Clinical Case Summary

Achalasia is a rare motor disorder characterized by esophageal aperistalsis and incomplete relaxation of the lower esophageal sphincter, commonly complicated by esophageal dilation with subsequent sigmoidization of the esophagus, pneumonia or lung abscesses, due to food regurgitation and aspiration[1]. Although an esophageal hemorrhage may occasionally occur due to esophageal stasis and esophagitis, severe bleeding from achalasia is exceedingly rare[2],[3].
We present the case of a 52-year-old female with a history of stroke and long-lasting achalasia which presented with sudden onset of hematemesis and altered general status. On admission the patient required fluid resuscitation and blood transfusion but without evidence of active bleeding. An upper gastrointestinal endoscopy was performed that revealed a dilated esophagus with a large adherent blood clot and no signs of active bleeding. After fragmenting and extracting the clot using a Dormia basket, multiple irregular ulcers were observed in the middle and lower esophagus, one with a visible vessel without active bleeding, presumed to be the bleeding source. Endoscopic treatment consisted of hemostasis using a metal clip for the esophageal ulcer with high-risk stigmata and balloon dilation with a 35mm polyethylene balloon for the achalasia. Further treatment included high-dose proton pump inhibitors, parenteral nutrition and the patient was scheduled to return for another session of pneumatic dilation.
The particularity of the case lies in the presence of a rare complication of achalasia, which can predispose to stasis ulcers, leading to possibly life-threatening esophageal bleeding.

References

1. Savarino E, Bhatia S, Roman S, Sifrim D, Tack J, Thompson SK, Gyawali CP. Achalasia. Nat Rev Dis Primers. 2022 May 5;8(1):28.
2. Momodu II, Wallen JM. Achalasia. [Updated 2023 Jul 31]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-
3. Cho JH. Massive esophageal bleeding in long-standing achalasia complicated by esophageal carcinoma and aspirin-induced stasis ulcer: Case report. Medicine (Baltimore). 2019 Jul;98(30):e16519.

Clinical Case Summary

Background: Pancreatic Adenocarcinoma (PA) represents an aggressive disease affected by very poor prognosis worldwide. In the early phases, PA remains asymptomatic favoring delayed diagnosis and affecting the disease prognosis. Recently, Acute Pancreatitis (AP) has been described by some anecdotical cases as a potential early manifestation of PA, in particular if case of pancreatic head development.
Case presentation: An 80-year-old female patient affected by diabetes mellitus type 2 complaining of acute epigastric pain was admitted to the emergency room receiving an AP diagnosis. After 2 days she was hospitalized at Gastroenterology Department to receive medical treatment. On admission, the patient presented hyper-neutrophilia, elevated levels of pancreatic enzymes, cholestasis indexes, and phlogosis markers. Abdominal ultrasound revealed a hypoechoic nodular lesion of 40 mm located at the pancreas body, associated with ectasia of the upstream Wirsung duct. The contrast enhancement US (CEUS), evidenced a hypoenhancement pattern in the arterial phase suggesting the need for a second-level imaging examination. The abdominal CT scan revealed the lesion ventral to the splenic-portal confluence without signs of vascular infiltration, presenting a late contrast enhancement suggestive of a hetero-primitive lesion of the pancreatic body. Echo-endoscopy-guided sampling confirmed the adenocarcinoma diagnosis. After this, the patient received a distal pancreatectomy to achieve surgical radicality. No progression of disease was observed after surgery, and she is currently on stable oncological follow-up.
Conclusion: Even when the neoplasm is not located in the head of the organ, AP has herein been reported as a rare exordial manifestation of PA whose onset can help clinicians to achieve early diagnosis of this aggressive tumor.

References

1. Saluja A, Maitra A. Pancreatitis and Pancreatic Cancer. Gastroenterology. 2019 May;156(7):1937-1940. doi: 10.1053/j.gastro.2019.03.050. Epub 2019 Mar 30. PMID: 30940522.
2. Bezuidenhout AF, Wei PK, Burstein D, Brook A, Brook OR, Freedman SD, Tsai LL. Unexplained Acute Distal Pancreatitis: Association With Subsequent Diagnosis of Pancreatic Cancer. AJR Am J Roentgenol. 2023 Aug;221(2):196-205. doi: 10.2214/AJR.23.28999. Epub 2023 Mar 22. PMID: 36946899.
3. Walling A, Freelove R. Pancreatitis and Pancreatic Cancer. Prim Care. 2017 Dec;44(4):609-620. doi: 10.1016/j.pop.2017.07.004. Epub 2017 Oct 5. PMID: 29132523.

Clinical Case Summary

Background: Mucinous cystic neoplasms are rare benign tumours of the liver, which occur in less than 5% of all liver cysts [1]. However, their etiology is not quite clear, female dominance and estrogen receptor positivity suggest their embryological origin. Without any specific laboratory, biomarker, imaging and cytological characteristics, the diagnosis left for histology, most of the time after variously radical operations.
Aim: The aim of our presentation is to show the severity of evaluating this rare condition through a case report.
Methods: A 27-year-old woman was admitted to our department with jaundice and abdominal pain. Past medical history was irrelevant, she had obesity and smoking as risk factors. Laboratory results showed total bilirubin level of 92.6 μmol/l with elevated obstructive liver enzymes. Abdominal ultrasound revealed a dilatated common bile duct and a 60 mm large septated cystic lesion in the liver. To evaluate the indeterminate etiology, abdominal CT was performed, which described an unspecific hypodensity in the head of the pancreas, and suggested Echinococcus cyst in the liver. Serology was negative twice. Due to progressing jaundice an ERCP was performed. An unremovable mass was found in the hepatic bifurcation, plastic stents were placed. CA19-9 was moderately elevated (156,3 U/ml). For further identification cholangioscopy was performed, where a soft, regular surfaced tissue was found at the level of hepatic bifurcation. Targeted biopsies were undiagnostic. Ultrasound follow up showed slight enlargement of the cystic lesion. Fine needle sampling was postponed several times due to the suspicion of echinococcoid cyst, eventually it was done. No specific cytological diagnosis was made, microbiology identified Acinetobacter baumannii. Lack of clinical and laboratory signs of systematic inflammation, no antibiotic was added. Follow up CT described further enlargement. After almost a half year of diagnostic steps, surgical resection was left to step forward. During the laparotomy huge pathological lymph nodes were seen, therefore left hepatolobectomy, radical lymphadenectomy, and hepato-jejunostomy were performed. Macroscopically two bigger parts were recognizable, one of them grown inside the bile duct. No early complications were noted.
Results: Histological process at first sight seen ovarial-like strome. Estrogen dye was added, which shown high density for oestrogen receptors. Final diagnosis was concluded as mucinous cystic neoplasm with low grade dysplasia.
Conclusion: Mucinous cystic neoplasms are rare benign tumors of the liver. As the case above has shown, diverse and sophisticated diagnostic modalities aren’t sensitive enough to determinate and differentiate cystic liver deformations. Fortunately, no malignant focus was found this time.

References

1.Soni S, Pareek P, Narayan S, Varshney V. Mucinous cystic neoplasm of the liver (MCN-L): a rare presentation and review of the literature. Med Pharm Rep. 2021 Jul;94(3):366-371. doi: 10.15386/mpr-1543. Epub 2021 Jul 29. PMID: 34430860; PMCID: PMC8357353.

Introduction

According to guidelines, in order to strictly diagnose esophageal motility disorders, besides achalasia, high resolution manometry (HRM) findings should be documented in both the upright and the supine position. A common finding among patients with Gastro-Esophageal Reflux Disease GERD submitted to manometry is ineffective esophageal motility (IEM). The clinical impact of such a diagnosis in this setting is ambiguous though it may denote future disturbance of peristaltic reserve even if until now only inadequate response to multiple rapid swallows (MRS) has been correlated to post anti-reflux surgery dysphagia.

Aims & Methods

We aimed to evaluate differences in esophageal contractility in different positions in patients with definite GERD.We included consecutive patients with definite GERD according to Acid Exposure Time (>6%) submitted simultaneously to 24 hours pH impedance off PPI study and HRM in both the upright and supine position. Patients with previous esophago-gastric surgeries as also patients diagnosed with motility disorders besides IEM/absent contractility according to Chicago 4 guidelines were excluded.

Results

In total 49 patients (M/F:26 (53.1%)/23 (46.9%), mean age:51±14) were included. The main referral reason was typical reflux symptoms in 37/49 (75.5%) patients while 8/49 (16.3%) reported dysphagia. Mean Distal Contractile Integral (DCI) was significantly reduced in the upright position (1629.2±1175.6 mm²/s/mmHg) vs supine position (2762.4±2710.4 mm²/s/mmHg), p=0.010. The rate of total ineffective swallows in the upright position was significantly increased in the upright position (29.0%) vs the supine (12.3%), p=0.010 as was also the rate of weak contraction (10.3% vs 3.1%, p=0.033 ) and marginally not of absent contractions (17.0% vs 8.3%, p=0.078). The rate of diagnosis of IEM/absent contractility was doubled in the upright position (8/49, 16.3%) vs in the supine position (4/49, 8.2%) (k-value:0.473, p=0.001). Inadequate post MRS contraction was observed in 9/49 (18.4%) and more specifically among 3/4 (75%) patients with IEM/absent contractility diagnosis in the supine position (k:-0.436, p=0.002) vs 4/8 (50%) in the upright position (k:-0.361, p=0.011). Dysphagia was not correlated to diagnosis of IEM/absent contractility neither in the supine (p=0.675) nor the upright position (p=0.586).

Conclusion

Upright position in HRM is correlated to a decreased mean DCI and an increased rate of ineffective esophageal contractions leading to an increased rate of diagnosis of IEM/absent contractility. Such a diagnosis in the upright position is correlated in a lesser extent to inadequate post-MRS swallow and not correlated to symptom of dysphagia. Until new data arise such an overdiagnosis among patients with GERD may be misleading and therefore the procedure in such patient may be better to be undertaken only in the supine position.

Clinical Case Summary

Background: Eosinophilic oesophagitis (EoE) is a type-2 mediated chronic inflammatory disease affecting the oesophagus, characterized by symptoms related to oesophageal dysfunction, such as dysphagia or persistent reflux symptoms despite optimal PPI therapy, and an elevation of eosinophil counts at different levels of the digestive tract wall, in the absence of other causes of eosinophilia^1,2.
A relationship has been found between EoE and allergic comorbidities and a certain degree of heredity in the pathogenesis of the disease^3,4,5. Histologic diagnosis: ≥15 eosinophils /HPF^6,7,8.
Case report: We evaluated a 28-year-old woman for suspected oesophageal rupture. In anamnesis: hypothyroidism, lactose intolerance, milk protein allergy.
On arrival, the patient complained of retrosternal pain, dysphagia, emesis, and sialorrhea. Immediate EGDS found oesophageal fissuration, extending 26 to 33 cm from dental arch, with rigidity of viscera and vertical grooves from middle oesophagus to cardia (Image 1, 2); multiple biopsies were performed in proximal and distal oesophagus for suspected EoE; naso-digiunal feeding tube was placed for proper feeding and the patient was kept on nihil per os. Histology confirmed the clinical suspicion of eosinophilic esophagitis. After 3 days, progressive reintroduction of diet per os was carried out. Upon histological confirmation, steroid therapy and PPI were set. A chest-CT scan, with and without iv and oral contrast showed spillage of contrast from the oesophageal lumen, as for mucosal tear, without clear signs of perforation (Image 3). Upon remission of symptoms, the patient was discharged with a soft diet, full dose proton pump inhibitors an systemic steroid on a sliding scale. Oral Budesonide was introduced in one week when the systemic steroid was suspended. One month after discharge, the patient had a complete remission of symptoms. Three months endoscopic follow-up confirmed total regression of the lesion (Image 4).

References

1. Hahn J.W., et al. Global Incidence and Prevalence of Eosinophilic Esophagitis, 1976–2022: A Systematic Review and Meta-analysis. CGH 2023 doi: 10.1016/j.cgh.2023.06.005
2. Dellon E.S. et al. International Consensus Recommendations for Eosinophilic Gastrointestinal Disease Nomenclature. CGH Off. Clin. Pract. J. Am. Gastroenterol. Assoc. 2022;20:2474–2484.e3. doi: 10.1016/j.cgh.2022.02.017.
3. Guajardo J.R., et al. Eosinophil-associated gastrointestinal disorders: A world-wide-web based registry. J. Pediatr. 2002;141:576–581. doi: 10.1067/mpd.2002.127663.
4. Díaz del Arco C., et al. Eosinophilic colitis: Case series and literature review. Pathol. Res. Pract. 2018;214:100–104. doi: 10.1016/j.prp.2017.09.029.
5. Visaggi P., Savarino E. Et al Six-Food Elimination Diet is Less Effective During Pollen Season in Adults with Eosinophilic Esophagitis Sensitized to Pollens. Am. J. Gastroenterol. 2023 doi: 10.14309/ajg.0000000000002357.
6. Lucendo A.J. et al. Guidelines on eosinophilic esophagitis: Evidence-based statements and recommendations for diagnosis and management in children and adults. UEG J. 2017;5:335–358. doi: 10.1177/2050640616689525
7. Hirano I., et al. AGA Institute and the Joint Task Force on Allergy-Immunology Practice Parameters Clinical Guidelines for the Management of Eosinophilic Esophagitis. Gastroenterology. 2020;158:1776–1786. doi: 10.1053/j.gastro.2020.02.038.
8. de Bortoli N., et al. Eosinophilic esophagitis: Update in diagnosis and management. Position paper by SIGE Dig. Liver Dis. 2017;49:254–260. doi: 10.1016/j.dld.2016.11.012

Disclosure

None