk factors for PVT in patients with cirrhosis and AD. The 1-year mortality rate did not differ significantly between patients with vs without PVT. CONCLUSIONS In an analysis of data from 2826 patients with cirrhosis, a significantly higher proportion of those with AD had PVT than those without AD. PVT was associated with increased variceal bleeding, which would increase the risk for AD. Strategies are needed to prevent PVT in patients with cirrhosis, through regular screening, to reduce portal hypertension.

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