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The upper cervical spine remains the most susceptible region for spinal trauma in children, with odontoid fractures being the most common ones in this age group. Concomitant traumatic brain injuries or skull fractures can pose therapeutic challenges in such cases. We report a case of an 11-year-old boy who presented in the semiconscious state with a deep scalp laceration in the left frontoparietal region. A computed tomography (CT) scan revealed a depressed skull fracture requiring an emergency left frontoparietal decompressive craniectomy and an associated fracture of the odontoid process at the base with anterior displacement. Odontoid fractures usually heal well after immobilization, and the use of instrumented fusion is still debated in view of skeletal immaturity. The usual management of Halo vest could not be instituted in his case owing to the skull fracture, and a single cannulated screw fixation was done under fluoroscopic guidance. Direct operative fusion of the odontoid process has been described in younger children with apophyseal fractures, but the evidence of such procedures is rare in elder children with a fused odontoid process. This case report, thus, confirms the anterior odontoid screw fixation as an effective mode of treatment in children of the 7- to 12-year age group.

Objectives: We sought association of genetic variants in the renin-angiotensin system (RAS) and vitamin D system with acute pancreatitis (AP) development and severity. Background: The endocrine RAS is involved in circulatory homeostasis through the pressor action of angiotensin II at its AT1 receptor. However, local RAS regulate growth and inflammation in diverse cells and tissues, and their activity may be suppressed by vitamin D. Intrapancreatic angiotensin II generation has been implicated in the development of AP. Methods: Five hundred forty-four white patients with AP from 3 countries (United Kingdom, 22; Germany, 136; and The Netherlands 386) and 8487 control subjects (United Kingdom 7833, The Netherlands 717) were genotyped for 8 polymorphisms of the RAS/vitamin D systems, chosen on the basis of likely functionality. Results: The angiotensin-converting enzyme I (rather than D) allele was significantly associated with alcohol-related AP when all cohorts were combined (P = 0.03). The renin rs5707 G (rather than A) allele was associated with AP (P = 0.002), infected necrosis (P = 0.025) and mortality (P = 0.046). Conclusions: The association of 2 RAS polymorphisms with AP suggests the need for further detailed analysis of the role of RAS/vitamin D in the genesis or severity of AP, particularly given the ready potential for pharmacological manipulation of this system using existing marketed agents. However, further replication studies will be required before any such association is considered robust, particularly given the significant heterogeneity of AP causation and clinical course.

Status epilepticus care and treatment are already being touched by the revolution in data science. New approaches designed to leverage the tremendous potential of "big data" in the clinical sphere are enabling researchers and clinicians to extract information from sources such as administrative claims data, the electronic medical health record, and continuous physiologic monitoring data streams. Algorithmic methods of data extraction also offer potential to fuse multimodal data (including text-based documentation, imaging data, and time-series data) to improve patient assessment and stratification beyond the manual capabilities of individual physicians. Still, the potential of data science to impact the diagnosis, treatment, and minute-to-minute care of patients with status epilepticus is only starting to be appreciated. In this brief review, we discuss how data science is impacting the field and draw examples from the following three main areas: (1) analysis of insurance claims from large administrative datasets to evaluate the impact of continuous electroencephalogram (EEG) monitoring on clinical outcomes; (2) natural language processing of the electronic health record to find, classify, and stratify patients for prognostication and treatment; and (3) real-time systems for data analysis, data reduction, and multimodal data fusion to guide therapy in real time. While early, it is our hope that these examples will stimulate investigators to leverage data science, computer science, and engineering methods to improve the care and outcome of patients with status epilepticus and other neurological disorders. This article is part of the Special Issue "Proceedings of the 7th London-Innsbruck Colloquium on Status Epilepticus and Acute Seizures".

ObjectiveCurrent surveillance strategies for duodenal adenomatosis in familial adenomatous polyposis (FAP) miss malignancies and underestimate cancer risk in ampullary disease. This study aimed to evaluate the utility of endoscopic ultrasound (EUS) in the assessment of FAP patients with duodenal and/or ampullary polyposis referred for surgical intervention.DesignA retrospective analysis of FAP patients undergoing index EUS between December 2006 and May 2015 was performed. Follow-up was completed in January 2018, including review of all EUS procedures and surgical interventions (median follow-up 6 years).ResultsFifty-five patients underwent 188 EUS procedures. Six patients (11%) developed malignancy (three duodenal, three ampullary). Ampullary cancer risk was underestimated by Spigelman stage and overestimated by Kashiwagi classification. Ultrasound findings were poor predictors of malignancy, with common bile duct dilatation being the only finding present in one EUS prior to a diagnosis of ampullary cancer. The best predictors of ampullary malignancy were an ampullary polyp size >3 cm and an increase >1 cm in ampullary polyp size. Ampullary polyp size >3 cm provided the best predictive value, correctly identifying two of the three cases of ampullary cancer and both patients with high-grade dysplasia. EUS biopsy failed to detect malignancy later confirmed by surgical histology in two patients.ConclusionEUS surveillance confers little additional benefit to standard endoscopic surveillance in FAP patients. The best predictor of ampullary malignancy is an ampullary polyp >3 cm; this could be regarded as a relative indication for surgery.

Background: Port-site metastases (PSMs) are a rare occurrence after robotic surgery. For robot-assisted radical cystectomy (RARC), isolated cases have been reported but management has not been previously described. We present a case of PSM that occurred after RARC and report the results of our systematic review of previously reported PSMs and describe the treatment options. Search Criteria and Methods: We describe a case of a PSM in a 55-year-old man who had undergone intracorporeal RARC. We performed a systematic review of MEDLINE and Embase databases for previously reported PSMs, detailing the stage and grade of the primary tumor, time to presentation of PSM, treatment offered, and outcomes for the identified cases. Results: We identified 4 cases of PSMs after RARC in published studies and also included our case for analysis. All 5 patients had muscle-invasive bladder cancer at cystectomy (stage ≥ T2) and 3 had local lymph node-positive disease. Our aggressive treatment of chemotherapy, wide surgical excision of PSM, and radiotherapy provided our patient with a 2-year disease-free status. Conclusion: PSMs are a rare event in RARC, with only 4 other cases reported in published studies. The outcomes have not been well reported for these cases. We propose that multimodality treatment consisting of salvage chemotherapy, surgery, and radiotherapy should be considered, although concessions could be needed after consideration of patient factors.

Little is known about the clonal structure or stem cell architecture of the human small intestinal crypt/villus unit, or how mutations spread and become fixed. Using mitochondrial DNA (mtDNA) mutations as a marker of clonal expansion of stem cell progeny, we aimed to provide answers to these questions. Enzyme histochemistry (for cytochrome c oxidase and succinate dehydrogenase) was performed on frozen sections of normal human duodenum. Laser-capture microdissected cells were taken from crypts/villi. The entire mitochondrial genome was amplified using a nested PCR protocol; sequencing identified mutations and immunohistochemistry demonstrated specific cell lineages. Cytochrome c oxidase-deficient small bowel crypts were observed within all sections: negative crypts contained the same clonal mutation and all differentiated epithelial lineages were present, indicating a common stem cell origin. Mixed crypts were also detected, confirming the existence of multiple stem cells. We observed crypts where Paneth cells were positive but the rest of the crypt was deficient. We have demonstrated patches of deficient crypts that shared a common mutation, suggesting that they have divided by fission. We have shown that all cells within a small intestinal crypt are derived from one common stem cell. Partially-mutated crypts revealed some novel features of Paneth cell biology, suggesting that either they are long-lived or a committed Paneth cell-specific long-lived progenitor was present. We have demonstrated that mutations are fixed in the small bowel by fission and this has important implications for adenoma development.

A 52-year old man developed hyperkalaemia on the 11th postoperative day following an extensive open retroperitoneal liposarcoma resection that included splenectomy. Despite thorough investigations, no aetiology for the hyperkalaemia was identified and standard empirical treatment was ineffective. On reconsideration, in view of the patient's concurrent thrombocytosis, a pseudofactual or artefactual hyperkalaemia was suspected. This was confirmed by contemporaneous testing of serum and plasma potassium levels, with the latter value lying within the normal range. Treatment for hyperkalaemia was discontinued, thus averting an iatrogenic and potentially dangerous hypokalaemia. This case highlights pseudohyperkalaemia as an often-neglected cause of elevated serum potassium levels and discusses its association with thrombocytosis following splenectomy.

BACKGROUND: Patients with advanced, metastatic sarcoma have a poor prognosis, and the overall benefit from the few standard-of-care therapeutics available is small. The rarity of this tumor, combined with the wide range of subtypes, leads to difficulties in conducting clinical trials. The authors previously reported the outcome of patients with a variety of common solid tumors who received treatment with drug regimens that were first tested in patient-derived xenografts using a proprietary method ("TumorGrafts"). METHODS: Tumors resected from 29 patients with sarcoma were implanted into immunodeficient mice to identify drug targets and drugs for clinical use. The results of drug sensitivity testing in the TumorGrafts were used to personalize cancer treatment. RESULTS: Of 29 implanted tumors, 22 (76%) successfully engrafted, permitting the identification of treatment regimens for these patients. Although 6 patients died before the completion of TumorGraft testing, a correlation between TumorGraft results and clinical outcome was observed in 13 of 16 (81%) of the remaining individuals. No patients progressed during the TumorGraft-predicted therapy. CONCLUSIONS: The current data support the use of the personalized TumorGraft model as an investigational platform for therapeutic decision-making that can guide treatment for rare tumors such as sarcomas. A randomized phase 3 trial versus physician's choice is warranted.

Repair of incisional hernias is complex in the setting of previous/current infection, loss of domain and bowel involvement, and is often on the background of significant co-morbidities. Reported repair techniques are associated with significant morbidity and led our unit to develop a novel technique for complex incisional hernia repair.A retrospective case notes review of all high-risk (Ventral Hernia Working Group grade 2-4) incisional hernia repairs was undertaken. Standardized repair involved resection of attenuated soft tissue and hernia sac (bioburden reduction), component separation (where necessary), intra-peritoneal Strattice™ biological mesh insertion, midline fascial closure, and soft-tissue reconstruction, performed in combination with a plastic surgeon as a single-stage procedure.A total of 58 patients underwent hernia repair between February 2009 and September 2012 (median age 59 years; 59 % female). Eleven patients (19 %) were grade 4, 19 (33 %) were grade 3, and 28 (48 %) were grade 2. Nineteen (33 %) were recurrent hernias, and midline fascial closure was achieved in 52 (90 %). Early complications included 15 (26 %) surgical-site occurrences, three (5 %) respiratory complications, two (3 %) cardiac complications, and two (3 %) urinary tract infections. Follow-up has revealed three (5 %) asymptomatic hernia recurrences and no patients requiring mesh explantation.This technique was associated with a low risk of surgical site occurrences and hernia recurrence, with no requirements for mesh explantation. Repair of such complex incisional hernias remains challenging, and further randomized controlled trials are required to elucidate the optimal method of closure and mesh type.

Introduction We describe a case of metallic, angiographic coil migration, following radiological exclusion of a gastroduodenal artery pseudoaneurysm secondary to chronic pancreatitis. Patients and Methods A 55-year-old man presented to the out-patient clinic with chronic, intermittent, post-prandial, abdominal pain, associated with nausea, vomiting and weight loss. He was known to have chronic pancreatitis and liver disease secondary to alcohol abuse and previously underwent angiographic exclusion of a gastroduodenal artery pseudoaneurysm. During subsequent radiological and endoscopic investigation, an endovascular coil was discovered in the gastric pylorus, associated with ulceration and cavitation. This patient was managed conservatively and enterally fed via naso-jejunal catheter endoscopically placed past the site of the migrated coil. This patient is currently awaiting biliary bypass surgery for chronic pancreatitis, and definitive coil removal will occur concurrently. Conclusions Literature review reveals that this report is only the eighth to describe coil migration following embolisation of a visceral artery pseudoaneurysm or aneurysm. Endovascular embolisation of pseudoaneurysms and aneurysms is generally safe and effective. More common complications of visceral artery embolisation include rebleeding, pseudoaneurysm reformation and pancreatitis.

Background: Patients with familial adenomatous polyposis (FAP) develop duodenal and ampullary polyps that may progress to malignancy via the adenoma-carcinoma sequence. Objective: The aim of this study was to review a large series of FAP patients undergoing pancreaticoduodenectomy for advanced duodenal and ampullary polyposis. Methods: A retrospective case notes review of all FAP patients undergoing pancreaticoduodenectomy for advanced duodenal and ampullary adenomatosis was performed. Results: Between October 1993 and January 2010, 38 FAP patients underwent pancreaticoduodenectomy for advanced duodenal and ampullary polyps. Complications occurred in 29 patients and perioperative mortality in two. Postoperative histology revealed five patients to have preoperatively undetected cancer (R = 0.518, P < 0.001). Conclusions: Pancreaticoduodenectomy in FAP is associated with significant morbidity, but low mortality. All patients under consideration for operative intervention require careful preoperative counselling and optimization.

We present the case of a 45-year-old man, who presented to his local casualty department with severe epigastric pain following an alcohol binge, and was subsequently diagnosed with acute pancreatitis. Pancreatic necrosis with multiple collections ensued, necessitating transfer to an intensive care unit (ITU) in a tertiary hepatopancreaticobiliary centre. Initially, the patient appeared to slowly improve and was discharged to the ward, albeit following a prolonged ITU admission. However, during his subsequent recovery, he suffered multiple episodes of haematemesis and melaena associated with haemodynamic instability and requiring repeat admission to the ITU. Computerised tomographic angiography, followed by visceral angiography, was used to confirm the diagnosis of multisite visceral artery pseudoaneurysms, secondary to severe, necrotising pancreatitis. Pseudoaneurysms of the splenic, left colic and gastroduodenal arteries were sequentially, and successfully, radiologically embolised over a period of 9 days. Subsequent sequelae of radiological embolisation included a clinically insignificant splenic infarct, and a left colonic infarction associated with subsequent enterocutaneous fistula formation. The patient made a prolonged, but successful, recovery and was discharged from hospital after 260 days as an in-patient. This case illustrates the rare complication of three separate pseudoaneurysms, secondary to acute pancreatitis, successfully managed radiologically in the same patient. This case also highlights the necessity for multidisciplinary involvement in the management of pseudoaneurysms, an approach that is often most successfully achieved in a tertiary setting.