Torticollis with Atlantoaxial Rotatory Subluxation in Children: A Clinical Review.

A small proportion of children with a sudden onset torticollis ("wry neck") presents with an atlantoaxial rotatory subluxation, usually after mild trauma or recent head or neck infection. Torticollis is a clinical diagnosis and imaging is usually not indicated, though often performed in clinical practice. Atlantoaxial rotatory subluxation on imaging is often a physiological phenomenon in torticollis, and concomitant neurological symptoms are therefore rare. Treatment is primarily conservative, with analgesics, a rigid neck collar, and if needed benzodiazepines to counteract muscle spasms and anxiety. In case of treatment failure or chronic subluxation, cervical repositioning and fixation under general anesthesia may be considered. Surgical treatment is only indicated in a small percentage of patients with chronic refractory subluxation, concomitant cervical fractures, or congenital anomalies. Early diagnosis and treatment are important, since this is associated with a more successful conservative outcome than a prolonged approach.

Quality of Life in Patients With Definite Chronic Pancreatitis: A Nationwide Longitudinal Cohort Study.

INTRODUCTION: Chronic pancreatitis (CP) has a negative impact on quality of life (QoL). Because CP is a chronic condition, multiple assessments of QoL are required to obtain a thorough understanding of its impact on patients. Such studies are currently lacking. This study aims to gain insight into the course and predictors of QoL in patients with CP using prospective longitudinal data from a large cohort of patients. METHODS: Post hoc analysis of consecutive patients with definite CP registered in a prospective database between 2011 and 2019 in the Netherlands. Patient and disease characteristics, nutritional status, pain severity, medication usage, pancreatic function, and pancreatic interventions were assessed from medical records and through standard follow-up questionnaires. The physical and mental component summary scales of the Short-Form 36 were used to assess physical and mental QoL at baseline and during follow-up. The course of both physical and mental QoL and their associated factors were longitudinally assessed by using generalized linear mixed models. RESULTS: Overall, 1,165 patients with definite CP were included for this analysis. During 10-year follow-up, generalized linear mixed model analyses revealed improvements in both physical (41.6-45.2, P < 0.001) and mental (45.9-46.6, P = 0.047) QoL. Younger age, current alcohol consumption, employment, no need for dietetic consultation, no steatorrhea, lower Izbicki pain score, and pain coping mechanism were positively associated with physical QoL ( P < 0.05). For mental QoL, a positive correlation was found between employment, nonalcoholic CP, no need for dietetic consultation, no steatorrhea, lower Izbicki pain score, pain coping mechanism, and surgical treatment. No association was observed between disease duration and longitudinal QoL per patient. DISCUSSION: This nationwide study provides insight into the dynamics of physical and mental QoL in patients with CP over time. Important and potentially influenceable factors to improve QoL are nutritional status, exocrine pancreatic function, employment status, and patients' coping strategy.

Long-term outcomes of pancreatoscopy-guided electrohydraulic lithotripsy for the treatment of obstructive pancreatic duct stones.

Background and study aims Pancreatoscopy-guided electrohydraulic lithotripsy (EHL) has proven to be an effective first-line therapy in symptomatic chronic pancreatitis (CP) patients with obstructing pancreatic duct (PD) stones 1 . However, long-term outcomes of endoscopic EHL remain unknown. The aim of the present study was to evaluate the long-term treatment effects of EHL as first-line therapy and to compare with those obtained in a historical cohort of patients who underwent extracorporeal shockwave lithotripsy (ESWL) as primary treatment. Patients and methods An observational retrospective single-center long-term follow-up study was performed including 19 consecutive patients who previously underwent endoscopic EHL compared to 18 patients who underwent ESWL followed by endoscopic retrograde pancreatography (ERP). The primary endpoint was long-term treatment success after EHL or ESWL defined as no recurrence of symptomatic intraductal stones confirmed on imaging. Secondary endpoints for the EHL-population included long-term clinical success (i. e., a similar or lower Izbicki Pain Score or reduction in opiate usage as compared to 6-month follow-up), quality of life (QoL), pancreatic function and hospital re-admission rate. Results In the EHL group, 37 % of the patients developed recurrent symptomatic PD stones versus 61 % in the ESWL group after a median follow-up of 35.0 and 76.5 months. Of the patients with recurrence, 71 % versus 100 % underwent a reintervention. Median time to recurrence was 12.0 versus 13.0 months. Clinical success sustained in 58 % of the EHL patients. QoL was not significantly different compared with 6-month follow-up and baseline. Conclusions Also at long-term follow-up, endoscopic EHL as first-line treatment is moderately effective for symptomatic CP patients with treatment success rates that seems at least equally effective as ESWL.

Cost-effectiveness of Early Surgery Versus Endoscopy-first Approach for Painful Chronic Pancreatitis in the ESCAPE Trial.

OBJECTIVE: Economic evaluation of early surgery compared to the endoscopy-first approach in CP. BACKGROUND: In patients with painful CP and a dilated main pancreatic duct, early surgery, as compared with an endoscopy-first approach, leads to more pain reduction with fewer interventions. However, it is unknown if early surgery is more cost-effective than the endoscopy-first approach. METHODS: The multicenter Dutch ESCAPE trial randomized patients with CP and a dilated main pancreatic duct between early surgery (surgery within 6 weeks) or the endoscopy-first approach in 30 centers (April 2011-September 2016). Healthcare utilization was prospectively recorded up to 18 months after randomization. Unit costs of resources were determined, and cost-effectiveness and cost-utility analyses were performed from societal and healthcare perspectives. Primary outcomes were the costs per unit decrease on the Izbicki pain score and per gained quality-adjusted life-year. RESULTS: In total, 88 patients were included in the analysis, with 44 patients randomized to each group. Total costs were lower in the early surgery group but did not reach statistical significance (mean difference €-4,815 (95% bias-corrected and accelerated confidence interval €-13,113 to €3411; P = 0.25). Early surgery had a probability percentage of 88.4% of being more cost-effective than the endoscopy-first approach at a willingness-to-pay threshold of €0 per day per unit decrease on the Izbicki pain score. The probability percentage per additional gained quality-adjusted life-year was 75.7% at a willingness-to-pay threshold of €50,000. CONCLUSION: In patients with painful CP and a dilated main pancreatic duct, early surgery was more cost-effective than the endoscopy-first approach.

Short- and long-term outcomes of selective use of Frey or extended lateral pancreaticojejunostomy in chronic pancreatitis.

BACKGROUND: Surgery is the most effective treatment in patients with painful chronic pancreatitis and a dilated pancreatic duct. Studies reporting the outcomes of selected surgical approach according to the pancreatic head size in these patients are lacking. METHOD: This was a retrospective, observational single-centre study of consecutive patients who underwent either a Frey procedure or extended lateral pancreaticojejunostomy (eLPJ) for pain due to chronic pancreatitis with a dilated main pancreatic duct (5 mm or more) between 2006 and 2017. A Frey procedure was used in patients with pancreatic head enlargement (40 mm or more) and eLPJ (full-length pancreaticojejunostomy, including transection of the gastroduodenal artery) in others. A biliodigestive bypass was added in the case of biliary obstruction. RESULTS: Overall, 140 of 220 patients met the eligibility criteria: 70 underwent a Frey procedure and 70 an eLPJ. Hepaticojejunostomy was added in 17.1 per cent of patients (Frey: 24.3 per cent; eLPJ: 10.0 per cent (P = 0.025)). Major morbidity occurred in 15.0 per cent of patients (Frey: 21.4 per cent; eLPJ: 8.6 per cent (P = 0.033)). After a median 7.8 years of follow-up, the mean (s.d.) decrease in Izbicki pain score was 33 (27) points (34 (28) points after a Frey procedure; 32 (26) points after an eLPJ). Pain relief was reported as 'very much' by 87.5 per cent of patients (Frey: 86.1 per cent; eLPJ: 88.9 per cent) and as 'partial' by 11.1 per cent (Frey: 13.8 per cent; eLPJ: 8.3 per cent). CONCLUSION: Selective-use of either a Frey procedure or eLPJ in patients with symptomatic chronic pancreatitis was-associated with low morbidity and long-term pain relief. Adding a-biliodigestive bypass did not increase morbidity.

A Classification Algorithm for Types of Diabetes in Chronic Pancreatitis Using Epidemiological Characteristics: Outcome of a Longitudinal Cohort Study.

OBJECTIVES: We developed an epidemiological algorithm to classify types of diabetes mellitus (DM) in chronic pancreatitis (CP), and applied it to a nationwide prospective longitudinal cohort of CP patients. METHODS: Patients with definite CP (M-ANNHEIM criteria) were classified as having DM types 1, 2, or 3c, or no DM using an algorithm based on epidemiological characteristics: DM onset in relation to age, CP onset, exocrine insufficiency. Variables associated with development of DM were identified. RESULTS: Of 1130 included patients with CP between 2011 and 2018, 368 patients (33%) had DM at inclusion. Among patients with DM, 11 were classified as having type 1 (3%), 159 as type 2 (43%), and 191 as type 3c (52%). Patients with DM type 3c had longer duration of CP, more severe pain and lower physical quality of life. During longitudinal follow-up of median 47 months, 120 (20%) patients developed DM, of which 99 patients were classified as type 3c. This was independently associated with pancreatic endoscopy and surgery. CONCLUSIONS: The described algorithm based on epidemiological characteristics can help to classify types of DM in patients with CP. Diabetes mellitus type 3c is associated with longer duration of CP and more severe CP sequelae.

Pain patterns in chronic pancreatitis: a nationwide longitudinal cohort study.

OBJECTIVE: Pain in chronic pancreatitis is subdivided in a continuous or intermittent pattern, each thought to represent a different entity, requiring specific treatment. Because evidence is missing, we studied pain patterns in a prospective longitudinal nationwide study. DESIGN: 1131 patients with chronic pancreatitis (fulfilling M-ANNHEIM criteria) were included between 2011 and 2018 in 30 Dutch hospitals. Patients with continuous or intermittent pain were compared for demographics, pain characteristics, quality of life (Short-Form 36), imaging findings, disease duration and treatment. Alternation of pain pattern and associated variables were longitudinally assessed using a multivariable multinomial logistic regression model. RESULTS: At inclusion, 589 patients (52%) had continuous pain, 231 patients (20%) had intermittent pain and 311 patients (28%) had no pain. Patients with continuous pain had more severe pain, used more opioids and neuropathic pain medication, and had a lower quality of life. There were no differences between pain patterns for morphological findings on imaging, disease duration and treatment. During a median follow-up of 47 months, 552 of 905 patients (61%) alternated at least once between pain patterns. All alternations were associated with the Visual Analogue Scale pain intensity score and surgery was only associated with the change from pain to no pain. CONCLUSION: Continuous and intermittent pain patterns in chronic pancreatitis do not seem to be the result of distinctly different pathophysiological entities. The subjectively reported character of pain is not related to imaging findings or disease duration. Pain patterns often change over time and are merely a feature of how severity of pain is experienced.

Suboptimal care for chronic pancreatitis patients revealed by moderate to low adherence to the United European Gastroenterology evidence-based guidelines (HaPanEU): A Netherlands nationwide analysis.

BACKGROUND AND OBJECTIVE: The 2016, United European Gastroenterology evidence-based guidelines for the diagnosis and therapy of chronic pancreatitis (HaPanEU) provided evidence-based recommendations for the management of chronic pancreatitis and allowed for the objective evaluation of the quality of care in several domains of disease management through assessment of guideline adherence. Therefore, the aim of this study is to evaluate the current level and the variety of care for chronic pancreatitis patients in the Netherlands using the HaPanEU guidelines as a reference standard. The majority of these patients were diagnosed before the publication of these guidelines. Therefore, in most patients, the results of the present study with respect to those recommendations regarding the diagnostic process of chronic pancreatitis represent guideline correspondence and not adherence. METHODS: A subgroup of patients from the Dutch nationwide chronic pancreatitis registry (CARE) was included in a retrospective cross-sectional observational cohort study. A total of 39 recommendations concerning the non-invasive management of chronic pancreatitis were appointed as quality indicators (QIs). Per patient, the number of relevant QIs was determined and guideline adherence was assessed. Data were analyzed to identify factors associated with guideline adherence. RESULTS: Overall, 97 patients with chronic pancreatitis from 11 hospitals were included. Per patient, a mean number of 26 relevant QIs was applicable, with an average adherence rate of 53%. In 45% of the patients, guideline adherence was less than 50%. The majority of suboptimal managed QIs concerned the management of chronic pancreatitis complications. Guideline adherence was not associated with hospital type, sex, age or etiology of pancreatitis. CONCLUSION: In the Netherlands, adherence to the HaPanEU recommendations for the management of chronic pancreatitis is moderate to low for all non-invasive domains, which may indicate suboptimal care for these patients. Closer guideline adherence could improve the level of care and the clinical outcomes of these patients. A nationwide approach to increase awareness of the key guideline recommendations among clinicians and patients is needed.

Natural Course and Treatment of Pancreatic Exocrine Insufficiency in a Nationwide Cohort of Chronic Pancreatitis.

OBJECTIVES: Pancreatic exocrine insufficiency (PEI) is a common complication of chronic pancreatitis. However, little is known about the natural course of PEI and the effect of pancreatic enzyme replacement therapy on symptoms. The aim of this study was to evaluate the natural course and treatment of PEI in a nationwide cohort of patients with chronic pancreatitis. METHODS: Patients with chronic pancreatitis were selected from the multicenter Dutch Chronic Pancreatitis Registry. Patients were classified in 3 groups: definite PEI, potential PEI, and no PEI. Definite PEI and no PEI were compared regarding the course of disease, symptoms, treatment, and quality of life. RESULTS: Nine hundred eighty-seven patients were included from 29 centers, of which 304 patients (31%) had definite PEI; 451 (46%), potentially PEI; and 232 (24%), no PEI. Patients with definite PEI had significantly more malabsorption symptoms, a lower body mass index, and aberrant defecation. Lowered quality of life was not independently associated with PEI. Of the PEI patients using pancreatic enzyme replacement therapy, 47% still reported steatorrhea. CONCLUSIONS: Pancreatic exocrine insufficiency is associated with malabsorption symptoms and a lower body mass index. Some form of pancreatic enzyme replacement therapy is reasonably effective in alleviating malabsorption symptoms, but improvement of treatment is needed.

Effect of Early Surgery vs Endoscopy-First Approach on Pain in Patients With Chronic Pancreatitis: The ESCAPE Randomized Clinical Trial.

Importance: For patients with painful chronic pancreatitis, surgical treatment is postponed until medical and endoscopic treatment have failed. Observational studies have suggested that earlier surgery could mitigate disease progression, providing better pain control and preserving pancreatic function. Objective: To determine whether early surgery is more effective than the endoscopy-first approach in terms of clinical outcomes. Design, Setting, and Participants: The ESCAPE trial was an unblinded, multicenter, randomized clinical superiority trial involving 30 Dutch hospitals participating in the Dutch Pancreatitis Study Group. From April 2011 until September 2016, a total of 88 patients with chronic pancreatitis, a dilated main pancreatic duct, and who only recently started using prescribed opioids for severe pain (strong opioids for ≤2 months or weak opioids for ≤6 months) were included. The 18-month follow-up period ended in March 2018. Interventions: There were 44 patients randomized to the early surgery group who underwent pancreatic drainage surgery within 6 weeks after randomization and 44 patients randomized to the endoscopy-first approach group who underwent medical treatment, endoscopy including lithotripsy if needed, and surgery if needed. Main Outcomes and Measures: The primary outcome was pain, measured on the Izbicki pain score and integrated over 18 months (range, 0-100 [increasing score indicates more pain severity]). Secondary outcomes were pain relief at the end of follow-up; number of interventions, complications, hospital admissions; pancreatic function; quality of life (measured on the 36-Item Short Form Health Survey [SF-36]); and mortality. Results: Among 88 patients who were randomized (mean age, 52 years; 21 (24%) women), 85 (97%) completed the trial. During 18 months of follow-up, patients in the early surgery group had a lower Izbicki pain score than patients in the group randomized to receive the endoscopy-first approach group (37 vs 49; between-group difference, -12 points [95% CI, -22 to -2]; P = .02). Complete or partial pain relief at end of follow-up was achieved in 23 of 40 patients (58%) in the early surgery vs 16 of 41 (39%)in the endoscopy-first approach group (P = .10). The total number of interventions was lower in the early surgery group (median, 1 vs 3; P < .001). Treatment complications (27% vs 25%), mortality (0% vs 0%), hospital admissions, pancreatic function, and quality of life were not significantly different between early surgery and the endoscopy-first approach. Conclusions and Relevance: Among patients with chronic pancreatitis, early surgery compared with an endoscopy-first approach resulted in lower pain scores when integrated over 18 months. However, further research is needed to assess persistence of differences over time and to replicate the study findings. Trial Registration: ISRCTN Identifier: ISRCTN45877994.

Surgery in Chronic Pancreatitis: Indication, Timing and Procedures.

Chronic pancreatitis is a chronic inflammation of the pancreas with pain as its severest symptom and often an impaired quality of life. Surgical intervention plays an important role in the management of pain but is generally kept as a last resort when conservative measures and endoscopy have failed. However, in the last few years multiple studies suggested the superiority of (early) surgical treatment in chronic pancreatitis for multiple end points, including pain relief. In this paper we highlight the most recent high-quality evidence on surgical therapy in chronic pancreatitis and the rationale for early (surgical) intervention.

Efficacy of total pancreatectomy with islet autotransplantation on opioid and insulin requirement in painful chronic pancreatitis: A systematic review and meta-analysis.

BACKGROUND: The rationale for total pancreatectomy in painful, treatment refractory, chronic pancreatitis is pain control. Concomitant islet cell autotransplantation can prevent the loss of islet cell function. This study aimed to systematically examine the impact of total pancreatectomy with islet cell autotransplantation on pain and quality of life. METHODS: This meta-analysis was conducted according the Meta-analyses of Observational Studies in Epidemiology guideline. The Cochrane Library, PubMed, and Embase were searched for the following terms (1990 through April 2018): total pancreatectomy and chronic pancreatitis. Studies were included when addressing total pancreatectomy with islet cell autotransplantation for chronic pancreatitis in adults. Studies that reported no data on pain, endocrine function, or quality of life were excluded. Quality was assessed using the Newcastle-Ottawa scale for evaluation of all studies. RESULTS: We included 15 observational studies evaluating 1,255 patients, of whom 28% had had endoscopic and 23% operative therapy. One year after total pancreatectomy with islet cell autotransplantation, the opioid-free rate had improved from between 0% and 15% to 63% (95% CI 46-77), and the insulin-free rate had decreased from between 89.5% and 100% to 30% (95% CI 20-43). An alcoholic etiology was associated with a lesser insulin-free rate after total pancreatectomy with islet cell autotransplantation. Quality of life improved statistically after total pancreatectomy with islet cell autotransplantation. Publication bias was present for both opioid and insulin outcomes. CONCLUSION: In selected patients with painful, treatment refractory, chronic pancreatitis, evidence shows that total pancreatectomy with islet cell autotransplantation is effective for pain control in almost two-thirds of patients, whereas the insulin-free rate is relatively low.