Clinical Case Summary

Background: Dysphagia represents a relatively common entity determined by a heterogeneous spectrum of frequent conditions ranging predominantly from oesophageal organic lesions (benign or malignant) to motility dysfunctions. However, rare extra-oesophageal causes of dysphagia, including neuromuscular disorders or mediastinum disorders, have been described.
Case presentation: A 61-year-old male patient was admitted to our department complaining of progressive worsening oropharyngeal dysphagia, especially for solid food. Six months before, he received an upper gastrointestinal barium X-ray showing no specific signs.
On admission, the diagnostic work-up included esophagogastroduodenoscopy (EGDS) and manometry: EGDS highlighted a hypopharyngeal sub-stenosis requiring the use of a paediatric endoscope to pass through it, while manometry evidenced an unspecific triple pressure trace at the upper oesophageal sphincter level suggesting an ab extrinsic compression. Moreover, an expert multidisciplinary “dysphagia” team composed of gastroenterologists, endocrinologists, otolaryngologists, and neurologists, excluded thyroid-related determinants, and ruled out other common causes of dysphagia.
Considering these findings, a neck-column CT scan was performed and the following radiological criteria of Diffuse Idiopathic Skeletal Hyperostosis (DISH) or “Forestier Syndrome” were revealed: flowing ossifications mainly along the anterior longitudinal ligament of at least four contiguous vertebrae with preserved inter-articular discs height and absence of apophyseal joints. Finally, the patient was surgically managed gaining the complete resolution of his symptoms.
Conclusion: DISH represents a rare cause of dysphagia. Clinicians should be aware that the diagnostic work-up of “unexplained” dysphagia should routinely include extra-oesophageal conditions, especially the rare ones.

References

1. Mazières B. Diffuse idiopathic skeletal hyperostosis (Forestier-Rotes-Querol disease): what's new? Joint Bone Spine. 2013 Oct;80(5):466-70. doi: 10.1016/j.jbspin.2013.02.011. Epub 2013 Apr 6. PMID: 23566663.
2. Karaarslan N, Gürbüz MS, Çalışkan T, Simsek AT. Forestier syndrome presenting with dysphagia: case report of a rare presentation. J Spine Surg. 2017 Dec;3(4):723-726. doi: 10.21037/jss.2017.11.05. PMID: 29354755; PMCID: PMC5760404
3. Legaye J. Forestier's syndrome : a rare cause of dysphagia. A case report and review of the literature. Acta Orthop Belg. 2020 Jun;86(2):216-219. PMID: 33418609.

Clinical Case Summary

A 67-year-old man initially presented in 2020 with intermittent dysphagia for solid food. An endoscopy of the upper gastrointestinal tract revealed an inflammatory, presumed peptic stenosis at the level of the Z-line. No hiatus hernia was present. A balloon dilatation was performed and therapy with proton pump inhibitors was started. This maintained remission until 2023, when he represented with frequent bolus retention, retrosternal chest pain and regurgitation. A repeat endoscopy appeared normal; however, biopsies revealed lymphocytic esophagitis. A high-resolution oesophageal manometry was performed to clarify the cause of dysphagia. Water swallows showed no abnormal findings; however, the patient had repeated, symptomatic outlet-obstruction during the solid test meal. This finding is often seen in eosinophilic esophagitis.¹ The diagnosis of primary lymphocytic esophagitis, considered to be a variant of this condition,² was proposed. Treatment with oro-dispersible budesonide (1mg bid) was started.
At the same time, an abnormal AV conduction pattern was noted on ECG. Echocardiography and a cardiac stress test were normal; however, CT imaging showed bihilar lymphadenopathy with peri-lymphatic pulmonary nodules. Broncho-alveolar lavage showed lymphocytic alveolitis (29% lymphocytes) and an elevated CD4/CD8 ratio (ratio = 4). ACE (51 U/l) was normal and granulomas were not seen on fine needle aspiration of the lymph nodes; however, the diagnosis of stage 2 pulmonary sarcoidosis was confirmed on PET-CT. Oral prednisolone (30mg od) replaced locally active steroids. The patient was symptom free after one month treatment.
Sarcoidosis is a granulomatous disease that often affects the lungs, skin and lymph nodes; however, involvement of the digestive tract is rare, and secondary lymphocytic esophagitis has hardly ever been described.³ The investigation, primary or secondary aetiology, and management of dysphagia in this case of lymphocytic esophagitis will be discussed.

References

1. Sykes C, Fairlamb G, Fox M, et al. Assessment of Esophageal Motility in Patients With Eosinophilic Esophagitis: A Scoping Review. J Clin Gastroenterol 2023;57:10-30.
2. Greuter T, Straumann A, Fernandez-Marrero Y, et al. Characterization of eosinophilic esophagitis variants by clinical, histological, and molecular analyses: A cross-sectional multi-center study. Allergy 2022;77:2520-2533.
3. Brito-Zeron P, Bari K, Baughman RP, et al. Sarcoidosis Involving the Gastrointestinal Tract: Diagnostic and Therapeutic Management. Am J Gastroenterol 2019;114:1238-1247.

Disclosure

None of the authors have any conflict of interest to disclose

Clinical Case Summary

Closure of mucosal defects after endoscopic submucosal dissection (ESD) may reduce the risk of postprocedural perforation and bleeding. However, defect closure with traditional through-the-scope (TTS) or over-the-scope (OTS) clips and OTS suturing devices may be technically limited by defect size and location. The development of a new through-the-scope helix tack suture system (TTSS) has emerged as a promising tool for overcoming these limitations, particularly when combined with TTS clips.

We present two cases of gastric ESD defect closure with TTSS and adjunctive TTS clip application, highlighting the outcomes and main challenges to achieve complete defect closure.

CASE 1: 73-year-old woman with a 15mm Paris 0-IIb dysplastic lesion of the posterior wall of the antrum. En-bloc ESD was performed and the 25mm mucosal defect was closed with TTSS in a ‘Z’ pattern. However, suboptimal tack placement resulted in incomplete closure of the central portion of the scar, leaving two areas of exposed submucosa at the edges. Complete defect closure was accomplished with the additional placement of four TTS clips at the edges, only possible due to the previously achieved proximity.

CASE 2: 55-year-old man referred for ESD of a 13mm Paris 0-IIa dysplastic lesion in the lesser curvature of the antrum. En-bloc ESD was accomplished, and the resulting 25mm mucosal defect was closed using TTSS in a 'Z' pattern. Complete defect closure was achieved with the additional placement of one TTS clip.

Both patients were discharged the following day and presented with no complications at 1-month follow-up.

These cases highlight the potential of TTSS becoming a practical solution for the closure of complex post-ESD defects, allowing earlier patient discharge. While this novel TTSS with adjunctive TTS clip application appears to be effective in the management of sizable defects, proper suture pattern and tack placement are paramount for achieving complete defect closure.

References

1. Kobara H, Tada N, Fujihara S, Nishiyama N, Masaki T. Clinical and technical outcomes of endoscopic closure of postendoscopic submucosal dissection defects: Literature review over one decade. Dig Endosc. 2023 Jan;35(2):216-231.
2. Hernandez-Lara A, Garcia Garcia de Paredes A, Rajan E, Storm AC. Step-by-step instruction: using an endoscopic tack and suture device for gastrointestinal defect closure. VideoGIE. 2021;6(6):243-245.
3. Hernandez A, Marya NB, Sawas T, Rajan E, Gades NM, Wong Kee Song LM, et al. Gastrointestinal defect closure using a novel through-the-scope helix tack and suture device compared to endoscopic clips in a survival porcine model (with video). Endosc Int Open. 2021 Apr;9(4):E572-E577.
4. Canakis A, Dawod SM, Dawod E, Simons M, Di Cocco B, Westerveld DR, et al. Efficacy, Feasibility, and Safety of the X-Tack Endoscopic HeliX Tacking System: A Multicenter Experience. J Clin Gastroenterol. 2024 Jan 29.
5. Canakis A, Deliwala SS, Frohlinger M, Twery B, Canakis JP, Shaik MR, et al. Endoscopic outcomes using a novel through-the-scope tack and suture system for gastrointestinal defect closure: a systematic review and meta-analysis. Endoscopy. Published online March 22, 2024.

Clinical Case Summary

We report the case of a 64-year-old man with preexisting hepatic sarcoidosis complicated by liver cirrhosis, who presented with confusion as an initial manifestation of COVID-19 confirmed by rapid Antigenic test. His Child Pugh score was 8 (Class B) and MELD was 16. Chest radiographs showed bilateral patchy interstitial opacities. Computed tomography (CT) of the brain was normal. Lumbar puncture was not performed because of a marked thrombocytopenia. Urine cytobacteriological examination did not show a urinary tract infection. An abdominal ultrasound showed normal hepatoportal flow and a mild ascites. Ascitic fluid analysis did not show spontaneous bacterial peritonitis. Since the patient was cirrhotic, we have considered the diagnosis of hepatic encephalopathy as a possible explanation for the neurological symptoms, so he started Lactulose and Rifaximin. However, his clinical condition showed no improvement, the neurological disorders remained the same. A brain MRI showed in FLAIR MR sequences a few hyperintensities punctiform in the white matter of the supratentorial region and the subcortical of the left insula region. There were no signs of a cerebral granuloma nor toxoplasmosis. It did not show venous thrombosis. There were no microheamorragies neither ischemic lesions. After careful evaluation by neurologic experts, encephalitis associated with SARS-CoV-2 infection was concluded. The patient was treated by intravenous Dexamethasone and he fully recovered without sequelae. This case illustrates the fact that neurological manifestations associated with COVID-19 infection are not necessarily a reflection of critical illness, and should incite clinicians to actively look for any evidence of COVID-19 infection as a differential diagnosis in patients with preexisting cirrhosis, presenting with neurological symptoms, during the epidemic period of COVID-19.

Introduction

Chronic liver injury ultimately progresses to the development of cirrhosis. Patients with cirrhosis can be in a compensated or decompensated phase, the latter marked by clinical events such as ascites, hepatic encephalopathy and variceal bleeding. These events are associated with significant morbidity and mortality and the management is challenging and labor-intensive. Due to ongoing unhealthy lifestyle factors resulting in chronic liver injury, the burden of cirrhosis on healthcare systems in Europe is increasing. There is however limited data on the impact of cirrhosis on Dutch healthcare resources.

Aims & Methods

We aimed to determine the point-prevalence and claimed health costs of adults (≥ 18 years) registered as patients with cirrhosis at Dutch hospitals. To this end we extracted health claims data (timeframe 2017-2021) from the records of the Dutch health claims database (Vektis), which covers almost all inhabitants of the Netherlands. We used diagnosis codes ‘compensated cirrhosis’ and ‘decompensated cirrhosis’ to identify patients.

Results

The point prevalence of patients with cirrhosis increased from 48,7 patients per 100.000 adult Dutch inhabitants in 2017 to 75,2 per 100.000 in 2021 (+54%). The point-prevalence for cirrhosis was highest in the province of Limburg with 105,6 patients per 100.000 adult Dutch inhabitants. The annual increase in unique new patients for which hospitals claimed costs was n=3.725 in 2018, n=3.840 in 2019 (+3%), n=3.749 in 2020 (-2%) and n=3.695 in 2021 (-1%). The largest increase was observed in the province of Zuid-Holland (approximately 5 new patients per 100.000 adult Dutch inhabitants per year). Total number of hospital admissions increased with 19% from 2.443 admissions in 2017 to 2.899 admissions in 2021. The median length of stay for admitted patients with cirrhosis in 2017-2021 was four days [IQR 2-7 days]. The annual reported costs for patients with cirrhosis increased from €35 million in 2017 to €78 million in 2021 (+120%).

Conclusion

The point-prevalence of Dutch adults registered as a patient with cirrhosis in Dutch hospitals increased by more than fifty percent, with remarkable regional differences. Consequently, the total healthcare costs claimed for these patients more than doubled in less than five years.

Introduction

Functional gastrointestinal disorders (FGIDs) are more prevalent in female than in male patients [1,2]. This triggers the question whether this is also reflected in medical care, especially also in self-medication, where often botanicals are used. A potential source of data to address this question is the PhytoVIS study, a pharmacoepidemiological study conducted in Germany in more than 20,000 patients taking herbal medicinal products.

Aims & Methods

To have a large and homogenous data base for a gender-related evaluation, records for a clinically proven herbal medicinal product (HMP) often used in FGIDs, STW 5 [3,4], were evaluated to uncover potential gender-related differences in the pharmacotherapy of FGIDs.

Results

1513 records were evaluated, 71 % of them from females and 29 % from males, i.e. a 4 % higher proportion of females compared to the overall population of the PhytoVIS study. Main criterium for the evaluation was the Clinical Global Impression Scale – Efficacy (CGI-E). No major differences between females and males were found, apart that women perceived a minimally better effect of the therapy. The reported symptoms were differentiated into symptoms associated with functional dyspepsia (FD), irritable bowel syndrome (IBS), or symptoms that can be associated with both. As far as the records allow a differentiation, no gender differences could be observed. This applies also to the perceived efficacy, the tolerability and the onset of action.

Conclusion

So, while there are differences in the prevalence of FGIDs in women and men, there are no therapeutically relevant pharmacoepidemiological differences in patients with functional gastrointestinal diseases treated with a medicinal product indicated in these diseases, so suggesting that there is no gender gap in this highly relevant therapeutic field.

References

1. Kim YS, Kim N. Sex-Gender Differences in Irritable Bowel Syndrome. J Neurogastroenterol Motil. 2018;24:544-558
2. Houghton LA, Heitkemper M, Crowell M, Emmanuel A, Halpert A, McRoberts JA, Toner B. Age, Gender and Women's Health and the Patient. Gastroenterology. 2016; S0016-5085(16)00183-9;
3. Malfertheiner P. STW 5 (Iberogast) Therapy in Gastrointestinal Functional Disorders. Dig Dis. 2017;35 Suppl 1:25-29
4. Madisch A, Vinson BR, Abdel-Aziz H, Kelber O, Nieber K, Kraft K, Storr M. Modulation of gastrointestinal motility beyond metoclopramide and domperidone : Pharmacological and clinical evidence for phytotherapy in functional gastrointestinal disorders. Wien Med Wochenschr. 2017;167:160-168

Disclosure

JW and EC were interns, OK, is employee of Steigerwald Arzneimittelwerk GmbH, Darmstadt, Germany, KN und KK have received fees from the same company.