Introduction

The Chicago classification 4.0 (CC4) definition of esophagogastric junction (EGJ) outflow obstruction (EJGOO) was made more stringent than the CC3 iteration to limit overdiagnosis of a disorder of undetermined significance. A clinically relevant EGJOO (Cr-EGJOO) diagnosis in CC4 requires a combination of relevant symptoms, high resolution manometric (HRM) criteria, and adjunctive tests instead of elevated supine median integrated relaxation pressure (IRP) alone in CC3.

Aims & Methods

We aimed to explore the importance of each component (symptoms, HRM, and adjunctive tests) in excluding or establishing Cr-EGJOO.
We included all patients undergoing esophageal HRM since 01/2020-12/2021, (both CC3 and CC4 protocols).
We excluded patients with inadequate tests. Positive symptoms were dysphagia/chest pain.
Adjunctive tests: Barium esophagrams were either timed or with a barium tablet. Functional lumen imaging probe (FLIP) tests were categorized as limited, borderline, or normal EGJ opening.
We also applied the same Cr-EGJOO criteria on the CC3 group (mandating relevant symptoms and adjunctive tests). We examined the proportion of EGJOOs excluded by each component.

Results

We included 486 (CC3, n=198, CC4, n=288) patients, with a median age of 58.7 years, 59% were female patients.
There were 100 (20.6%) with an elevated IRP in at least one position and normal peristalsis. Symptom data were available for all 100 patients and excluded Cr-EGJOO in 24%, barium esophagrams available for 85 and excluded Cr-EGJOO in 78.8%, FLIP was available in 11 patients (all had an esophagram). It rejected Cr-EGJOO in 63.6%, was borderline in 9.1%, and positive in the 27.3%. HRM criteria excluded Cr-EGJOO in 37.1% of the CC4 group. Note, most (93.1%) elevated IRPs were in the supine position.
Consequently, we could apply the Cr-EGJOO criteria on 85 patients with complete data (68 and 17 patients in the CC4 and CC3 groups, respectively). In the CC4 group the EGJOO rate was reduced from 27.3% to 3.1%, and in the CC3 group from 11.1% to 5.3%. Patients with an elevated IRP in at least one position and a positive adjunctive test were invariably symptomatic, but most symptomatic patients did not have Cr-EGJOO.

Conclusion

The updated CC 4.0 criteria considerably reduce the diagnosis rate of EGJOO diagnosis. Barium esophagrams had the highest yield in excluding clinically irrelevant cases, highlighting the importance of utilizing this non-invasive, readily available test to avoid unnecessary worry and invasive treatments.

Clinical Case Summary

Muir-Torre syndrome (MTS) is a rare autosomal-dominant genodermatosis characterized by sebaceous neoplasms and multiple visceral malignancies.
A 72-year-old man was admitted to our Emergency room for multiple episodes of lower gastrointestinal bleeding. His personal history was characterized by the diagnosis of MTS. In the previous years he underwent surgery several times, first for distal esophagectomy and total gastrectomy for adenocarcinoma pT3N0, then excision of right and left thigh cancers, atypical left hemicolectomy for adenocarcinoma pT3N0 and partial transversal colectomy for cancer. All of these operations caused anatomical changes and significant non-cirrhotic portal hypertension. At blood test acute severe anemia was found. Esophagogastroduodenoscopy and ileo-colonscopy revealed normal esophageal-digiunal anastomosis and blood in the colon without finding an active source of bleeding. Secondly, abdominal angio-CT scan showed a varix of periumbilical collateral circulation bulging into the ileal lumen, without signs of active hemorrhage. A multidisciplinary team (endoscopists, interventional radiologists and surgeons) evaluated this patient's condition. The surgical technique was not feasible due to post-operative morphological and vascular changes. The radiologists were also unable to perform transjugular intrahepatic portosystemic shunt and embolization for the same reasons. An antegrade double balloon enteroscopy was carried out to identify and stop the bleeding, but it failed. As a result, the source of the ematic loss was not discovered because it was impossible to completly examine the small bowel due to a severe adverse syndrome and the patient eventually died. Bleeding from ectopic varices is a rare and a life-threatening situation. This is one of the rare case describing ileal bleeding varices in a patient with the rare MTS syndrome.
Image: ANGIO-CT SCAN: varix of periumbilical collateral circulation bulging into the ileal lumen

References

Bhaijee F, Brown AS. Muir-Torre syndrome. Arch Pathol Lab Med. 2014 Dec;138(12):1685-9. doi: 10.5858/arpa.2013-0301-RS. PMID: 25427047.
Haddad A, Beji H, Chaker Y, Maghrebi H, Jouini M, Kacem M. Surgical treatment for bleeding ileal varices: A case report. Int J Surg Case Rep. 2021 Nov;88:106573. doi: 10.1016/j.ijscr.2021.106573. Epub 2021 Nov 3. PMID: 34753100; PMCID: PMC8585655.

Introduction

Colonic diverticular bleeding (CDB) is the most common cause of acute lower gastrointestinal bleeding (ALGIB) and often requires blood transfusion. There are several studies regarding the use of restrictive blood transfusion for acute upper gastrointestinal bleeding, but there is only one previous study regarding the use of restrictive blood transfusion for ALGIB [1]. Therefore, the efficacy of restrictive blood transfusion for CDB remains unclear.

Aims & Methods

This study sought to elucidate the association between the restrictive blood transfusion strategy and the clinical outcomes in patients with CDB. We retrospectively analyzed 475 patients who were urgently hospitalized for CBD and received blood transfusions at two facilities between November 2013 and December 2021. Transfusion strategy was defined as the restrictive group when patients received transfusion for hemoglobin (Hb) ≤ 7 g/dl, and the liberal group when patients received for Hb > 7 g/dl. The hemoglobin cut-off value for restrictive blood transfusion was determined based on a prior randomized clinical trial regarding UGIB [2]. Rebleeding within 30-days, mortality within 30-days, and composite outcome within 30-days were evaluated. The composite outcome comprised the following endpoints: rebleeding, need for surgery or IVR for hemostasis, and mortality within 30 days. We also investigated medical costs during hospitalization. Logistic regression analysis was performed to calculate the odds ratios (OR) with 95% confidence interval (CI) to analyze associations between restrictive transfusion strategy and outcomes.

Results

Of 475 patients, 184 (38.7%) (124 males, 60 females, mean age 76.5±11.5 years) patients received blood transfusion. The mean hemoglobin levels at the time of transfusion in the restrictive and liberal groups were 6.3±0.6 g/dl and 8.2±1.2 g/dl, In the restrictive and liberal group, rebleeding within 30-days was 53.9% and 45.3% (P = 0.24), the mortality within 30-days was 1.1% and 0% (P = 0.30), the composite outcome within 30-days was 56.1% and 52.6% (P = 0.63), respectively. After adjusting for potential confounders, multivariate analysis revealed that restrictive transfusion strategy not associated with decreased rebleeding risk within 30-days, (P = 0.42 ; odds ratio [OR], 1.3), whereas endoscopic hemostasis significantly associated with decreased rebleeding risk (P < 0.01 ; OR 0.2). There was no statistically significant difference in total medical costs between the two groups (P = 0.68).

Conclusion

There were no statistically significant differences in rebleeding within 30-days, mortality within 30-days, and compositeoutcome within 30-days between the restrictive and liberal blood transfusion strategies. Restrictive blood transfusion may be a reasonable therapeutic strategy for patients with CDB, except for patients in which shock status cannot be withdrawn. However, further evidence is needed to advocated the efficacy of restrictive transfusion strategies for lower gastrointestinal bleeding.

References

[1] Kherad O, Restellini S, Martel M, Sey M, Murphy MF, Oakland K, et al. Outcomes following restrictive or free red blood cell transfusion in patients with lower gastrointestinal bleeding. Aliment Pharmacol Ther. 2019 Apr;49(7):919–25.
[2] Villanueva C, Colomo A, Bosch A, Concepción M, Hernandez-Gea V, Aracil C, et al. Transfusion strategies for acute upper gastrointestinal bleeding. N Engl J Med. 2013 Jan;368(1):11–21.

Introduction

Although the recommended method of an initial examination of hematochezia is colonoscopy, computed-enhanced CT (CECT) is an appealing diagnostic modality for the same disorder, because it has a superior ability of detecting active bleeding and it is widely available, fast, and minimally invasive. In many centers, CECT has already become an established method for characterizing the location of hematochezia. However, its impact on clinical courses of patients with colon diverticular bleeding is fully known. We conducted this study to assess them on the basis of single-center experience in Japan.

Aims & Methods

Consecutive 370 patients with colon diverticular bleeding from April 2002 to January 2023 were enrolled. Diagnosis of colon diverticular bleeding was based on total colonoscopy. Patients were divided into two groups who underwent initial examinations via CECT and colonoscopy. 
Patient attributes, clinical data, and outcomes (length of hospital stay, re-bleeding rate, blood transfusion units and 30 days survival rate) were retrospectively analyzed. The patient characteristics and outcomes were compared between two groups using χ2 test or Fisher’s exact test.

Results

279 patients were men (75.4%). The average age was 70.0(30-94) years old.  Initial CECT was performed to 204 patients (Group A) (54.9%) and remaining 166(group B).  
Patients in group A were more likely to have cerebrovascular disorder.
Findings for other characteristics were similar between two groups. 
In Group A, CECT revealed extravasation in 110 patients (53.9%). 
Emergent colonoscopy was performed in 118 patients (extravasation positive 100: negative 18) and found bleeding point in 46 patients (extravasation positive 43: negative 3). 
In Group B, emergent colonoscopy was performed in 70 patients and found bleeding point in 19(27.1%) patients. 
Patients in group A required more blood transfusion units than group B significantly (3.36 ± 6.28 units in group A vs. 1.53 ± 2.96 units in Group B, p=0.001).
No significant difference was observed in terms of length of hospital stay (10.64 ± 7.11 days in group A vs.9.95 ± 5.37 days in Group B ,p=0.32) , recurrent bleeding rate (23.6 % in group A vs. 23.4 % in Group B, OR 1.016 ,p=1) , and 30 days survival rate (99.5% in Group A vs. 100 % in Group B, OR 1.005 ,p=1)

Conclusion

Emergent colonoscopy, subsequent to initial CECT which revealed extravasation, can detect bleeding point more efficiently in patients with colon diverticular bleeding. But, CECT did not improve clinical outcomes of these patients in this study.

Clinical Case Summary

A 57- year- old female with past medical history of NF1 and diverticulitis presented with one day history of left lower quadrant abdominal pain, nausea, and vomiting. CT Abdomen and Pelvis demonstrated sigmoid diverticulitis without abscess, and an incidental mesenteric mass 4.4 x 3.1 x 3.7 cm in the left lower quadrant. She was medically managed with intravenous antibiotics and discharged five days later. She was scheduled for a 1 month follow up with general surgery, who recommended resection of the mass. She underwent a small bowel resection of the mass and was discharged on postoperative day three. Pathology was consistent with gastrointestinal stromal tumor with two foci, 5.4 cm, and 0.6 cm in size with negative margins. The overall mitotic index was low (2/50 HPF) with Ki-67 staining of 5%. Tumor cells were positive for CD117, DOG1, SMA and negative for CD 31, CD 34, MSA, S100 and Desmin. Onkosight NGS KIT sequencing revealed no clinically relevant mutations in KIT exons 2, 9, 10, 11, 13, 14, 15, 17 and 18. Oncology recommended CT surveillance scans every 6 months without adjuvant therapy since the tumor developed in the setting of NF1. Our patient unfortunately deferred routine screening due to loss of insurance and inability to afford the out-of-pocket costs of CT imaging. In summary, our case illustrates the rare occurrence of GIST in a patient with NF1. Since NF1-associated GISTs typically present in female patients in their fourth decade of life, our case highlights the importance of early screening. Surgical resection is the mainstay of treatment for this condition as seen in our patient while tyrosine kinase inhibitors are typically reserved for sporadic cases or those with high-risk features [1,2]. Thus, further research into cost effective diagnostic surveillance and non-surgical treatment modalities for those with NF1 associated GIST is warranted.

References

1. von Mehren M, Joensuu H. Gastrointestinal Stromal Tumors. J Clin Oncol. 2018 Jan 10;36(2):136-143. doi: 10.1200/JCO.2017.74.9705. Epub 2017 Dec 8. PMID: 29220298; PMCID: PMC6553810.
2. Rutkowski P, Skoczylas J, Wisniewski P. Is the Surgical Margin in Gastrointestinal Stromal Tumors Different? Visc Med. 2018 Oct;34(5):347-352. doi: 10.1159/000491649. Epub 2018 Aug 24. PMID: 30498701; PMCID: PMC6257087.

Clinical Case Summary

Ballon-occluded retrograde transvenous occlusion (BRTO) is a minimal invasive procedure to treat mainly the gastric varices but it may be used in ectopic varices as well. We report a case of 70years old male known to have Alcoholic liver disease (cirrhosis) with portal hypertension, presented with malena. He underwent an urgent Gastroscopy which showed GAVE (Gastric antral vascular ectasia) and Grade 1 varix not needing any therapy. Due to persistent malena and haemoglobin drop, CT mesenteric angiogram was performed which reported multiple enlarged varices in the abdomen with no active site of haemorrhage. Video capsule endoscopy was performed which showed small bowel varices with evidence of fresh blood. Unfortunately, the ultrasound liver showed thrombosis of portal vein. Due to portal vein thrombosis TIPSS (Transjugular intrahepatic porto systemic shunt) was not performed and MDT decision for BRTO (Balloon-occluded retrograde transvenous occlusion) was made which the patient underwent successfully.
BRTO is advantageous over TIPSS because this can be performed with poor hepatic reserve and with hepatic encephalopathy however it increases the risk of bleeding from oesiphageal varices and ascites. Gastro-renal shunts are the most sites for use of BRTO however, this procedure was successfully done in this case.
Based on literature review BRTO procedure has been successfully used in gastric varices.
I’ve found few cases reported with small bowel varices treated with BRTO.
Learning points:

1. Whether BRTO is superior to TIPPS for ectopic varices
2. Whether BRTO can be considered in ectopic varices in patients without portal vein thrombosis

References

https://www.sciencedirect.com/science/article/pii/S1930043317305022


https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3444869/#!po=0.806452

Disclosure

No conflict of interest