Introduction

Malnutrition is a common complication in patients with inflammatory bowel disease(IBD) that leads to a worse long-term prognosis, but is frequently underdiagnosed. The "Malnutrition Universal Screening Tool"(MUST) ia a simple, rapid, reproducible and already validated tool for screening. We describe the prevalence of patients at risk of malnutrition in a single tertiary IBD outpatient clinic using the self-administered MUST and we assess the applicability of the test evaluating the patients' opinion about its difficulty.

Aims & Methods

We conducted a transversal study at a tertiary Spanish center that included consecutive adult outpatients with IBD. Variables related to their IBD such as type, phenotype, location, current treatment, number of previous biological drugs and surgical interventions were collected. Disease activity was evaluated with the partial Mayo index for ulcerative colitis(UC), Harvey-Brasdhaw for Crohn's disease(CD), fecal calprotectin and CRP. Remission was defined as both Partial Mayo≤1 for UC and Harvey-Bradshaw≤4 for CD and CRP<5mg/dL and fecal calprotectin<150mcg/g. All patients with IBD completed a self-administered nutrition screening assessment using the MUST score. The risk of malnutrition was classified as low(score=0), medium/high(score≥1).

Results

106 patients were included, 51 men(48.11%) and 55 women(51.89%), with a mean age of 54 years(SD 17.95). 38(35.85%) had UC and 68(64.15%) had CD. Regarding treatment, 40patients(37.38%) had received at least one biological drug previously and 29(27.35%) had prior surgery. In relation to disease activity, at the time of the survey, 84(79.25%) were in remission. 21 patients(19.81%) had an intermediate/high risk of malnutrition. Regarding posible predisposing risk factors, remission was significantly associated with a lower malnutrition risk. Among patients without remission 81.81% had an medium/high risk of malnutrition whereas only the 3.57% of patients in remission presented medium/high risk(p<0.001). No statistically significant differences were found in univariate analysis when comparing the IBD type(OR 0.08 CI 0.004-1.61), the number of biological drugs received(OR 0.96 CI 0.67-1.4) or whether they had undergone previous surgery(OR 1.1 CI 0.78-1.55). All patients were able to screen themselves and 105/106 reported the MUST questionnaire as either very easy/easy to understand and to complete.

Conclusion

The self-administered MUST is an useful and easy to apply tool in malnutrition screening, being able to detect 20% of patients at risk of malnutrition in an outpatient setting.

Introduction

The mainstay of treatment of Zenker’s diverticulum is cricopharyngeal myotomy. Although flexible endoscopic septotomy is widely implemented, complete myotomy is difficult to assess resulting in recurrence rates of 11%. On the other hand, ZD peroral endoscopic myotomy allows complete myotomy visual confirmation, at the expense of procedure length and complexity. To overcome these limitations, a hybrid technique that allies FES simplicity and fastness to zPOEM efficacy is proposed: 1-septum isolation with overtube; 2-standard FES until middle of the diverticulum; 3-muscular septum isolation by zPOEM until the base of the diverticulum; 4-complete myotomy; 5-residual mucosal flaps section; 6-mucosotomy closure with TTS clips. We aim to demonstrate the feasibility, effectiveness and safety of the hybrid zPOEM technique for the treatment of ZD.

Aims & Methods

A retrospective analysis including patients with ZD treated by hybrid zPOEM procedure from April 2021 to February 2023 was made. Technical success, early clinical success (dysphagia score ≤ 1), adverse events and recurrence rates were evaluated.

Results

11 patients (male 54.5%, mean age 72.9 years) underwent hybrid zPOEM procedure for treatment of ZD (mean size 23.18mm, range 15-30mm). The procedure was technically successful in all patients, with a mean procedure time of 21.6 minutes. Early clinical success was achieved in all patients, although 1 patient maintained occasional regurgitation due to incomplete mucosal flaps section. Mean symptoms scores improved from baseline for dysphagia (1.5 vs 0), regurgitation (1.5 vs 0.1), halitosis (0.5 vs 0), cough (0.5 vs 0) and hoarseness (0.3 vs 0). During a mean follow-up time of 10 months, no recurrence was recorded. No intra- or post-procedure AE were recorded.

Conclusion

Hybrid zPOEM technique is a fast, safe and effective procedure, where, by combining classic FES and zPOEM, one can have the best of both techniques. This procedure should be considered as a first-line therapeutic option in third-space-endoscopy experienced centers.

References

Zhang LY, Nieto J, Ngamruengphong S, Repici A, Khashab MA. Zenker’s diverticulum: advancing beyond the tunnel. VideoGIE. 2021;6(12):562–7
Lupu A, Lafeuille P, Yzet C, Benech N, Jacques J, Rivory J, et al. Hybrid peroral endoscopic myotomy for large Zenker’s diverticulum. Endoscopy. 2022;54:E862–3.
Krishnan A, Ichkhanian Y, Runge TM, Khashab MA. Hybrid endoscopic approach for submucosal tunneling septum division for Zenker s diverticulum. Endoscopy. 2020;52(12):E457–8.

Introduction

Primary cancer of the appendix is rare and accounts for 0.4% of all gastrointestinal (GI) malignancies with an incidence of approximately 1.2 cases per 100,000 people per year in the United States. Patients with inflammatory bowel disease (IBD), including ulcerative colitis (UC), Crohn’s disease (CD), and indeterminate colitis (IC), face increased risk for the development of colorectal dysplasia and carcinoma. Although appendiceal inflammation occurs histologically in 40-80% of colectomy specimens from patients with IBD, appendiceal neoplasms have been reported only infrequently. A direct association between IBD and appendiceal neoplasia, including whether IBD predisposes to the development of appendiceal neoplasia, is unclear.

Aims & Methods

We aimed to describe the clinical characteristics, outcomes, and incidence of appendiceal neoplasms in patients with IBD. Using bioinformatics and natural language processing tools to mine the electronic medical record at our large referral center, all patients > 18-years-old with IBD who had an appendiceal neoplasm were identified from January 1992 to April 2023. Relevant demographic and clinical data were abstracted.

Results

In total, 44 patients were included (61.4% female) with a median age of 53 years at appendix neoplasm diagnosis. A majority had UC (68.2%). Of these, four (13.3%) had backwash ileitis, 27 (90%) had extensive colitis, 12 (40%) had a history of colorectal neoplasia, and 6 (20%) had primary sclerosing cholangitis (PSC). Median duration of IBD prior to appendix neoplasm diagnosis was 17 years. The most common type of appendix neoplasm was adenocarcinoma (36.4%), followed by appendiceal mucinous neoplasm (34.1%), and carcinoid or neuroendocrine tumor (22.7%). The most common presenting symptom associated with diagnosis of appendix neoplasm was abdominal pain/distension (36.4%). Appendix neoplasm diagnosis was made by cross-sectional imaging in a majority of patients (47.7%). The most common type of IBD-directed therapy at the time of appendix neoplasm diagnosis was 5-aminosalicylate (5-ASA) agents (25%). All patients had appendix neoplasm surgically resected. Median appendix neoplasm size was 2.7 cm. The most common histologic tumor grade was grade 1 (31.8%). Two (4.5%) patients had lymph node involvement and 7 (15.9%) patients had metastatic disease at the time of appendix neoplasm diagnosis. Twelve (27.2%) patients received adjuvant chemotherapy. Three patients with UC (6.8%) had recurrence of appendix neoplasm after surgical resection due to peritoneal seeding with a median time to recurrence from initial diagnosis of 78 months.

Conclusion

Appendix neoplasms are more commonly seen in patients with longstanding extensive UC in those presenting with abdominal pain/distension. The most common types of appendix neoplasia included adenocarcinoma and appendiceal mucinous neoplasms. A minority of patients had metastatic disease at the time of appendiceal neoplasia diagnosis. Although the incidence and recurrence of appendix neoplasia in patients with IBD is relatively low, likely related to the small area of mucosa at-risk relative to the colorectum, the recognition of appendix neoplasms as a potential complication of longstanding disease remains unclear. Further studies to compare appendiceal neoplasia in patients with and without IBD are needed to determine if IBD predisposes to the development of this neoplastic complication.

References

1. Ruoff C, Hanna L, Zhi W, Shahzad G, Gotlieb V, Saif MW. Cancers of the appendix: review of the literatures. ISRN Oncol. 2011;2011:728579. doi:10.5402/2011/728579
2. Nehme F, Schneider A, Hamid F. Appendiceal Adenocarcinoma Associated With Ulcerative Colitis. ACG Case Rep J. 2019;6(11):e00255. Published 2019 Nov 25. doi:10.14309/crj.0000000000000255
3. Van de Moortele M, De Hertogh G, Sagaert X, Van Cutsem E. Appendiceal cancer : a review of the literature. Acta Gastroenterol Belg. 2020;83(3):441-448.
4. Orta L, Trindade AJ, Luo J, Harpaz N. Appendiceal mucinous cystadenoma is a neoplastic complication of IBD: case-control study of primary appendiceal neoplasms. Inflamm Bowel Dis. 2009;15(3):415-421. doi:10.1002/ibd.20764

Disclosure

Siri A. Urquhart, M.D.: Discloses no conflicts.
John B. Kisiel, M.D.: Research funding and intellectual property licensed to Exact Sciences.
Nayantara Coelho-Prabhu, M.B.B.S.: Research funding from Fujifilm, Cook Endoscopy, and Alexion Pharma.

Clinical Case Summary

Biliary pathology is a frequent motive for consultation in hospitals, and abdominal ultrasound is the first line-imaging test for its study. The genetic syndrome LPAC (Low Phospholipid-Associated Cholelithiasis) leads to the development of intrahepatic lithiasis. In this syndrome, there is a decrease in biliary phospholipids secretion caused by a mutation in the ABCB4 gene, responsible for a dysfunction of the MDR3 transporter. Despite being considered a rare entity, it accounts for 1-5% admissions for biliary pathology, affecting young women more frequently.
Two of three clinical criteria must be met for its diagnosis: symptoms onset before the age of 40, recurrence after cholecystectomy and compatible ultrasound appearance (presence of intrahepatic hyperechogenic spots, &quot;comet tail&quot; images or intrahepatic lithiasis with acoustic shadowing). Identification of the ABCB4 gene mutation is not a diagnostic criterion since it only appears in 50% of patients. Medical treatment with ursodeoxycholic acid is effective and cholecystectomy can be avoided.
We present a 23-year-old male who consulted for epigastric pain associated with vomiting. Laboratory tests showed elevation of amylase greater than three times above the upper limit of normal, which allowed the diagnosis of acute pancreatitis. Liver profile, calcium and triglycerides were within normal limits. The patient had no previous alcoholic intake. Abdominal ultrasound ruled out cholelithiasis but identified multiple intrahepatic hyperechogenic spots and &quot;comet tail&quot; signs. The patient was diagnosed with LPAC syndrome and treatment with ursodeoxycholic acid was initiated. During follow-up he did not present new episodes of biliary symptomatology.
The detection of the typical ultrasound signs of LPAC syndrome is the main tool for its suspicion. This disease is not as uncommon as it may seem, so it is essential to think about it in order to make a correct diagnosis and treatment of these patients.

References

Ultrasound image of intrahepatic lithiasis

Clinical Case Summary

Pancreatic cystic lymphangioma is extremely rare, accounting for less than 5% of pancreatic cystic neoplasms and less than 1% of all lymphangiomas.¹
A 51-year-old female presented with complaints of recurrent abdominal pain for 4 weeks. She was previously misdiagnosed as acute pancreatitis complicated with pancreatic pseudocyst. There was no history of trauma. Dynamic MRI on the abdomen revealed a large, encysted collection at the pancreatic body suggesting a query pancreatic pseudocyst. EUS examination revealed a large cystic lesion at the body of the pancreas. The cyst was multilocular with characteristic multiple thin septa within the lesion (Figure 1). EUS-FNA with 22-G needle was done with aspiration of about 20 ml of cyst fluid, divided into two bottles, one for cytology examination and mucin stain while the second bottle for Amylase, CEA and TG levels. Aspiration fluid was serous yellowish in colour, with negative string sign. Also, a large focal lesion within the spleen was also seen.The biochemical evaluation of the aspirated cystic fluid revealed low amylase and CEA, and high TG levels. Cytopathological examination showed fairly cellular material featuring numerous predominantly small lymphocytes. There were no epithelial, atypical, or malignant cells. IHC staining was positive for CD-31 and CD2-40. Alcian blue mucin stain was negative. The diagnosis of pancreatic cystic lymphangioma was made based on EUS, cytological and biochemical examination with IHC features. Since, lymphangiomas are benign tumors without malignant potential, treatment depends on the symptoms. Lymphangioma can encroach on adjacent organs and need surgical resection. To relieve symptoms of abdominal, distal pancreatectomy and splenectomy was discussed, but patient refuse surgery. Multidisciplinary team decided a conservative management with a follow-up by dynamic MRI and EUS within 6 months.

References

1. Chen D, Feng X, Lv Z, Xu X, Ding C, Wu J. Cystic lymphangioma of pancreas: A rare case report and review of the literature. Medicine (Baltimore). 2018 Jul;97(28): e11238. doi: 10.1097/MD.0000000000011238.

Clinical Case Summary

Caudate lobe hypertrophy is a common finding in cirrhotic liver, although giant variants are rare. Nevertheless, any lesion on a cirrhotic liver warrants consideration for hepatocellular carcinoma (HCC).
This case involves a 28-year-old male patient irregularly followed up at our center for secondary biliary cirrhosis. With a history of intrahepatic bile duct paucity due to Alagille syndrome since neonatal age, he is a current smoker (10 pack-years) with no alcohol abuse. After three years lost to follow-up, the patient presented with a gradual 4-month onset of jaundice, dark urine, and upper right quadrant discomfort. Laboratory findings revealed worsening liver function tests: total bilirubin 11.5 mg/dl (vs 2.5 mg/dl), predominantly conjugated 7.6 mg/dl (vs 1.7 mg/dl), alkaline phosphatases 3.3x ULN (vs 2.6x ULN), gamma-glutamyl transferase 6.4x ULN (vs 4.5x ULN), aspartate aminotransferase 3.8x ULN (vs 1.7x ULN), alanine aminotransferase 1.5x ULN (vs 1.3x ULN).
The 4-phase CT scan revealed a dysmorphic liver with a large mass spanning segments 4, 5, 6, 7, and 8, measuring 130x100mm. The mass exhibited arterial enhancement without late phase washout and no evidence of a capsule. This mass displaced the hepatic veins and abutted the inferior vena cava and portal branches, without signs of invasion. MRI showed a similar appearance with no diffusion restriction. Initially suspected as HCC, the mass was classified as LIRADS-4. Alpha-foetoprotein level was normal. CT-guided biopsy retrieved multiple 41mm samples. Histopathological findings indicated annular fibrosis with histological cholestasis and no malignant cells. Therefore, the established diagnosis was caudate lobe hypertrophy, following a multidisciplinary team discussion and review of radiological features.
Despite this entity's rarity, biopsy seems crucial to rule out HCC, especially with abnormal vascular behavior like arterial enhancement, to avoid overtreatment.