Introduction

The aberrant right subclavian artery (ARSA) is the most frequent vascular aortic anomaly with an incidence of 0.4% - 2% in the general population. In 80% of the cases it has a retroesophageal location, also 30% of ARSA is found in patients with trisomy 21. Endoscopic ultrasound (EUS) enables the diagnosis of multiple pathologies and the assessment of vascular structures, in which anomalies such as ARSA can be detected. The main objective of our research is to evaluate the presence of the ARSA as an infrequent finding in upper EUS.

Aims & Methods

The main objective of our research is to evaluate the presence of the ARSA as an infrequent finding in upper EUS. The population of the study consists in all of the patients referred for upper EUS, for different reasons, excluding those with esophageal stenosis, those referred for linear endosonography and patients under 18y.o, at the Gastroenterology Division of both the Centro Medico Docente La Trinidad and the Clinica Atias, health centers located in Caracas, Venezuela. This is a retrospective, observational descriptive, cross-sectional study

Results

A total of 9,966 patients with upper EUS using a radial transducer were studied, six of them were diagnosed with ARSA, two males and four females, between the ages 39 to 72 (mean of age: 55 for males, 60 for females), all cases were confirmed with computed tomography angiography and none of them were previously diagnosed during their upper endoscopy or any other techniques before EUS. Furthermore, one of the patients with ARSA also had Kommerell's diverticulum. However, of the six patients only one reported intermittent dysphagia to solids.

Conclusion

Despite being the most common aortic vascular anomaly, ARSA is still a rare finding; it may go unnoticed during upper endoscopy and EUS. With the radial echoendoscope transducer an anechoic tubular structure can be seen emerging from the distal arch of the aorta crossing to the right between the vertebral column and the posterior wall of the esophagus. When comparing the incidence of ARSA with other reports, we had 0.06% versus 0.33% found in another study.

References

1. Tony E Yusuf,* Michael J Levy,* Maurits J Wiersema et al. Endoscopic ultrasound in the diagnosis of aberrant right subclavian artery
2. Maeder M, Binek J. Impact of endoscopic ultrasonography in the diagnosis of aberrant right subclavian artery: a case report. Ultraschall.Med 2004, 25:296–8
3. De Luca L, Bergman JJ, Tytgat GN, Fockens PE. US imaging of the arteria lusoria: case series and review.Gastrointest. Endosc.2000; 52:670–3
4. Stone WM, Brewster DC, Moncure AC et al. Aberrant right subclavian artery: varied presentations and management options. J Vasc Surg [Internet]. 1990; 11(6):812–7
5. Domínguez-Massa C, Berbel-Bonillo A, Pérez-Guillen M, Montero-Argudo JA. Dissected aberrant right subclavian artery with Kommerell diverticulum. Rev Port Cardiol (Engl Ed) [Internet]. 2019; 38(10):737.e1-737.e4
6. Mark NM, Madtes DK. Aberrant right subclavian artery on endobronchial ultrasound. Am J Respir Crit Care Med [Internet]. 2017; 196(6):e14.

Disclosure

NONE

Clinical Case Summary

A 71-year-old female presented to the gastroenterology clinic following a two year history of dysphagia and sensation of food sticking in the oesophagus. Her dysphagia was limited to solid foods, and symptoms were not exacerbated by positional change. Pain was mostly localised around the sternal notch where she would have the sensation of a food bolus. Over the preceding 2 years, she had experienced about 7.5kg of weight loss with no constitutional symptoms of fevers or fatigue. Her past medical history included hypertension, dyslipidaemia, lower back pain, cold agglutinin disease, monoclonal B lymphocytosis and peripheral arterial disease. On examination, there were no signs of malnourishment or palpable lymph nodes in the axilla, neck or supraclavicular region. Her abdomen was soft and non-tender. Given largely non-illuminating blood results, an oesophageal-gastroduedonoscopy was performed. However, no findings were noted, specifically no oesophageal webs, Zenker diverticulum, gastroparesis, trachealisation of the oesophagus or corrosive pathology. H. pylori was also not isolated on histopathology. A barium swallow was then performed which showed extensive external compression of the proximal third of the oesophagus at the level of the arch of the aorta, most indicative of dysphagia lusoria (Fig 1). Computed tomography angiography (CTA) showed retroesophageal aberrant right subclavian artery bifurcating from the ascending aorta independent of a brachiocephalic artery and causing external oesophageal compression (Fig 2 & 3). Given this definitive diagnosis, the patient was then seen in clinic where management of dysphagia lusoria was explained. Despite ongoing symptoms, the patient decided to continue with conservative management and lifestyle modifications, which included eating smaller bites and increase in fluid intake. Although an extensive compression typically requires surgical intervention, our patient was stable at two-year follow-up (Fig 4 A-B).

References

Figure 1: Barium swallow examination showing extrinsic compression of the proximal third of the oesophagus at the level of the aortic arch (white arrow).
Figure 2: Axial view of computed tomography angiography (CTA) showing the posterior course of the retroesophageal aberrant right subclavian artery.
Figure 3: Axial view of computed tomography angiography (CTA) showing compression of oesophagus (white arrow) by the retroesophageal aberrant right subclavian artery (white arrowhead).
Figure 4: Computed tomography angiography (CTA) multi-planar reconstruction (MPR) showing the posterior course of the right subclavian artery (white arrow), crossing the mediastinum and compressing the oesophagus. A. coronal view B. Sagittal view

Disclosure

None.

Introduction

​Routine mucosal sampling from pre-defined anatomical sites is recommended during pediatric upper gastrointestinal endoscopy regardless of macroscopic appearance, but evidence for this recommendation remains limited. We investigated this issue in a large and well-defined patient cohort.

Aims & Methods

Concordances between endoscopic and histologic findings and their association to the diagnosis after the first endoscopy was studied in 1181 consecutive children undergoing endoscopy with systematic biopsy sampling during years 2007-2014. Furthermore, the associations between endoscopic and histologic findings and a gastrointestinal diagnosis received during follow-up up to 11 years were studied.

Results

Systematic sampling was conducted in 98.4% of the children in esophagus, 99.3% in stomach, and 98.8% in duodenum. Any endoscopic findings were reported in 51.7% and histologic abnormalities in 59.3% of the children. The Cohen’s k coefficient between endoscopic and histologic findings was 0.272 overall, 0.190 in esophagus, 0.148 in stomach, and 0.520 in duodenum. The most common diagnoses were celiac disease (26.0%), inflammatory bowel disease (11.2%) and gastroesophageal reflux disease (5.2%). Significant association between endoscopic abnormalities and presence of a diagnosis was seen in any endoscopic site and in duodenum, but not in esophagus or stomach, while histologic abnormalities associated with a diagnosis in each biopsy location (Table 1.). Histologic abnormalities were seen in 146 (25.4%) children without diagnosis at the first endoscopy, the most common being chronic inactive gastritis (7.5%) and mild chronic esophagitis (5.8%). Furthermore, in patients without initial diagnosis histologic abnormalities were associated with a follow-up diagnosis (odds ratio 2.59, 95% confidence interval 1.40-4.79), whereas endoscopic abnormalities did not (OR 0.61, 95% CI 0.31-1.18).

Table 1. Relationships between endoscopic and histologic findings and diagnosis received in 1181 children at the time of their first upper gastrointestinal endoscopy.
		N	%	Gastrointestinal diagnosis Odds ratio	95% confidence interval
	Endoscopic findings Any site	610	64.3	2.98	2.35-3.77
Esophagus		125	47.2	0.83	0.57-1.20
Stomach		190	52.6	1.06	0.78-1.45
Duodenum		384	73.4	4.02	3.08-5.24
Histologic findings Any site		700	79.1	30.6	21.8-43.0
Esophagus		238	78.2	4.47	3.20-6.24
Stomach		363	76.3	4.78	3.62-6.33
Duodenum		367	90.1	19.4	13.3-28.4

Conclusion

The low concordance between endoscopic and histologic findings supports systematic biopsy sampling in pediatric upper gastrointestinal endoscopy. Histologic findings are strongly associated with initial gastrointestinal diagnosis and to a lesser extent with a follow-up diagnosis.

Disclosure

Author has no conflict of intrest

Clinical Case Summary

Splenic artery (SA) pseudoaneurysms are rare but potentially fatal complications of pancreatitis, with a mortality rate up to 52%. A good prognosis relies on early diagnosis and treatment.
A 54-year-old man, recently splenectomized due to spleen rupture, and with a history of alcohol and tobacco abuse presented with hematemesis, melena and acute anemia. Upper endoscopy revealed a blood vessel without ulcer in the posterior gastric body (PGB), suggestive of Dieulafoy lesion, treated with adrenaline and through-the-scope clips (TTSC). During hospitalization there were 2 more hemorrhagic events: the first treated with TTSC; the second with an over-the-scope clip (OTSC).
Weeks after being safely discharged, he presented with hematemesis, epigastric pain and acute anemia. A presumed Dieulafoy lesion in the PGB was treated with an OTSC. A computed tomography (CT) scan was performed to exclude vascular malformations, revealing an hipodense heterogeneous area in the pancreas tail contacting the SA and the PGB, suggestive of intraductal papillary mucinous neoplasm with malignant transformation.
An endoscopic ultrasound with biopsy was scheduled but, before that happened, another episode of hematemesis with hemodynamic instability required a CT scan, revealing a pseudocyst adjacent to the PGB. Reviewing his medical records from another hospital: a CT scan performed at the time of the spleen rupture described this collection, consistent with a pseudocyst based on cytology.
Subsequent angiography confirmed a SA pseudoaneurysm communicating with PGB, treated with microcoil embolization. The patient was discharged after an uneventful recovery. No more hemorrhagic events were recorded for 10 months.
SA pseudoaneurysms require a high index of suspicion for early diagnosis. This case emphasizes the importance of reviewing medical records and considering previous evidence of pancreatitis in at risk patients. Prompt diagnosis and intervention can prevent life-threatening hemorrhage.

References

Endoscopic images (a total of 10-12 pictures); CT scan images (a total of 3-4).

Introduction

Though small bowel capsule endoscopy (SBCE) has revolutionised the investigation of the small bowel, small lesions may still go undetected.

Aims & Methods

This was a multicentre study whose primary aim was to assess rates of detection of ampulla of Vater during SBCEs and identify any statistically significant variation between different levels of expertise and different reading speeds.
De-identified SBCE videos, selected randomly, each of 15 minutes duration from the 1^st duodenal image, were uploaded on secure virtual cloud following ethical approval, data protection clearance and patient consent. Patient age, indication for endoscopy and findings were recorded. The videos were reviewed by 7 gastroenterology experts and 6 trainees from 6 European centres. Data regarding detection of ampulla of Vater (AoV) was collected. Each video was analysed initially using the mouse-wheel and then at a speed of 10 frames per second (fps). Data was inputted into SPSS and statistical significance assessed via Kappa coefficient.

Results

Data was collected from 30 patients of whom 63.3% were males and whose median age was 63.5 years +/- 7.164. Commonest indications were iron deficiency anaemia (56.7%), small bowel Crohn’s disease (16.7%) and overt GI bleeding (13.3%). All patients were given a macrogol based osmotic laxative for bowel preparation.
The following mean detection rates of Ampulla of Vater were observed:

• trainees using mouse-wheel; 35% (10.5)
• trainees using 10fps; 21.6% (6.5)
• experts using mouse-wheel; 33.8% (10.1)
• experts using 10fps; 20.4% (6.1)

On only taking into consideration the ampullas detected by the standard, mean detection rates using mouse-wheel for trainees is 68.7% and 82.1% for experts, as compared to 50% for trainees and 55% for experts using 10fps, showing a decline in detection rate with higher reading speed.
Table 1. denotes Kappa and p values obtained by comparison of standard/trainee/expert rates in detection of ampulla of Vater.

	Mouse-wheel		10fps
	Kappa	P value	Kappa	P value
Standard vs Trainees	0.347	0.2	0.400	0.2
Standard vs Experts	0.621	0.2	0.513	0.2
Trainee group	0.135	0.75	0.109	0.2
Expert group	0.493	0.98	0.482	0.2
Trainees vs Experts	0.260	0.2	0.284	0.2

Table 1. Comparison of AoV detection

Mathematical comparison of ampulla detection in mouse-wheel vs 10fps amongst trainees resulted in Kappa 0.323 (p=0.018); and amongst experts resulted in Kappa 0.414 (p=0.01).

Conclusion

The results demonstrate that the rate of concordance in the detection of the ampulla amongst experts declines with a higher speed of reviewing a SBCE. This suggests that pathology may be potentially missed at higher reading speeds especially in the first part of the small bowel where sometimes a SBCE rushes through. A slower reading speed is advisable especially in those with limited experience in reviewing a SBCE.

Clinical Case Summary

Superior mesenteric artery (SMA) syndrome is a rare cause of upper gastrointestinal obstruion.Nutcracker syndrome occurs as the result of external compression of the left renal vein. Although they share a similar pathophysilogy,occurring simultaneously is rare.
Case report 1: 41 years old female presented with chronic postprandial upper abdominal pain, frequent vomiting and marked weight loss.Barium meal showed thickened gastric and duodenal mucosa.Post contrast triphasic CT abdomen followed by 3D CT angiography revealed: dilated stomach, decreased aorta- SMA distance 5.5 mm& decreased aorta – SMA angle 20 degrees, compressed dilated left renal vein between aorta and SMA. She started conservative treatment with dietitian to gain more weight which relieved the pain and decreased vomiting episodes.
Case report 2: 16 years old female presented with persistant vomiting.Upper endoscopy showed dilated stomach with superficial erosions. CT aortic angiography revealed: reduced aorto mesentric angle to 19.8 and aorto mesentric distance reduced to 3.5 cm, proximal dilatation of the stomach, compression of left renal vein between aorta and SMA as well as mild dilated tortous ovarian and pelvic veins.Conservative treatment failed at first and due to refusal of surgery, she was sent for nasojejunal tube to optimize nutrition.
Case report 3: 36 years female patient presented with acute onset abdominal pain, vomiting with history of marked weight loss due to abdominal pain with attacks of hematuria. Barium meal and endoscopy suggested superior mesentric artery syndrome. CT angiography of the abdomen was ordered and showed decreased aorto mesentric angle to 16 and aorto mesentric distance to 2.8 cm, as well as compression of left renal vein between aorta and SMA suggesting SMA syndrome and nutcracker syndrome. Patient refused conservative treatment and refered for gastrojejunostomy and stent placement in the left renal vein for relief of obstruction of nutcracker syndrome.

Introduction

Superior Mesenteric Artery Syndrome (SMAS), also known as Wilkie’s syndrome, is a rare gastrointestinal condition characterized by compression of the distal duodenum between the abdominal aorta (AA) and the superior mesenteric artery (SMA). Evidence on Wilkie’s syndrome is limited, reviews are based on small cohorts and case studies^1-3. Patients with SMAS typically present with postprandial epigastric discomfort, nausea, vomiting, stomach distention, and reflux, leading to malnutrition and weight loss. As mesenteric fat decreases due to the weight loss, the duodenal obstruction worsens, increasing SMAS symptoms. SMAS mostly affects young females aged 20-30 years and is challenging to diagnose due its overlap with dyspepsia, anorexia nervosa and median arcuate ligament syndrome^1-3. SMAS can present acutely, after weight loss from bariatric surgery, malnutrition or (hyper)metabolic disorders. It also presents chronically, due to an anatomically high duodenal position or a low aortic SMA origin^1-3. Diagnostic imaging, including computed tomography (CT) typically reveals a reduced SMA-AA angle <25° and a characteristic vertical, abrupt duodenal obstruction on barium X-ray series^1,2. Conservative treatment focuses on improving gastrointestinal motility and increasing the SMA-AA angle by weight gain to regain mesenteric fat¹. If conservative treatment fails, surgical intervention is considered, with duodenojejunostomy being most commonly performed. Yet, this carries potential severe complications such as anastomotic leakage and non-physiological gastro-duodenal changes. Recent evidence suggests laparoscopic Treitz ligament release may offer a viable and low-risk alternative^3,4.

Aims & Methods

This study aims to introduce a standardized, low risk treatment protocol for SMAS. The protocol includes: 1) Jejunal tube feeding for six-eight weeks, 2) Laparoscopic release of the Treitz ligament, and 3) SMA reimplantation⁵. The protocol was implemented at three hospitals in the Netherlands, Medisch Spectrum Twente, Franciscus, and Erasmus MC.

Results

A total of 12 patients were treated according to this protocol from 2020 until 2024. The cohort consisted of 7 (58.3%) female patients, with a mean age of 29.2 years (SD 11.8). The average weight and body mass index were 60.33 kg (SD 6.5) and 20.01 (SD 3.4). The mean SMA-AA angle measured on CT scans was 22.55° (SD 13.1). All patients started treatment with jejunal feeding. Nine patients underwent laparoscopic Treitz ligament release, with 7 experiencing complete resolution of symptoms. One patient with co-existing Nutcracker syndrome received primary SMA infra-renal reimplantation, with complete symptom release after the surgery. In two patients, symptoms persisted following Treitz ligament release, resembling gastroparesis and irritable bowel syndrome. The 2 remaining patients are planned for laparoscopic Treitz ligament release.

Conclusion

The combination of jejunal feeding followed by laparoscopic Treitz ligament release, represents a safe and effective treatment approach for SMAS; 7/9 patients who underwent the Treitz ligament release experienced resolution of all symptoms, demonstrating the efficacy of protocol. This approach offers an alternative to traditional surgical procedures, such as duodenojejunostomy, and reduces the risks of substantial short- and long-term complications. Further research with larger sample sizes is required to confirm the long-term outcomes and advantages of this protocol compared to other treatment strategies for SMAS.

References

1. Oka A, Awoniyi M, Hasegawa N, Yoshida Y, Tobita H, Ishimura N, Ishihara S. Superior mesenteric artery syndrome: Diagnosis and management. World J Clin Cases. 2023 May 26;11(15):3369-3384. doi: 10.12998/wjcc.v11.i15.3369. PMID: 37383896;
2. Navandhar PS, Shinde RK, Gharde P, Nagtode T, Badwaik N. Understanding Superior Mesenteric Artery Syndrome: Etiology, Symptoms, Diagnosis, and Management. Cureus. 2024 Jun 2;16(6):e61532. doi: 10.7759/cureus.61532. PMID: 38957238; PMCID: PMC11218894
3. Beita AKV, Whayne TF. The Superior Mesenteric Artery: From Syndrome in the Young to Vascular Atherosclerosis in the Old. Cardiovasc Hematol Agents Med Chem. 2019;17(2):74-81. doi: 10.2174/1871525717666190920100518. PMID: 31538906.
4. Fang JF, Wei B, Zheng ZH, Lei PR, Chen TF, Wei HB. Laparoscopic lysis of the ligament of treitz for superior mesenteric artery syndrome. Dig Surg. 2014;31(4-5):291-6. doi: 10.1159/000367670. Epub 2014 Oct 17. PMID: 25342434.
5. Ali T, Tomka J, Bakirli I, Bakirov I. Surgical Treatment of Wilkie's Syndrome by Vascular Transposition. Cureus. 2022 Apr 18;14(4):e24251. doi: 10.7759/cureus.24251. PMID: 35475250; PMCID: PMC9018456.
6. Pourhassan S, Grotemeyer D, Fürst G, Rudolph J, Sandmann W. Infrarenal transposition of the superior mesenteric artery: a new approach in the surgical therapy for Wilkie syndrome. J Vasc Surg. 2008 Jan;47(1):201-4. doi: 10.1016/j.jvs.2007.07.037. Epub 2007 Oct 18. PMID: 17949939