Intrahepatic cholangiocarcinoma in a non-cirrhotic liver in a patient with homozygous ZZ alpha-1 antitrypsin deficiency

Alpha-1 antitrypsin (AAT) deficiency, which is an under-recognised metabolic genetic disorder, is known to cause severe lung disease and liver cirrhosis in about 10%-15% of cases. Patients with AAT deficiency are at a higher risk for developing hepatocellular carcinoma, both in cirrhotic and in non-cirrhotic livers. In this case report, a 48-year-old woman with homozygous ZZ AAT deficiency presented with abdominal pain, and by imaging, an abnormal area in the liver was found. The initial differential diagnosis consisted of benign abnormalities but a malignancy could not be ruled out. Finally, this abnormality turned out to be an intrahepatic cholangiocarcinoma (iCCA) in a non-cirrhotic liver. Since this type of tumour has been very infrequently described to be associated with AAT deficiency, the question remains whether alpha-1 trypsin accumulation in the hepatocytes was responsible for the development of iCCA. However, other associated factors for developing an iCCA were ruled out

Surgery for Ampullary Cancer in a Patient with Pancreatic Lipomatosis Caused by Cystic Fibrosis

A patient with cystic fibrosis (CF) with pancreatic insufficiency presented with jaundice due to an ampullary tumour. CF is known for a higher incidence of gastrointestinal malignancies. The patient suffered from pancreatic insufficiency. At computed tomography (CT), pancreatic lipomatosis with absence of the pancreatic duct was seen. This is uncommon, also in patients with CF. During surgery, a total pancreatectomy was performed, because there was no possibility to construct a duct to mucosa anastomosis due to the absence of the pancreatic duct and more importantly the pancreas was already afunctional. The presence of lipomatosis increases the risk of leakage at the pancreaticojejunal anastomosis. Therefore, it is important to take this phenomenon, in this case already visible on the preoperative CT scan, into account during the workup for surgery

Ankle Ligament Injuries

This chapter presents a clinical scenario of a 23-year-old male athlete who had suffered an inversion injury during a cutting movement while trying to pass an opponent. It also focuses on lateral ankle sprains (LAS). Many patients who present to the Emergency Department after sustaining an LAS mainly suffer from pain and are unable to bear weight on the affected ankle. Patients that present to the Emergency Department are often unable to bear weight on their affected ankle. To provide some stability, immobilization by means of a cast may be chosen as an acute treatment method. In patients that suffer from a severe lateral ligament injury, only elite athletes generally qualify for surgical treatment to enable quick recovery. The chapter then provides recommendations for implementing evidence-based practice in the clinical setting

Weight, BMI and stability are risk factors associated with lateral ankle sprains and chronic ankle instability: A meta-analysis

Importance Lateral ankle sprains (LAS) are common in the general population and may lead to chronic ankle instability (CAI). If patients at risk could be identified, they could receive adequate and on-time treatment. Objective The purpose of the current review was to identify all reported intrinsic factors associated with sustaining a LAS or progressing to CAI after an initial sprain. Evidence review PubMed, Embase, MEDline, Cochrane and PEDro were searched for studies published until July 2019. Articles were selected if they included intrinsic factors related to LAS or CAI, subjects of at least 16 years old, and contained a minimum of 10 patients and 10 controls. Studies were excluded if they concerned reviews or case reports, included patients with previous surgical interventions, concomitant injuries or joint pathology other than ankle instability. Quality of included studies was assessed using the Quality in Prognostic Studies tool and quality of evidence was assessed using the GRADEpro tool. In case outcomes were described by at least three studies, data were pooled and assessed by performing a meta-analysis. Based on the pooled data, either a fixed-effects model or random-effects model was selected to correct for the degree of heterogeneity. Findings The search resulted in a total of 4154 studies. After title and abstract screening and subsequent full-text screening, 80 relevant studies were included. Results of the meta-analyses indicated that, compared with healthy controls, patients with LAS had a higher mean body mass index (BMI). In patients with CAI, a higher weight and a longer time to stabilise after performing a task (eg, jumping) were found compared with healthy controls. Other outcomes could not be compared using a meta-analysis due to heterogeneity in outcome measurement and the great number of different outcomes reported. Identification of the risk factors when patients present themselves after a LAS may help to determine which patients are at risk of recurrent sprains or developing CAI. Conclusions and relevance Based on the findings in this review, a higher BMI, and a higher weight and neuromuscular stability deficits may be regarded risk factors for sustaining a LAS or developing CAI, respectively. Level of evidence III

Clinical efficacy of the Ankle Spacer for the treatment of multiple secondary osteochondral lesions of the talus

BACKGROUND The Ankle Spacer was developed as a joint-sparing alternative to invasive endstage surgeries. Currently, there are no clinical studies on the Ankle Spacer. AIM To describe the operative technique and the clinical efficacy of the Ankle Spacer for the treatment of multiple, cystic osteochondral lesions of the talus in patients with failed prior operative treatment. METHODS This is a prospective study during which patients were assessed preoperatively, at 2- and 6 wk, and at 3, 6, 12 and 24 mo postoperatively. Patients with multiple, cystic or large (≥ 15 mm) osteochondral lesions of the talus after failed prior surgery were included. The primary outcome measure was the numeric rating scale (NRS) for pain during walking at 2 years postoperatively. Secondary outcome measures included the NRS in rest and during stair climbing, the American Orthopaedic Foot and Ankle Society Hindfoot Score, the Foot and Ankle Outcome Score, the Short- Form 36 physical and mental component scale, and the Range of Motion (ROM). Radiographic evaluations were conducted to evaluate prosthetic loosening and subsidence. Revision rates and complications were also assessed. RESULTS Two patients underwent an Ankle Spacer implantation on the talus. The NRS during walking improved from 6 and 7 preoperatively to 2 and 2 points postoperatively at 2 years, in patient 1 and 2, respectively. The other patient-reported outcome measures also improved substantially. There were no re-operations nor complications. Radiological imaging showed no loosening of the implant and no change of implant position. CONCLUSION The Ankle Spacer showed clinically relevant pain reduction during walking, improvement in clinical outcomes as assessed with PROMs, and no complications or re-operations. This treatment option may evolve as a joint-sparing alternative to invasive end-stage surgeries

Treatment of anastomotic leak after oesophagectomy for oesophageal cancer : large, collaborative, observational TENTACLE cohort study

Background: Anastomotic leak is a severe complication after oesophagectomy. Anastomotic leak has diverse clinical manifestations and the optimal treatment strategy is unknown. The aim of this study was to assess the efficacy of treatment strategies for different manifestations of anastomotic leak after oesophagectomy. Methods: A retrospective cohort study was performed in 71 centres worldwide and included patients with anastomotic leak after oesophagectomy (2011-2019). Different primary treatment strategies were compared for three different anastomotic leak manifestations: interventional versus supportive-only treatment for local manifestations (that is no intrathoracic collections; well perfused conduit); drainage and defect closure versus drainage only for intrathoracic manifestations; and oesophageal diversion versus continuity-preserving treatment for conduit ischaemia/necrosis. The primary outcome was 90-day mortality. Propensity score matching was performed to adjust for confounders. Results: Of 1508 patients with anastomotic leak, 28.2 per cent (425 patients) had local manifestations, 36.3 per cent (548 patients) had intrathoracic manifestations, 9.6 per cent (145 patients) had conduit ischaemia/necrosis, 17.5 per cent (264 patients) were allocated after multiple imputation, and 8.4 per cent (126 patients) were excluded. After propensity score matching, no statistically significant differences in 90-day mortality were found regarding interventional versus supportive-only treatment for local manifestations (risk difference 3.2 per cent, 95 per cent c.i. -1.8 to 8.2 per cent), drainage and defect closure versus drainage only for intrathoracic manifestations (risk difference 5.8 per cent, 95 per cent c.i. -1.2 to 12.8 per cent), and oesophageal diversion versus continuity-preserving treatment for conduit ischaemia/necrosis (risk difference 0.1 per cent, 95 per cent c.i. -21.4 to 1.6 per cent). In general, less morbidity was found after less extensive primary treatment strategies. Conclusion: Less extensive primary treatment of anastomotic leak was associated with less morbidity. A less extensive primary treatment approach may potentially be considered for anastomotic leak. Future studies are needed to confirm current findings and guide optimal treatment of anastomotic leak after oesophagectom

Practice variation in anastomotic leak after esophagectomy: Unravelling differences in failure to rescue

Introduction: Failure to rescue (FTR) is an important outcome measure after esophagectomy and reflects mortality after postoperative complications. Differences in FTR have been associated with hospital resection volume. However, insight into how centers manage complications and achieve their outcomes is lacking. Anastomotic leak (AL) is a main contributor to FTR. This study aimed to assess differences in FTR after AL between centers, and to identify factors that explain these differences. Methods: TENTACLE – Esophagus is a multicenter, retrospective cohort study, which included 1509 patients with AL after esophagectomy. Differences in FTR were assessed between low-volume (<20 resections), middle-volume (20–60 resections) and high-volume centers (≥60 resections). Mediation analysis was performed using logistic regression, including possible mediators for FTR: case-mix, hospital resources, leak severity and treatment. Results: FTR after AL was 11.7%. After adjustment for confounders, FTR was lower in high-volume vs. low-volume (OR 0.44, 95%CI 0.2–0.8), but not versus middle-volume centers (OR 0.67, 95%CI 0.5–1.0). After mediation analysis, differences in FTR were found to be explained by lower leak severity, lower secondary ICU readmission rate and higher availability of therapeutic modalities in high-volume centers. No statistically significant direct effect of hospital volume was found: high-volume vs. low-volume 0.86 (95%CI 0.4–1.7), high-volume vs. middle-volume OR 0.86 (95%CI 0.5–1.4). Conclusion: Lower FTR in high-volume compared with low-volume centers was explained by lower leak severity, less secondary ICU readmissions and higher availability of therapeutic modalities. To reduce FTR after AL, future studies should investigate effective strategies to reduce leak severity and prevent secondary ICU readmission

Erratum to “Practice variation in anastomotic leak after esophagectomy:Unravelling differences in failure to rescue (vol 49, pg 974, 2023)

The publisher regrets that when the article was published the following collaboration authors from the “TENTACLE – Esophagus collaborative group” appeared incorrectly in the main author list due to a technical error: Writing Committee, Joos Heisterkamp, Fatih Polat, Jeroen Schouten, Pritam Singh, Study collaborators. This has now been corrected. The publisher would like to apologise for any inconvenience caused