Introduction

Enteropathy Associated T Cell Lymphoma (EATL) is a rare and highly aggressive T-cell non-Hodgkin’s lymphoma (NHL)¹ that is strongly associated with refractory coeliac disease (RCD), particularly type 2 RCD ², and possibly inflammatory bowel disease (IBD). The incidence and prevalence of both coeliac disease³ and IBD⁴ have been rising around the world for several decades, including in both Englandand Denmark^​. Some of the potential drivers of EATL are the antigen-driven proliferation of intra-villous T-cells from coeliac disease and chronic inflammation in IBD, so it is important to understand if changes in these diseases are altering clinical presentations of EATL and its survival.

Aims & Methods

Our aim was to describe the contemporary incidence and survival of EATL from two European countries on a national scale, given the rising incidence and prevalence of coeliac disease and use of immunosuppression in inflammatory bowel disease, to give further insights into epidemiology of this rare and often fatal malignancy.
People with EATL were identified from national cancer registries from 2013-2019 in England and 2004-2020 in Denmark. Within each country we used direct standardisation to estimate incidence, Poisson regression to compare incidence between groups and Cox regression to assess overall survival.

Results

172 and 39 patients were identified in England (E) and Denmark (DK) respectively with a mean age at diagnosis of 66 and 68 years. Approximately half of all cases had coeliac disease (E 52%, DK 41%), and ~7% (E 6%, DK 8%) had inflammatory bowel disease (Table). The overall age-standardised incidence rate was (E) 0.48 (95% CI 0.41-0.56) and (DK) 0.44 (95% CI 0.30-0.58) per million population per year. In both countries incidence was higher in older age groups. The 5-year overall survival was 10% (95% CI: 6%-18%) in England and 20% (95% CI: 9%-33%) in Denmark. Those with coeliac disease fared better than those without: (adjusted Hazard Ratio E 0.65 (95% CI 0.45-0.93), DK 0.57 (95% CI 0.26-1.27)).

Table: Characteristics of people with EATL in England (2013-2019) and Denmark (2004-2020)

Category	England (n=172)	Denmark (n=39)
Mean Age (95% CI)	66 (44, 89)	68 (65, 72)
Male (%)	108 (63%)	18 (46%)
Ethnicity, white (%)	160 (93%)	Not available
Stem cell transplant (%)	30 (17%)	Not available
Chemotherapy (%)	89 (52%)	Not available
Coeliac disease (%)	89 (52%)	16 (41%)
Inflammatory bowel disease (%)	11 (6%)	3 (8%)
Small bowel resection (%)	112 (65%)	7 (18%)
Died (%)	144 (84%)	33 (85%)

Conclusion

This analysis of two nationwide cancer registries is the largest population-based study to date to provide contemporary and comprehensive epidemiologic estimates of all ICD-O-3 coded EATL. It shows that a higher incidence is associated with older age, male sex (in England) and that half of cases also have coeliac disease. Our finding of the high proportion of people with inflammatory bowel disease compared to the background prevalence in both countries requires further investigation, as reasons for this are unclear, but it could be related to chronic inflammation or immunosuppression. Overall survival is poor and gets worse in older age groups but is better in those with coeliac disease, following surgical resection and those able to tolerate intensive therapies.

References

1. Ferreri AJ, Zinzani PL, Govi S, et al. Enteropathy-associated T-cell lymphoma. Crit Rev Oncol Hematol 2011;79:84-90.
2. Pelizzaro F, Marsilio I, Fassan M, et al. The Risk of Malignancies in Celiac Disease-A Literature Review. Cancers (Basel) 2021;13.
3. King JA, Jeong J, Underwood FE, et al. Incidence of Celiac Disease Is Increasing Over Time: A Systematic Review and Meta-analysis. Am J Gastroenterol 2020;115:507-525.
4. Ng SC, Shi HY, Hamidi N, et al. Worldwide incidence and prevalence of inflammatory bowel disease in the 21st century: a systematic review of population-based studies. Lancet 2017;390:2769-2778.

Introduction

Dysphagia is a common condition with consequences surpassing the physical dimension and extending to the patient’s psychological, personal, and professional life. However, when referring to non-obstructive esophageal dysphagia (NOD) and motility disorders, data is still scarce.

Aims & Methods

Our study consists in a prospective and multicentric evaluation of the severity of dysphagia and its impact on quality of life, that was measured with the Eckardt, EuroQol-5D (TTO and VAS) and PROMIS GI Disrupted Swallowing (PDS) scores, in patients with NOD and underlying motility disorders. We included 373 individuals (250 with NOD and 123 without dysphagia).

Results

Among patients with NOD, 97 had esophagogastric junction (EGJ) outflow disorders on manometry according to Chicago V4.0, 24 spastic motility disorders, 38 hypomotile disorders and 91 normal motility. Compared with non-dysphagic individuals, dysphagic patients had significantly worse scores of both EuroQol-5D and PDS (p-values <0.001).
Among esophageal motility disorders, Eckardt score was significantly different between groups (p-value <0.001) as determined by ANOVA. Specifically, it was significantly higher in EGJ outflow disorders (mean of 5.2), when compared to spastic motility disorders (mean of 3.3) or dysphagic patients with normal motility (mean of 3.2).
With respect to Patient-reported outcomes, PDS score was statistically different between groups (p-value 0.003), being higher in the EGJ outflow disorders (mean T-score of 64.1) when compared to hypomotiity disorders (mean of 60.2) and dysphagic patients with normal motility (mean of 60.3).
Regarding patient-reported quality of life, there were no differences between groups in terms of EuroQol-5D TTO or VAS score (p-value of 0.156 and 0.368, respectively).

Conclusion

Our findings highlights the impact of NOD on the quality-of-life of patients. Although physician and patient reported symptoms’ severity were worse on EGJ outflow disorders, overall quality of life seems equally affected among all esophageal motility profiles.

Introduction

The ergonomics has been considered important in practicing surgical laparoscopy, and prior studies have shown significance of relevant factors e.g. operating room setup and design of surgical devices. However, little has been known in flexible endoscopy; How do endoscopists control flexible endoscopes? Which muscles are loaded during endoscopy? The deeper understanding of ergonomic aspects in flexible endoscopy is indispensable, since the number of endoscopies performed and its complexity are rapidly increasing.

Aims & Methods

We aimed to identify the differences of ergonomics between diagnostic endoscopy (EGD) and therapeutic endoscopy (ESD), by 1) multiple motion capture technology, and 2) wireless surface electromyography (EMG), in pre-clinical setting.
1) The multiple motion capture assessment
The control section of standard flexible endoscope (GIF-Q260J; Olympus, Tokyo, Japan), from the boot to the angulation knobs, was marked with 6 optical reflective markers. These markers were traced using a motion capture system (OptiTrack Flex13; NaturalPoint, OR) and the data were processed to generate 3-D coordinate data (The sternum manubrium of the endoscopist was setup as the coordinate origin; X: left-right axis, Y: cranio-caudal axis, Z: antero-posterior axis). EGD and ESD were performed with this motion capture system using live porcine models. For each endoscopist, mean±2SD for plotted coordinates from the origin was calculated in the three axes and described as a range of his/her arm/hand movement. The data were compared between EGD and ESD.
2) EMG assessment
Muscle activation of the left biceps brachii muscle, trapezius muscle, extensor carpi ulnaris muscle, flexor carpi radialis muscle, pronator teres muscle, thenar muscle, back neck muscle, and erector spinae muscle were recorded during endoscopic procedures using wireless surface EMG (BioLog DL-5500, DL-510A; S&ME, Tokyo, Japan). The maximal voluntary contraction (MVC) was obtained to normalize root-mean-square muscle activation as %MVC. We compared differences in muscle activity between EGD and ESD using %MVC.

Results

1) The motion capture was performed on 15 board-certified endoscopists conducting EGD (n=13) and ESD (n=12). The movement range of endoscopist’s left hand/arm holding endoscope is shown in the Table. On all three axes, the range of movement was significantly narrower during ESD than during EGD (X, p<0.001; Y, p=0.015; Z, p<0.001).

	EGD			ESD
	X axis	Y axis	Z axis	X axis	Y axis	Z axis
Mean ± 2SD (cm)	-0.32±14.2	-24.3±11.0	12.8±11.6	-1.3±9.9	-22.8±9.8	7.9±8.9

2) The EMG was recorded during 15 EGDs and 8 ESDs. The %MVC was higher during ESD than EGD in an analysis including all muscles (LS-mean±SE; EGD, 28.9±1.5%; ESD, 34.1±2.6%; p=0.078). The comparison by each specific muscle showed that the extensor carpi ulnaris muscle (EGD, 34.6±3.5%; ESD, 47.8±5.8%; p=0.093) and the pronator teres muscle (EGD, 42.8±6.6%; ESD, 67.6±10.9%; p=0.092) had significantly larger %MVC during ESD (p<0.1 was considered statistically significant).

Conclusion

Our study has first shown that the range of endoscopis’s movement was significantly narrower in therapeutic endoscopy than in diagnostic endoscopy. Furthermore, it was demonstrated that muscle activity, especially in left forearm muscles, was larger during therapeutic endoscopy. These data may help understanding the ergonomics in flexible endoscopy, and potentially contribute to optimize its working environment and further development of relevant medical devices.

Introduction

Cardiorespiratory arrest is a recognized complication of endoscopic procedures, particularly when sedation is administered. Hospitalized patients, especially those with a prescribed Do Not Resuscitate (DNR) order, are more likely to have severe underlying illness, and consequently a higher risk of cardiorespiratory complications. Additionally, the request for endoscopic exams, especially urgent ones, in this group of patients is becoming increasingly common.

Aims & Methods

The aim of this study was to asses the current clinical practices, as well as the beliefs of gastroenterologists in Portugal regarding the management of the code status in hospitalized patients with prescribed DNR undergoing endoscopic procedures.
Self-reported data was obtained through a voluntary and anonymous survey of gastroenterologists and gastroenterology trainees in Portugal.

Results

One hundred and forty five gastroenterologists, of which 26 (17.9%) were trainees, responded to the survey, with an average age of 42 years (27-83 years), and 59.3% female. The majority are aware of the patient's code status prior to the endoscopic procedure (73.1%, n=106). However, DNR is rarely discussed (<25% of cases) with the patient/legal representative (66.9%, n=97) or attending physician (58.6%, n=85). In patients with prescribed DNR, the rate of reversal prior to the procedure is rarely performed (reversal in all or almost all in 8,3%, n=12). Most respondents are unaware of the procedures necessary for DNR prescription (81.3%, n=118), as well as existing national/international recommendations on DNR reversal (96.5%, n=140). Regarding personal beliefs, the majority consider that in these patients DNR should be reversed to an intermediate option that includes limited resuscitation maneuvers according to the patient's preference (62.1%, n=90). They also believe that the urgency of a procedure should not influence the decision to reverse DNR (n=89, 61.4%), and that if DNR is reversed, it should be done by the attending physician who initially prescribed it (91.7%, n=133). Finally, respondents are divided when asked if they would perform an endoscopic procedure on a patient with prescribed DNR that has not been previously reversed: 49.7% answered yes (n=72), 22.1% no (n=32), and 28.3% have no formed opinion (n=41). There is a consensus on the need for recommendations on this topic for gastroenterologists (97.9%, n=142).

Conclusion

The majority of Gastroenterologists rarely reverse DNR before endoscopic procedures, however, it is mostly advocated that limited resuscitation maneuvers should be offered according to patient preference. Most are unaware of the existence of institutional, national, or international policies regarding DNR. These findings suggest considerable variation in knowledge and approach to hospitalized patients with DNR, highlighting the need for specific recommendations for Gastroenterology in this area.

References

H. Carneiro A, Carneiro R. DNR A Decisão de Não Reanimar. Revista Portuguesa de Medicina Interna [Internet]. 5 de Dezembro de 2021
Braga I, Dutschmann L, Fiuza T, Almeida H, Gil V, Brandão T. Implementação da Ordem de Não Reanimar. Revista Ordem Médicos. 2012;133:20-23.
Wada K, Szego MJ. Revisiting code status in patients undergoing GI endoscopy with a "do not resuscitate" order. Gastrointest Endosc. 2019 Feb;89(2):380-382.
Feld LD, Mergener K, Rubin DT, Rao VL. Management of code status in the periendoscopic period: a national survey of current practices and beliefs of U.S. gastroenterologists. Gastrointest Endosc. 2021 Jul;94(1):172-177.e2.

Clinical Case Summary

Case: W present a case of a 43-year-old man with hepatic cirrhosis complicated by hypertensive gastrointestinal bleeding, in a program to eradicate esophageal varices. One week after an elective endoscopic variceal ligation (EVL), he presented with fever, anorexia, pain in the dorsolumbar region, headaches, and psychomotor retardation. Subsequently, there was the appearance of focal motor seizures. The analyses showed C-reactive protein of 113 mg/L, without leukocytosis. Peripheral blood cultures were negative. A head computed tomography (CT) showed mild left parietal and occipital cortical hypodensities. Analysis of the cerebrospinal fluid was compatible with central nervous system (CNS) infection. Cerebral magnetic resonance revealed multiple supra and infratentorial rounded lesions, compatible with abscesses and ventriculitis. He started antibiotics with a meningeal dose. Hospitalization was complicated by SARS-COV 2 infection, so a chest CT was performed after 1 month of hospitalization, and was identified a mediastinal abscess with 5 cm with fistulization from the esophagus, compatible with iatrogenic esophageal perforation after EVL. Other possible causes of brain abscesses were excluded. He completed several weeks of antibiotic and parenteral nutrition, with normalization of the neurological status and resolution of brain lesions. About 1 month after targeted antibiotic treatment, he performed an endoscopy, revealing a deformed area in the distal esophagus but no evidence of an orifice or suppuration. Conclusion: Neurological manifestations in patients with liver cirrhosis are usually due to hepatic encephalopathy (HE). However, CNS infection, especially in patients who underwent recent procedures, such as EBL, should be considered in the differential diagnosis, given that early treatment influences the prognosis. Clinically, CNS infection can be very variable, however, it is important to be aware of atypical signs of HE, such as focal neurological deficits.

References

Krige JE, Bornman PC, Shaw JM, Apostolou C. Complications of endoscopic variceal therapy. S Afr J Surg. 2005 Nov;43(4):177-88, 190-4.

Disclosure

Nothing to disclosure

Introduction

Endoscopic submucosal dissection (ESD) for esophageal squamous cell carcinoma (ESCC) with esophageal varices (EVs) is one of the most challenging endoscopic procedures due to the high risk of bleeding and fibrosis caused by prior treatment of varices. However, due to the small number of such cases, the clinical outcomes of ESD for ESCC with EVs have been reported in only a limited number of cases at single centers^​1-3.

Aims & Methods

We conducted a multicenter, retrospective, cohort study to clarify the safety and efficacy of ESD for ESCC with EVs.Eleven institutions in the Tohoku region of Japan participated in this multicenter, collaborative, retrospective study. We performed endoscopic resection for 3619 patients with esophageal carcinoma at the participating institutions during the period from January 2010 to April 2022. Among those, 30 patients who underwent ESD for ESCC with EVs were recruited for the present study.
Rates of en bloc resection and R0 resection, procedure time and adverse events were evaluated as indicators of the feasibility and safety of ESD. Additional treatment, recurrence and metastasis of the lesions were evaluated as indicators of the long-term efficacy of ESD.

Results

Portal hypertension was caused by cirrhosis, of which alcohol was the most common cause. En bloc resection was achieved in 93.3% and R0 resection in 80.0% of the patients. The median procedure time was 92 minutes. Adverse events included a case of uncontrolled intraoperative bleeding leading to discontinuation of ESD and a case of esophageal stricture due to extensive resection. During the follow-up period of a median for 42 months, a patient with local recurrence and another patient with liver metastasis were observed. One patient died of liver failure after receiving chemoradiotherapy as an additional treatment after ESD. No patient died of ESCC.

Conclusion

This multicenter, retrospective cohort study demonstrated the safety and efficacy of ESD for ESCC with EVs. Further studies are needed to establish appropriate treatment methods for EVs before ESD and additional treatments for patients with insufficient ESD.

References

1. Sawaguchi M, Jin M, Matsuhashi T, et al. The feasibility of endoscopic submucosal dissection for superficial esophageal cancer in patients with cirrhosis (with video). Gastrointest Endosc 2014;79:681-5.
2. Choi YK, Noh JH, Kim DH, et al. Outcomes of endoscopic submucosal dissection for superficial esophageal neoplasms in patients with liver cirrhosis. Clin Endosc 2022;55:381-9.
3. Zhang S, Liu YD, Chai NL, et al. Endoscopic submucosal dissection for early cancers or precancerous lesions of the upper gastrointestinal tract in cirrhotic patients with esophagogastric varices: Ten-year experience from a large tertiary center in China. Gastrointest Endosc 2023 Online ahead of print.

Disclosure

The authors declare that they have no conflicts of interest.