Clinical Case Summary

An 81years-old man with a medical history of recently diagnosed bullous pemphigoid, without skin lessions at presentation and reporting corticoid withdrawal, presented to the emergency department with hemodynamic instability and melena, complaining for vague chest pain. Patient was admitted to the hospital and gastroscopy was performed. Obliteration of oesophageal mucosa throughout the oesophagus and deep, diffuse esophageal lacerations extending down to the muscle layer with coexisting hemorrhagic content and thrombi in the lumen, were noted (picture 1). Differential diagnosis included either traumatic oesophageal rupture or bullous pemphigoid with oesophageal involvement. Urgent computed tomography revealed dilated oesophagus, filled with content but no free air or liquid in the mediastinum (picture 2). Based on clinical history, pemphigoid oesophageal involvement was the most prominent diagnosis. Laboratory workup revealed persistent activated partial thromboplastin time (aPTT), with an otherwise normal coagulation panel. Since there was no history of heparin treatment, other disorders such as antiphosholipid syndrome were excluded. Laboratory tests confirmed the presence of a factor VIII acquired inhibitor and ultimately led to the diagnosis of acquired haemophillia A, which was triggrered by patient’s autoimmube disorder. Therefore a six-week course of oral corticoids, cyclophosphamide and eptacog alfa as bolus intravenous infusion (an activated recombinant coagulation factor VII) were administered. Repeat endoscopy a month after admission revealed complete mucosal healing of the oesophagus (picture 3). Oral diet was re-initiated and the patient was soon discharged with no further complications. Our case indicates that systemic diseases can have a severe impact on gastrointestinal tract and past medical history must always be taken under consideration no matter how severe the endoscopic findings may be [1].

References

1) Gaspar, R et al. Bullous pemphigoid: extensive esophageal involvement. Gastrointest Endosc 2017; 86: 400–402.

Clinical Case Summary

A 38-year-old man consulted the emergency department for acute retrosternal pain and haemoptysis for 72 hours. In the anamnesis he reported intermittent dysphagia for the last 10 years. The X-ray showed a pneumonic infiltrate, and the study was completed with a thoracic CT scan showing pneumomediastinum as indirect evidence of oesophageal perforation (Image 1). The oesophagogram showed no evidence of a solution of continuity (Image 2). Conservative treatment was started with absolute diet, piperacillin-tazobactam, linezolid and high-dose omeprazole. On admission, gastroscopy showed pseudodiverticula in the distal oesophagus (Image 3), with one of them showing friability and fibrin remnants (Image 4) as well as a partially stenosing oesophageal ring (Image 5). Biopsies were taken, ruling out eosinophilic oesophagitis and malignancy. A new oesophagogram was performed showing slow oesophageal emptying and gastro-oesophageal reflux (Image 6). In view of the good clinical evolution, the patient was discharged with omeprazole at standard doses.
In our case, the patient presumably had an oesophageal motor disorder and gastro-oesophageal reflux, in fact indirectly visualised in the oesophagorama. This could have conditioned the development of diverticula and oesophageal ring of probable peptic origin (1). As a consequence, he presented an oesophageal impaction that ultimately precipitated the perforation (2).

References

Image 1: Contrast-enhanced thoracoabdominal CT: concentric thickening of the lower third of the oesophagus with moderate amount of adjacent pneumomediastinum.
Image 2: Oesophagogram with barium contrast, with no evidence of a solution of continuity in the oesophagus.
Image 3: Distal oesophagus with presence of diverticula.
Image 4: Diverticulum in distal oesophagus with haematic debris covered with fibrin.
Image 5: Distal oesophagus with partially stenosing oesophageal ring.
Image 6: Barium contrast esophagogram: irregular esophageal surface with slowed emptying, suggestive of motor disorder. Moderate gastro-oesophageal reflux.
1. Herbella F et al. Dis Esophagus 2012
2. Smith C.D. et al. The Surgical Clinics of North America 2015

Disclosure

I don't declare any Conflict of Interest.

Clinical Case Summary

A 65-years-old male with a history of familial adenomatous polyposis (FAP) was scheduled for duodenal endoscopic mucosal resection (EMR) of a 2 cm lateral spreading tumor (IIa in Paris classification), located in the lower segment of the duodenal bulb with a small fraction of intrapyloric extension. Due to lesion’s location, initial attempts to adequately lift or visualize the polyp in full extent using a gastroduodenoscope with or without a mounted cap were unsuccessful (Figure 1). Colonoscope use had no effect as well. Only when we mounted an endocuff (ENDOCUFF VISION™ ) on the tip of the colonoscope we did manage to get a better fixed scope position, maximization of the endoscopic view and properly lift and resect the lesion in piecemeal EMR fashion (Figure 2). This was due to the stabilization of the scope intrapylorically with the help of the endocuff arms (Figure 3). Argon plasma coagulation was performed at the rims of the polypectomy, so as to reduce recurrence rate. Pathology report noted a tubulovillous adenoma with low and focal high-grade dysplasia. Patient is re-scheduled for repeat endoscopy in 3 months. To our knowledge this is the first documented case of a duodenal EMR performed with an endocuff assisted colonoscope in the literature. This report underlines the ability of the endocuff to achieve better fixed scope position in defiant duodenal polyps [1,2].

References

[1]. GA Paspatis et al. The use of endocuff for the facilitation of endoscopic mucosal resection in defiant colonic polyps. A case series. Hippokratia 2021; 25: 134–137.
[2]. Vanbiervliet Geoffroy et al. Endoscopic management of superficial nonampullary duodenal tumors: European Society of Gastrointestinal Endoscopy (ESGE) Guideline. Endoscopy 2021; 53: 522–534.

Introduction

Autoimmune bullous diseases (AIBDs) are a group of rare dermatoses characterized by the presence of blisters on the skin and erosions on mucous membranes, induced by autoantibodies against cell adhesion molecules. The two main categories of AIBDs are pemphigus and pemphigoid diseases, which differ in the specific proteins targeted and the level of blister formation.
Pemphigus disorders are intraepithelial blistering diseases where autoantibodies target desmosomal proteins, leading to loss of cell-cell adhesion within the epidermis. This results in flaccid blisters and erosions on the skin and mucous membranes and does not heal with scarring.
Pemphigoid disorders, on the other hand, are subepithelial blistering diseases characterized by autoantibodies against components of the basement membrane zone. These interactions lead to tense blisters and erosions, primarily affecting the skin and sometimes mucosal surfaces. Lesions appear at sites subjected to trauma, and usually heal with scarring and may occur on the mucous membranes of the eyes, nose, oral cavity, laryngopharynx, and esophagus and can lead to serious complications such as scar formation with stenosis, sometimes requiring tracheostomy and esophageal dilatation.
Esophageal erosions, strictures and stenosis can be found on endoscopy and can significantly affect the quality of life of patients. Due to the rarity of these diseases, endoscopic prevalence and features of AIBD-associated esophageal lesions are not well known.

Aims & Methods

The aim of the present study is to assess the esophageal involvement of AIBD-associated mucosal lesions.

Results

A total of 68 patients with AIBD were selected. They were 37 female and 31 male, mean age 56.7 years. Among the patients, 31 (45.6%), 26 (38.2%) and 11 (16,2%) were classified into the epidermolysis bullosa acquisita, mucous membrane pemphigoid, and pemphigus vulgar groups, respectively. Esophageal involvement was seen in 33 (48.5%) patients, most of them with epidermolysis (n=18, 26.5%), followed by pemphigus (n=8, 11.8%) and mucous membrane pemphigoid (n=5, 7.3 %). All patients with esophageal involvement had oral ulcerations and 51% also had pharyngeal lesions. Most of lesions such as erosion, ulcers and vesicles were seen in the proximal esophagus as well as esophageal strictures that were observed in 3 (9%) patients with epidermolysis. Fluoroscopy-guided dilatations were performed in these patients without any complications. Gastroesophageal reflux disease was only noticed in 2 patients.

Conclusion

Odynophagia and/or dysphagia does not predict AIBD-associated esophageal injury since involvement of this organ is usually associated with oral and pharyngeal lesions. Upper endoscopy should be done in all symptomatic patients. Esophageal biopsies should be avoided in order to prevent scar formation and stenosis especially in the group with pemphigoid disorders (subepithelial blistering disease).

References

1. van Beek N, Holtsche MM, Atefi I, Olbrich H, Schmitz MJ, Pruessmann J, Vorobyev A, Schmidt E. State-of-the-art diagnosis of autoimmune blistering diseases. Front Immunol. 2024 Jun 6;15:1363032. doi: 10.3389/fimmu.2024.1363032
2. Daniel BS, Murrell DF. Review of autoimmune blistering diseases: the Pemphigoid diseases. J Eur Acad Dermatol Venereol. 2019 Sep;33(9):1685-1694. doi: 10.1111/jdv.15679.

Clinical Case Summary

We present the case of a 37-year-old man referred to our outpatient clinic with a 5-year-history of eosinophilic gastritis (EoG) refractory to proton pump inhibitors, sodium cromoglicate, corticosteroids and elimination diet. He had watery diarrhea [Bristol Stool Scale (BSS)=7], vomiting and dysphagia for solid food [Dysphagia symptom questionnaire (DSQ)=63]. An esophagogastroduodenoscopy (EGD) showed erythema and granularity of the duodenal and gastric mucosa and white exudates on esophageal mucosa. Histologically, >20 eosinophils (eos)/high power field (HPF) were found in esophageal mucosa (fig.1A) and >30 eos/HPF in gastric and duodenal mucosa (fig.1B). Therefore, EoG was confirmed and a novel diagnosis of eosinophilic esophagitis (EoE) and eosinophilic duodenitis (EoD) was made. The allergology evaluation showed multiple allergies, severe atopic dermatitis (AD) [Eczema Area Severity Index (EASI)=60] and chronic rhinosinusitis with nasal polyps (CRSwNP). To exclude other causes of eosinophilic infiltration ileocolonoscopy, abdominal MRI and tests for hematologic malignancies were performed.
A therapy with dupilumab 300 mg every other week was started for AD, and then optimized for EoE (300 mg/week) after EMA approval. After six months, the patient achieved remission of AD (EASI=8) and improvement of CRSwNP, dysphagia (DSQ=35), diarrhea (BSS=4), vomiting and dyspepsia. New EGDs performed after six and twelve months from dupilumab start showed a normal esophagus and erosions of the gastric mucosa. Eosinophils were still present in the duodenal and gastric mucosa (>30 eos/HPF) with an isolated eosinophilic foveolar abscess; interestingly, no eosinophils were found in the esophageal mucosa (fig.2).
In conclusion, our case support the efficacy of dupilumab for the treatment of concurrent EoE, AD and CRSwNP. Moreover, it gives an intriguing starting point to discuss about the pathogenesis and the different therapeutic response of eosinophilic gastrointestinal diseases.

References

Figure 1: Hematoxylin and eosin stain before dupilumab therapy shows esophageal mucosa with papillary hyperplasia, acanthosis and diffuse intraepithelial eosinophilic exocytosis (eosinophilic count: 20/HPF) (A). Gastric antral and oxyntic mucosa and duodenal mucosa with marked inflammatory infiltrate consisting mainly of eosinophilic granulocytes and lymphatic nodular aggregates (severe eosinophilic esophagitis and gastro-duodenitis) (B).
Figure 2: Hematoxylin and eosin stain six (A) and twelve (B) months after dupilumab induction shows esophageal mucosa with acanthosis, spongiosis, mild dilation of the intercellular spaces of the epithelium and absence of intraepithelial eosinophils; antral and oxyntic gastric mucosa with moderate inflammatory lymphoplasmacytic and eosinophilic infiltrate (>30 eosinophilic granulocytes/HPF), isolated eosinophilic micro-abscess and hyperplastic-regenerative aspects of gastric pits. The H. Pylori Giemsa stain was negative.

Introduction

Gastroesophageal reflux disease (GORD) is a recognised complication of bariatric surgery; however, the clinical phenotype of patients who develop postoperative GORD remains poorly characterised. Improved understanding of this cohort may aid in risk stratification and management.

Aims & Methods

This study aimed to identify the epidemiological and clinical features of patients with upper gastrointestinal symptoms following bariatric surgery and to evaluate whether these vary by surgery type. This retrospective study examined 31 post-bariatric surgery patients with upper gastrointestinal symptoms referred to a quaternary gastroenterology service between 2004 and 2023. Data collected included age, sex, comorbidities, type and number of bariatric procedures, time from surgery to symptom onset, and symptom profile.

Results

Patients were predominantly female (80.6%) with high prevalence of comorbid depression (45.2%) and anxiety (53.3%). Sleeve gastrectomy was the most common procedure (70.9%), followed by adjustable gastric banding (35.5%); 16.1% patients underwent more than one bariatric surgery. On average, symptoms developed 0.19 years postoperatively and persisted for a mean duration of 6.8 years before referral. Dysphagia and heartburn were reported in 77.4% and 74.2% of patients, respectively. All patients who underwent Roux-en-Y gastric bypass (RYGB) reported heartburn. Patients with a history of sleeve gastrectomy appeared to have the highest symptom burden.

Conclusion

Patients who develop GORD after bariatric surgery are predominantly female, frequently have pre-existing mental health conditions, and often undergo sleeve gastrectomy. Symptoms typically emerge early and persist for several years before being investigated. Upper gastrointestinal symptoms appeared more prevalent than previously reported, suggesting possible under-recognition in clinical practice. Although RYGB is the preferred bariatric procedure in patients with co-existing GORD, our findings indicate that significant reflux symptoms may still occur postoperatively. These results support the need for early identification perioperatively and tailored follow-up of at-risk patients.

References

1. Klaus, A., & Weiss, H. (2008). Is preoperative manometry in restrictive bariatric procedures necessary? Obesity Surgery, 18(8), 1039–1042. https://doi.org/10.1007/s11695-007-9399-1
2. Tolone, S., Savarino, E., & Yates, R. B. (2016). The impact of bariatric surgery on esophageal function. Annals of the New York Academy of Sciences, 1381(1), 98–103. https://doi.org/10.1111/nyas.13107
3. Savarino, E., Marabotto, E., & Savarino, V. (2018). Effects of bariatric surgery on the esophagus. Current Opinion in Gastroenterology, 34(4), 243–248. https://doi.org/10.1097/mog.0000000000000439
4. Nguyen, A. D. (2021). Impact of bariatric surgery on gastroesophageal reflux disease and esophageal motility. Current Opinion in Gastroenterology, 37(4), 364–371. https://doi.org/10.1097/mog.0000000000000745
5. Kalarchian, M. A., King, W. C., Devlin, M. J., White, G. E., Marcus, M. D., et al. (2017). Surgery-related gastrointestinal symptoms in a prospective study of Bariatric Surgery Patients: 3-year follow-up. Surgery for Obesity and Related Diseases, 13(9), 1562–1571. https://doi.org/10.1016/j.soard.2017.03.028
6. O’Rourke, R. W., Deveney, C. W., McConnell, D. B., Wolfe, B. M., & Jobe, B. A. (2007). Esophageal motility disorders after gastric banding. Diseases of the Esophagus, 20(3), 269–273. https://doi.org/10.1111/j.1442-2050.2007.00685.x

Disclosure

Nil

Clinical Case Summary

Introduction: Oesophageal perforation secondary to endoscopic variceal ligation (EVL) is extremely rare and most reported instances uniformly resulted in fatal outcomes, ultimately succumbing to septic shock [1, 2, 3, 4].
Discussion: A 61-year-old man with Child B HBV-related cirrhosis and clinically significant portal hypertension underwent EVL as primary prevention for high-risk varices. After 3 weeks, an urgent endoscopy performed for melena revealed active bleeding with severe spurting from one of two band ulcers and an underlying visible muscle layer with a suspected perforation.
Previous orotracheal intubation and hemodynamic support due to the onset of hemodynamic instability, two Over-The-Scope Clips (OTSC) were deployed onto the bleeding ulcer to entirely encompass and close the defect. Due to the persistent variceal oozing a partially covered oesophageal metal stent (SEMS, 18 mm x 12 cm) was inserted with immediate haemostasis.
A subsequent computed tomography (CT) scan confirmed the presence of an inferior paraesophageal extraluminal area (30x15mm) consistent with the perforation.
Thus, an urgent trans jugular intrahepatic portosystemic shunt (TIPS) was performed, lowering the portosystemic gradient from 16 to 9 mmHg. The oesophageal SEMS was removed during an esophagogastroduodenoscopy (EGD), and the site of the oesophageal perforation progressively healed.
The clinical course was characterized by severe sepsis, which improved over time, and after 44 days the patient was discharged.
No further episodes of gastrointestinal bleeding occurred and at the 6-month follow-up EGD after the acute event only residual low-risk varices were observed, with no OTSC in place.
Conclusion: This case highlights how, in select scenarios, such a severe complication can be effectively managed through a prompt endoscopic treatment of post EVL perforation and successful resolution of portal hypertension. This may help alter the otherwise dreadful course of this complication.

References

Images:
- Active oesophageal bleeding with severe spurting from one band ulcer ;

1. Wu C-M, Ging Ho Lo, Lin S-L et al. Esophageal Rupture After Endoscopic Banding Ligation for Esophageal Varices Bleeding. Journal of Intensive Care Medicine. 2002;17(4):195-198. doi:10.1177/0885066602017004007
2. Schoonbroodt D; A Zipf; M Jung et al. Local necrosis and fatal perforation of oesophagus after endoscopic ligation. Lancet (London, England) vol. 344,8933 (1994): 1365. doi:10.1016/s0140-6736(94)90723-4
3. Hou M C; Lin H C; Chang F Y et al. Oesophageal perforation following endoscopic variceal ligation and balloon tamponade. Journal of gastroenterology and hepatology vol. 9,6 (1994): 659-62. doi:10.1111/j.1440-1746.1994.tb01582.x
4. Vijayvargiya Prakhar; Bhamidimarri Kalyan; Grewal Priya et al. Delayed Esophageal Perforation after Treatment for Recurrent Esophageal Variceal Bleeding: 484. American Journal of Gastroenterology 106(): p S188, October 2011. doi: 10.14309/00000434-201110002-00484